text
stringlengths
0
868k
Mitral valve repair with artificial chordae is considered as a good choice for correcting mitral regurgitation (mr) caused by mitral valve prolapse due to chordal elongation or rupture . The polytetrafluoroethylene (ptfe) suture has been applied as artificial chordae in large series with good outcomes . However, artifi...
Bone mass loss and muscle atrophy are the frequent complications occurring after spinal cord injury (sci).12 although unloading is an important factor in the pathogenesis of bone loss in sci patients, neuronal lesion and hormonal changes also seem to be involved in this process.3 inactivation and extreme unloading in s...
Feeling stigmatized is common among those living with mental illness.15 around the world many people with mental illness are discriminated against, have restricted work opportunities, feel stigmatized at work,68 and are even denied the basic rights afforded to other members of society.9 where studies have been conducte...
The diameter of the blood vessel capillaries in most vertebrates is about 25% smaller than the mean diameter of the erythrocytes, causing blood cell deformation during the capillary flow [1, 2]. Thus, the blood flow in the capillaries is that of a series of the red blood cells, separated by the plasma trapped between t...
Poor quality drug or substandard product encounters a major stringent issue for the global health system and it cannot be ignored . In most streamlined regions of the globe like japan, canada, australia, new zealand, the united states of america and most of the european union, hardly 1% of the market value products are...
Primary lesions are choristomas that involve displacement of epithelial elements during closure of the neural groove or other epithelial fusion lines between the third and the fifth weeks of gestation [1, 2]. Those located in the orbit may arise from cutaneous, conjunctival, respiratory, or lacrimal gland epithelium . ...
Signet ring cell lymphoma (srcl) are proliferations of malignant lymphoid cells containing cytoplasmic inclusions or vacuoles that displace the nucleus, imparting it a signet ring appearance . It is a rare morphological variant of non - hodgkin lymphoma (nhl), which can be confused with other more common tumors such as...
Computed tomography angiography (cta) is currently the standard non - invasive imaging method used for the assessment of the aorta . This is mainly due to high image quality, fast acquisition and easy post processing . Indications for cta are broad, including preoperative evaluation, imaging of aortic aneurysm, aortic ...
Barotrauma - induced pneumocephalus resulting from a rapid change in the surrounding pressure is a major concern for airline passengers and scuba divers5,6,9 - 11,14). Recent advances in aircraft technology have led to a tremendous increase in air travel for the general population and for neurosurgical patients . Neuro...
Despite important developments in the medical treatment of rheumatoid arthritis (ra), this disease has a major impact on important life areas of many patients . Over the past two decades, a growing emphasis has been put on arthritis self management strategies to help patients with ra cope with the consequences of the d...
Peritoneal dialysis (pd) is a first choice and successful home - based dialysis modality for patients with end - stage kidney disease (eskd), with great advantages for their quality of life: preservation of residual renal function, no vascular access requirement, and possibility of continuing scholar or professional ac...
The device consists of three layers made from pdms prepolymer and curing agent (sylgard 184, dow corning co., midland, mi) at a 10:1 ratio . The top and bottom layers are molded against a master mold made by standard photolithography using the negative - photoresist su-8 (su-8, microchem co., newton, ma). The master mo...
Effective water disinfection from fecal coliforms is a problem whose solution, leads to the improvement of environmental health conditions . Presently, water disinfection of fecal coliforms commonly uses chemical and conventional radiant heating methods . . Bacterial deactivation by conventional radiant heating is achi...
The first report on the immunostimulatory properties of bacterial dna date back to tokunaga and colleagues in 1984 due to the presence of unmethylated cpg dinucleotides in a particular base sequence context termed cpg motif . Importantly, the immunostimulatory activity is found in bacterial, yeast, viruses, insect and ...
The use of gene transfer as a therapeutic tool requires a regulatory system allowing control of the expression of the therapeutic gene by the administration of a small inducer molecule . The treatment could then be adapted to the needs of the patients and, should complications arise, the therapy could be stopped or int...
The aim of the present study was to investigate whether epidural administration of a xylazine - lidocaine combination accompanied by xylazine sedation would provide satisfactory analgesia for some surgical procedures on 10 calves admitted to the department of veterinary surgery, university of kafkas with perineal uroli...
Biliary cystadenomas are rare cystic lesions of the liver . They account for less than 5% of nonparasitic cysts of the liver and occur frequently in middle - aged women . These cysts need to be differentiated from other cystic lesions such as simple cysts, hydatid cysts, abscesses, hematomas, and polycystic liver disea...
Central venous catheters (cvc) are widely used in clinical practice for total parenteral nutrition along with other functions, such as central venous pressure monitoring, fluid and blood transfusion, medication and hemodialysis . Central venous catheter thrombosis is one of the most serious, life - threatening, and cos...
Iridoschisis is a rare condition in which the iris stroma is cleaved in two portions and is defined as a separation of the anterior iris stroma from the posterior stroma and muscle layers . The anterior layer splits into strands, and the free ends float freely in the anterior chamber.1 the condition was first described...
The majority of thyroid cancers are classified as papillary (88%) or follicular (9%); together these two histological types are grouped as differentiated thyroid cancers (dtc). While the annual rate of cancer incidence is decreasing, there has been a 2.4-fold increase in thyroid cancer from 1973 to 2002 . Dtc occurs mo...
A 37-year - old healthy male presented with irritation, pain and reduced vision in his left eye after mud contamination . Corneal scrapings demonstrated numerous septate hyphae, and specimen cultures were positive for chrysosporium sp . The lesion did not respond to aggressive topical 5% natamycin, 0.15% topical amphot...
After undergoing a mastectomy, the patients experience lymphedema and sensory changes due to physical changes along with restriction of the range of motion of the shoulder joints and upper extremity muscle weakening1 . Although treatments such as radiation therapy and anti - cancer therapy are implemented as a prescrip...
Supplementary material is available online and contains detailed methods, supplementary table 1, supplementary fig . 1 and a list of all investigated sequences determined in this study.supplementary material . Supplementary material.
Dehydration is the main cause of morbidity and mortality in critically ill patients and assessment of intravascular volume status (ivs) is essential for their management (1, 2). However, diagnosis of dehydration and intravascular volume loss is sometimes difficult (3). Some clinical signs and symptoms are assessed for ...
Almost six million people each year all over the world die because of tobacco related smoking as well exposure to tobacco of other s smoke is significant health hazard . Significant part of children and youth are daily expose to environmental tobacco smoke, which influence on their health and increases risk of tobacco ...
The study database consisted of 398 unique human infectious disease outbreak events collected from disease outbreak news, published online by the world health organization during 19962009 (9). For each outbreak, we defined the initial source or sources of the first public communication as the individual, organization, ...
Considerable evidence supports that increasing consumption of dietary fiber is associated with lower risk of obesity in adults . Based on these data, the dietary reference intakes (dris) for americans two years and older is to consume 14 grams (g) of total fiber per 1000 kilocalories (kcal) of total energy intake . In ...
Bisphosphonates have been considered both effective and cost - effective in preventing vertebral, peripheral, and hip fractures also in older populations [13]. However, oral bisphosphonates may cause gastrointestinal problems, esophagitis, and, in rare cases, atypical fractures of the femur . Thus, it is recommended th...
Epithelioid osteoblastoma (eo) is an uncommon histologic variant of osteoblastoma (ob) and derives its name from the characteristic presence of plump epithelioid osteoblasts . Eo is also named as aggressive osteoblastoma (ao) due to its propensity for local invasion and high rate of recurrence . Conventional osteoblast...
Impaired glucose tolerance (igt) is a less severe form of hyperglycemia that represents an intermediate stage in the development of type 2 diabetes.13 twenty - five percent to 75% of individuals with igt progress to diabetes within a decade of diagnosis.4 in recent years, the numbers of new patients with type 2 diabete...
The value given to traditional medicine (tm) is a function of public health and drug policies at a national level . Its potential contribution to conventional allopathic medicine can be characterized by how it is defined, the motivation for its use, and country - specific integration strategies . The world health organ...
Type one diabetes mellitus (t1 dm), one of the most common chronic diseases in childhood, affects 15 million children in the world and is rapidly increasing in specific regions [1, 2] so that an annual increase of 2 to 5 percent in europe, the middle east, and australia is reported . In addition, wide range of serious ...
In 1999, a dutch surgeon transmitted hepatitis b virus (hbv) to at least eight patients and possibly to a total of 28 patients . Literature shows that this form of transmission is not unique, numerous cases of hbv transmission from health care workers to patients have been reported, as reviewed by mele and gunson [2, 3...
Contrast induced nephropathy (cin) is one of the most common complications after radiographic procedures using intravascular radiocontrast media . Cin after percutaneous coronary intervention (pci) is common (5 - 50% according to characteristics of patients) and is associated with increased rate of morbidity and mortal...
Food access is defined as having sufficient resources to obtain appropriate foods for a nutritious diet and is one dimension of food security (defined as including both physical and economic access to food that meets people's dietary needs as well as their food preferences). Difficulty accessing food may negatively aff...
After completion of the experiment, students should have a fundamental understanding of weber s law and its applications to tactile stimuli . They should be able to describe the relationship between the physical intensity of a stimulus and perceived intensity . . They should be familiar with the operation procedures of...
Diabetes mellitus (dm) is an endocrine disease characterized by a deficit in the production of insulin with consequent alteration of the process of assimilation, metabolism, and balance of blood glucose concentration ., the global prevalence of dm has increased substantially, reaching 8.3% in 2014, which corresponds to...
So far, only four case reports and one case series has been published . Due to the paucity of data, not much is known about the biological and clinical behavior of ahl . Both ahl and ah are considered to have a better prognosis and considerably less cardiac involvement when compared with its more frequent counterpart, ...
The interest in understanding the genetic basis of diseases and drug regimens has increased, requiring dna isolation . However the extraction of dna from buccal cells (bc) is bringing a new perspective to obtaining dna8 . Compared to other methods, such as blood collection, it is a noninvasive collection method and is ...
Essential hypertension (eh) is a major risk factor for cardiovascular disease (cvd), and eh incidence among youth is increasing . African americans (aas) experience a higher prevalence, earlier onset, and greater severity of eh - related complications than other ethnic groups . From late childhood onward, aas display i...
Dictybase () is the model organism database (mod) for the social amoeba dictyostelium discoideum . Like other mods such as flybase (1), wormbase (2,3), mouse genome informatics (4) and the saccharomyces genome database (sgd) (5), dictybase (6) uses the organism's genome sequence to organize the biological knowledge res...
Everyday life experience shows that playing a video game, learning how to drive a car, doing math homework, or performing another cognitively demanding task may affect breathing . In some cases respiration tends to be inhibited; in other cases it seems to accelerate and/or its volume changes . In general, little is kno...
It is the destruction or removal of all pathogenic micro - organisms which give rise to infection, but not necessarily their spore forms . It is the process by which an article, surface, or medium is freed of all microorganisms either in the vegetative or spore state . With the whole world looking at the eradication of...
A 37-year - old woman presented to us complaining of sudden right flank pain and lower abdominal pain that had lasted for 1 week, as well as nausea and vomiting . Two years previously she had undergone a laparoscopic right partial nephrectomy at another hospital owing to a small right renal mass that was diagnosed as r...
Despite enormous improvement of diagnostic and therapeutic possibilities, 2 of every 3 cases of head and neck cancer are detected in advanced stage and, even after curative treatment, 30% to 50% of patients sustain a locoregional recurrence with worse prognosis.1 in addition to long periods without symptoms, the diffic...
Alzheimer's disease is the most common form of neurodegenerative disorders characterized by the formation of extracellular deposits composed of amyloid beta peptide (a) and masses of paired, helically wound protein filaments in the cytoplasm of neuronal cell bodies and neuritic processes called neurofibrillary tangles ...
Schools are an ideal setting where health promotion strategies can be used to enhance the oral health of children and an indirect channel to target community for promoting oral health . Oral health promoting schools that incorporate health education, promotion, prevention, and curative services are effective in lowerin...
Under the influence of some emergencies (mad cow disease, dioxin in feed), there has been a significant development of food laws which led to the enhancement of some normative papers that make up the basis of food security such as the council regulation (ec) n. 178 of 28 january 2002 (european commission, 2002). This l...
Adjuvant radiotherapy after breast - conserving surgery constitutes an indispensable part of breast conserving therapy in early breast cancer . The standard technique of radiation therapy is to treat the entire breast up to a total dose of 40 - 50 gy, with or without a boost dose of 10 - 15 gy to the tumor bed dependin...
Mechanical stress caused by flexibility training can affect hemodynamic responses.1 it has been shown that stretched muscle fibers activate mechanoreceptors, which elicit cardiovascular adjustments through parasympathetic withdrawal and sympathetic activation.26 in this context, previous studies with animal models have...
The platinum - based doublet is the chemotherapy of choice in patients with stage iiib / iv non small cell lung carcinoma (nsclc).1 pemetrexed is given with platinum compounds in patients with the nonsquamous type of nsclc.2 the pemetrexed - platinum combination has an overall survival advantage in patients with nsclc....
Mood disorders, principally major depressive disorder and bipolar disorder, are associated with about 60% of all suicides.16 an often - quoted figure is that about 15% of patients with major depression will eventually die by suicide.7,8 this estimation is, however, derived largely from studies of severely depressed inp...
Vascular leiomyoma (vl) or angioleiomyoma comparable to the one described in this study is extremely rare . Only 8.5% to 10% of vl are found in the head and neck area and, among these, only 3% occur in the nasal cavity [1, 2]. We describe the clinical and histological findings and the feasibility of surgical treatment ...
In fact, it is reported to be one of the three most frequent disabling symptoms in ms with as many as 90% of patients reporting fatigue . Clinically, patients with ms report fatigue as exhaustion, lack of energy, increased somnolence or worsening of symptoms and weakness exacerbated by activity and heat (uthoff phenome...
Early childhood caries is again on the rise in australian children despite considerable public health initiatives, including fluoridation of drinking water and use of fluoridated toothpaste . Dental caries continues to affect large numbers of children with nearly 50% of australian 6-year - olds having a history of deca...
Depression is a widespread condition (1, 2) that can cause significant worsening of comorbid medical conditions, socioeconomic burden resulting from functional disability, and reduced quality of life (3 - 5). The world health organization has predicted that by the year 2030, depression will be among the top three cause...
And gary ruvkun (wightman et al ., 1993) first discovered that a small non - coding rna lin-4 was able to repress the translation of lin -14, a gene important for developmental timing of c. elegans, the scientific community accepted the findings as an oddity of worm biology . Since then a plethora of studies have solid...
In eukaryotes, the basic repeating unit of chromatin is the nucleosome, consisting of 147 base pairs of dna wrapped around an octamer of histone proteins . The octamer contains two copies of each of the core histones, h2a, h2b, h3, and h4 . Histone proteins are dynamically post - translationally modified to regulate a ...
According to global initiative for asthma (gina) guideline, patient who do not reach an acceptable level of control with highest possible step four treatment, i.e., high dose inhaled corticosteroids (ics), inhaled long acting beta2 agonist (laba), montelukast, and oral sustained release theophylline, can be considered ...
Considerations of suicidality as a phenomenon developing along a continuum arose several decades ago as an opposition to - at the time conventional - static medical models, which focused mainly on completed suicides with little or no attention to the events and processes that lead to these deaths . Among others, mikawa...
Cardiovascular disorders are one of the the leading causes of mortality and morbidity today all over the world . They are endemic, and have been seen to increasingly affect a considerable number of people . They are also responsible for a major financial burden on the community and cause healthcare expenses . Although ...
The operative methods of this procedure are generally grouped into cold steel / dissection methods and more modern methods such as coblation, use of a harmonic scalpel, suction diathermy, radiofrequency ablation, electrocautery, microdebrider, and laser . Proponents and opponents of these modern methods have hinged the...
Obstetric injury is the most common cause of sphincter injury and pelvic floor failure resulting in lack of bowel control . The pudendal nerve may be damaged during the course of prolonged vaginal delivery consequent to stretching [13]. A third / fourth - degree tear (involving the anal sphincter complex) occurs in 0.5...
Poor sleep quality and sleep disorders particularly insomnia, osa, and rls are common problems in people with type 2 diabetes (3,6,8,12,13). Sleep quality provides a global subjective assessment of sleep that includes features such as sleep duration, time needed to initiate sleep (i.e., sleep latency), percentage of ti...
The protozoan trypanosoma cruzi, the causative agent of chagas' disease in humans, parasitizes several other mammalian species . After infection with t. cruzi, the parasites survive and multiply in nucleated cells as amastigotes, eventually reaching the bloodstream as trypomastigote forms . The acute infection phase is...
Malignant brain cancer persists as a catastrophic illness and is the second leading cause of cancer death in children [14]. The failure to effectively manage malignant brain cancer has been due in large part to the highly invasive nature of the disease and to the unique anatomical and metabolic environment of the brain...
To ultimately understand the structure - function relationship resulting from protein isoform variation, mutation, and posttranslational modification, one must be able to quantify the functional effect of the structural alteration on the interaction of the protein with its binding proteins . Traditional methodologies u...
Ethiopia has one of the worst maternal mortality ratios in the world, at 673 per 100,000 (range 54810).1 concealed within this figure is a marked urban / rural split . The 2011 ethiopian demographic health survey carried out by the ministry of health showed that in the capital, addis ababa, 82% of mothers deliver in a ...
Asthma has been shown to have prevalence of 7 - 10% world - wide and 9% in malta and is a common cause of emergency room visits . Recent studies reveal that management of adult asthma in the accident and emergency (a&e) department setting often differs from that which is recommended in clinical practice guidelines . Co...
Orthopaedic surgery, such as total hip and total knee replacement surgery, is associated with an increased risk of life - threatening and morbidity - associated venous thromboembolism (vte) during the peri- and post - operative periods . As a result, current evidence - based clinical guidelines recommend the administra...
In fact electron microscopy has been used in the pathological diagnosis of glomerular diseases over past few decades and its diagnostic value has been strongly emphasized . Ultrastructural features may enable a diagnosis to be made where the light microscopy is apparently normal, for example minimal change, thin membra...
Periodontal diseases, the most prevalent diseases throughout the world, are predominantly caused by gram - negative, anerobic bacteria present on the tooth root surfaces as a biofilm . Inflamed periodontal tissue produces significant amounts of pro - inflammatory cytokines, mainly il-1, il-6, pge2 and tumor necrosis fa...
Scrub typhus infection is a common febrile disorder presenting in the tropical region with propensity to involve different systems . Amongst them the cardio - respiratory system is involved in a significant proportion resulting in various manifestations . Herein we describe a case of scrub typhus presenting as acute ri...
Exudative retinal detachment, a very rare ocular finding in leukemia, has only been described in a few cases and has not been previously reported in india.12 this case is also the first case to document common acute lymphoblastic leukemia - associated antigen (calla) positive precursor b - cell lymphoblastic leukemia m...
The concept of volume transmission, introduced by luigi agnati and kjell fuxe in 1986, designates is a form of communication in brain tissue in which modulators act across wide distances when their sites of release and removal are further apart than for wired transmission, characteristic of focused synaptic action (fux...
Multiplexing biological assays on microarrays has been instrumental for generating the data required to understand biology at a systems - level . Although microarrays have contributed to the exponential growth of biological data over the past decade, there remain bottlenecks in the use of this technology . A primary ch...
The story begins in the 1960s when post - retrieval amnesia was first revealed by donald lewis . He showed that temporally graded retrograde amnesia could be obtained for a well - established memory in rats, if that memory were activated by a reminder of the original learning experience . These studies are the real ori...
Cysticercosis, a parasitic disease caused by taenia solium cysticercus, is one of the important parasitic diseases . Neurocysticercosis (ncc) is accepted to refer to cysts in the central nerve system, including the parenchyma and ventricles of the brain and the spinal cord . Subcutaneous cysticercosis (scc) is used for...
Diffuse normolipemic plane xanthoma (dnpx) was first described by altman and winkelmann in 1962 . The features of this entity are: (1) xanthelasma palpebrarum; (2) diffuse xanthoma planum of the head, neck, trunk, and extremities, and (3) plasma lipid values that are within normal limits . Several associations have bee...
Bronchiectasis is defined as irreversible, abnormal dilatation of one or more bronchi with chronic airway inflammation and results in a chronic cough, sputum production, recurrent chest infection, and airflow obstruction.1,2 according to us data, the estimated prevalence of bronchiectasis is 53 cases per 100,000 adults...
Cardiovascular diseases (cvd) are the leading cause of morbidity and mortality in patients with chronic kidney disease (ckd), and these people have about twenty times higher risk of cardiac death compared to people of the same sex and age without ckd (1). Some studies have shown that among older patients with ckd, trad...
Diabetic macrovascular complications are the leading cause of death and disability causing 7080% of deaths in diabetic patients . At present, there are many theories about the pathogenesis of diabetic vascular diseases, including insulin resistance, oxidative stress, lipid metabolism disorder, and inflammation theory ....
Caries is today, regardless of familiarity of its multi causal etiology of the disease and the possibility of its effective prevention still the most widespread disease of our civilization, which affects about 95% of our population . It covers all populations and age groups (1), and is a disease that is very difficult ...
One of the most common causes of pain in patients with malignant disease is cancer - induced bone pain (cibp), affecting patients with primary bone sarcomas and those with bone metastases . The pain consists of a triad of states; background pain, spontaneous pain, and movement - induced (incident) pain . Current interv...
To detect the expression of hsp25 in rat dental follicles both in vivo and vitro, and explore the underlying mechanism of hsp25 on the proliferation and differentiation of rat dental follicle cells (dfcs). Immunohistochemistry was performed to detect the expression of hsp25 in mandibles of postnatal rats on days 1, 3, ...
Lung cancer is one of the most frequent cancers and the leading cause of cancer - related mortality in denmark and worldwide.1 on a global basis approximately 1.6 million people are afflicted by lung cancer annually1 and the 5-year survival rate is less than 15%.24 lung cancer is associated with high age and presence o...
It has become clear that cells of the immune system can affect the brain and a host of behavioral processes (blalock, 1984). For instance, it is now accepted that circulating t lymphocytes and other peripheral immune cells routinely enter the central nervous system (cns), albeit in limited concentrations, and perform a...
Type 1 diabetes is a heterogeneous autoimmune disorder characterized by severe pancreatic -cell loss [14]. Discoveries of genetic variants associated with risk to t1d have provided new insights into their functional outcomes and affected pathways and until now more than 60 loci across the human genome have been associa...
The nature of fast pathway conduction, especially during atypical avnrt of the fast - slow type, is poorly understood . There is evidence that atypical fast - slow and typical atrioventricular avnrt do not utilize the same limb for fast conduction, but no data exist on patients who have presented with both typical and ...
Cytomegalovirus (cmv) infections are commonly reported in severely immunocompromized patients, including those with acquired immunodeficiency syndrome and patients who have received immunosuppressive therapy after transplantation or chemotherapy for malignant disease . Cmv is a ubiquitous member of the herpes virus fam...
In the beginning of october 2014, six children who were vomiting and experiencing abdominal cramping and diarrhea were admitted to the local hospital in gyeongsangnam - do, republic of korea (south korea). All patients attended the same daycare center that provided care and food to children from low - income families ....
A throwing event is a sports competition that displays a player s ability to throw an object, such as a shot put, discus, javelin, or hammer . To achieve a high level of performance in this sport, total physical fitness, including explosive muscle power, flexibility, agility, and coordination is required1 . In particul...
They have closer resemblance to the cells growing in the in vivo tissue environment (2). They further contain specific characteristics similar to the corresponding tissues in vivo and can stay active and functional for weeks (3). It is important to study the cells growth, proliferation and response to chemical agents i...
The most important reason for this is the apparent development of a nondepolarizing, relaxant - induced neuropathy, which leads to difficulty in weaning patients from artificial ventilation, among other problems . Some frequently used drugs, such as corticosteriods and aminoglycoside antibiotics, contribute to this neu...
In 2010, hispanics accounted for more than half of the growth in the total us population between 2000 and 2010, making them the fastest growing ethnic group . This expansion of hispanics is largely due to the natural increase (births minus deaths) of the existing population . Since 2000, of the total hispanic growth in...
A 76-year - old male ex - smoker presented with a recent history of hematuria and transient right - flank pain . On physical examination, no mass was palpable in the abdomen, and no costovertebral angle tenderness was found . Hemogram and blood chemistry results were normal, except for azotemia (serum creatinine, 2.2 n...
Deposited data can be found in the gene expression omnibus (geo) database: http://www.ncbi.nlm.nih.gov/geo/query/acc.cgi?acc=gse77286 a. thaliana ecotype columbia (col-0), and an arabidopsis t - dna insertion double mutant (bzip19 bzip23), were grown in a climate chamber with a long day photoperiod (16 h light at 22 c,...
Neurofibromatosis (nf) is considered one of a group of multisystem hereditary diseases that also include tuberous sclerosis, von hippel this heterogeneous group of diseases has been alternatively defined as phakomatoses, neurocutaneous syndromes, tumor predisposition syndromes, and ectodermal dysplasia by different aut...