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T-cell translocation gene 1 (Ttg-1) encodes a nuclear protein normally expressed in neural lineage cells. We previously identified and cloned T-cell translocation gene 1 (Ttg-1), a putative zinc finger protein, as a result of its deregulated expression in a T-cell acute lymphoblastic leukemia cell line (RPMI 8402) with a t(11;14)(p15;q11). We have now characterized its genomic organization and identified the major transcriptional start site to lie within an initiator-like motif. Ttg-1 is normally expressed in mouse brain and not in thymus. The mouse neuroblastoma cell line, N2a, also expresses Ttg-1. Antibodies raised against a TrpE-Ttg-1 fusion protein precipitate an 18-Kd nuclear protein from metabolically labeled 8402 cells. Immunofluorescence of N2a cells shows a nuclear pattern. The two potential zinc finger domains in Ttg-1 are highly homologous to similar regions in lin-11, mec-3, and lsl-1. This data suggests that Ttg-1 may be involved in gene regulation. | 4 |
Right-to-left shunting through a patent foramen ovale caused by pulmonary hypertension associated with rheumatoid arthritis and Sjogren's syndrome: a case report. This case report presents a fifty-four-year-old woman with right-to-left shunt in the atrium. It seemed clinically at first that the shunt was due to atrial septal defect. However, she also had pulmonary disease associated with rheumatoid arthritis and Sjogren's syndrome. At autopsy atrial septal defect was not evident, but a patent foramen ovale and pulmonary artery disease were observed. This case suggests that pulmonary hypertension secondary to rheumatoid arthritis and Sjogren's syndrome could lead to right-to-left shunting through a patent foramen ovale. | 3 |
Palliation of malignant esophageal obstruction. Progressive dysphagia is common in patients with advanced esophageal carcinoma. Multiple nonsurgical techniques are available to provide palliation and improved nutrition. Simple dilatation is the oldest technique and newer methods may offer greater efficacy. Laser therapy now provides an excellent opportunity to treat obstructing tumors. Placement of an esophageal prosthesis may become particularly useful when dilatation must be performed too frequently or has become ineffective or in the patient with an esophageal-pulmonary fistula. Newer techniques including BICAP tumor probe, intracavitary radiotherapy, or absolute alcohol injection offer promise. This review discusses the advantages and disadvantages of these different palliative techniques for patients with the extremely poor prognosis associated with esophageal cancer. | 0 |
Myotonic heart disease: a clinical follow-up. We followed 37 patients with myotonic dystrophy for a mean of 6 years. Two developed atrial flutter or fibrillation, 6 developed a new bundle branch block, 1 developed complete heart block requiring a pacemaker, and another with progressive 1st-degree heart block and a widening QRS interval had a sudden death. Most patients had predictable, gradually progressive disease of their cardiac conduction system. We recommend that patients with progressive atrioventricular block or widening QRS interval due to myotonic heart disease have yearly ECGs and be questioned about syncope or presyncope to determine the need for a cardiac pacemaker. | 2 |
Defective dystrophin in Duchenne and Becker dystrophy myotubes in cell culture. We examined normal and dystrophic human myotubes in cell culture for expression of dystrophin, the protein product of the Duchenne muscular dystrophy locus. Dystrophin levels in developing myotubes detected by Western blotting increased after 24 hours and reached maximum levels after 10 days in fusion medium. We did not detect dystrophin in myotubes cultured from Duchenne myoblasts (7 cases). Myotubes from a Becker muscular dystrophy patient's biopsy produced a lower molecular weight (approximately 408 kd) dystrophin, which was the same size in a whole muscle preparation from the same biopsy. This 408-kd dystrophin was the expected size for this Becker patient whose DNA was deleted for exons 45-48 of the Duchenne gene. This cell culture system will allow a detailed analysis of the effects of potential pharmacologic agents on steady-state dystrophin levels. | 4 |
The use of fibrin sealant to reduce blood loss during Cotrel-Dubousset instrumentation for idiopathic scoliosis. The increased complexity of Cotrel-Dubousset instrumentation has, as an unexpected side effect, a potential increase in blood loss. A prospective randomized study was undertaken to test the hypothesis that application of a fibrin sealant to exposed cancellous bone can significantly reduce blood loss during Cotrel-Dubousset instrumentation for idiopathic scoliosis. A significant difference was demonstrated in total body loss, loss per level fused, and loss per kilogram of body weight when comparing the sealant group with random controls. A significant difference was demonstrated in loss per level fused when comparing the sealant control with a historical control group. Thirty-three patients were randomly assigned to the fibrin sealant or nonsealant groups; another 10 patients operated on before planning the study were included as historical controls. The sealant was used to control bleeding at the bone graft donor site and in the spine after decortication. All patients underwent Cotrel-Dubousset instrumentation for idiopathic scoliosis. There were no significant differences between groups with respect to degree of curvature, number of levels fused, age or weight of patient, or operating time. Total blood loss in the sealant group averaged 672 ml compared with 894 ml in the sealant control group. No patient in the sealant group required homologous blood. Given the increasing awareness of the complications of blood transfusion, the authors conclude that fibrin sealant is a useful adjunct to spinal surgical technique. | 4 |
Temporal arteritis presenting as a submandibular swelling. Temporal arteritis (giant cell arteritis) is a disease of protean manifestation. A case which presented as a submandibular swelling is reported. | 3 |
Lack of bone accretion and amenorrhea: evidence for a relative osteopenia in weight-bearing bones. Bone mineral density (BMD) was studied in young exercising amenorrheic girls to determine if density was compromised and the change related to injury. Ninety-eight volunteers (professional ballet dancers and controls) were studied in a cross-sectional study. Dancers and controls were further subdivided into normally cycling and amenorrheic subjects. Amenorrhea significantly lowered bone density of the spine (P less than 0.0001), wrist (P less than 0.03), and metatarsal (P less than 0.01); effects on the wrist were eliminated by controlling for age while controlling for weight eliminated all effects of amenorrhea at three sites. BMD of the metatarsal, a weight-bearing bone, showed an interaction between amenorrhea and dancing (exercising) P less than 0.035); surprisingly, dancing was associated with a further lowering of bone density. This interaction was eliminated when controlling for age, but not when controlling for weight. With multiple comparisons of the groups, spine, wrist, and metatarsal bone density was significantly lower in amenorrheic dancers when compared to normal dancers (P less than 0.05), even when controlling for age and weight in the metatarsal (P less than 0.05), and age in the spine (P less than 0.05). Estradiol levels correlated with bone density of both the wrist and the spine (r = 0.25, r = 0.23, P less than 0.02). Metatarsal density correlated with estradiol levels only in the dancers (r = 0.34, P less than 0.02). The only variable found to correlate with the occurrence of stress fractures was age of menarche. This was also the only variable of 9 (BMD of the wrist, spine or foot, calories ingested and expended, amount of calcium ingested, involvement in high energy activity, age of menarche or presence of amenorrhea) to predict stress fractures. Thus, BMD is significantly affected by the presence of amenorrhea but the effects are generally weight dependent. The compensatory increase in bone density generally seen in stressed bones, such as the metatarsal in ballet dancers, is deficient in amenorrheic premenopausal women even when controlling for weight but this effect may be age and estrogen dependent. Bone mass may not accumulate in the same manner in adolescents as in the mature women, thus putting them at risk for injury. | 4 |
Increased Ca2+ signaling after alpha-adrenoceptor activation in vascular hypertrophy. In an effort to explain the increased sensitivity to agonists of hypertrophic vascular muscle, intracellular Ca2+ concentration ([Ca2+]i)-signaling mechanisms were studied in normal and hypertrophic rat aortas from normotensive and coarctation-hypertensive rats. Based on both fura 2 fluorescence and aequorin luminescence measurements, qualitatively different patterns of Ca2+ mobilization occur in normal and hypertrophic rat aortic muscle. Normal rat aortic muscle contracts to phenylephrine with little or no increase in [Ca2+]i, whereas the angiotensin II-induced contraction is accompanied by a marked [Ca2+]i transient. In contrast, hypertrophic rat aortic muscle shows a dramatic increase in Ca2+ signaling after phenylephrine stimulation. Moreover, both the amplitude of the angiotensin-induced [Ca2+]i transient and the contractile sensitivity to this agonist are decreased in the hypertrophic muscle. Our results strongly suggest that the amplitude of the [Ca2+]i transient after agonist stimulation determines the contractile sensitivity and that there is an altered coupling of the alpha-adrenoceptor in the hypertrophic vascular muscle. | 3 |
The effect of intravenous fixed-dose heparin during total hip arthroplasty on the incidence of deep-vein thrombosis. A randomized, double-blind trial in patients operated on with epidural anesthesia and controlled hypotension. Heparin was given in fixed doses intravenously during unilateral primary total hip-replacement operations in a prospective, double-blind trial to assess the effect on the incidence of deep-vein thrombosis. One hundred and fifty patients were randomly assigned to one of two groups before the operation. Twenty-four patients were excluded from the study, leaving 126 patients. Group I consisted of sixty-six patients who received saline solution intravenously, and Group II comprised sixty patients who received heparin. All patients had epidural anesthesia with controlled hypotension. Fixed doses of heparin were administered five minutes before the operative incision was made and every thirty minutes throughout the operation. Mean arterial pressures were maintained at between fifty and sixty millimeters of mercury in all patients. Ascending venography was done on the seventh day after the operation. The incidence of deep-vein thrombosis was 24 per cent (sixteen of sixty-six patients) in Group I and 8 per cent (five of sixty patients) in Group II; the difference is significant (p = 0.03). The intraoperative loss of blood averaged 220 +/- 79 milliliters in Group I compared with 269 +/- 109 milliliters in Group II. An average of less than one unit of blood was transfused for each patient in each group. Postoperatively, there was no difference between the groups with regard to the amount of drainage that was collected in a Hemovac device or the values for hematocrit. | 4 |
Node-negative breast cancer: prognostic subgroups defined by tumor size and flow cytometry Adjuvant systemic therapy for women with node-negative breast cancer is most easily justified for those patients at highest risk of relapse. We have examined the impact of tumor size, histologic grade, estrogen receptor (ER) status, tumor ploidy, and S-phase fraction (SPF) on relapse-free survival (RFS) for 169 patients with node-negative breast cancer in order to identify groups of patients at high and low risk of relapse. Patients with small tumors (less than or equal to 1.0 cm) had a significantly better RFS than those with larger tumors (P = .005), with 96% remaining relapse-free at 5 years. Patients with tumors less than or equal to 1.0 cm were thus excluded from analysis when attempting to define a group with a poor prognosis. Within the group of patients with tumors greater than 1.0 cm, tumor ploidy (P = .63), ER status (P = .3), or progesterone receptor (PgR) status (P = .24) did not predict for RFS. Patients with grade 1 or 2 infiltrating ductal tumors had a significantly better prognosis than those with grade 3 tumors (P = .04). The prognostic factor that gave the widest separation between subgroups, however, was SPF. Patients whose tumors were greater than 1.0 cm with an SPF less than or equal to 10% had a 5-year RFS of 78% compared with a 5-year RFS of 52% for those with an SPF greater than 10% (P = .006). We have combined tumor size and SPF to identify three prognostic groups: (1) tumor less than or equal to 1.0 cm, 5-year RFS 96%; (2) tumor greater than 1.0 cm plus SPF less than or equal to 10%, 5-year RFS 78%; 3) tumor greater than 1.0 cm plus SPF greater than 10%, 5-year RFS 52%. These prognostic groupings may help identify patients most suitable for adjuvant therapy. | 0 |
Primary bone tumours of the thoracic skeleton: an audit of the Leeds regional bone tumour registry. An audit of the Leeds regional bone tumour registry found that primary bone tumours of the thoracic skeleton constituted 90 of the 2004 cases (4.5%). Thirty seven per cent occurred in the ribs, 32% in the scapulae, 11% in the thoracic vertebrae, 11% in the sternum, and 9% in the clavicles. Malignant tumours were more common than benign (54 v 36) and occurred in an older population (mean ages 47 and 31 years). The scapula was the most common site for malignant lesions and the ribs the most common site for benign tumours. Chondrosarcoma was the commonest tumour in older patients, fibrous dysplasia and plasmacytoma in the middle age group, and eosinophilic granuloma in children. Presenting symptoms were a poor guide to whether the lesion was malignant or not. This and the small proportion of correct preoperative diagnoses indicate the need for early biopsy. Bone tumour registries provide a valuable source of cumulative information about uncommon tumours and facilitate accurate diagnosis, teaching, and research. | 0 |
Postangiographic femoral artery injuries: nonsurgical repair with US-guided compression Ultrasound-guided compression repair (UGCR) of catheterization-related femoral artery injuries was evaluated as a possible new imaging-guided interventional procedure. Thirty-nine femoral artery injuries (35 pseudoaneurysms, four arteriovenous fistulas) were detected with color Doppler flow imaging in patients with enlarging groin hematomas and/or groin bruits 6 hours to 14 days after catheterization procedures. UGCR was not performed in 10 patients due to spontaneous thrombosis (n = 4), infection (n = 1) or skin ischemia (n = 1), unsuitable anatomy (n = 3), or excessive discomfort (n = 1). The remaining 29 patients underwent a full trial of compression therapy, and the lesion was eliminated in 27. Follow-up color flow scans were obtained after 24-72 hours in all 27 successful cases and at 1-15 months in 19; no recurrences or complications occurred. UGCR for acute injuries is safe and technically simple and is promising as a cost-effective, first-line treatment for uncomplicated catheterization-related femoral artery injuries. UGCR is probably not appropriate for long-standing injuries. | 3 |
Macrophages stimulate bone resorption when they phagocytose particles. We investigated in vitro a mechanism by which particulate debris may induce bone resorption and cause implant loosening. We first studied two standard particles: latex, which is considered to be inert, and zymosan, which is inflammatory. Macrophages that phagocytosed either particle became activated, and stimulated 15 times as much bone resorption as did control macrophages. For activation to occur, 100 times more latex than zymosan had to be phagocytosed. We also found that bone cement and polyethylene particles activated macrophages in a similar manner, and that the necessary amounts of these were intermediate between those of latex and zymosan. None of the particles were toxic. It was concluded that implant loosening may result from bone resorption stimulated by mediators released by macrophages that have phagocytosed particles of bone cement or polyethylene. | 4 |
Dysplasia and DNA aneuploidy in a pelvic pouch. Report of a case. A patient with an 18-year history of ulcerative colitis was operated on with colectomy, mucosal proctectomy, ileoanal anastomosis, and an S-type pelvic pouch due to intractable chronic continuous disease. The patient was followed by endoscopic controls and biopsy sampling from the pouch at regular intervals. A gradual development of severe atrophy in the ileal mucosa was followed by the development of low grade dysplasia. At the most recent endoscopic control, 4 years after the construction of the pouch, biopsies were sampled also for flow cytometric DNA analyses. DNA aneuploidy was detected in a biopsy from the center of the pouch, and a biopsy taken immediately adjacent showed low grade dysplasia. These findings underline the importance of endoscopic follow-up after construction of a pelvic pouch and focus attention to the potential of malignant transformation of the mucosa. | 4 |
Hepatic fibrin-ring granulomas in a patient with hepatitis A. Hepatic fibrin-ring granulomas were found in a 30-year-old patient with serologically confirmed hepatitis A. Other causes associated with the presence of fibrin-ring granulomas in the liver, such as Hodgkin's and non-Hodgkin's lymphoma, cytomegalovirus infection, visceral leishmaniasis, and consumption of allopurinol, were ruled out. It is suggested that hepatitis A must be included in the differential diagnosis of a patient with hepatic fibrin-ring granulomas. | 4 |
Radiation-associated valvular disease. The prevalence of radiation-associated cardiac disease is increasing due to prolonged survival following mediastinal irradiation. Side effects of radiation include pericarditis, accelerated coronary artery disease, myocardial fibrosis and valvular injury. We evaluated the cases of three young patients with evidence of significant valvular disease following mediastinal irradiation. One patient underwent the first reported successful aortic and mitral valve replacement for radiation-associated valvular disease (RAVD) as well as concurrent coronary artery revascularization. A review of the literature revealed 35 reported cases of RAVD, with only one successful case of valve replacement that was limited to the aortic valve. Asymptomatic RAVD is diagnosed 11.5 years after mediastinal irradiation compared with 16.5 years for symptomatic patients, emphasizing that long-term follow-up is important for patients receiving mediastinal irradiation. This study defines a continuum of valvular disease following radiation that begins with mild asymptomatic valvular thickening and progresses to severe valvular fibrosis with hemodynamic compromise requiring surgical intervention. | 3 |
Spontaneous sump syndrome: Successful treatment by duodenoscopic sphincterotomy. "Sump syndrome" is a rare complication of side-to-side choledochoenterostomy operations which develops in the distal, nonfunctioning limb of the common bile duct where lithogenic bile, gastrointestinal contents, and debris accumulate. We report here a patient who developed spontaneous sump syndrome as a result of the formation of choledochoduodenal fistula, and who presented with multiple pyogenic liver abscesses. The patient's symptoms and liver abscesses resolved completely after treatment by endoscopic sphincterotomy and antibiotics. This case demonstrates that sump syndrome may occur spontaneously, that it can be a cause for pyogenic liver abscess formation, and that it may be treated effectively by endoscopic sphincterotomy. | 4 |
Signs, complications, and platelet aggregation in familial exudative vitreoretinopathy. Between 1979 and 1989, I examined 106 patients (16 pedigrees) with signs of familial exudative vitreoretinopathy. Of these patients, 101 had familial exudative vitreoretinopathy, and five had a sporadic manifestation. The complications of familial exudative vitreoretinopathy, deformation of the posterior retina, vitreous hemorrhage, amblyopia, and retinal detachment, caused diminished visual acuity. Of 170 eyes, retinal neovascularization was observed in 18 eyes (11%), and retinal exudates were observed in 16 eyes (9%). Several forms of retinal detachment occurred in 37 of 180 eyes (21%), which often took an unfavorable course. A falciform retinal fold was observed in 14 eyes (8%). Retinal surgery was performed in 14 eyes; reattachment of the retina was successful in only seven eyes. Platelet aggregation studies disclosed no significant differences between seven patients with familial exudative vitreoretinopathy and ten control subjects. The pathogenesis of the disease is based on a premature arrest of the vascular development of the retina. | 4 |
Endothelial-derived relaxing factor released by endothelial cells of human umbilical vessels and its impairment in pregnancy-induced hypertension. The principal objective of this study was to determine and compare the capability of human umbilical vessels of normal and pregnancy-induced hypertensive parturients to produce and to release the endothelial-derived relaxing factor. A bolus of bradykinin injected in the perfusion system of human umbilical vessels induces a release of a relaxant factor, detectable by bioassay, that is pharmacologically similar to the endothelial-derived relaxing factor. Human umbilical cords were collected from normal and pregnancy-induced hypertensive parturients. In the latter group the release of endothelial-derived relaxing factor is extremely reduced. In fact, in umbilical vessels collected from normal parturients, bradykinin at a dose of 20 pmol produces a release of endothelial-derived relaxing factor equivalent to a relaxation induced by 59.9 +/- 11.0 and 30.8 +/- 11.4 pmol of glyceryl trinitrate for the artery and vein, respectively. The same dose of bradykinin in umbilical vessels, collected from pregnancy-induced hypertensive parturients, produces a release equivalent to 6.6 +/- 2.2 and 5.7 +/- 3.5 pmol of glyceryl trinitrate equivalent for the artery and vein, respectively. Neither an increasing bolus of exogenous bradykinin or an infusion of superoxide dismutase or L-arginine was able to restore the production of endothelial-derived relaxing factor to normal levels. Our results indicate a probable alteration of endothelial cell numbers or an alteration of the enzymatic pathway, probably due to cytotoxic endogenous factors produced in pregnancy. | 3 |
Left recurrent laryngeal nerve paralysis: an unusual presentation of histoplasmosis. A case of histoplasmosis seen as left recurrent laryngeal nerve paralysis in a 49-year-old man is described. The patient had roentgenographic findings of a solitary, noncalcified left upper lobe mass and mediastinal adenopathy. Tissue diagnosis of histoplasmosis was obtained using a thoracotomy, allowing institution of appropriate treatment. | 4 |
Digitalis-like activity in human plasma: relation to blood pressure and sodium balance. PURPOSE: On the assumption that renal tubular cells are more important as the target cells for a natriuretic factor than blood cells, we used a well-characterized cultured renal tubular cell line, Madin-Darby canine kidney (MDCK), cells to monitor the circulating digitalis-like factor in human plasma and examine its role in the regulation of blood pressure and sodium balance. SUBJECTS AND METHODS: We investigated the effects of plasma on binding of radioactive ouabain to monolayered MDCK cells in order to determine the level of a circulating digitalis-like factor. First, we measured specific 3H-ouabain binding to MDCK cells in the presence of plasma from 71 outpatients (34 normotensive subjects and 37 hypertensive patients) after incubation for 4 hours. Second, we measured specific 3H-ouabain binding after incubation of cells with plasma from 16 hospitalized subjects (eight normotensive subjects and eight hypertensive patients) receiving low and high sodium diets. RESULTS: In Study 1, ouabain binding was lower by 30% with plasma from hypertensive patients than with plasma from normotensive subjects (p less than 0.01). There was a significant negative correlation between individual subject's systolic or mean blood pressure and ouabain binding (r = -0.34, p less than 0.01 or r = -0.29, p less than 0.01). In Study 2, ouabain binding was also significantly reduced by 25% in the presence of plasma from hypertensive subjects as compared with plasma from normotensive subjects irrespective of sodium intake (p less than 0.01). A significant negative correlation was also found for all subjects between either systolic, diastolic, or mean blood pressure and ouabain binding (r = -0.58, p less than 0.01, r = -0.51, p less than 0.01, or r = -0.55, p less than 0.01, respectively). With the changes from low to high sodium intake, there was a corresponding decrease in ouabain binding (p less than 0.01) and an increase in sodium excretion (p less than 0.01). A significant negative correlation was observed between these two parameters (r = -0.47, p less than 0.05). CONCLUSIONS: These findings suggest that a circulating digitalis-like factor, which may act on renal tubular cells as the ouabain-displacing compound, is increased in patients with essential hypertension and also demonstrate that plasma levels may be influenced by changes in dietary sodium intake. | 3 |
Catheterization laboratory events and hospital outcome with direct angioplasty for acute myocardial infarction To assess the safety of direct infarct angioplasty without antecedent thrombolytic therapy, catheterization laboratory and hospital events were assessed in consecutively treated patients with infarctions involving the left anterior descending (n = 100 patients), right (n = 100), and circumflex (n = 50) coronary arteries. The groups of patients were similar for age (left anterior descending coronary artery, 59 years; right coronary artery, 58 years; circumflex coronary artery, 62 years), patients with multivessel disease (left anterior descending coronary artery, 55%; right coronary artery, 55%; circumflex coronary artery, 64%), and patients with initial grade 0/1 antegrade flow (left anterior descending coronary artery, 79%; right coronary artery, 84%; circumflex coronary artery, 90%). Cardiogenic shock was present in eight patients with infarction of the left anterior descending coronary artery, four with infarction of the right coronary artery, and four with infarction of the circumflex coronary artery. Major catheterization laboratory events (cardioversion, cardiopulmonary resuscitation, dopamine or intra-aortic balloon pump support for hypotension, and urgent surgery) occurred in 10 patients with infarction of the left anterior descending coronary artery, eight with infarction of the right coronary artery, and four with infarction of the circumflex coronary artery (16 of 16 shock and six of 234 nonshock patients, p less than 0.001). There was one in-laboratory death (shock patient with infarction of the left anterior descending coronary artery). | 4 |
Neuroendocrine carcinoma of the ampulla of vater. A case of absence of somatostatin in a vasoactive intestinal polypeptide-, bombesin-, and cholecystokinin-producing tumor. A 31-year-old patient with a clinical picture of obstructive jaundice had surgical treatment, and a primary carcinoid of the ampulla of Vater (VA) was found. The tumor was studied with light microscopy, immunohistochemistry, and electron microscopy. The neoplasm had histopathologic and cytopathologic features similar to those encountered in typical neuroendocrine neoplasms. It is interesting that immunohistochemical techniques disclosed the presence of vasointestinal polypeptide, cholecystokinin, and bombesin; however, unlike most neuroendocrine neoplasms arising in VA, no somatostatin-immunoreactive cells were found. | 0 |
Referral of patients with primary biliary cirrhosis for liver transplantation. All patients with primary biliary cirrhosis referred to this unit for consideration for transplantation between April 1981 and January 1989 were analysed retrospectively to assess whether disease stage at referral affects the outcome after grafting and whether greater awareness of the benefits of the procedure means that patients are now being referred at an earlier stage. Seventy of the 107 patients have been grafted, with an overall one year actuarial survival of 62%. A better prognosis at the time of referral, as assessed by both serum bilirubin concentration and a mathematically derived prognostic index, was associated with a greater probability of survival after grafting. Patients in the tertile with the best prognosis (median serum bilirubin concentration at referral 84 mumol/l and estimated survival in the absence of transplantation of more than nine months) had a 78% one year actuarial survival after transplantation, whereas those in the tertile with the worst prognosis (median serum bilirubin concentration 467 mumol/l and estimated survival of less than four months) had a one year actuarial survival of only 50%. No trend towards earlier referral of patients, however, was shown using either of these two markers. This retrospective analysis suggests that many patients are being referred too late for an optimal outcome. We recommend that patients with primary biliary cirrhosis who are potential candidates for liver grafting should be referred to a transplant centre before the serum bilirubin concentration approaches 150 mumol/l. | 1 |
US-guided fine-needle aspiration biopsy of gallbladder masses. Ultrasonically (US) guided fine-needle aspiration biopsy was performed in 88 patients who had gallbladder masses. All masses were less than 4.8 cm in diameter. A 22- or 23-gauge, Teflon-coated needle was placed into the mass with the transhepatic or transperitoneal route. By means of this technique, gallbladder malignancy was confirmed in 69 of the 78 cases of malignancy (88.5%). Ten of 10 benign lesions were properly categorized. One patient developed bile peritonitis following a single needle pass with the transperitoneal approach. Pain, a minor complication of the procedure, was noted in four cases. No instances of hemorrhage or vasovagal reactions occurred. From their experience in this large series, the authors conclude that US-guided fine-needle aspiration biopsy of gallbladder masses is a safe, reliable, and accurate technique for the diagnosis of malignancy. | 1 |
Frequency of three Hex A mutant alleles among Jewish and non-Jewish carriers identified in a Tay-Sachs screening program. Mutations in the HEX A gene, encoding the alpha-subunit of beta-hexosaminidase A (Hex A), are the cause of Tay-Sachs disease as well as of juvenile, chronic, and adult GM2 gangliosidoses. We have examined the distribution of three mutations--a 4-nucleotide insertion in exon 11, a G----C transversion at a 5' splice site in intron 12, and a 269Gly----Ser amino acid substitution in exon 7--among individuals enzymatically diagnosed as carriers of Hex A deficiency. Mutation analysis included polymerase chain reaction (PCR) amplification of the relevant regions of genomic DNA, followed by allele-specific oligonucleotide hybridization; another test for heterozygosity of the exon 11 insertion was based on the formation of heteroduplex PCR fragments of low electrophoretic mobility. The percentage distribution of the exon 11, intron 12, exon 7, and unidentified mutant alleles was 73:15:4:8 among 156 Jewish carriers of Hex A deficiency and 16:0:3:81 among 51 non-Jewish carriers. Regardless of the mutation, the ancestral origin of the Jewish carriers was primarily eastern and (somewhat less often) central Europe, whereas for the non-Jewish carriers it was western Europe. Because a twelfth of the Jewish carriers and four-fifths of the non-Jewish carriers of Hex A deficiency had mutant alleles other than the three common ones tested, enzyme-based tests cannot be replaced by DNA-based tests at the present time. However, DNA-based tests for two-carrier couples could identify those at risk for the chronic/adult GM2 gangliosidoses rather than for infantile Tay-Sachs disease. | 2 |
Flow cytometric analysis of terminal deoxynucleotidyl transferase. Terminal deoxynucleotidyl transferase (TdT) is a useful marker for lymphoid precursor cells. In this study, the authors used flow cytometry (FCM) to analyze TdT expression in human hematopoietic malignancies. Cells were fixed in 0.5% formaldehyde, briefly exposed to nonionic detergent, and subsequently labeled with mouse monoclonal anti-TdT antibodies followed by fluorescein-conjugated antimouse IgG and propidium iodide (PI), which was used for the simultaneous analysis of DNA content. Cells from 20 of 22 acute lymphoblastic leukemias (ALL), 4 of 7 mixed lineage leukemias, 2 of 21 acute myeloid and myelomonocytic leukemias, 1 of 2 chronic myeloid leukemias in blast crisis, 1 lymphoblastic lymphoma, and 1 thymoma were TdT positive. Cells from 13 nonlymphoblastic lymphomas, 3 myelodysplastic bone marrows, and peripheral blood mononuclear cells from 29 normal individuals were negative. An excellent correlation was seen between this assay, the conventional slide immunofluorescence technique, and an enzyme immunoassay method. The FCM assay detected as few as 2% blasts in mixing experiments of TdT-containing leukemic cells with normal peripheral lymphocytes. Furthermore, the combined analysis of TdT and DNA allowed the recognition of aneuploid TdT positive cells in four cases of ALL. The high sensitivity, the quantitative information obtained, and the capability of simultaneously analyzing DNA content make this method of TdT analysis more valuable than conventional techniques. | 0 |
Newer antihypertensive agents. Three recent additions to the list of antihypertensive agents have been approved for use as monotherapy or in combination with other drugs. Betaxolol hydrochloride (Kerlone) maintains its effect for 24 hours, making it a true once-a-day beta blocker. Penbutolol sulfate (Levatol) is as effective as other beta blockers and diuretics. Doxazosin mesylate (Cardura), a selective alpha 1 blocker, also allows once-a-day dosing and has produced favorable changes in lipid profiles. Two new drug delivery systems, one for verapamil hydrochloride (Verelan) and one for extended-release nifedipine (Procardia XL), allow less frequent dosing and may offer other advantages, such as greater compliance and a more tolerable side-effect profile. | 3 |
Patients' expectations of patient-controlled analgesia. Patient-controlled analgesia is an increasingly popular method of postoperative pain relief. However, patients often worry about new therapies. Eighty ASA 1 and 2 patients aged 18-65 years were asked to list the advantages and disadvantages of using patient-controlled analgesia. The most important advantage as perceived by patients was the reduced time spent by nurses in giving medication, but there was concern that direct personal contact would also be lessened. Preservation of self control, autonomy, rapid onset of analgesia, ability to titrate analgesia and lack of injections were seen as an advantage. Addiction and machine faults were seen as minimal problems. Preservation of patient-nurse contact is of great importance to ensure success of postoperative analgesia. | 4 |
Experimental intracerebral hemorrhage: early removal of a spontaneous mass lesion improves late outcome. The purpose of the present study was to determine whether early removal of an experimental intracerebral mass altered cerebral blood flow, brain water content, neuropathological findings, or neurological function 24 hours later. In three experimental series, a 50-microliter balloon was inflated within the right caudate nucleus in rats. At 24 hours after inflation, we studied cerebral blood flow by quantitative autoradiography, brain specific gravity, and qualitative histopathology by light microscopy. The animals were also assessed using a simple neurological deficit scale. In each series, half of the animals had the balloon inflated for 10 minutes (Group 1), and half had the balloon inflated permanently (Group 2). After transient inflation, there were surprisingly small differences in the blood flow between the two cerebral hemispheres at 24 hours. By contrast, in animals with permanent inflation, several indices of blood flow were significantly worse in the hemisphere ipsilateral to the balloon: the mean and median blood flow levels in the caudate nucleus; the mean blood flow in the cerebral cortex; and the area of cortex with ischemic levels of blood flow. The specific gravity was reduced in areas surrounding the site of balloon insertion after either transient or permanent inflation, and there was evidence of ischemic cell damage in all animals studied. These changes were more severe after permanent inflation, but the differences were not significant. Neurological outcome was significantly better after transient as compared with permanent inflation. The present findings contradict previous results and suggest that early removal of an intracerebral mass may have subsequent benefits. | 4 |
The effect of thromboxane synthetase inhibition on cardiopulmonary function during endotoxemia in sheep. The early pulmonary hypertension seen with endotoxin (lipopolysaccharide) has been reported as resulting from the release of thromboxane A2. We studied the cardiopulmonary response to endotoxin in sheep with and without treatment with a thromboxane synthetase inhibitor, OKY-046. The animals were implanted with instruments for crystallographic dimension analysis of the left ventricle and measurement of left ventricular, aortic, left atrial, and pulmonary arterial pressures and cardiac index. Thirteen sheep received 1.0 micrograms/kg of Escherichia coli endotoxin with (n = 6) and without (n = 7) OKY-046 (10 mg/kg bolus, then 10 micrograms/kg/min). OKY-046 prevented the increase in pulmonary arterial pressure and decrease in cardiac index usually seen during the early phase of endotoxemia. Between 8 and 12 hours after the administration of endotoxin, cardiac index increased from 6.4 +/- 0.8 to 8.4 +/- 0.8 L/min/m2. Concomitantly, the end-systolic pressure/diameter relationship (a sensitive myocardial contractility index) significantly decreased from 14.7 +/- 0.6 to 7.7 +/- 0.7 mm Hg/mm. Another index of the left ventricular contractility, the maximum rate of pressure rise was also reduced. OKY-046 prevented decreases in end-systolic pressure/diameter relationship and maximum rate of pressure rise. | 3 |
Genetics and psychiatry: an unheralded window on the environment. Two recent reviews in the American Journal of Psychiatry and the British Journal of Psychiatry reported on progress in understanding the genetics of psychiatric disorder. Both reviews focused on this progress as a prelude to psychiatric diagnostics and therapeutics based on molecular biology. Neither review recognized that the latest data in behavioral genetics support environmental causes for abnormal development and psychopathology as much as they support genetic causes. Moreover, these genetic data point clearly to a type of environmental cause with central importance: the environment that is specific or unique to each sibling in a family. | 4 |
Orofacial metastasis of pulmonary giant cell carcinoma. A patient was admitted to the hospital with multiple skin nodules of recent origin and signs and symptoms suggestive of acute pulmonary infection. Because one of the skin masses was located overlying the left mandibular body region, the patient was referred to the hospital dentistry clinic for evaluation. Historical, clinical, and radiographic assessments were consistent with reactive lymphadenopathy, and an intraoral excisional biopsy was performed. The biopsy results were indicative of giant cell carcinoma, which was confirmed by biopsy results from a similar skin lesion on the shoulder and by malignant cells recovered in the sputum and pleural fluids. Although this carcinoma has been shown to rarely metastasize to the skin and lymphatics of the neck, this is the first case report of metastasis to facial lymphatics. | 0 |
Levels of the human hepatocyte growth factor in serum of patients with various liver diseases determined by an enzyme-linked immunosorbent assay. We have found a hepatotrophic factor in plasma or sera of patients with fulminant hepatic failure and have purified human hepatocyte growth factor from plasma of these patients. In this study we developed an enzyme-linked immunosorbent assay with high specificity and sensitivity for human hepatocyte growth factor in human serum. This assay for serum human hepatocyte growth factor is a sandwich method consisting of three steps. The standard curve for human hepatocyte growth factor appeared to be linear in the range of 0.20 to 12.50 ng purified human hepatocyte growth factor/ml (2.35 to 147 pmol/L). The assay took about 4 hr. Serum human hepatocyte growth factor values in patients with fulminant hepatic failure measured by enzyme-linked immunosorbent assay showed a strong positive correlation with that by bioassay using rat hepatocytes in primary culture. The mean value of serum human hepatocyte growth factor for 30 normal subjects was 0.24 +/- 0.12 (S.D.) ng/ml; that for 23 patients with fulminant hepatic failure was 8.06 +/- 1.76 (S.E.M.) ng/ml- greater than 30 times greater than the mean value for normal subjects. Serum human hepatocyte growth factor levels in patients with acute hepatitis, chronic hepatitis and cirrhosis were found to be slightly higher than those in normal subjects, but only the increase in serum human hepatocyte growth factor of acute hepatitis patients was statistically significant. The enzyme-linked immunosorbent assay for serum human hepatocyte growth factor should prove useful for serum human hepatocyte growth factor level measurement in patients with various liver diseases. | 2 |
MY7 monoclonal antibody for diagnosis of cutaneous T-cell lymphoma. Infiltrate in cutaneous T-cell lymphomas (CTCLs) is composed mainly of CD4 helper cells with a phenotype very similar to that of benign cutaneous lymphoid infiltrate. MY7 (CD13) is a monoclonal antibody that is normally expressed on peripheral granulocytes and monocytes but also cross-reacts with an antigen expressed on epidermal basal cells. We studied MY7 expression on basal cells of the epidermis and CD4 cell infiltrate in 34 CTCLs, 11 pseudolymphomas, and 29 other benign cutaneous lesions. An indirect immunofluorescence technique with double labeling and an immunoperoxidase technique were used. We found that in benign inflammatory infiltrate, less than 10% of CD4 cells expressed MY7 antigen associated with normal MY7 monoclonal antibody labeling of basal cells, whereas in CTCLs more than 50% of CD4 tumoral cells in dermis expressed MY7 antigens; however, basal cells were MY7 negative. Thus, it is demonstrated that MY7 monoclonal antibody with its double modulation on epidermis (basal cells) and dermis (CD4 cells) has diagnostic value for differentiating CTCLs with CD4+ MY7+ tumor cells in dermis and MY7-negative basal cells from benign inflammatory lesions with CD4+ MY7- cells in dermis and MY7-positive basal cells. This modulation of MY7 labeling could be related to the secretion of epidermal cytokines. | 0 |
Increased risk of leukemia relapse with high-dose cyclosporine A after allogeneic marrow transplantation for acute leukemia. Eighty-one patients with acute myeloid leukemia (ANLL, n = 44) or acute lymphoblastic leukemia (ALL, n = 37), aged 10 to 50 years were randomized to receive 1 mg/kg per day (n = 41, group A) or 5 mg/kg per day (n = 40, group B) of cyclosporine A (CyA) from day -1 to day +20 after bone marrow transplant (BMT). All patients received CyA orally thereafter. All patients were prepared with cyclophosphamide (CY) 120 mg/kg and fractionated total body irradiation (TBI), and received unfractionated BM from an HLA-identical sibling. The two groups were comparable for diagnosis, disease status, French-American-British (FAB) classification, WBC count at diagnosis, cytogenetic abnormalities, extramedullary disease before BMT, donor/recipient age and sex, number of cells infused, and number of days with intravenous (IV) CyA. Median follow-up for surviving patients in group A was 983 v 632 days in group B. Patients in group A had lower serum levels of CyA (295 v 686 ng/mL, P = .004), lower bilirubin levels (1.9 v 2.6 mg/dL, P = .07), lower creatinine levels (0.9 v 1.4 mg/dL, P = .06), and a lower proportion of CD8+ cells in the peripheral blood (PB) within day +21 (19% v 28%, P = .07). First day to 0.5 x 10(9)/L neutrophils was comparable in the two groups (13 v 14 days; P = .1). In a Cox model, the actuarial risk of acute graft-v-host disease (GVHD) grade II+, after stratification for age (less than 20 years greater than) was significantly lower in group B patients (0.54, P = .04). The actuarial risk of developing chronic GVHD was comparable (P = .9). Actuarial transplant-related mortality (TRM) at 240 days was 28% and 26% (P = .8) in group A and B: the major cause of death was GVHD in group A (P = .02) and multiorgan toxicity in group B (P = .07). The actuarial risk of relapse at 2 years overall was 20% in group A and 52% in group B (P = .001); it was 9% v 43%, respectively, for patients in first remission (P = .0001) and 48% v 63% for patients in non-first complete remission (CR) (P = .1). Actuarial 2-year disease-free survival (DFS) in group A and B was 58% v 32% (P = .02) for all patients, 71% v 35% (P = .01), in first remissions, and 30% v 23% (P = .2) in advanced disease.(ABSTRACT TRUNCATED AT 400 WORDS). | 4 |
Intra-abdominal "angiosarcomatosis" report of two cases after pelvic irradiation. Angiosarcomas account for only 1% to 2% of all soft tissue sarcomas with the most common site of origin being skin and subcutaneous tissue particularly on the face and scalp of elderly patients. These neoplasms rarely arise in the small or large bowel with presentation as diffuse abdominal "angiosarcomatosis." Two patients are reported who were treated with adjuvant pelvic irradiation for gynecologic malignancies (squamous cell carcinoma of the cervix and adenocarcinoma of the endometrium) in whom diffuse abdominal angiosarcoma of intestinal origin developed 7 and 18 years later. Both patients were treated with standard megavoltage irradiation. The clinical course was rapid in both cases with death due to intra-abdominal hemorrhage and bowel obstruction. The pertinent literature relating to the association between radiation therapy and subsequent development of angiosarcomas of soft tissues and other sites is briefly reviewed. | 0 |
Radiation necrosis of the optic chiasm, optic tract, hypothalamus, and upper pons after radiotherapy for pituitary adenoma, detected by gadolinium-enhanced, T1-weighted magnetic resonance imaging: case report. A 26-year-old woman was treated for a prolactin secreting pituitary adenoma by surgery and radiotherapy (5860 rads). Fourteen months later, she developed right hemiparesis and dysarthria. A T1-weighted magnetic resonance imaging scan using gadolinium contrast showed a small, enhanced lesion in the upper pons. Seven months later, she had a sudden onset of loss of vision, and radiation optic neuropathy was diagnosed. A T1-weighted magnetic resonance imaging scan showed widespread gadolinium-enhanced lesions in the optic chiasm, optic tract, and hypothalamus. Magnetic resonance imaging is indispensable for the early diagnosis of radiation necrosis, which is not visualized by radiography or computed tomography. | 0 |
The frequency, causes and timing of death within 30 days of a first stroke: the Oxfordshire Community Stroke Project. In a prospective, community-based study of 675 consecutive patients with a first-ever stroke, of whom over 90% had computed tomography (CT) and/or necropsy examinations, 129 deaths occurred within 30 days of the onset of symptoms, a case fatality rate (CFR) of 19%. The 30 day CFR for patients with cerebral infarction was 10% (57 of 545, for primary intracerebral haemorrhage 52% (34 of 66), for subarachnoid haemorrhage 45% (15 of 33) and for those of uncertain pathological type 74% (23 of 31). The CFR for patients who had been functionally dependent pre-stroke was 33% compared with 17% for those who had been independent pre-stroke. The age-adjusted relative risk of death for patients who had been functionally dependent pre-stroke was not significantly greater (1.8, 95% confidence interval 0 to 4.3). There was a significant trend for CFR to increase with age (Chi square for trend = 4.0, p less than 0.05). This relationship was found in those patients who had been functionally independent prestroke (Chi square for trend = 7.9, p less than 0.005) but not in those who had been dependent pre-stroke (Chi square for trend = 0.5, NS). The pattern of increasing CFR with increasing age amongst those who had been independent prestroke was seen particularly in patients with cerebral infarction (Chi square for trend = 8.6, p less than 0.005). The age-adjusted relative risk of death for patients with cerebral infarction who had been functionally dependent pre-stroke was 2.2 (95% confidence interval 1.2 to 4.1). Fifty three percent of all deaths within 30 days of stroke were due to the direct neurological sequelae of the stroke. | 2 |
Course of diabetic retinopathy following cataract surgery Five patients with mild to moderate retinopathy to both eyes underwent complication-free cataract surgery in one eye. Within three months of surgery deterioration of the retinopathy was observed in the operated eye only. In four patients there was an increase of intraretinal haemorrhages and hard exudates, accompanied by clinically significant macular oedema manifested as retinal thickening and extensive fluorescein leakage from both the macular and the peripapillary capillary networks. Of these four patients one also developed retinal ischaemia, evident ophthalmoscopically by flame-shaped haemorrhages and cotton-wool spots and angiographically by areas of capillary non-perfusion. The fifth patient showed proliferation of new blood vessels and vitreous haemorrhage. Diabetic patients scheduled for cataract surgery should undergo a thorough preoperative evaluation of any existing retinopathy. Postoperatively they should be followed up at close intervals so that any progression of retinopathy can be promptly detected and considered for laser treatment. | 3 |
Cholesterol cysts of the temporal bone: diagnosis and treatment. Cholesterol cyst (or granuloma) of the temporal bone, a recognized clinical entity distinct from cholesteatoma, is more common than previously thought. Apparently it is caused by obstruction of previously pneumatized temporal bone air cells. Surgical cure is achieved by drainage and reestablishment of normal pneumatization. This paper reviews 14 cholesterol cysts of the temporal bone, emphasizing the importance of preoperative imaging and surgical approach. Use of magnetic resonance imaging differentiates cholesterol cysts from cholesteatoma or other neoplasms. Computed tomography delineates the location of the lesion and defines temporal bone anatomy essential to surgical approach. The two studies together allow the surgeon to properly plan drainage, as in the case of a cholesterol cyst, versus excision or exteriorization, as in the case of cholesteatoma. The infralabyrinthine approach to a petrous apex cholesterol cyst is the procedure of choice when hearing preservation is desired. | 4 |
Persistence of arrhythmia exercise response in healthy young men. This study assesses the persistence of arrhythmia at rest or during exercise tests, or both, after a mean follow-up period of 6.7 years in 76 young men (mean age 21.5 years) without evidence of organic heart disease. The exercise test was performed using a near-maximal protocol based on progressively increasing intermittent work loads, each of 5 minutes' duration. The initial work load was 50 W. The electrocardiogram was continuously registered throughout all stages of the examination. Arrhythmia was defined as the occurrence of greater than or equal to 1 supraventricular or 1 ventricular premature beat at any stage of the examination. At the follow-up examination, the rate of persistence of arrhythmia did not differ significantly among the subgroups, irrespective of follow-up interval, type of arrhythmia, or arrhythmia patterns of response to exercise. Two-dimensional echocardiography did not show any structural abnormalities and Doppler examination did not demonstrate significant abnormal flow patterns. Our data show that almost all patients continued to present arrhythmia after the follow-up period, without any evidence of development of organic heart disease. Moreover, the arrhythmia pattern of response to exercise remained constant throughout the years. At this time, arrhythmia without underlying heart disease seems to be of a benign natural course in these young men. | 4 |
Biology of basal cell carcinoma (Part I). Basal cell carcinoma is the most common malignancy in humans. Although rarely metastatic, it is capable of significant local destruction and disfigurement. This two-part article reviews the current understanding of basal cell carcinoma biology. Part I examines significant clinical, histologic, and ultrastructural features that relate to invasive potential. Genetic characteristics, including tumor growth rate, chromosomal abnormalities, and oncogene presence, are discussed, and expression of important cell and matrix proteins, including keratin, fibronectin, and HLA antigens, are reviewed. Further topics to be explored in Part II include host immunologic responses, theories of pathogenesis, and valuable second-line therapeutic regimens for treatment of multiple cancers. | 0 |
Two-dimensional echocardiographic features of double outlet left ventricle. In a cyanotic newborn infant, the diagnosis of double outlet left ventricle was made from the two-dimensional echocardiographic examination. The diagnosis was later confirmed at cardiac catheterization and surgery. The parasternal and subcostal views were especially useful for identification of the origin of both great arteries from the morphologic left ventricle. A review of the medical literature since 1967 revealed 77 cases of double outlet left ventricle, most of which were diagnosed only at surgery or postmortem examination. The anatomic features demonstrated with two-dimensional echocardiography in this case are representative of the findings cited most often in the cases reported in the medical literature. | 3 |
The role of alpha adrenoceptor subtypes in sympathetic control of the acral-cutaneous microcirculation. In pithed and anesthetized rats, laser-Doppler flowmetry was used to evaluate the role of alpha-1 and alpha-2 adrenoceptors in mediating sympathetic responses in acral regions of the cutaneous circulation. The intravenous administration of the selective alpha-1 agonist, phenylephrine, was a more potent vasopressor agent than BH-T 933 (a selective alpha-2 adrenoceptor agonist) in pithed rats. However, BH-T 933 was more potent and more efficacious than phenylephrine in reducing cutaneous microvascular perfusion (CP). BH-T 933 also caused a greater increase in cutaneous microvascular resistance. Neural and humoral sympathetic effects on CP were characterized with selective alpha-1 and alpha-2 adrenoceptor antagonists (prazosin and rauwolscine, respectively). It was found that frequency-related reductions in CP elicited by sciatic nerve stimulation were antagonized by prazosin, but not by rauwolscine. In fact, rauwolscine enhanced neurally evoked reductions in CP at the highest stimulation frequencies. However, both prazosin and rauwolscine antagonized reductions in CP elicited by electrical stimulation of the thoracolumbar outflow (sympathoadrenal activation). Ganglionic stimulation (intravenous 1,1-dimethyl-4-phenylpiperazinium) also caused a profound, transient reduction in CP that was abolished by rauwolscine, but was not significantly altered by prazosin. In contrast, 1,1-dimethyl-4-phenylpiperazinium-induced increases in mean arterial pressure were reduced by prazosin, but not by rauwolscine. In ketamine-anesthetized rats, rauwolscine caused a dose-related increase in CP without altering mean arterial pressure, whereas prazosin lowered mean arterial pressure but did not alter CP. We conclude that acral regions of the cutaneous vasculature are more sensitive to alpha-2 vis-a-vis alpha-1 adrenoceptor-mediated vasoconstriction. | 3 |
Genetics of common diseases of adulthood. Implications for prenatal counseling and diagnosis. Genetic factors play an important role in the development of many common diseases of adulthood that result in early morbidity and mortality. Prevention of these disorders and their sequelae is best established through early detection and early intervention. Although it may be feasible to screen the entire population for some disorders (e.g., hypertension), this approach would be expensive and impractical for others (e.g., colon cancer). The family history provides an inexpensive and convenient method of identifying families at risk for premature diseases of adulthood. Family screening for a disorder should be recommended if there is increased risk for the disorder among family members, if screening methods are available to detect the condition at an early age or preclinical stage, and if early intervention will alter the course of the disease. For many disorders screening and intervention can prevent the occurrence of clinical disease. The prenatal counseling session affords an ideal setting for identifying families at risk for diseases of adulthood with major genetic components. By reviewing the family history, key family members can be identified and investigated, in order to establish a specific genetic diagnosis. At-risk relatives can then be counseled and screened for the disorder preclinically and premorbidly. The screening and intervention available for a disease depends on the nature of the disorder, our understanding of its physiology and etiology, and our current technology. The disorders discussed earlier are typical of conditions of adulthood that are influenced strongly by genetic factors, especially when they appear in younger adults. Atherosclerosis, colon cancer, and diabetes are complex phenotypes. Each can be caused by single-gene defects, but commonly the genetics are more complex. Empiric data help to establish the risk to an individual in the latter cases. In all three examples, early detection should lead to treatment, which can prevent more serious sequelae: by treating the dyslipidemia, coronary artery disease can be prevented; by removing the benign polyp, malignant cancer can be avoided; and when impaired glucose tolerance is detected, diet and exercise can prevent or delay frank diabetes and its complications. The complete evaluation of individuals at risk for disorders such as those in Table 1 and their families can be a complicated task. Referral to a center experienced in the genetics of common diseases often may be necessary.(ABSTRACT TRUNCATED AT 400 WORDS). | 0 |
Fine structure of active and healed duodenal ulcer. In order to characterize the fine structure of active and healed duodenal ulcers, we examined tissue specimens of patients with active duodenal ulcer disease (n = 30) before and after treatment with either antacids (n = 16) or H2-receptor antagonists (n = 14), by light microscopy and various electron microscopic techniques, e.g., scanning and transmission electron microscopy. The characteristic histological feature of both the active and healed duodenal ulcer was the appearance of periodic acid-Schiff (PAS)-positive epithelial cells at the edge of the ulcers. Electron microscopy revealed that these cells were similar to a special type of mucus-secreting cell in the antrum (surface mucous cell). Their mucus granules contained mainly neutral glycoproteins. Helicobacter pylori were found attached to these cells in tissue specimens from 12 of 30 patients (40%). The mucous structure destroyed during the ulcerative phase regained its normal net-like structure after treatment. The ultrastructural healing process of duodenal ulcer was characterized by the presence of gastric metaplasia, by stunted microvilli of the duodenal epithelium (p less than 0.001 vs. control group), and an increased number of lysosome-like bodies (p less than 0.001 vs. control group) of the epithelial cells. These results were independent of the type of treatment, and showed that the repair mechanisms were incomplete after a 4-wk period of treatment. | 4 |
Bone sarcomas as second malignant neoplasms following childhood cancer. This study explores the relationship between histologic variants of bone sarcomas and previous therapy in patients in whom an unrelated malignant neoplasm had been diagnosed during childhood. Sarcomas of bone were the most common second malignant neoplasm (SMN) reported to the Late Effects Study Group, a 13-institution consortium consisting of pediatric oncology centers from western Europe, Canada, and the United States. The authors attempted to relate the histologic subtypes of the 91 bone tumors to clinical factors such as previous therapy and genetic predisposition because morphologic variants have been shown to have biologic significance in other tumors and may have etiologic import. The literature concerning the subtypes of bone tumors, clinical and experimental, is also reviewed. The authors also investigated the effect of several factors on the time interval from the first diagnosis to the SMN (i.e., the bone sarcoma). Anthracyclines significantly shortened the interval by about 3 years. The primary diagnosis also significantly affected the interval, with leukemia/lymphomas having the shortest interval and retinoblastoma the longest. The authors could not demonstrate any significant relationship between morphologic characteristics of the osteosarcoma and predisposing conditions. However, lesions diagnosed as chondrosarcoma and malignant fibrous histiocytoma occurred almost exclusively in patients who had received radiation therapy to the site in which the SMN developed. | 0 |
Factors predictive of results of direct ablative operations for drug-refractory ventricular tachycardia. Analysis of 80 patients. During a 9-year period 80 patients with drug-resistant sustained ventricular tachycardia underwent direct surgical ablation of arrhythmogenic myocardium. Sixty-nine were male (86%) and 11 female (14%), with 1.9 +/- 1.1 (standard deviation) ventricular tachycardia morphologies per patient. The mean number of drugs failed was 3.7 +/- 1.6 per patient. The preoperative left ventricular ejection fraction was 36.4% +/- 14.4%. Complete preoperative endocardial mapping data (greater than 4 endocardial sites in each ventricular tachycardia) were available for 60 of the 80 patients (75%) and intraoperative endocardial data in the clinical ventricular tachycardia was obtained in 37 (46.3%) of the patients. In 17 patients mapped intraoperatively by computer-assisted techniques, complete epicardial and endocardial data in the clinical ventricular tachycardia were obtained in 14 patients (82.4%). Overall, 73 of 80 (91.3%) had some mapping data available. Hospital mortality occurred in 10 patients (12.5%) at a mean interval of 13.5 days, range 0 to 62 days. Postoperatively the clinical ventricular tachycardia has not recurred in 65 of 70 surviving patients (92.9%). Nonclinical ventricular tachycardia occurred in another four patients. All nine patients with postoperative ventricular tachycardia responded to drugs. The major factors predictive of hospital mortality were prolonged cardiopulmonary bypass (greater than 150 minutes), preoperative ejection fraction less than 31%, and incomplete preoperative mapping. Hospital mortality in patients with an ejection fraction below 31% was significantly associated with a history of amiodarone usage. At 3 years of follow-up, freedom from sudden cardiac death was 95.7%, and 86.7% of patients were free of ventricular tachycardia on no antiarrhythmic drugs. These results suggest that direct ventricular tachycardia operations are an effective form of therapy for patients with sustained monomorphic ventricular tachycardia. | 3 |
Excision repair of pyrimidine dimers induced by simulated solar radiation in the skin of patients with basal cell carcinoma. One prominent lesion induced in DNA by ultraviolet (UV) radiation is the cyclobutyl pyrimidine dimer formed between adjacent pyrimidines on the same DNA strand. We investigated whether people who have developed basal cell carcinoma on sun-exposed skin have an altered ability to repair UV-induced pyrimidine dimers in DNA. Twenty-two patients with at least one basal cell carcinoma, aged 31-84 years, and 19 healthy volunteers, aged 25-61 years, took part in the study. Both groups were given one minimal erythema dose (MED) of simulated solar radiation on the lower back. DNA was extracted from the irradiated skin 0 to 6 h later, and the number of UV-induced pyrimidine dimers was determined using a dimer-specific endonuclease. At time 0, the average number of dimers per unit of DNA was similar in the two groups. After 6 h, an average of 22 +/- 4% of the dimers were removed in the group with basal cell carcinoma compared to 33 +/- 4% in the cancer-free group. In the basal cell carcinoma group, only 23% of the patients repaired more than 30% of the dimers after 6 h, compared with 53% of the cancer-free subjects (p less than 0.05). We conclude that patients who develop basal cell carcinoma on sun-exposed skin may have a decreased ability to repair pyrimidine dimers induced in skin exposed to simulated solar radiation. | 0 |
Hypoplastic transverse arch and coarctation in neonates. Surgical reconstruction of the aortic arch: a study of sixty-six patients. From Jan. 1, 1983, to Jan. 1, 1988, 66 consecutive neonates with coarctation and severe hypoplasia of the transverse arch underwent coarctation repair by resection of the coarctation and reconstruction of the aortic arch. Mean age at operation was 14 +/- 8 days, ranging from 2 to 30 days; 63% of the newborn infants were less than 2 weeks of age. The coarctation was isolated in 23%, associated with a ventricular septal defect in 39%, and associated with complex anomalies in 38%, including 16 cases of transposition of the great arteries or doublet-outlet right ventricle plus ventricular septal defect, two cases of simple transposition, two of corrected transposition plus ventricular septal defect, and five cases of "hypoplastic" left ventricle. The surgical technique comprises a wide resection of the coarctation extended to the contiguous ductal tissue followed by the reconstruction of the aortic arch in bringing the descending aorta into the concavity of the aortic arch. This technique is able to relieve the obstruction of the aortic arch provided that (1) the descending aorta is widely dissected to allow mobilization and (2) the incision of the transverse arch is extended proximal to the ostium of the left carotid artery. The operation was performed through a left thoracotomy in 62 patients and through a sternotomy in four additional neonates with transposition and ventricular septal defect who underwent a one-stage repair with aortic reconstruction, closure of the defect, and arterial switch. The overall early mortality rate (less than 30 days) was 14% (9/66; 95% confidence limits = 5% to 22%), including four deaths occurring within the first month, at a concomitant or subsequent repair of the associated anomaly. There were six late deaths, all related to the associated lesions. The overall mortality rate was 23% (15/66; 95% confidence limits = 13% to 33%). The mean follow-up was 21 +/- 10 months, ranging from 6 to 66 months. Actuarial survival rates at 5 years are 72% +/- 10% for the overall group; 87% +/- 17% for simple coarctation; 88% +/- 12% for coarctation and ventricular septal defect; and 52% +/- 18% for complex coarctation. The rate of recurrent coarctation was 12.5% (95% confidence limits = 2% to 23%), leading to five reoperations with no deaths. Freedom from reoperation was 89.5% +/- 9% at 5 years. This technique of coarctation repair offers several advantages: low operative mortality, complete relief of the left ventricular obstruction, wide resection of the ductus tissue, absence of prosthetic material, and preservation of the left subclavian artery. | 4 |
Chronic dehydration stone disease. A study was made of 819 patients attending a metabolic stone clinic. A firm diagnosis was made in 708 (86%) and in 132 of these (19%) the diagnosis was thought to be chronic dehydration. The records were available for study for 87 males and 11 females in the chronic dehydration group. The mean age at presentation was 43 years. The causes of chronic dehydration were hot climate (62%), with hot occupation and low water intake almost equal in second place. In patients with a single cause of chronic dehydration, 57% also had a dietary risk factor for urolithiasis and this was most commonly high oxalate intake. Following dietary advice, the mean urinary volume increased from 1720 to 2475 ml/24 h. This was accompanied by a rise in mean urinary calcium from 6.02 to 6.96 mmol/24 h, presumably due to the calcium in the additional water drunk. Urinary oxalate did not change significantly. The mean follow-up time was 4.85 years and the stone recurrence rate was low. It was concluded that chronic dehydration is a common cause of urolithiasis; this can be treated satisfactorily by increasing water intake plus dietary advice in certain cases. | 4 |
A newly established human osteosarcoma cell line with osteoblastic properties. A human osteosarcoma cell line, HuO9, was established from a tumor that was heterotransplanted into athymic nude mice. Antiserum against nude mouse spleen cells was added to the early passage cultures to eliminate the host fibroblastic cells. The cell line retained a high activity of liver/bone/kidney-type alkaline phosphatase (ALP) and secreted osteocalcin, i.e., bone gamma-carboxyglutamic acid-containing protein (BGP), into the medium. The addition of 1,25-dihydroxyvitamin D3 (1,25(OH)2D3) increased the ALP activity as well as the level of BGP secreted into the medium. The ALP of 1,25(OH)2D3-treated cells has the same inhibition characteristics to heat and amino acids as that of untreated cells. Synthetic human parathyroid hormone stimulated the production of intracellular adenosine 3',5'-cyclic monophosphate (cAMP) approximately 100-fold within five minutes. However, the stimulation was not observed with a synthetic human thyrocalcitonin. When HuO9 cells were transplanted into the back of a nude mouse, a tumor with an abundant osteoid formation and mineralization was produced. The results indicate that the HuO9 cell line expresses well-differentiated osteoblastic phenotypes. HuO9 is the first established human cell line to produce BGP, and it provides a useful model for the studies of osteoblasts and the regulatory mechanisms of BGP production. | 0 |
Multiple granular cell tumors associated with giant speckled lentiginous nevus and nevus flammeus in a child. We describe an 11-year-old girl in whom multiple cutaneous granular cell tumors were associated with a giant speckled lentiginous nevus and an extensive nevus flammeus. An association between granular cell tumors and pigmented skin lesions has been reported twice previously and supports a neural origin for these tumors. An abnormality of neural crest development is proposed to explain the coexistence of three uncommon and unusually extensive cutaneous disorders in this patient. This case may represent a further variant of phakomatosis pigmentovascularis. | 0 |
Prospective study on prevalence of esophageal chest pain in patients referred on an elective basis to a cardiac unit for suspected myocardial ischemia. The prevalence of esophageal chest pain was studied prospectively in patients referred on an elective basis to a cardiac unit for suspected myocardial ischemia. A group of 248 consecutive patients without previously documented heart disease was admitted for elective diagnostic coronary angiography. The clinical history classified 185 patients as having anginal pain and the coronary angiogram was normal in 48 of them. In 37 of these 48 patients full esophageal testing was performed including 24-hr intraesophageal pH and pressure recordings with indication of chest pain episodes as well as a number of esophageal provocation tests, ie, acid perfusion, edrophonium stimulation, balloon distension, and ergonovine stimulation, all performed under continuous esophageal manometric and electrocardiographic monitoring. In 19 of these 37 patients, the familiar chest pain could be reproduced by esophageal provocative testing without ischemic ST-T segment alterations; six of these 19 patients had also a positive 24-hr pH and pressure recording. These data strongly suggest an esophageal origin of chest pain in half the patients with typical angina and a normal coronary angiogram. | 1 |
Endoscopic coagulation of the choroid plexus using the Nd:YAG laser: initial experience and proposal for management. Although most patients with hydrocephalus are treated effectively with ventriculoperitoneal shunts, a small group fails to respond to diversion procedures. In some patients within this group, hydrocephalus may be caused in part by the overproduction of the cerebrospinal fluid (CSF). In other patients, previous shunt infections may limit the ability of the peritoneum to absorb fluid, and patient response to a ventriculoperitoneal shunt may be improved by reducing CSF production. We discuss a case of a 29-month-old hydrocephalic infant with a ventriculoperitoneal shunt who had ascites. Computed tomography showed bilateral symmetrical enlargement of the choroid plexus. Repeated cultures of the CSF and shunt tubing were sterile, and the daily production of the CSF exceeded 1000 ml. Therapy with acetazolamide decreased the CSF output temporarily, but it was discontinued after serious electrolyte abnormalities occurred. The endoscopic coagulation of the choroid plexus with a neodymium:YAG laser transmitted through a flexible quartz fiber decreased CSF production effectively. This procedure may be useful in a variety of clinical conditions associated with hydrocephalus or with other intraventricular lesions. | 4 |
Spinal cord stimulation: a contemporary series Forty-three patients with chronic pain disorders of different causes were selected for spinal cord stimulation. All underwent implantation of a ribbon electrode through a small laminotomy, under general anesthesia. Thirteen patients (30%) failed to obtain significant pain relief during a period of trial stimulation, and their electrodes were removed. The remainder underwent a definitive implant and were followed for a mean of 13 months (range, 3-33 months). Nineteen of them (63%) continued to experience pain relief. A detailed analysis of this series, as well as a literature review, is presented. | 2 |
Anorectal function in incontinent patients with cerebrospinal disease. Anorectal manometry and the electrical activity of the external anal sphincter were measured in 20 patients with well-defined, incomplete spinal lesions who were referred because of fecal incontinence and in 30 normal subjects. Six patients had a high spinal lesion, 11 had a low spinal lesion, and 3 had mixed high and low spinal lesions. Patients with high spinal lesions had normal basal pressures but abnormally low squeeze pressures and impaired rectal sensation. Unlike normal subjects, there was no relationship between the depth of sphincter relaxation and the distention volumes. The external sphincter responses to rectal distention and increases in intraabdominal pressure were enhanced, and leakage of perfusion fluid was uncommon. Patients with low spinal lesions had abnormally low basal and squeeze pressures, blunted rectal sensation, and showed impaired external anal sphincter responses to rectal distention or increases in intraabdominal pressures. Most of these patients leaked the infused fluid during these maneuvers. Sphincter function in patients with mixed lesions was more severely impaired than in patients with low and high spinal lesions. Patients with mixed lesions showed abnormally low basal and squeeze pressures, impaired rectal sensation, and no external anal sphincter responses to either rectal distention or increases in intraabdominal pressure. Leakage occurred during these maneuvers in all patients with mixed lesions. | 2 |
Lymphomas of the breast. A clinicopathologic and immunohistochemical study of primary and secondary cases. Primary mammary lymphoma is rare, and little is known about the immunophenotype of such cases. The authors studied both primary and secondary breast lymphomas with a broad panel of T- and B-cell markers using paraffin-embedded tissue and the avidin-biotin immunoperoxidase method. Cases of primary B-cell lymphoma were further tested to determine light and heavy chain type. Thirty-five cases were analyzed, including 16 primary lymphomas. Diffuse large cell lymphoma was present in ten of 16 primary and 14 of 18 secondary cases. Lymphoepithelial lesions in ducts and lobules and frequent vascular involvement were found in both primary and secondary cases. Immunohistochemistry studies on 13 tumors revealed all of the primary tumors to be B-cell in origin, except for one case of primary T-cell lymphoma; to the authors' knowledge, this represents the first description of this entity. Fifteen of 17 secondary tumors exhibited B-cell markers and one of 17 exhibited T-cell markers; in only one case could lineage not be determined. Among primary B-cell cases, IgM was found to be the most frequent heavy chain type; IgA reactivity was found in only one case. Survival was related to stage and histologic characteristics; patients with Stage II disease and higher grade histologic lesions had a worse prognosis. Half of the patients with primary lymphoma have had recurrent disease. Although local recurrences were observed, the authors also saw a tendency for recurrence in other extranodal sites. The authors conclude that, although primary lymphoma of the breast is compatible with long-term survival, a significant number of patients eventually die of their disease. As with other extranodal lymphomas, survival rate appears most related to stage of disease. Also, the vast majority of primary tumors are B-cell in origin, express IgM heavy chain, and can be considered tumors of mucosa-associated lymphoid tissue. | 0 |
Postoperative analgesic requirements in patients exposed to positive intraoperative suggestions. OBJECTIVE--To establish whether positive suggestions given to a patient under general anaesthesia reduce postoperative pain and analgesic requirements. DESIGN--Prospective double blind randomised study. SETTING--Operating theatre and gynaecology ward of a teaching hospital. PATIENTS--63 Woman undergoing elective abdominal hysterectomy were randomised to be played either a tape of positive suggestions or a blank tape during the operation through a personal stereo system. INTERVENTIONS--Three women were withdrawn from the study. Anaesthesia was standardised for all of the women. Postoperative analgesia was provided through a patient controlled analgesia system for the first 24 hours. Pain scores were recorded every six hours. MAIN OUTCOME MEASURES--Morphine consumption over the first 24 hours after the operation; pain scores. RESULTS--Mean morphine requirements were 51.0 mg (95% confidence interval 42.1 to 60.0 mg in the women played positive suggestions; and 65.7 mg (55.6 to 75.7 mg) in those played a blank tape. The point estimate (95% confidence interval) for the difference of means was 14.6 mg (22.4%) (1.9 (2.9%) to 27.3 mg (41.6%] (p = 0.028). Pain scores were similar in the two groups. CONCLUSION--Positive intraoperative suggestions seem to have a significant effect in reducing patients' morphine requirements in the early postoperative period. | 4 |
Concordant Graves' disease after bone marrow transplantation: implications for pathogenesis. The current working hypothesis on the pathogenesis of autoimmune disease focuses on the interactions between susceptibility genes and environmental stimuli. In Graves' disease it is postulated that aberrant expression of HLA class II antigens on thyroid epithelial cells permits the presentation of specific thyroid antigen to activated lymphocytes. Evidence suggests that thyrocyte HLA-DR expression is secondary to the production of cytokines by presensitized T-lymphocytes. A 20-yr-old woman and her 18-yr-old brother presented with classical findings of Graves' disease with ophthalmopathy within a year of each other. Diagnosis was confirmed by demonstration of elevated serum levels of T4 and T3, strongly positive titers of TSH binding inhibitory immunoglobulins, and histological examination after subtotal thyroidectomy. Eight years previously, acute life-threatening aplastic anemia in the brother led to therapeutic transplantation of bone marrow from his sister. After the procedure, 100% of his peripheral leucocytes were genotype 46,XX. HLA typing performed before transplantation and 2 months after thyroidectomy in the female indicated complete identity with her brother's leukocytes for class I and class II antigens. Thyroid autoantibodies at this time were weakly positive. Although the concordance of thyroid disease in these patients could be due to chance, the patients were of different sexes, the family history was negative, and neither the probands nor the first degree relatives bore the HLA-DR3/B8 antigens. We propose that the male passively acquired a clone of programmed or activated lymphocytes from his sister and that his hyperthyroidism was not primarily dependent on exposure to specific thyroid-derived antigen. | 4 |
Marfanoid children. Etiologic heterogeneity and cardiac findings. The clinical, cardiac, and echocardiographic test results of 20 children with marfanoid features are reviewed. Fifteen were diagnosed as having Marfan syndrome, two had "possible" Marfan syndrome, and three had other diagnoses. On first evaluation, eight patients with Marfan syndrome (53%) had mitral regurgitation and none had aortic regurgitation. Echocardiography showed aortic root enlargement in 12 (80%) of 15 patients and mitral valve prolapse in 12 (80%) of 15. None had a normal echocardiogram. At follow-up examination, one patient had developed aortic root enlargement, and one patient, mitral valve prolapse. Thus, although aortic root enlargement is usually present in early childhood in patients with Marfan syndrome, it is not considered specific because in this study it also occurred in one child with Alport's syndrome and in one with marfanoid features. Four patients with aortic root enlargement were treated with propranolol and their echocardiograms showed no further increase in the aortic root diameter for several years. We recommend echocardiography in the diagnosis and routine management of children in whom Marfan syndrome is suspected. | 4 |
Recurrent lateral rectus palsy in childhood. Five patients with recurrent, lateral rectus palsy in childhood, examined at the University of Iowa Hospitals over a period of 22 years, are reported. During the same period, 131 abducens nerve palsy patients, younger than 18 years of age, were evaluated. Eighteen similar patients, most single case reports, are reviewed from the literature. All reported patients, including our own, shared the following features: spontaneous recovery within 6 months in the majority of patients, ipsilateral recurrence, and painless palsy. There is female and left-sided preponderance. Etiology is undetermined. Hypotheses include viral etiology, neurovascular compression by aberrant artery, and migraine. | 2 |
Use of technetium-99m sestamibi to determine the size of the myocardial area perfused by a coronary artery. The value of the new radionuclide tracer, technetium-99m (Tc-99m) sestamibi, to demonstrate myocardial perfusion in areas supplied by specific coronary arteries was evaluated in patients injected with the agent during cardiac catheterization. Tc-99m sestamibi differs from thallium-201 in its physical characteristics (photon energy 140 keV), half-life (6 hours) and lack of significant redistribution, allowing its administration during an episode of chest pain or ischemia occurring outside the nuclear medicine laboratory with later imaging to visualize the distribution. In 13 patients Tc-99m sestamibi was administered intravenously during balloon-occlusion angioplasty. In 11 of 13 patients, defects of the single photon emission computed tomography images corresponded to the area made ischemic during angioplasty. In the remaining 2 patients, abundant collateral flow was present and no defects were seen. In a second study, 15 patients had Tc-99m sestamibi selectively injected into a coronary artery during angiography. Later imaging identified the area supplied by the artery injected. Tc-99m sestamibi imaging can detect perfusion defects associated with short episodes of ischemia, and the area supplied by the different coronary arteries. | 3 |
Giardiasis [published erratum appears in Prim Care 1991 Jun;18(2):following xii] Giardiasis represents a major protozoan infection for the 1990s. This article reviews this organism for the primary care physician, discussing the characteristics, clinical manifestations, diagnosis, and treatment. Prevention and patient education needs are also presented. | 1 |
Fasciculations due to verapamil in a patient with neuropathy. A patient with pre-existing peripheral neuropathy developed generalized fasciculations under verapamil which ceased after discontinuation of the drug. It is suggested that the increase in acetylcholine (ACh) release at the neuromuscular junction by verapamil may trigger fasciculation in the state of regeneration at ACh-sensitive sites of axon terminals and immature axon sprouts. | 2 |
Hyponatraemia secondary to an inappropriately high release of antidiuretic hormone in cardiac tamponade. A spontaneous intrapericardial haemorrhage caused cardiac tamponade in a 29 year old paraplegic man who was being treated with warfarin. The associated persistent hyponatraemia, which was believed to be caused by an inappropriately high release of antidiuretic hormone, rapidly resolved after pericardiocentesis. | 3 |
Does intracoronary infusion of Fluosol-DA 20% prevent left ventricular diastolic dysfunction during coronary balloon angioplasty? Distal intracoronary infusion of the perfluorochemical Fluosol-DA 20% has been shown to prevent systolic dysfunction during coronary artery balloon occlusion in coronary angioplasty. To assess its effect on global diastolic dysfunction, a randomized, single-blind, crossover protocol comparing intracoronary infusion of Fluosol or no infusion (control) was performed during 60 s balloon inflations in 10 patients (mean age 67 years) undergoing coronary angioplasty. Assessment of global systolic and diastolic function was obtained with high fidelity micromanometer measurements of left ventricular pressure. Eighteen pairs of balloon inflations (Fluosol versus control) were analyzed. Patients reported significantly less severe chest pain during inflations accompanied by Fluosol compared with control. However, during coronary balloon occlusion, no significant differences in the changes from baseline values were observed between Fluosol and control with regard to ventricular relaxation, including the time constant of early ventricular relaxation (tau) and maximal rate of fall in left ventricular pressure (maximal negative dP/dt). No differences between Fluosol and control were observed in terms of the increase in end-diastolic pressure or minimal diastolic pressure during balloon inflation. Mean systolic pressure decrease from baseline values was greater during control than during Fluosol inflations (-9.0 +/- 3.3 mm Hg, p = 0.013), but no significant difference was observed in the change in maximal rate of rise in left ventricular pressure (maximal positive dP/dt). These results suggest that Fluosol does not preserve global left ventricular diastolic function during coronary balloon occlusion, possibly because of its limited oxygen delivery capability relative to arterial blood. | 3 |
Coffee, caffeine, and cardiovascular disease in men BACKGROUND. For many years, an association between coffee consumption and the risk of coronary heart disease has been suspected. Although based on small numbers of end points, a prospective study has suggested a particularly strong association between recent coffee drinking and the incidence of cardiovascular disease. METHODS. We examined prospectively the relation of coffee consumption with the risk of myocardial infarction, need for coronary-artery bypass grafting or angioplasty, and risk of stroke in a cohort of 45,589 U.S. men who were 40 to 75 years old in 1986 and who had no history of cardiovascular disease. RESULTS. During two years of follow-up observation, 221 participants had a nonfatal myocardial infarction or died of coronary heart disease, 136 underwent coronary-artery surgery or angioplasty, and 54 had a stroke. Total coffee consumption was not associated with an increased risk of coronary heart disease or stroke. The age-adjusted relative risk for all cardiovascular disease among participants who drank four or more cups of coffee per day was 1.04 (95 percent confidence intervals, 0.74 to 1.46). Increasing levels of consumption of caffeinated coffee were not associated with higher risks of cardiovascular disease. Higher consumption of decaffeinated coffee, however, was associated with a marginally significant increase in the risk of coronary heart disease (relative risk, 1.63; 95 percent confidence interval, 1.02 to 2.60). Finally, we observed no pattern of increased risk across the subgroups of participants with increasing intakes of caffeine from all sources. Adjustment for major cardiovascular-risk indicators, dietary intake of fats, and cholesterol intake did not materially alter these associations. CONCLUSIONS. These findings do not support the hypothesis that coffee or caffeine consumption increases the risk of coronary heart disease or stroke. | 2 |
Education and epilepsy: assessment and remediation. Learning difficulties in children with epilepsy may be caused by brain damage and should be investigated. In many cases, however, seizures and/or electroencephalographic (EEG) findings are the only signs of pathology. Frequency and type of seizures may be determining factors that should, if necessary, be evaluated by long-term EEG monitoring, preferably during school performance or in conjunction with neuropsychological assessment. This may prove that subclinical epileptiform discharges in the EEG can adversely affect the child's performance. Secondary psychological problems in epilepsy patients, combined with side effects of antiepileptic drugs, may cause or heighten learning problems. Prophylactic control of seizures with one appropriate drug may alleviate learning problems. Computerized neuropsychological testing with simultaneous EEG recording may reveal the influence of epileptiform discharges on cognitive function and also help to evaluate the effects of antiepileptic drugs. Objective assessment of subclinical epileptiform activity makes it easier to treat the pathology identified by the EEG with optimal dosage of the most appropriate drug. A balance is required because epileptiform discharges and even occasional seizures may be less disabling than side effects from large doses of several drugs. Information to the school and the parents concerning the patient's abilities and limitations may be as important as seizure control. Specialized teaching should be started early, when necessary, with the patient integrated into a normal school if possible. However, good functioning in a special school is preferable to marginal functioning in a normal school. | 2 |
Visual evoked potential monitoring of optic nerve function during surgery. A study was made with intra-operative flash--visual evoked potentials (VEP) monitored using a fibre-optic/contact lens photo stimulator in 57 patients undergoing intra-orbital surgical procedures with potential risk to the optic nerve. The VEPs recorded under enflurane and nitrous oxide anaesthesia did not differ significantly in latency or amplitude from the pre-operative recordings. Transient abolition of the VEP was seen under many circumstances and did not correlate with the outcome of surgery, but absence of a previously normal VEP for more than four minutes during surgical manipulation within the orbit did show a correlation with post operative impairment of vision. The technique provides early warning to the surgeon of threats to the integrity of the optic nerve. | 2 |
The effect of posterior rectopexy on fecal continence. A prospective study. Twenty-three patients with rectal prolapse or intussusception were studied to specifically focus on the effect of posterior rectopexy on fecal continence, anal pressure, and rectal capacity. Before operation, five patients were fully continent (A), 10 were continent for solid stools (B) and eight patients were fully incontinent (C). Group A remained fully continent; continence was regained nine times in group B and in group C, three patients regained full continence, two became continent for solid stools, three patients remained incontinent. Other symptoms such as constipation, false urgency, and a feeling of incomplete evacuation were not beneficially influenced by rectopexy. The patients' continence status was correlated to anorectal manometry and rectal capacity measurement. In group B, incremental pressure (P = squeeze - basal P) increased significantly (P less than 0.02) as well as incremental volume (V = maximum tolerated volume - volume of first sensation) (P less than 0.05). We conclude that, by an increase of incremental anal pressure and incremental rectal volume, posterior rectopexy offers an 83 percent chance of regaining full continence, or a major improvement, and a 17 percent chance of stabilization of fecal incontinence. | 1 |
Genotypic analyses of Richter's syndrome. The authors report the immunogenotype of two cases of Richter's syndrome. The immunoglobulin gene rearrangement pattern obtained on Southern Blot analysis was found in both cases to be the same in leukemic blood cells and in the tissue involved by the lymphoma. The beta chain and gamma chain T-cell receptor gene rearrangement pattern exhibited a germ-line configuration in the peripheral blood cells and in the lymph node in Case 2, whereas in Case 1 the lymph node had a gene rearrangement in the beta chain, as well as in the gamma chain T-cell receptor, and the leukemic cells from bone marrow were found to be in a germ-line configuration for T-cell receptors (beta and gamma chains). | 0 |
Alterations in brain electrical activity may indicate the onset of malignant hyperthermia in swine. The time course of changes in brain electrical activity during halothane anesthesia was examined in 12 malignant hyperthermia-susceptible (MHS) and 14 normal (nMHS) swine. Power densities in selected frequency bands were calculated from the electroen-cephalogram (EEG). EEG and systemic variables were determined over a period of 60 min after starting halothane (1% inspired). Malignant hyperthermia (MH) was triggered in all susceptible pigs. Initial changes in the EEG during development of MH consisted of a decrease in total power and a shift to lower frequencies (delta-theta activity) in all animals. These EEG alterations were noted when there was an increase in heart rate, but other systemic variables were still normal. EEG changes in all MHS animals started at an arterial oxygen tension (PaO2) greater than 90 mmHg and an arterial carbon dioxide tension (PaCO2) less than 50 mmHg. In 5 MHS animals EEG became isoelectric at a PaO2 of 61-82 mmHg and a PaCO2 of 53-68 mmHg. Mean arterial blood pressure at this time was 54-66 mmHg. To determine the effects of hypoxia on the EEG in 7 nMHS animals, oxygen was decreased over a period of 45-60 min to 7% inspired. In 7 other nMHS animals, hypercarbia was produced by admixture of carbon dioxide to the fresh gas supply to achieve incremental increases of PaCO2 to 110-120 mmHg. Significant EEG changes during hypoxia comparable to those seen at the onset of MH were noted at a PaO2 below 40 mmHg and during hypercarbia at a PaCO2 greater than 68 mmHg. | 4 |
Receptor changes in the spinal cord of sheep associated with exposure to chronic pain. There is evidence that post-injury hypersensitivity is partly due to changes in the central nervous system. Sheep with foot rot were used to investigate the effect of chronic pain on some receptors thought to be involved in spinal nociceptive processing systems (alpha 2 adrenoceptor and mu and delta opioid receptors). Saturation binding studies showed a variable distribution of [3H] clonidine (alpha 2 adrenoceptor agonist) in the spinal cord of normal sheep. The number of receptors (Bmax) present in areas thought to be involved in nociceptive processing, laminae I and II and lamina X, increased to 131% and 169% of control sheep values respectively in animals exposed to chronic pain. The affinity of the receptors (KD), however, remained unchanged at approximately 2 nM. There was less [3H]DAGO (mu opioid agonist) and [3H]DPDPE (delta opioid agonist) binding in the sheep spinal cord. Both opioid receptor types being mainly located in the superficial dorsal horn. The [3H]DPDPE binding was unchanged in the sheep with foot rot, whilst the number (Bmax), but not the affinity, of the [3H]DAGO binding sites increased in laminae I and II in lame animals to 130% of the control sheep values. Hence, in animals in chronic pain, the number of alpha 2 adrenoceptors and mu opioid receptors increased mainly in areas of the sheep spinal cord associated with nociception. | 4 |
Uniparental paternal disomy in Angelman's syndrome. Angelman's syndrome and Prader-Willi syndrome are both causes of mental retardation with recognisable, but quite different, clinical phenotypes. Both are associated with deletions of chromosome 15q11-13, of maternal origin in Angelman's and paternal in Prader-Willi. Prader-Willi can arise by inheritance of two chromosomes 15 from the mother and none from the father (uniparental maternal disomy). In 2 patients with Angelman's syndrome we found evidence of uniparental paternal disomy. The phenotypic effects of maternal and paternal disomy of chromosome 15 are very different and inheritance of two normal 15s from one parent does not lead to normal development--strong evidence in man for genomic imprinting, in which the same gene has different effects dependent upon its parental origin. | 2 |
Coronary morphology after percutaneous directional coronary atherectomy in humans: autopsy analysis of three patients. The morphologic basis of angiographically successful percutaneous directional atherectomy and subsequent restenosis in human coronary arteries is unknown. The clinical and pathologic features of three patients who died after coronary atherectomy are described. Tissue fragments obtained with atherectomy demonstrated atheromatous and fibroproliferative intima, media and adventitia. At autopsy, treated vascular segments (from the left anterior descending artery in two patients and a vein graft in one patient) demonstrated discrete defects in the vascular wall. Defects extending into atheroma, media or adventitia corresponded with the presence of these tissues in the atherectomy specimens. Tissues were otherwise not disrupted in the manner associated with balloon angioplasty. Acute mural thrombus deposition was evident in the resection zone in one patient. Late findings included fibroproliferative intimal tissue extending from the resected areas into the vascular lumen. In one patient intimal hyperplasia was sufficient to narrow the vascular lumen by 82% and was implicated in subsequent myocardial ischemia and infarction. The study indicates that 1) the vascular injury associated with atherectomy is distinct from that associated with balloon angioplasty, 2) acute mural thrombus deposition may occur even with resection limited to the intima, and 3) intimal hyperplasia may develop in regions treated with atherectomy and may be associated with late myocardial ischemia and infarction. | 4 |
Expression of X protein and hepatitis B virus replication in chronic hepatitis. The X protein can act on the enhancer of hepatitis B virus in an in vitro system and elevate the transcriptional level of hepatitis B virus. However, because no relationship had been reported between X protein expression and hepatitis B virus replication in patients with chronic hepatitis B, we focused on its expression in the liver in comparison with markers of hepatitis B virus replication. Liver biopsy samples and sera from 59 carriers with HBsAg were examined immunohistochemically for X protein using rabbit IgG against recombinant X protein. There was a significant difference in the serum hepatitis B virus DNA level between X protein-positive and -negative patients (p less than 0.001). Serum pre-S1 and pre-S2 antigens were also measured quantitatively by enzyme immunoassay using monoclonal antibodies specific against each antigen. The titers of pre-S1 antigen in patients positive for X protein were significantly higher (p less than 0.001) than those of the X protein-negative patients (3.02 +/- 0.99 vs. 2.00 +/- 0.59, respectively). Similarly, the titers of pre-S2 antigen were 2.98 +/- 0.91 vs. 1.94 +/- 0.54, respectively (p less than 0.001). The rate of positivity of the X protein was higher (38 of 49; 77.6%) in the replicative group (serum HBeAg, serum hepatitis B virus DNA or HBcAg in liver positive) compared with that observed in the nonreplicative group (3 of 10; 30%--serum HBeAg, serum hepatitis B virus DNA and HBcAg in liver negative) (p less than 0.01). | 4 |
Acute silicosis due to inhalation of a domestic product. A 20-yr-old woman with no history of pulmonary disease presented with acute dyspnea and fever. After various investigations and treatments the symptoms persisted and a lung biopsy was performed. Pathologic analysis disclosed the presence of silicotic crystals within lung tissue and mediastinal lymph nodes. The patient admitted having repeatedly inhaled a domestic scouring powder rich in silica during the preceding 6 months. This is the first report of acute pulmonary silicosis developing after exposure to a common household product. | 4 |
Angiodysplasia: characterization, diagnosis, and advances in treatment. Gastrointestinal bleeding accounts for 2% of all adult hospital admissions each year. Angiodysplasia is one of the most frequently reported causes of lower gastrointestinal bleeding. In 80% of patients with bleeding angiodysplasia, the bleeding will stop spontaneously, but will often recur. Although angiography may detect bleeding in 86% of actively bleeding patients, the bleeding rate must be at least 0.5 mL/min. The treatment of choice for control of bleeding has been resection of the suspected segment of bowel, but this procedure is associated with a 10% rebleeding rate and 7% to 8% mortality. Superselective catheterization with infusion of vasoconstrictors has been used to control bleeding, but effects are short-lived. Superselective embolization occludes an offending vessel, but is difficult to perform accurately and is associated with risk of infarction. Laser photocoagulation controls bleeding in 84% of patients, with a 6% complication rate. This method of controlling bleeding offers distinct advantages, especially for the hemodynamically unstable patient. Except for cases in which surgical resection is indicated, Nd:YAG laser photocoagulation is the safest, least invasive, and probably most effective means of treating angiodysplasia. | 1 |
Prognosis and prognostic factors of retinal infarction: a prospective cohort study. OBJECTIVE--To determine the prognosis and adverse prognostic factors in patients with retinal infarction due to presumed atheromatous thromboembolism or cardiogenic embolism. DESIGN--Prospective cohort study. SETTING--University hospital departments of clinical neurology. PATIENTS--99 patients with retinal infarction, without prior stroke, referred to a single neurologist between 1976 and 1986 and evaluated and followed up prospectively until death or the end of 1986 (mean follow up 4.2 years). INTERVENTIONS--Cerebral angiography (55 patients), aspirin treatment (37), oral anticoagulant treatment (eight), carotid endarterectomy (13), cardiac surgery (six), and peripheral vascular surgery (two). MAIN OUTCOME MEASURES--Death, stroke, coronary events, contralateral retinal infarction; survival analysis confined to 98 patients with retinal infarction due to presumed artheromatous thromboembolism or cardiogenic embolism (one patient with giant cell arteries excluded), and Cox's proportional hazards regression analysis, including age as a prognostic factor. RESULTS--During follow up 29 patients died (21 of vascular causes and eight of non-vascular or unknown causes), 10 had a first ever stroke, 19 had a coronary event, and only one developed contralateral retinal infarction. A coronary event accounted for more than half (59%) of the deaths whereas stroke was the cause of only one death (3%). Over the first five years after retinal infarction the actuarial average absolute risk of death was 8% per year; of stroke 2.5% per year (7.4% in the first year); of coronary events 5.3% per year, exceeding that of stroke; and of stroke, myocardial infarction, or vascular death 7.4% per year. Prognostic factors associated with an increased risk of death were increasing age, peripheral vascular disease, cardiomegaly, and carotid bruit. Adverse prognostic factors for serious vascular events were increasing age and carotid bruit for stroke, and increasing age, cardiomegaly, and carotid bruit both for coronary events and for stroke, myocardial infarction, or vascular death. CONCLUSIONS--Patients who present with retinal infarction due to presumed atherothromboembolism or cardiogenic embolism are at considerable risk of a coronary event. The risk of stroke, although high, is not so great. Not all strokes occurring after retinal infarction relate directly to disease of the ipsilateral carotid system, although this is probably the most common cause. Few patients experience contralateral retinal infarction. Non-arteritic retinal infarction should be diagnosed or confirmed by an ophthalmologist, and the long term care of patients with the condition should involve a physician who has an active interest in managing vascular disease. | 3 |
Progression of stage T1 bladder tumors after intravesical bacillus Calmette-Guerin [published erratum appears in J Urol 1991 Apr;145(4):840] Of 221 patients with superficial bladder tumors (stages Ta, Tis and T1) treated by transurethral resection and an initial 6-week course of intravesical bacillus Calmette-Guerin (BCG) 195 were evaluated after 3 months for local response. Of these patients 17 (8.7%) had a stage T1 tumor, of whom 14 (82%) subsequently had muscle invasion (7), uncontrolled local disease (6) or metastasis (1). The median interval to progression was 8.4 months, which was significantly (p = 0.0001) less than among the other 178 patients with a nonstage T1 lesion or no tumor found at 3 months, of whom 44 (25%) had progression (median more than 5 years). These data suggest that patients with stage T1 tumor 3 months after BCG therapy require additional therapy other than simple tumor resection and meticulous followup. | 0 |
The influence of neutralizing heparin after carotid endarterectomy on postoperative stroke and wound hematoma. The influence of neutralizing or not neutralizing heparin after carotid endarterectomy on postoperative stroke and wound hematoma is unknown. During the past 6 years some of the authors frequently gave protamine sulfate to neutralize heparin, whereas others did not unless a patch was used or wound hemostasis was not readily obtained. To determine the influence of protamine sulfate on stroke and wound hematoma the records of 697 patients having a carotid endarterectomy from January 1984 to September 1989 were reviewed. Protamine sulfate was given to 328 patients, and 369 did not receive protamine sulfate. The incidence of stroke in the two groups was 1.8% (n = 6) and 2.7% (n = 10), respectively, and the difference was not significant (p = 0.6019). Excluding three strokes that could not be related to neutralizing or not neutralizing heparin, the difference remained insignificant (1.5% vs 2.2%, p = 0.7290). The incidence of wound hematoma was 1.8% (n = 6) in patients given protamine sulfate and 6.5% (n = 24) in patients not given protamine sulfate, and this difference was significant (p = 0.0044). The difference remained significant when three hematomas not related to protamine sulfate were excluded (1.2% vs 6.2%, p = 0.0013). In patients not given protamine sulfate draining the wound lessened the incidence of wound hematoma (4.4% vs 8.6%), but this difference was not statistically significant (p = 0.1475). In patients given protamine sulfate the dose of protamine sulfate (15 to 45 mg vs 50 to 75 mg) had no statistically significant effect on the incidence of stroke (0.8% vs 2.0%, p = 0.6530) or wound hematoma (1.6% vs 1.0%, p = 1.000). | 2 |
A novel metalloproteinase gene specifically expressed in stromal cells of breast carcinomas. A gene has been identified that is expressed specifically in stromal cells surrounding invasive breast carcinomas. On the basis of its sequence, the product of this gene, named stromelysin-3, is a new member of the family of metalloproteinase enzymes which degrade the extracellular matrix. The suggestion is that stromelysin-3 is one of the stroma-derived factors that have long been postulated to play an important part in progression of epithelial malignancies. | 0 |
Nasopalatine duct cyst: an analysis of 334 cases. The nasopalatine duct cyst (NPDC) is the most common cyst of nonodontogenic origin in the maxilla. However, the information reported about this lesion consists primarily of small surveys and isolated case reports. The purpose of this retrospective investigation was to gather demographic, clinical, and histologic data on a large series of NPDCs, and to compare the findings with those of previous studies. In this study, the overall mean age at diagnosis was 42.5 years, and there was a slight male predilection. The mean radiographic diameter was 17.1 mm, but 75% of the lesions were 20 mm or less in diameter. Symptoms were present in at least 70% of the cases. Only 28% of the specimens contained respiratory epithelium. There was no correlation between radiographic size, patient's age, presenting symptoms, or type of epithelium. Recurrence was noted in only 2% of the cases. | 0 |
Management of hemorrhagic complications from preoperative embolization of arteriovenous malformations. Endovascular embolization procedures have undergone dramatic evolution and improvement in recent years. Despite these advances, controversy remains regarding the optimal role of these procedures in treating cerebral arteriovenous malformations (AVM's) and whether their purpose should be as a presurgical adjunct or as primary therapy. This controversy risks fragmentation between disciplines in the broader efforts to improve management of cerebrovascular disorders. The authors report seven cases of life-threatening hemorrhages that occurred during staged invasive therapy for AVM's which illustrate the value of a unified team approach to optimize patient care. Each patient underwent at least one embolization procedure using polyvinyl alcohol particles, followed in two cases by the occlusion of proximal feeding vessels by platinum microcoils and in one case by the attempted detachment of an endovascular balloon. In three patients, catheter penetration into the subarachnoid space resulted in subarachnoid hemorrhage. One patient suffered rupture of a large feeding vessel during balloon inflation. The final three patients sustained intracranial hemorrhage 2 hours, 8 hours, and 5 days, respectively, following embolization. All but two patients underwent emergency craniotomy at the time of the complication. These cases underscore the advantages of interdisciplinary management optimizing decision-making and providing expeditious care when life-threatening complications develop. | 4 |
Treatment of cutaneous T cell lymphoma with a combination of low-dose interferon alfa-2b and retinoids. In a pilot study the therapeutic effect and side effect profile of low-dose interferon alfa-2b in combination with a retinoid for the treatment of cutaneous T cell lymphoma were evaluated. Seven patients (four women, three men) with histologically confirmed cutaneous T cell lymphoma were included. Four patients had received therapy previously. The treatment schedule consisted of 2 million U of interferon alfa-2b administered subcutaneously three times per week and oral 13-cis-retinoic acid, 1 mg/kg/day, with subsequent dose reduction in case of response. The combination therapy produced two complete and two partial remissions. Responses were maintained by continuous therapy for up to 15 months even after dose reduction of both agents by 50%. Side effects were negligible and did not result in discontinuation of treatment in any patient. | 0 |
Effects of intravenous immunoglobulin on hemorrhage-induced alterations in plasma cell repertoires. Hemorrhage produces decreases in serum immunoglobulin (Ig) levels and alterations in the number and frequency of B cells producing antibodies against bacterial antigens. These abnormalities in immune response may contribute to the increased susceptibility to infection after injury and hemorrhage. To examine the relationship between serum Ig levels and bacterial antigen-specific plasma cell numbers and frequencies after blood loss, we treated hemorrhaged mice with intravenous Ig (IVIG). Hemorrhaged mice given IVIG had increased total numbers of splenic plasma cells compared with normal or hemorrhaged, untreated mice. Immunization with the bacterial polysaccharide antigen levan immediately after hemorrhage resulted in approximately 60% fewer levan-specific splenic plasma cells than those seen in normal unhemorrhaged mice. Treatment of hemorrhaged mice with IVIG did not correct the decrease in levan-specific plasma cells. These results demonstrate that hemorrhage-induced alterations in the numbers and frequencies of bacterial antigen-specific B cells are not related to changes in serum Ig levels and cannot be corrected through administration of IVIG. | 4 |
Reappraisal of surgical treatment of traumatic transection of the thoracic aorta. Since Crawford's report in 1973, repair of traumatic transection of the thoracic aorta without shunt or bypass has emerged as a popular technique which simplifies the operation and avoids use of heparin. Growing evidence, however, indicates that the incidence of paraplegia is higher with this method and may outweigh its advantages. With this in mind, we have examined our experience with 40 patients who underwent repair of aortic transection from 1975-1988. The operated patients in our series all survived. Fourteen were repaired using some type of bypass or shunt, none of whom developed paraplegia. The remaining 26 patients were repaired without a shunt and 9 (34.6%) developed paraplegia or paraparesis (p less than 0.02). Paraplegia was related to aortic occlusion time (p less than 0.002). It did not occur in 11 patients with times less than 27 minutes, but happened in 2 of 8 patients with times between 28 and 35 minutes and in all 7 patients with clamp times over 35 minutes. These data suggest that shunt or bypass should be used in most cases of aortic transection. | 3 |
Occult vascular malformations of the optic chiasm: magnetic resonance imaging diagnosis and surgical laser resection. Angiographically occult vascular malformations of the optic nerve and chiasm are extremely rare. Before the advent of magnetic resonance imaging (MRI), it was difficult to diagnose these lesions preoperatively. We report MRI scan findings of optic chiasm cavernous angiomas in two patients with chiasmal syndrome. MRI was useful in localizing the vascular malformation and delineating its characteristics, especially chronic hemorrhage. One patient underwent biopsy of the lesion. The other patient underwent complete microsurgical resection of the malformation with the carbon dioxide laser with preservation of vision. Occult vascular malformations of the optic nerve and chiasm may be a more common cause of visual deterioration than previously recognized. The MRI scan is the imaging modality of choice for diagnosing and following these lesions. In certain patients, these vascular malformations may be amenable to complete surgical removal with stabilization or improvement of visual function. | 3 |
Botulinum A toxin for the treatment of adult-onset spasmodic torticollis. Thirty-five patients with adult-onset idiopathic torticollis were treated by local injections of botulinum A toxin into dystonic cervical muscles. Substantial improvement with respect to reduction and elimination of pain was found in 81 percent, improvement in posture deformity and involuntary spasms in 70 percent, increased range of motion of the neck in 78 percent, reduction in visible sternocleidomastoid hypertrophy in 86 percent, and improvement in tremor in 65 percent. The syndrome was divided into four subtypes based on pattern of dystonic muscle groups involved in the dystonia, head and shoulder posture, and sternocleidomastoid muscle hypertrophy. Injection strategy based on this subdivision is described. | 4 |
Outcome of renal transplantation after urinary diversion and enterocystoplasty: a retrospective, controlled study. A total of 17 patients with intestinal urinary diversion of enterocystoplasty underwent renal transplantation between 1970 and 1988. Patient age ranged from 4 to 35 years (mean age 20 years). The patients were divided into 2 groups. In group 1 (10 patients, 2 of whom required retransplantation) the ureter of the transplanted kidney was implanted into an ileal (7) or colonic (1) conduit or enterocystoplasty (2). In group 2 (7 patients, 1 of whom required a second transplant) the diversion was taken down and the transplanted ureter was implanted into the defunctionalized bladder. There were 14 living related and 6 cadaveric kidneys transplanted. Graft survival rates were 58 and 87% in groups 1 and 2, respectively, with an over-all rate of 70% (14 of 20 kidneys). There was no statistical difference in the graft survival rate between the 2 groups. The complications in group 1 included ureteroileal anastomotic leak (3 patients), ureteroileal stenosis (1), calculus formation (1), urosepsis (1), hyperchloremic metabolic acidosis (1), and wound infection and dehiscence (1). There were no complications in group 2. Renal transplantation into a pre-existing urinary intestinal conduit or augmented bladder does not statistically adversely affect patient or graft survival. However, the complication rate is much higher when the ureter is implanted into an intestinal segment. Therefore, it is preferable whenever possible to implant the ureter into the native bladder. | 4 |
Carbohydrate malabsorption. Its measurement and its contribution to diarrhea. The major purpose of this research was to gain insight into the effect of carbohydrate malabsorption on fecal water output. To do this we measured daily fecal output of total carbohydrate, reducing sugars, and organic acids (a product of bacterial fermentation). Normal subjects were studied in their native state and when diarrhea was induced by mechanisms that did and did not involve carbohydrate malabsorption. Patients with malabsorption syndrome were also studied. We concluded that: (a) Excretion of carbohydrate and its breakdown products can be expressed as a single number by converting organic acids to their monosaccharide equivalents. (b) Diarrhea per se causes only a trivial increase in fecal carbohydrate excretion. (c) The molar output of osmotic moieties in feces due to unabsorbed carbohydrate can be determined by adding fecal reducing sugars to organic acids and their obligated cations. This expression parallels almost exactly the effect of increasing doses of lactulose (a nonabsorbable sugar) on fecal water output; one excreted millimole obligates 3.5 g of stool water. This relationship can be used to predict the effect of carbohydrate malabsorption on stool water output in patients with diarrhea. (d) 12 of 19 patients with malabsorption syndrome due to various diseases had excessive fecal excretion of carbohydrate and its breakdown products; of the diseases that cause malabsorption syndrome, combined small and large bowel resection is most likely to result in excessive fecal excretion of carbohydrate and monosaccharide equivalents. In 6 of these 19 patients carbohydrate malabsorption appeared to be the major cause of diarrhea. | 1 |
Effect of intraarterial versus intravenous cisplatin in addition to systemic doxorubicin, high-dose methotrexate, and ifosfamide on histologic tumor response in osteosarcoma (study COSS-86). In osteosarcoma, intraarterial (IA) administration of systemic treatment has been advocated to improve local tumor response preparing for, or even obviating, definitive surgery. Because data from the literature did not unequivocally support the local superiority of IA infusion, a comparative study was started in 1986. Preoperative chemotherapy consisted of 45 mg/m2 of doxorubicin on days 1 and 2; 12 g/m2 of high-dose methotrexate on days 15 and 22; and 3 g/m2 of ifosfamide on days 29, 30, 50, and 51 followed on days 31 and 52 by intravenous (IV) versus IA tourniquet infusion of cisplatin (DDP). A strict randomization of patients was not feasible. A balanced distribution of risk factors was strived for by stratifying and allocating the appropriate patients centrally. The infusion time was prolonged from 1 to 5 hours in the IV group, and the DDP dose was reduced from 150 to 120 mg/m2 in both arms when intolerable ototoxicity became apparent. A multivariate analysis was performed to exclude a bias on the response rates from risk factor distribution and from modifications of DDP infusion time and dosage. The overall fraction of histologic good responders (greater than 90% necrosis) was not found to be different after IA versus IV treatment (34/50 [68%] vs. 41/59 [69%]). Intraarterial instead of IV use of DDP within an aggressive systemic treatment does not seem to improve the local tumor response. | 0 |
Relation of central obesity and insulin resistance with high diabetes prevalence and cardiovascular risk in South Asians. The hypothesis that the high mortality from coronary heart disease (CHD) in South Asians settled overseas compared with other populations is due to metabolic disturbances related to insulin resistance was tested in a population survey of 3193 men and 561 women aged 40-69 years in London, UK. The sample was assembled from industrial workforces and general practitioners' lists. In comparison with the European group, the South Asian group had a higher prevalence of diabetes (19% vs 4%), higher blood pressures, higher fasting and post-glucose serum insulin concentrations, higher plasma triglyceride, and lower HDL cholesterol concentrations. Mean waist-hip girth ratios and trunk skinfolds were higher in the South Asian than in the European group. Within each ethnic group waist-hip ratio was correlated with glucose intolerance, insulin, blood pressure, and triglyceride. These results confirm the existence of an insulin resistance syndrome, prevalent in South Asian populations and associated with a pronounced tendency to central obesity in this group. Control of obesity and greater physical activity offer the best chances for prevention of diabetes and CHD in South Asian people. | 4 |
Fine needle aspiration of pancreatic fluid collections. The complications of pancreatitis remain diagnostic and therapeutic challenges. Patients with simple pancreatitis, pancreatic necrosis, pseudocyst, noninfected fluid collection or abscess may all have similar clinical pictures. During the last decade computerized tomography (CT)-guided needle aspiration of peripancreatic fluid collections has been increasingly used as a diagnostic tool for pancreatic abscesses. This study reviewed the effectiveness of CT-guided needle aspiration of peripancreatic fluid collections at our institution in terms of diagnostic accuracy, correlation with eventual outcome, and safety. Charts of patients who underwent needle aspiration of de novo peripancreatic fluid collections over the past 2 years were reviewed. Thirty-five patients underwent 50 aspirations. Eleven patients (31%) had a positive aspirate culture; seven of these had a positive Gram's stain. There were no false positive Gram's stains but four false negatives. Twenty-four (69%) patients had negative aspirate Gram's stains and cultures. Patients with positive aspirate cultures were treated with antibiotics only (2), CT-guided drain placement (7), or surgical drainage (3). The mortality related to each of these therapeutic modalities was 50 per cent, 0 per cent, and 33 per cent, respectively. Of the 24 patients with negative aspirate cultures, five had no further treatment, 12 received antibiotics, and six underwent CT drain placement. All these patients survived. There was one false negative aspirate culture. This patient underwent delayed surgical drainage but expired. There were no major complications from CT-guided aspiration. CT-guided aspiration appears to be a safe and reliable method of diagnosing infection in peripancreatic fluid collections. | 4 |
Mortality and treated blood pressure in patients of the European Working Party on High Blood Pressure in the Elderly. Elderly hypertensive patients were randomly assigned to treatment with diuretics and methyldopa (n = 352) or placebo (n = 339), and divided into three groups, each according to their blood pressures, after nine months of treatment. Subsequently, 65 placebo patients and 56 treated patients died. A U-shaped relation was seen in treated patients between mortality and systolic blood pressure, and in placebo patients between mortality and diastolic blood pressure. Whereas in treated patients, the highest mortality was seen in patients with the lowest diastolic pressure, the lowest mortality was seen in the group with the highest diastolic pressure. The increased mortality in treated patients with the lowest blood pressure may not be drug-induced, but an expression of deterioration in general health, as indicated by the decreases in body weight and hemoglobin levels found in patients with the lowest blood pressures in previous analyses of these data. | 3 |
Occlusive arterial disease as a form of presentation of homocystinuria. Homocystinuria was first described in 1962. The disease affects several systems including the eyes, skeletal and nervous and vascular systems. We present the case of a 28-year old woman who presented with a severe occlusive arteriopathy. Screening for the most usual causes of arterial disease in young patients was negative, but a study of aminoaciduria and column chromatography confirmed the diagnosis of B6-responsive homocystinuria. Although it was necessary to amputate her left leg, the patient remains asymptomatic 16 months after B6 therapy was started. | 2 |
Surgical experience with cerebral amyloid angiopathy. Cerebral amyloid angiopathy can present as lobar intracerebral hemorrhage in an elderly person, presumably due to increased fragility of the vessels affected by amyloid deposition. For this reason, patients presenting with intracerebral hemorrhage and suspected of having cerebral amyloid angiopathy have often been treated nonsurgically. Since 1983 we have evaluated 11 patients with cerebral amyloid angiopathy (nine women and two men, mean age 73 years) who have undergone either intracerebral hematoma evacuation or brain biopsy. Nine of the 11 patients presented with intracerebral hemorrhage, which was unilobar in three patients and multilobar in six and involved the parietal lobes seven times, the frontal lobes four times, the temporal lobes four times, and the occipital lobes twice. These nine patients underwent hematoma removal, with no cases of abnormal intraoperative bleeding or recurrent hemorrhage. Six patients improved neurologically, and two were unchanged after hematoma evacuation; the remaining patient had a fatal cardiopulmonary arrest during the immediate postoperative period. During follow-up in seven patients (median 11 months, range 1 week to 74 months) none experienced a recurrent intracerebral hemorrhage and four continued to improve. Two of the 11 patients had cerebral amyloid angiopathy diagnosed by brain biopsy as part of an evaluation for dementia, also without surgical complications. This series suggests that patients with cerebral amyloid angiopathy may safely undergo operative procedures, and patients presenting with intracerebral hemorrhage may show neurologic improvement following evacuation of the hematoma. | 3 |
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