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The leukocyte group-5 antigenic system, first described by van Leeuwen et al. (1964)--leukocyte group-4 was the early designation for HLA-B and LA the designation for HLA-A--has 2 known alleles (a and b) that segregate independently of the HLA system (van Rood et al., 1967). It is expressed in leukocytes, placent...
LEUKOCYTE ANTIGEN GROUP FIVE
None
2,900
omim
https://www.omim.org/entry/151450
2019-09-22T16:38:56
{"omim": ["151450"], "synonyms": ["Alternative titles", "GRANULOCYTE ANTIGEN 5"]}
Type of acute lung injury Ventilator-associated lung injury SpecialtyPulmonology Ventilator-associated lung injury (VALI) is an acute lung injury that develops during mechanical ventilation and is termed ventilator-induced lung injury (VILI) if it can be proven that the mechanical ventilation caused the acute...
Ventilator-associated lung injury
c2350350
2,901
wikipedia
https://en.wikipedia.org/wiki/Ventilator-associated_lung_injury
2021-01-18T18:57:28
{"mesh": ["D055397"], "icd-9": ["J95859"], "wikidata": ["Q7920251"]}
12q14 microdeletion syndrome is characterised by mild intellectual deficit, failure to thrive, short stature and osteopoikilosis. It has been described in four unrelated patients. The syndrome appears to be caused by a heterozygous deletion at chromosome region 12q14, which was detected in three of the four patie...
12q14 microdeletion syndrome
c4305140
2,902
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=94063
2021-01-23T19:10:46
{"icd-10": ["Q93.5"], "synonyms": ["Del(12)(q14)", "Deletion 12q14", "Monosomy 12q14", "Osteopoikilosis-short stature-intellectual disability syndrome"]}
Gonadoblastoma Micrograph of a gonadoblastoma. H&E stain. SpecialtyUrology, oncology A gonadoblastoma is a complex neoplasm composed of a mixture of gonadal elements,[1] such as large primordial germ cells, immature Sertoli cells or granulosa cells of the sex cord, and gonadal stromal cells. Gonadoblastomas ...
Gonadoblastoma
c0206661
2,903
wikipedia
https://en.wikipedia.org/wiki/Gonadoblastoma
2021-01-18T18:54:44
{"mesh": ["D018238"], "umls": ["C0206661"], "orphanet": ["206484"], "wikidata": ["Q5581320"]}
## Summary ### Clinical characteristics. ELANE-related neutropenia includes congenital neutropenia and cyclic neutropenia, both of which are primary hematologic disorders characterized by recurrent fever, skin and oropharyngeal inflammation (i.e., mouth ulcers, gingivitis, sinusitis, and pharyngitis), and cervi...
ELANE-Related Neutropenia
None
2,904
gene_reviews
https://www.ncbi.nlm.nih.gov/books/NBK1533/
2021-01-18T21:29:35
{"synonyms": []}
Cogan syndrome is a rare autoimmune disease that affects the eyes and inner ears. Symptoms of the syndrome include irritation and pain in the eyes, decreased vision, hearing loss, and vertigo. Other symptoms may include joint or muscle pain or inflammation of the blood vessels. The exact cause of Cogan syndrome ...
Cogan's syndrome
c0271270
2,905
gard
https://rarediseases.info.nih.gov/diseases/1421/cogans-syndrome
2021-01-18T17:56:59
{"mesh": ["D055952"], "synonyms": ["Cogan syndrome"]}
During a large-scale survey of persons with Usher syndrome (retinitis pigmentosa and sensorineural deafness), Beighton et al. (1993) identified 14 children in 9 Afrikaner families in South Africa with a combination of progressive rod-cone dystrophy, sensorineural deafness, and renal dysfunction of the Fanconi type le...
ROD-CONE DYSTROPHY, SENSORINEURAL DEAFNESS, AND FANCONI-TYPE RENAL DYSFUNCTION
c1849333
2,906
omim
https://www.omim.org/entry/268315
2019-09-22T16:22:32
{"mesh": ["C564829"], "omim": ["268315"]}
Abdominal aura (also known as visceral aura and epigastric aura) is used to denote a type of somatosensory or somaesthetic aura that typically manifests itself as a rising epigastric sensation. The term is indebted to the Latin words abdomen (belly) and aura (wind, smell).[1] Other presentations of the abdominal aur...
Abdominal aura
c4023506
2,907
wikipedia
https://en.wikipedia.org/wiki/Abdominal_aura
2021-01-18T18:42:33
{"umls": ["C4023506"], "wikidata": ["Q4665116"]}
Thrombotic microangiopathy Micrograph of thrombotic microangiopathy with the characteristic onion-skin layering seen in older lesions. PAS stain. SpecialtyRheumatology Thrombotic microangiopathy (TMA) is a pathology that results in thrombosis in capillaries and arterioles, due to an endothelial injury.[1] It...
Thrombotic microangiopathy
c2717961
2,908
wikipedia
https://en.wikipedia.org/wiki/Thrombotic_microangiopathy
2021-01-18T19:07:11
{"gard": ["12465"], "mesh": ["D057049"], "umls": ["C2717961"], "icd-9": ["446.6"], "icd-10": ["M31.1"], "orphanet": ["93573"], "wikidata": ["Q3312044"]}
Osteopetrosis refers to a group of rare, inherited skeletal disorders characterized by increased bone density and abnormal bone growth. Symptoms and severity can vary greatly, ranging from neonatal onset with life-threatening complications (such as bone marrow failure) to the incidental finding of osteopetrosis o...
Osteopetrosis
c0029454
2,909
gard
https://rarediseases.info.nih.gov/diseases/4155/osteopetrosis
2021-01-18T17:58:32
{"mesh": ["D010022"], "umls": ["C0029454"], "orphanet": ["2781"], "synonyms": ["Osteopetroses", "Marble bones", "Marble bone disease", "Albers-Schonberg disease", "Osteosclerosis fragilis", "Albers-Schonberg osteopetrosis", "Albers-Schoenberg disease", "Osteopetrosis and related disorders"]}
Extravasation SpecialtyToxicology Extravasation is the leakage of intravenously (IV) infused, and potentially damaging, medications into the extravascular tissue around the site of infusion. The leakage can occur through brittle veins in the elderly, through previous venipuncture access, or through direct leak...
Extravasation (intravenous)
c0015376
2,910
wikipedia
https://en.wikipedia.org/wiki/Extravasation_(intravenous)
2021-01-18T18:48:34
{"mesh": ["D005119"], "umls": ["C0015376"], "wikidata": ["Q2051919"]}
A number sign (#) is used with this entry because of evidence that fibrosis, neurodegeneration, and cerebral angiomatosis (FINCA) is caused by compound heterozygous mutation in the NHLRC2 gene (618277) on chromosome 10q25. Description Fibrosis, neurodegeneration, and cerebral angiomatosis is characterized by severe...
FIBROSIS, NEURODEGENERATION, AND CEREBRAL ANGIOMATOSIS
None
2,911
omim
https://www.omim.org/entry/618278
2019-09-22T15:42:43
{"omim": ["618278"]}
A rare, genetic, syndromic intellectual disability disease characterized by global developmental delay, microcephaly, mild to moderate intellectual disability, truncal ataxia, trunk and limb, or generalized, choreiform movements, and elevated serum creatine kinase levels. Variably associated features include mild cer...
Intellectual disability-hyperkinetic movement-truncal ataxia syndrome
None
2,912
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=369847
2021-01-23T17:41:30
{"icd-10": ["G25.5"]}
This article needs additional citations for verification. Please help improve this article by adding citations to reliable sources. Unsourced material may be challenged and removed. Find sources: "Vesicoureteral reflux" – news · newspapers · books · scholar · JSTOR (July 2017) (Learn how and when to remove this tem...
Vesicoureteral reflux
c0042580
2,913
wikipedia
https://en.wikipedia.org/wiki/Vesicoureteral_reflux
2021-01-18T18:46:57
{"mesh": ["D014718"], "umls": ["C0042580"], "orphanet": ["289365"], "wikidata": ["Q1550521"]}
Birt-Hogg-Dubé syndrome is a rare disorder that affects the skin and lungs and increases the risk of certain types of tumors. Its signs and symptoms vary among affected individuals. Birt-Hogg-Dubé syndrome is characterized by multiple noncancerous (benign) skin tumors, particularly on the face, neck, and upper c...
Birt-Hogg-Dubé syndrome
c0346010
2,914
medlineplus
https://medlineplus.gov/genetics/condition/birt-hogg-dube-syndrome/
2021-01-27T08:25:34
{"gard": ["2322"], "mesh": ["D058249"], "omim": ["135150"], "synonyms": []}
Wolcott–Rallison syndrome Other namesEarly-onset diabetes mellitus with multiple epiphyseal dysplasia Radiograph of a WRS child presenting with dysplastic bone growth in various regions of the body. Wolcott–Rallison syndrome, WRS, is a rare, autosomal recessive disorder with infancy-onset diabetes mellitus, ...
Wolcott–Rallison syndrome
c0432217
2,915
wikipedia
https://en.wikipedia.org/wiki/Wolcott%E2%80%93Rallison_syndrome
2021-01-18T18:30:06
{"gard": ["5589"], "mesh": ["C536739"], "umls": ["C0432217"], "orphanet": ["1667"], "wikidata": ["Q8029730"]}
A number sign (#) is used with this entry because of evidence that short-rib thoracic dysplasia-18 with polydactyly (SRTD18) is caused by homozygous mutation in the IFT43 gene (614068) on chromosome 14q24. Description Short-rib thoracic dysplasia (SRTD) with or without polydactyly refers to a group of autosomal...
SHORT-RIB THORACIC DYSPLASIA 18 WITH POLYDACTYLY
c4693420
2,916
omim
https://www.omim.org/entry/617866
2019-09-22T15:44:36
{"omim": ["617866"]}
Synucleinopathy Other namesα-Synucleinopathies Positive α-Synuclein staining of a Lewy body in a patient with Parkinson's disease. SpecialtyNeurology Synucleinopathies (also called α-Synucleinopathies) are neurodegenerative diseases characterised by the abnormal accumulation of aggregates of alpha-synuclei...
Synucleinopathy
None
2,917
wikipedia
https://en.wikipedia.org/wiki/Synucleinopathy
2021-01-18T18:56:21
{"wikidata": ["Q2376264"]}
A number sign (#) is used with this entry because immunodeficiency-21 (IMD21) is caused by heterozygous mutation in the GATA2 gene (137295) on chromosome 3q21. Primary lymphedema with myelodysplasia (614038), or Emberger syndrome, is an allelic disorder with overlapping clinical features. Description This primary ...
IMMUNODEFICIENCY 21
c3280030
2,918
omim
https://www.omim.org/entry/614172
2019-09-22T15:56:14
{"mesh": ["D000077428"], "omim": ["614172"], "orphanet": ["228423"], "synonyms": ["DENDRITIC CELL, MONOCYTE, B LYMPHOCYTE, AND NATURAL KILLER LYMPHOCYTE DEFICIENCY", "MONOCYTOPENIA WITH SUSCEPTIBILITY TO MYCOBACTERIAL, FUNGAL, AND PAPILLOMAVIRUS INFECTIONS AND MYELODYSPLASIA", "Combined immunodeficiency with susceptibi...
SLC35A3-CDG is a form of congenital disorders of N-linked glycosylation characterized by distal arthrogryposis (mild flexion contractures of the fingers, deviation of the distal phalanges, swan-neck deformity), retromicrognathia, general muscle hypotonia, delayed psychomotor development, autism spectrum disorder (spe...
Autism spectrum disorder-epilepsy-arthrogryposis syndrome
c3809910
2,919
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=370943
2021-01-23T17:08:46
{"omim": ["615553"], "icd-10": ["Q87.8"], "synonyms": ["SLC35A3-CDG"]}
A number sign (#) is used with this entry because 3-methylglutaconic aciduria type V (MGCA5), also called dilated cardiomyopathy with ataxia, is caused by homozygous mutation in the DNAJC19 gene (608977) on chromosome 3q26. Description 3-Methylglutaconic aciduria type V is an autosomal recessive disorder characteri...
3-METHYLGLUTACONIC ACIDURIA, TYPE V
c1857776
2,920
omim
https://www.omim.org/entry/610198
2019-09-22T16:04:58
{"doid": ["0110000"], "mesh": ["C565706"], "omim": ["610198"], "orphanet": ["66634"], "synonyms": ["Alternative titles", "CARDIOMYOPATHY, DILATED, WITH ATAXIA", "MGA, TYPE V"]}
A neuropathy causing itching between the shoulder blades Notalgia paresthetica Other namesNotalgia paraesthetica Notalgia Paresthetica SpecialtyNeurology Notalgia paraesthetica (NP) or notalgia paresthetica (also known as "Hereditary localized pruritus", "Posterior pigmented pruritic patch", and "subscapu...
Notalgia paresthetica
c0457010
2,921
wikipedia
https://en.wikipedia.org/wiki/Notalgia_paresthetica
2021-01-18T19:06:03
{"gard": ["7225"], "wikidata": ["Q494925"]}
Belief that symptoms are caused by an unproven infection Not to be confused with Neuroborreliosis or Post-treatment Lyme disease syndrome. Chronic Lyme disease Pseudomedical diagnosis RisksNocebo Treatment risksDangers of long-term antibiotic therapy LegalitySome jurisdictions have legislated to protect...
Chronic Lyme disease
c3890422
2,922
wikipedia
https://en.wikipedia.org/wiki/Chronic_Lyme_disease
2021-01-18T18:38:19
{"mesh": ["D000077342"], "umls": ["C3890422"], "wikidata": ["Q17103401"]}
A rare, genetic, neuromuscular disease characterized by adult-onset muscle weakness and atrophy in a scapuloperoneal distribution, mild involvement of the facial muscles, dysphagia, and gynecomastia. Elevated serum CK levels and mixed myopathic and neurogenic abnormalities are associated clinical findings. *[v]: V...
Neurogenic scapuloperoneal syndrome, Kaeser type
c1867005
2,923
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=85146
2021-01-23T18:38:13
{"gard": ["10312"], "mesh": ["C566695"], "omim": ["181400"], "umls": ["C1867005"], "icd-10": ["G12.1"], "synonyms": ["Kaeser syndrome", "Stark-Kaeser syndrome"]}
Nephronophthisis Nephronophthisis has an autosomal recessive pattern of inheritance. SpecialtyMedical genetics SymptomsPolyuria[1] TypesInfantile, Juvenile and Adult NPH[2] Diagnostic methodRenal ultrasound[2] TreatmentHypertension and anemia management[2] Nephronophthisis is a genetic disorder of th...
Nephronophthisis
c0687120
2,924
wikipedia
https://en.wikipedia.org/wiki/Nephronophthisis
2021-01-18T18:37:21
{"gard": ["206"], "umls": ["C0687120"], "orphanet": ["655"], "wikidata": ["Q1257011"]}
Abandoned child syndrome SpecialtyPsychiatry Abandoned child syndrome is a proposed behavioral or psychological condition that results primarily from the loss of one or both parents, or sexual abuse. Abandonment may be physical (the parent is not present in the child's life) or emotional (the parent withho...
Abandoned child syndrome
None
2,925
wikipedia
https://en.wikipedia.org/wiki/Abandoned_child_syndrome
2021-01-18T19:05:10
{"wikidata": ["Q4663376"]}
Alpha-methylacyl-CoA racemase (AMACR) deficiency is a disorder that causes a variety of neurological problems that begin in adulthood and slowly get worse. People with AMACR deficiency may have a gradual loss in intellectual functioning (cognitive decline), seizures, and migraines. They may also have acute episodes o...
Alpha-methylacyl-CoA racemase deficiency
c3280428
2,926
medlineplus
https://medlineplus.gov/genetics/condition/alpha-methylacyl-coa-racemase-deficiency/
2021-01-27T08:24:36
{"mesh": ["C565768"], "omim": ["614307"], "synonyms": []}
Abortion in Alaska is legal. 63% of adults said in a poll by the Pew Research Center that abortion should be legal in all or most cases. Alaska was one of only four states to make abortion legal between 1967 and 1970, a few years before the US Supreme Court's decision in 1973's Roe v. Wade ruling. Alaska had consent ...
Abortion in Alaska
None
2,927
wikipedia
https://en.wikipedia.org/wiki/Abortion_in_Alaska
2021-01-18T18:30:22
{"wikidata": ["Q64876901"]}
Molar pregnancy is a condition in which the placenta does not develop properly. The symptoms of molar pregnancy, which may include vaginal bleeding, severe morning sickness, stomach cramps, and high blood pressure, typically begin around the 10th week of pregnancy. Because the embryo does not form or is malformed...
Hydatidiform mole
c0020217
2,928
gard
https://rarediseases.info.nih.gov/diseases/10263/hydatidiform-mole
2021-01-18T17:59:57
{"mesh": ["D006828"], "orphanet": ["99927"], "synonyms": ["HYDM", "Hydatid mole", "Molar pregnancy"]}
A number sign (#) is used with this entry because autosomal recessive deafness-63 (DFNB63) is caused by homozygous mutation in the LRTOMT gene (612414) on chromosome 11q13. Clinical Features Tlili et al. (2006) reported a consanguineous 6-generation Tunisian family segregating autosomal recessive congenital hearing...
DEAFNESS, AUTOSOMAL RECESSIVE 63
c1969621
2,929
omim
https://www.omim.org/entry/611451
2019-09-22T16:03:22
{"doid": ["0110515"], "mesh": ["C566951"], "omim": ["611451"], "orphanet": ["90636"], "synonyms": ["Autosomal recessive isolated neurosensory deafness type DFNB", "Autosomal recessive isolated sensorineural deafness type DFNB", "Autosomal recessive non-syndromic neurosensory deafness type DFNB"], "genereviews": ["NBK14...
"Shipping fever" redirects here. For a related family of diseases sometimes referred to as "shipping fever", see Pleuropneumonia. Bovine respiratory disease (BRD) is the most common and costly disease affecting beef cattle in the world.[1] It is a complex, bacterial infection that causes pneumonia in calves which ca...
Bovine respiratory disease
None
2,930
wikipedia
https://en.wikipedia.org/wiki/Bovine_respiratory_disease
2021-01-18T18:34:07
{"wikidata": ["Q17115892"]}
Endometrioid tumor Histopathology of a well-differentiated endometrioid adenocarcinoma in the ovary SpecialtyOncology, gynecology Endometrioid tumors are a class of tumor characterized by a resemblance to endometrium/[1] endometrial carcinoma, and over a third of cases have focal squamous differentiation. #...
Endometrioid tumor
c0474809
2,931
wikipedia
https://en.wikipedia.org/wiki/Endometrioid_tumor
2021-01-18T18:34:27
{"wikidata": ["Q5376366"]}
Moyamoya disease (MMD) is a rare intracranial arteriopathy involving progressive stenosis of the cerebral vasculature located at the base of the brain causing transient ischemic attacks or strokes. ## Epidemiology The incidence of MMD is highest in Asian populations but MMD occurs in many other ethnic groups. T...
Moyamoya disease
c0026654
2,932
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=2573
2021-01-23T18:16:24
{"gard": ["7064"], "mesh": ["D009072", "C536991"], "omim": ["252350", "607151", "608796", "614042"], "umls": ["C0026654", "C2931384"], "icd-10": ["I67.5"], "synonyms": ["Idiopathic Moyamoya disease"]}
Malonyl-CoA decarboxylase deficiency is a condition that prevents the body from converting certain fats to energy. The signs and symptoms of this disorder typically appear in early childhood. Almost all affected children have delayed development. Additional signs and symptoms can include weak muscle tone (hypotonia),...
Malonyl-CoA decarboxylase deficiency
c0342793
2,933
medlineplus
https://medlineplus.gov/genetics/condition/malonyl-coa-decarboxylase-deficiency/
2021-01-27T08:25:27
{"gard": ["3371"], "mesh": ["C535702"], "omim": ["248360"], "synonyms": []}
Okihiro syndrome is a syndrome of multiple congenital anomalies and is characterized by ocular manifestations (uni- or bilateral Duane anomaly (95% of cases), congenital optic nerve hypoplasia or optic disc coloboma), bilateral deafness and radial ray malformation that can include thenar hypoplasia and/or hypoplasia ...
Okihiro syndrome
c1623209
2,934
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=93293
2021-01-23T18:13:11
{"gard": ["9182"], "mesh": ["D004370"], "omim": ["607323"], "umls": ["C1623209"], "icd-10": ["Q87.8"], "synonyms": ["Duane-radial ray syndrome"]}
A number sign (#) is used with this entry because of evidence that cleft palate, cardiac defects, and mental retardation (CPCMR) is caused by heterozygous mutation in the MEIS2 gene (601740) on chromosome 15q14. Clinical Features Percin et al. (1995) reported a family in which members in 3 generations showed variou...
CLEFT PALATE, CARDIAC DEFECTS, AND MENTAL RETARDATION
c1832950
2,935
omim
https://www.omim.org/entry/600987
2019-09-22T16:15:32
{"mesh": ["C563414"], "omim": ["600987"], "synonyms": ["Alternative titles", "CARDIAC MALFORMATION, CLEFT LIP/PALATE, MICROCEPHALY, AND DIGITAL ANOMALIES"]}
A number sign (#) is used with this entry because Czech dysplasia is caused by heterozygous mutation in the COL2A1 gene (120140) on chromosome 12q13. Description Czech dysplasia is an autosomal dominant skeletal dysplasia characterized by early-onset, progressive pseudorheumatoid arthritis, platyspondyly, and short...
CZECH DYSPLASIA
c1836683
2,936
omim
https://www.omim.org/entry/609162
2019-09-22T16:06:34
{"mesh": ["C535766"], "omim": ["609162"], "orphanet": ["137678"], "synonyms": ["Alternative titles", "CZECH DYSPLASIA, METATARSAL TYPE", "PSEUDORHEUMATOID DYSPLASIA, PROGRESSIVE, WITH HYPOPLASTIC TOES", "SPONDYLOEPIPHYSEAL DYSPLASIA WITH PRECOCIOUS OSTEOARTHRITIS"]}
Contrast-induced nephropathy Other namesCIN SpecialtyNephrology Contrast-induced nephropathy (CIN) is a form of kidney damage in which there has been recent exposure to medical imaging contrast material without another clear cause for the acute kidney injury. CIN is classically defined as a serum creatinine ...
Contrast-induced nephropathy
c4055183
2,937
wikipedia
https://en.wikipedia.org/wiki/Contrast-induced_nephropathy
2021-01-18T18:58:16
{"umls": ["C4055183"], "icd-9": ["586"], "icd-10": ["N14.1"], "wikidata": ["Q1783300"]}
This article has multiple issues. Please help improve it or discuss these issues on the talk page. (Learn how and when to remove these template messages) This article needs attention from an expert on the subject. Please add a reason or a talk parameter to this template to explain the issue with the article. W...
Langer mesomelic dysplasia
c0432230
2,938
wikipedia
https://en.wikipedia.org/wiki/Langer_mesomelic_dysplasia
2021-01-18T18:35:48
{"gard": ["3553"], "mesh": ["C537267"], "umls": ["C0432230"], "icd-10": ["Q87.1"], "orphanet": ["2632"], "wikidata": ["Q16550087"]}
Vascular tumor A hemangioma, a benign type of vascular tumor SpecialtyOncology A vascular tumor is a tumor of vascular origin; a soft tissue growth that can be either benign or malignant, formed from blood vessels or lymph vessels.[1] Examples of vascular tumors include hemangiomas, lymphangiomas, hemang...
Vascular tumor
c0282607
2,939
wikipedia
https://en.wikipedia.org/wiki/Vascular_tumor
2021-01-18T19:02:45
{"mesh": ["D019043"], "umls": ["C0282607", "C0027668"], "orphanet": ["211237"], "wikidata": ["Q25422732"]}
Not to be confused with Homothorax. Blood accumulation in the pleural cavity Hemothorax Other namesHaemothorax Haemorrhagic pleural effusion Chest X-ray showing left sided hemothorax (arrowed) SpecialtyPulmonology SymptomsChest pain Difficulty breathing ComplicationsEmpyema Fibrothorax TypesTr...
Hemothorax
c0019123
2,940
wikipedia
https://en.wikipedia.org/wiki/Hemothorax
2021-01-18T19:00:51
{"mesh": ["D006491"], "umls": ["C0019123"], "icd-9": ["860", "511.8"], "icd-10": ["J94.2", "S27.1"], "wikidata": ["Q369073"]}
A number sign (#) is used with this entry because congenital adrenal hyperplasia (CAH) due to 11-beta-hydroxylase deficiency is caused by homozygous or compound heterozygous mutation in the CYP11B1 gene (610613) on chromosome 8q24. Description Congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency...
ADRENAL HYPERPLASIA, CONGENITAL, DUE TO STEROID 11-BETA-HYDROXYLASE DEFICIENCY
c0268292
2,941
omim
https://www.omim.org/entry/202010
2019-09-22T16:31:26
{"doid": ["0050811"], "mesh": ["C535978"], "omim": ["202010"], "orphanet": ["90795", "418"], "synonyms": ["Alternative titles", "ADRENAL HYPERPLASIA IV", "STEROID 11-BETA-HYDROXYLASE DEFICIENCY", "11-BETA-HYDROXYLASE DEFICIENCY", "ADRENAL HYPERPLASIA, HYPERTENSIVE FORM", "P450C11B1 DEFICIENCY"]}
Oral leukoplakia describes a white plaque that does not rub off and cannot be characterized as any other condition. Though it may occur in any part of the mouth, it generally affects the tongue, gums, and inner cheek. Physicians will usually biopsy oral leukoplakia lesions as 20-40% of cases are precancerous or cance...
Oral leukoplakia
c0023532
2,942
gard
https://rarediseases.info.nih.gov/diseases/7260/oral-leukoplakia
2021-01-18T17:58:34
{"mesh": ["D007972"], "umls": ["C0023532"], "synonyms": []}
Bartram et al. (1982) observed subacute sclerosing panencephalitis in a brother and sister of nonconsanguineous parents of 11 children living in rural Turkey. An interval of 4 years separated onset of symptoms in the 2 children. Fibroblast interferon had no beneficial effect. Neuro \- Subacute sclerosing panencep...
PANENCEPHALITIS, SUBACUTE SCLEROSING
c0038522
2,943
omim
https://www.omim.org/entry/260470
2019-09-22T16:23:47
{"doid": ["8970"], "mesh": ["D013344"], "omim": ["260470"], "icd-9": ["046.2"], "icd-10": ["A81.1"], "orphanet": ["2806"], "synonyms": ["Alternative titles", "SUBACUTE SCLEROSING PANENCEPHALITIS"]}
Abortion in Spain is legal upon request up to 14 weeks of pregnancy, and at later stages for serious risk to the health of the woman or fetal defects.[1] Abortion legislation in Spain has a fluctuating history. During the 1930s, abortion law was liberalized in the area controlled by the Republicans, but this was sho...
Abortion in Spain
None
2,944
wikipedia
https://en.wikipedia.org/wiki/Abortion_in_Spain
2021-01-18T18:29:00
{"wikidata": ["Q2874481"]}
Gaucher disease type 1 (GD1) is the most common form of Gaucher disease. Like other types of Gaucher disease, GD1 is caused when not enough glucocerebrosidase (GBA) is made. GBA is an important enzyme that breaks down a fatty chemical called glucocerebroside. Because the body cannot break down this chemical, fat-...
Gaucher disease type 1
c1961835
2,945
gard
https://rarediseases.info.nih.gov/diseases/2441/gaucher-disease-type-1
2021-01-18T18:00:22
{"mesh": ["D005776"], "omim": ["230800"], "orphanet": ["77259"], "synonyms": ["Gaucher disease, noncerebral juvenile", "GD 1", "Glucocerebrosidase deficiency", "Acid beta-glucosidase deficiency", "GBA DEFICIENCY"]}
Relatively benign brain cancer involving ependymal cells Subependymoma Micrograph of a subependymoma showing the characteristic clustering of nuclei. H&E stain. SpecialtyNeoplasms A subependymoma is a type of brain tumor; specifically, it is a rare form of ependymal tumor.[1] They are usually in middle aged...
Subependymoma
c0206725
2,946
wikipedia
https://en.wikipedia.org/wiki/Subependymoma
2021-01-18T18:51:10
{"gard": ["10070"], "mesh": ["D018315"], "wikidata": ["Q7631123"]}
A number sign (#) is used with this entry because autosomal dominant deafness-3A (DFNA3A) is caused by heterozygous mutation in the connexin-26 gene (GJB2; 121011) on chromosome 13q12. See also DFNA3B (612643), which is caused by mutation in the connexin-30 gene (GJB6; 604418) on chromosome 13q12. Clinical Feat...
DEAFNESS, AUTOSOMAL DOMINANT 3A
c2675750
2,947
omim
https://www.omim.org/entry/601544
2019-09-22T16:14:37
{"doid": ["0110564"], "mesh": ["C567277"], "omim": ["601544"], "orphanet": ["90635"], "synonyms": ["Autosomal dominant isolated neurosensory deafness type DFNA", "Autosomal dominant isolated neurosensory hearing loss type DFNA", "Autosomal dominant isolated sensorineural deafness type DFNA", "Autosomal dominant isolate...
For a general phenotypic description and a discussion of genetic heterogeneity of neuroblastoma, see NBLST1 (256700). Mapping Maris et al. (2008) provided evidence for 1 or more candidate neuroblastoma susceptibility genes on chromosome 6p22. Among 1,032 neuroblastoma patients and 2,043 controls of European descent...
NEUROBLASTOMA, SUSCEPTIBILITY TO, 4
c0027819
2,948
omim
https://www.omim.org/entry/613015
2019-09-22T16:00:06
{"mesh": ["D009447"], "omim": ["613015"], "orphanet": ["635"]}
Parkes Weber syndrome is a disorder of the vascular system, which is the body's complex network of blood vessels. The vascular system consists of arteries, which carry oxygen-rich blood from the heart to the body's various organs and tissues; veins, which carry blood back to the heart; and capillaries, which are tiny...
Parkes Weber syndrome
c1842180
2,949
medlineplus
https://medlineplus.gov/genetics/condition/parkes-weber-syndrome/
2021-01-27T08:24:55
{"gard": ["9787"], "mesh": ["C564254"], "omim": ["608354"], "synonyms": []}
A number sign (#) is used with this entry because several chromosome aberrations, including recurrent translocations and deletions, have been found to be related to the development or progression of multiple myeloma; see CYTOGENETICS section. Description Multiple myeloma is a neoplastic plasma cell disorder charact...
MYELOMA, MULTIPLE
c0026764
2,950
omim
https://www.omim.org/entry/254500
2019-09-22T16:24:41
{"doid": ["9538"], "mesh": ["D009101"], "omim": ["254500"], "icd-9": ["203.0"], "icd-10": ["C90.0", "C90.00"], "orphanet": ["29073", "314701", "85443"]}
Oodinium, a genus of parasitic dinoflagellates, causes velvet disease in fish Velvet disease (also called gold-dust, rust and coral disease) is a fish disease caused by dinoflagellate parasites of the genus Piscinoodinium, specifically Amyloodinium in marine fish, and Oodinium in freshwater fish. The disease gives i...
Velvet (fish disease)
None
2,951
wikipedia
https://en.wikipedia.org/wiki/Velvet_(fish_disease)
2021-01-18T18:52:07
{"wikidata": ["Q2217862"]}
A number sign (#) is used with this entry because of evidence that this form of autosomal recessive mental retardation (MRT6) is caused by homozygous mutation in the ionotropic glutamate receptor-6 gene (GRIK2; 138244) on chromosome 6q16. Clinical Features Motazacker et al. (2007) reviewed phenotypic features o...
MENTAL RETARDATION, AUTOSOMAL RECESSIVE 6
c1970198
2,952
omim
https://www.omim.org/entry/611092
2019-09-22T16:03:39
{"doid": ["0060308"], "mesh": ["C567017"], "omim": ["611092"], "orphanet": ["88616"], "synonyms": ["AR-NSID", "NS-ARID"]}
A rare ciliopathy characterized by progressive hearing and visual loss in the first decades of life and, in some cases, vestibular dysfunction. Patients have normal hearing at birth. Onset of hearing loss is usually in late childhood or adolescence after development of speech. Profound deafness is mostly reported by ...
Usher syndrome type 3
c1568248
2,953
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=231183
2021-01-23T17:37:50
{"gard": ["5442"], "mesh": ["D052245"], "omim": ["276902", "500004", "614504"], "umls": ["C1568248"], "icd-10": ["H35.5"], "synonyms": ["USH3"]}
Frontotemporal dementia (FTD) comprises a group of neurodegenerative disorders, characterized by progressive changes in behavior, executive dysfunction and language impairment, as a result of degeneration of the medial prefrontal and frontoinsular cortices. Four clinical subtypes have been identified: semantic dement...
Frontotemporal dementia
c0338451
2,954
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=282
2021-01-23T18:00:52
{"gard": ["8436"], "mesh": ["D057180"], "omim": ["172700", "600274", "600795", "607485"], "umls": ["C0338451"], "icd-10": ["G31.0"], "synonyms": ["FTD"]}
Jalili (1989) studied 2 female cousins, both the products of consanguineous marriages, affected with severe retinal dystrophy characterized by visual impairment from birth and profound photophobia in the absence of night blindness. The ophthalmologic characteristics suggested a cone-rod type of congenital amaurosis. ...
AMAUROSIS CONGENITA, CONE-ROD TYPE, WITH CONGENITAL HYPERTRICHOSIS
c1857588
2,955
omim
https://www.omim.org/entry/204110
2019-09-22T16:31:11
{"mesh": ["C536604"], "omim": ["204110"], "orphanet": ["1021"]}
Spinal enthesopathy SpecialtyRheumatology Spinal enthesopathy is a form of enthesopathy affecting the spine.[1] ## References[edit] 1. ^ Ball, John (1 January 1983). "The Enthesopathy of Ankylosing Spondylitis". Rheumatology. XXII (suppl_2): 25–28. doi:10.1093/rheumatology/XXII.suppl_2.25. ## External lin...
Spinal enthesopathy
c0152090
2,956
wikipedia
https://en.wikipedia.org/wiki/Spinal_enthesopathy
2021-01-18T18:48:02
{"icd-9": ["720.1"], "icd-10": ["M46.0"], "wikidata": ["Q7577458"]}
Contact granuloma Other namesContact ulcer, Vocal fold contact ulcer or Vocal process granuloma Healthy vocal folds. Contact granulomas may form in the posterior part of the larynx. SpecialtyOtolaryngologist Contact granuloma is a condition that develops due to persistent tissue irritation in the poste...
Contact granuloma
c4040427
2,957
wikipedia
https://en.wikipedia.org/wiki/Contact_granuloma
2021-01-18T19:10:03
{"umls": ["C4040427"], "wikidata": ["Q5164829"]}
A number sign (#) is used with this entry because of evidence that hereditary motor and sensory neuropathy type VIB with optic atrophy (HMSN6B) is caused by homozygous or compound heterozygous mutation in the SLC25A46 gene (610826) on chromosome 5q22. Description Hereditary motor and sensory neuropathy type VIB is ...
NEUROPATHY, HEREDITARY MOTOR AND SENSORY, TYPE VIB, WITH OPTIC ATROPHY
c0393807
2,958
omim
https://www.omim.org/entry/616505
2019-09-22T15:48:39
{"doid": ["0080068"], "mesh": ["C562851"], "omim": ["616505"], "orphanet": ["90120"], "synonyms": ["Alternative titles", "HMSN VIB", "CHARCOT-MARIE-TOOTH DISEASE, TYPE 6B"]}
Late-onset familial Alzheimer disease, is a form of familial Alzheimer disease, that begins after age 65. In general, Alzheimer disease (AD) is a degenerative disease of the brain that causes gradual loss of memory, judgement and the ability to function socially. The exact underlying cause of late-onset familial AD i...
Late-Onset Familial Alzheimer Disease
None
2,959
gard
https://rarediseases.info.nih.gov/diseases/12799/late-onset-familial-alzheimer-disease
2021-01-18T17:59:30
{"synonyms": []}
Rotor syndrome Other namesRotor type hyperbilirubinemia[1] Bilirubin SpecialtyPediatrics, hepatology Rotor syndrome (also known as Rotor type hyperbilirubinemia)[2] is a rare cause of mixed direct (conjugated) and indirect (unconjugated) hyperbilirubinemia, relatively benign, autosomal recessive[3] bilirub...
Rotor syndrome
c0220991
2,960
wikipedia
https://en.wikipedia.org/wiki/Rotor_syndrome
2021-01-18T18:39:30
{"gard": ["218"], "mesh": ["D006933"], "umls": ["C0220991"], "icd-9": ["277.4"], "icd-10": ["E80.6"], "orphanet": ["3111"], "wikidata": ["Q1512812"]}
Cutis laxa is a disorder of connective tissue, which is the tissue that forms the body's supportive framework. Connective tissue provides structure and strength to the muscles, joints, organs, and skin. The term "cutis laxa" is Latin for loose or lax skin, and this condition is characterized by skin that is sagg...
Cutis laxa
c3276539
2,961
medlineplus
https://medlineplus.gov/genetics/condition/cutis-laxa/
2021-01-27T08:25:11
{"gard": ["6227", "4017"], "omim": ["123700", "614434", "616603", "219100", "614437", "613177", "219200", "612940", "219150", "614438", "614100", "304150"], "synonyms": []}
Bangstad syndrome Other namesAtaxia-diabetes-goiter-gonadal insufficiency syndrome This condition is inherited in an autosomal recessive manner Bangstad syndrome is a severe, inherited congenital disorder associated with abnormalities of the cell membrane. It was characterized in 1989.[1] ## Contents * ...
Bangstad syndrome
c0342284
2,962
wikipedia
https://en.wikipedia.org/wiki/Bangstad_syndrome
2021-01-18T18:47:51
{"gard": ["812"], "mesh": ["C537902"], "umls": ["C0342284"], "orphanet": ["1227"], "wikidata": ["Q4855629"]}
A rare syndromic craniosynostosis with variable phenotypic expression characterized by craniosynostosis, intellectual disability, distinctive facies, abnormalities of the fingers and toes (brachydactyly, polydactyly and syndactyly), short stature, congenital heart disease, skeletal defects, obesity, genital abnor...
Carpenter syndrome
c1275078
2,963
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=65759
2021-01-23T18:52:39
{"gard": ["6003"], "mesh": ["C563187"], "omim": ["201000", "614976"], "umls": ["C1275078"], "icd-10": ["Q87.0"], "synonyms": ["ACPS2", "Acrocephalopolysyndactyly type 2"]}
Common variable immunodeficiency (CVID) is a group of disorders characterized by low levels of a type of protein known as immunoglobulins (Ig). Because of low level of Ig, the immune system cannot make antibodies that fight bacteria, viruses or other toxins in the body. This leads to frequent infections, particularly...
Common variable immunodeficiency
c0009447
2,964
gard
https://rarediseases.info.nih.gov/diseases/6140/common-variable-immunodeficiency
2021-01-18T18:01:13
{"mesh": ["D017074"], "omim": ["607594"], "orphanet": ["1572"], "synonyms": ["CVID", "Common variable hypogamma-globulinemia", "Hypogamma-globulinemia, acquired", "Immunoglobulin deficiency, late-onset", "Common variable immune deficiency", "Idiopathic immunoglobulin deficiency", "Primary antibody deficiency", "Primary...
Epithelial basement membrane dystrophy Other namesMap-dot-fingerprint dystrophy and Cogans's microcystic dystrophy SpecialtyOphthalmology Epithelial basement membrane dystrophy (EBMD), is a disorder of the eye that can cause pain and dryness. It is sometimes included in the group of corneal dystrophies.[1] ...
Epithelial basement membrane dystrophy
c0521723
2,965
wikipedia
https://en.wikipedia.org/wiki/Epithelial_basement_membrane_dystrophy
2021-01-18T19:01:02
{"gard": ["9732"], "mesh": ["C535477"], "umls": ["C0521723"], "orphanet": ["98956"], "wikidata": ["Q4162388"]}
Overview of obesity in Germany Obesity in Germany has been increasingly cited as a major health issue in recent years. The federal government has declared this to be a major issue.[1] Data released by the World Health Organisation in 2014 showed that while an issue of growing concern, within the European Union,...
Obesity in Germany
None
2,966
wikipedia
https://en.wikipedia.org/wiki/Obesity_in_Germany
2021-01-18T19:02:47
{"wikidata": ["Q7074854"]}
The examples and perspective in this article deal primarily with the United States and do not represent a worldwide view of the subject. You may improve this article, discuss the issue on the talk page, or create a new article, as appropriate. (March 2013) (Learn how and when to remove this template message) ...
Concussions in sport
None
2,967
wikipedia
https://en.wikipedia.org/wiki/Concussions_in_sport
2021-01-18T19:05:51
{"wikidata": ["Q5159124"]}
A rare, genetic form of obesity characterized by severe early-onset obesity, hyperphagia, insulin resistance with hyperinsulinemia, reduced adult final height, delayed speech and language development and a tendency for social isolation and aggressive behavior. *[v]: View this template *[t]: Discuss this temp...
Severe early-onset obesity-insulin resistance syndrome due to SH2B1 deficiency
None
2,968
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=329249
2021-01-23T17:10:12
{"icd-10": ["E66.8"]}
A number sign (#) is used with this entry because of evidence that multiple fibroadenomas of the breast (MFAB) are caused by heterozygous mutation in the PRLR gene (176761) on chromosome 5p13. Description Fibroadenoma represents a benign breast disease characterized by lobuloalveolar growth with abnormally high pro...
MULTIPLE FIBROADENOMAS OF THE BREAST
c3809918
2,969
omim
https://www.omim.org/entry/615554
2019-09-22T15:51:37
{"omim": ["615554"], "orphanet": ["50920"], "synonyms": ["Mammary polyadenomatosis"]}
Localized lichen myxedematosus with monoclonal gammopathy or systemic symptoms is a form of atypical lichen myxedematosus (see this term), characterized by the appearance of several 2-4 mm erythematous waxy papules confined to a few sites that may be associated with either an immunoglobulin A (IgA) nephropathy in pat...
Localized lichen myxedematosus with monoclonal gammopathy or systemic symptoms
None
2,970
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=90399
2021-01-23T17:33:45
{"icd-10": ["L98.5"]}
A number sign (#) is used with this entry because of evidence that the exclusively skeletal form of Antley-Bixler syndrome can be caused by heterozygous mutation in a fibroblast growth factor receptor gene, FGFR2 (176943), on chromosome 10q26. A form of Antley-Bixler syndrome that includes disordered steroidogenesis...
ANTLEY-BIXLER SYNDROME WITHOUT GENITAL ANOMALIES OR DISORDERED STEROIDOGENESIS
c2936791
2,971
omim
https://www.omim.org/entry/207410
2019-09-22T16:30:55
{"doid": ["0050462"], "mesh": ["D054882"], "omim": ["207410"], "orphanet": ["83"], "synonyms": ["Alternative titles", "TRAPEZOIDOCEPHALY-SYNOSTOSIS SYNDROME", "MULTISYNOSTOTIC OSTEODYSGENESIS WITH LONG BONE FRACTURES", "OSTEODYSGENESIS, MULTISYNOSTOTIC, WITH FRACTURES"]}
Hidradenoma Other namesAcrospiroma, cystadenoma, hydrocystadenomas Micrograph showing an acrospiroma. H&E stain. SpecialtyDermatology Hidradenoma refers to a benign adnexal tumor of the apical sweat gland.[1][2] These are 1–3 cm translucent blue cystic nodules. It usually presents as a single, small skin-c...
Hidradenoma
c0019522
2,972
wikipedia
https://en.wikipedia.org/wiki/Hidradenoma
2021-01-18T19:04:18
{"mesh": ["D006607"], "umls": ["C0019522"], "wikidata": ["Q12822297"]}
## Clinical Features Cantu et al. (1981, 1985) reported a second Guadalajara camptodactyly syndrome; see 211910 for a description of type I. Two sisters, aged 6 and 3 years, presented the same intrauterine growth retardation-malformation syndrome characterized by low birthweight dwarfism and a variety of dysmor...
CAMPTODACTYLY SYNDROME, GUADALAJARA, TYPE II
c2673861
2,973
omim
https://www.omim.org/entry/211920
2019-09-22T16:30:13
{"mesh": ["C567138"], "omim": ["211920"], "orphanet": ["1326"]}
"Mucous cyst" redirects here. For swelling of connective oral tissue, see Oral mucocele. Myxoid cyst Other namesDigital mucous cyst,[1] and Mucous cyst[1]) Digital mucous cyst in left index finger with nail depression SpecialtyOncology, rheumatology A Myxoid cyst is a cutaneous condition often characteriz...
Myxoid cyst
c1258666
2,974
wikipedia
https://en.wikipedia.org/wiki/Myxoid_cyst
2021-01-18T19:01:44
{"mesh": ["D045888"], "wikidata": ["Q6949364"]}
Metatropic dysplasia is a skeletal disorder characterized by short stature, shortened arms and legs, and a long narrow chest. The signs and symptoms of this condition can vary from life threatening to mild. Signs and symptoms may include worsening abnormal curvature of the spine (scoliosis and kyphosis), flatteni...
Metatropic dysplasia
c0265281
2,975
gard
https://rarediseases.info.nih.gov/diseases/3571/metatropic-dysplasia
2021-01-18T17:59:07
{"mesh": ["C537356"], "omim": ["156530"], "umls": ["C0265281"], "orphanet": ["2635"], "synonyms": ["Metatropic dwarfism", "Metatropic dysplasia, nonlethal dominant"]}
## Clinical Features Duncan et al. (1979) reported 2 female patients and 28 others in the literature with the combination of mullerian duct aplasia, unilateral renal aplasia, and cervicothoracic somite dysplasia (MURCS). The authors postulated an alteration affecting blastemas of the lower cervical-upper thoracic s...
MULLERIAN DUCT APLASIA, UNILATERAL RENAL AGENESIS, AND CERVICOTHORACIC SOMITE ANOMALIES
c1698581
2,976
omim
https://www.omim.org/entry/601076
2019-09-22T16:15:27
{"mesh": ["C537371"], "omim": ["601076"], "orphanet": ["3109", "2578"], "synonyms": ["Alternative titles", "MAYER-ROKITANSKY-KUSTER-HAUSER SYNDROME, TYPE II", "MRKH, TYPE II", "KLIPPEL-FEIL DEFORMITY, CONDUCTIVE DEAFNESS, AND ABSENT VAGINA"]}
Palilalia (from the Greek πάλιν (pálin) meaning "again" and λαλιά (laliá) meaning "speech" or "to talk"),[1] a complex tic, is a language disorder characterized by the involuntary repetition of syllables, words, or phrases. It has features resembling other complex tics such as echolalia or coprolalia, but, unlike oth...
Palilalia
c0392185
2,977
wikipedia
https://en.wikipedia.org/wiki/Palilalia
2021-01-18T18:53:29
{"wikidata": ["Q1757666"]}
Patients with acute lymphoblastic leukemia (ALL) who present with bulky disease of the lymph nodes, spleen, and mediastinum, so-called lymphomatous ALL (LALL), appear clinically to represent a distinct category of ALL of T-cell lineage. The biologic basis of this distinction was pointed out by Chilcote et al. (19...
LYMPHOBLASTIC LEUKEMIA, ACUTE, WITH LYMPHOMATOUS FEATURES
c0023449
2,978
omim
https://www.omim.org/entry/247640
2019-09-22T16:25:45
{"doid": ["9952"], "omim": ["247640"], "orphanet": ["513"], "synonyms": ["Alternative titles", "LYMPHOMATOUS ALL"]}
A number sign (#) is used with this entry because heme oxygenase-1 deficiency can be caused by compound heterozygous mutation in the HMOX1 gene (141250) on chromosome 22q13. Clinical Features Yachie et al. (1999) reported the first human case of heme oxygenase-1 deficiency. The patient was 26 months old when he was...
HEME OXYGENASE 1 DEFICIENCY
c1841651
2,979
omim
https://www.omim.org/entry/614034
2019-09-22T15:56:44
{"mesh": ["C564200"], "omim": ["614034"]}
A rare genetic disease associated with mild to severe intellectual deficit that may be associated with behavioral disorders and characteristic physical features including a high forehead, prominent and large ears, hyperextensible finger joints, flat feet with pronation and, in adolescent and adult males, macroorchidi...
Fragile X syndrome
c0016667
2,980
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=908
2021-01-23T16:52:56
{"gard": ["6464"], "mesh": ["D005600"], "omim": ["300624", "311360"], "umls": ["C0016667", "C0751156"], "icd-10": ["Q99.2"], "synonyms": ["FRAXA syndrome", "FXS", "FraX syndrome", "Martin-Bell syndrome"]}
A number sign (#) is used with this entry because of evidence that pyruvate dehydrogenase E2 deficiency is caused by homozygous mutation in the DLAT gene (608770) on chromosome 11q23. For a general phenotypic description and a discussion of genetic heterogeneity of pyruvate dehydrogenase deficiency, see 312170. Cli...
PYRUVATE DEHYDROGENASE E2 DEFICIENCY
c0034345
2,981
omim
https://www.omim.org/entry/245348
2019-09-22T16:26:03
{"doid": ["3649"], "mesh": ["D015325"], "omim": ["245348"], "orphanet": ["765", "79244"], "synonyms": ["Alternative titles", "LACTIC ACIDEMIA DUE TO DEFECT OF E2 LIPOYL TRANSACETYLASE OF THE PYRUVATE DEHYDROGENASE COMPLEX"]}
Isobutyryl-CoA dehydrogenase deficiency is an inborn error of valine metabolism. The prevalence is unknown. Only one symptomatic patient (with anaemia, failure to thrive, dilated cardiomyopathy and plasma carnitine deficiency) has been described so far, but several series of patients have been identified through newb...
Isobutyryl-CoA dehydrogenase deficiency
c1969809
2,982
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=79159
2021-01-23T17:31:09
{"gard": ["10223"], "mesh": ["C535541"], "omim": ["611283"], "umls": ["C1969809"], "icd-10": ["E71.1"], "synonyms": ["Isobutyric aciduria"]}
Oxygen shortage of the brain For other uses, see hypoxia (disambiguation). Cerebral hypoxia Circle of Willis Arteries beneath brain SpecialtyCritical care medicine Cerebral hypoxia is a form of hypoxia (reduced supply of oxygen), specifically involving the brain; when the brain is completely deprived of ...
Cerebral hypoxia
c1140716
2,983
wikipedia
https://en.wikipedia.org/wiki/Cerebral_hypoxia
2021-01-18T18:46:58
{"mesh": ["D002534"], "icd-9": ["437.9"], "wikidata": ["Q2249526"]}
A number sign (#) is used with this entry because of evidence that mutations in the NOD2/CARD15 gene (605956) are associated with susceptibility to Crohn disease in families linked to chromosome 16. A promoter polymorphism in the IL6 gene (147620) is associated with susceptibility to Crohn disease-associated grow...
INFLAMMATORY BOWEL DISEASE (CROHN DISEASE) 1
c0010346
2,984
omim
https://www.omim.org/entry/266600
2019-09-22T16:22:49
{"doid": ["0110892"], "mesh": ["D003424"], "omim": ["266600"], "icd-9": ["556.9", "556"], "icd-10": ["K50.90", "K51", "K50", "K50.9", "K51.9"]}
Erdheim-Chester disease (ECD), a non-Langerhans form of histiocytosis, is a multisystemic disease characterized by various manifestations such as skeletal involvement with bone pain, exophthalmos, diabetes insipidus, renal impairment and central nervous system (CNS) and/or cardiovascular involvement. ## Epidemiology...
Erdheim-Chester disease
c0878675
2,985
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=35687
2021-01-23T18:38:16
{"gard": ["6369"], "mesh": ["D031249"], "umls": ["C0878675"], "icd-10": ["D76.3"]}
Myxopapillary ependymoma (MEPN) describes a slow growing ependymoma located almost exclusively in the conus medullaris-cauda equina-filum terminale region of the spinal cord, presenting in all age groups, and manifesting with variable symptoms such as neck pain, vomiting and unsteady gait and metastasis. It has a mor...
Myxopapillary ependymoma
c0205769
2,986
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=251643
2021-01-23T16:59:13
{"gard": ["10633"], "mesh": ["D004806"], "umls": ["C0205769"], "icd-10": ["D43.2"]}
Hypochondroplasia is a form of skeletal disease characterized by very short stature. Hypochondroplasia is similar to achondroplasia, but the features tend to be milder. People with hypochondroplasia usually have very short stature, large head, accentuated lordosis, short arms and legs, and broad, short hands and feet...
Hypochondroplasia
c0410529
2,987
gard
https://rarediseases.info.nih.gov/diseases/6724/hypochondroplasia
2021-01-18T17:59:53
{"mesh": ["C562937"], "omim": ["146000"], "umls": ["C0410529"], "orphanet": ["429"], "synonyms": ["HCH"]}
Carcinoma in situ Other namesin situ neoplasm SpecialtyOncology Carcinoma in situ (CIS) is a group of abnormal cells.[1][2] While they are a form of neoplasm,[3] there is disagreement over whether CIS should be classified as cancer. This controversy also depends on the exact CIS in question (i.e. cervical, s...
Carcinoma in situ
c0007099
2,988
wikipedia
https://en.wikipedia.org/wiki/Carcinoma_in_situ
2021-01-18T19:09:20
{"mesh": ["D002278"], "umls": ["C0007099"], "wikidata": ["Q1035645"]}
Dermatologic terminology Atrophoderma refers to conditions involving skin atrophy.[1] Types include: * Follicular atrophoderma * Linear atrophoderma of Moulin * Atrophoderma of Pasini and Pierini ## References[edit] 1. ^ "Atrophoderma" at Dorland's Medical Dictionary * v * t * e Cutaneous ker...
Atrophoderma
c0151514
2,989
wikipedia
https://en.wikipedia.org/wiki/Atrophoderma
2021-01-18T18:58:34
{"wikidata": ["Q4072263"]}
Inherited isolated adrenal insufficiency due to partial CYP11A1 deficiency is a rare, genetic, chronic, primary adrenal insufficiency disorder, due to partial loss-of-function CYP11A1 mutations, characterized by early-onset adrenal insufficiency without associated abnormal external male genitalia. Patients presen...
Inherited isolated adrenal insufficiency due to partial CYP11A1 deficiency
c4707238
2,990
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=289548
2021-01-23T17:42:04
{"icd-10": ["E27.1"]}
In psychiatry, complicated grief disorder (CGD) is a proposed disorder for those who are significantly and functionally impaired by prolonged grief symptoms for at least twelve months after the bereavement.[1] It is distinguished from non-impairing grief[2] and other disorders (such as major depressive disorder[3][4]...
Complicated grief disorder
None
2,991
wikipedia
https://en.wikipedia.org/wiki/Complicated_grief_disorder
2021-01-18T18:53:56
{"wikidata": ["Q5156653"]}
A number sign (#) is used with this entry because susceptibility to thrombophilia (THPH1) can be conferred by heterozygous mutation in the thrombin gene (F2; 176930) on chromosome 11p11. Description Thrombophilia is a multifactorial disorder of inappropriate clot formation resulting from an interaction of genetic, ...
THROMBOPHILIA DUE TO THROMBIN DEFECT
c3160733
2,992
omim
https://www.omim.org/entry/188050
2019-09-22T16:32:38
{"omim": ["188050"], "synonyms": ["Alternative titles", "THROMBOPHILIA DUE TO FACTOR 2 DEFECT", "VENOUS THROMBOSIS", "VENOUS THROMBOEMBOLISM"], "genereviews": ["NBK1148"]}
Pelz et al. (1972) described cholesterol pneumonia in brother and sister, who died at 9.5 and 4 months, respectively. Tachypnea, cough and cyanosis were symptoms. Pulmonary \- Cholesterol pneumonia \- Tachypnea \- Cough Inheritance \- Autosomal recessive Misc \- Death in infancy Skin \- Cyanosis ▲ Close *[v]: ...
CHOLESTEROL PNEUMONIA
c0549472
2,993
omim
https://www.omim.org/entry/215030
2019-09-22T16:29:44
{"mesh": ["C535937"], "omim": ["215030"]}
Fructose malabsorption Other namesDietary fructose intolerance Chemical structure of fructose SpecialtyEndocrinology Fructose malabsorption, formerly named dietary fructose intolerance (DFI), is a digestive disorder[1] in which absorption of fructose is impaired by deficient fructose carriers in the small ...
Fructose malabsorption
c1531694
2,994
wikipedia
https://en.wikipedia.org/wiki/Fructose_malabsorption
2021-01-18T19:02:14
{"umls": ["C1531694"], "icd-9": ["271"], "icd-10": ["E74.3"], "wikidata": ["Q1671489"]}
Yokkaichi asthma (四日市ぜんそく, Yokkaichi zensoku) refers to cases of chronic obstructive pulmonary disease, chronic bronchitis, pulmonary emphysema, and bronchial asthma in humans and various environmental changes usually attributed to sulfur dioxide (SO2) emissions which appeared as smog over the city of Yokkaichi i...
Yokkaichi asthma
None
2,995
wikipedia
https://en.wikipedia.org/wiki/Yokkaichi_asthma
2021-01-18T19:10:43
{"wikidata": ["Q8054567"]}
## Description The type of diabetes mellitus called IDDM is a disorder of glucose homeostasis that is characterized by susceptibility to ketoacidosis in the absence of insulin therapy. It is a genetically heterogeneous autoimmune disease affecting about 0.3% of Caucasian populations (Todd, 1990). Genetic studie...
DIABETES MELLITUS, INSULIN-DEPENDENT
c0011854
2,996
omim
https://www.omim.org/entry/222100
2019-09-22T16:28:46
{"doid": ["9744"], "mesh": ["D003922"], "omim": ["222100"], "icd-10": ["E10"], "synonyms": ["Alternative titles", "DIABETES MELLITUS, TYPE I", "JUVENILE-ONSET DIABETES"]}
This article needs attention from an expert in medicine or Psychology. Please add a reason or a talk parameter to this template to explain the issue with the article. WikiProject Medicine or WikiProject Psychology may be able to help recruit an expert. (February 2009) Semantic dyslexia is, as the name suggests, ...
Semantic dyslexia
None
2,997
wikipedia
https://en.wikipedia.org/wiki/Semantic_dyslexia
2021-01-18T18:59:50
{"wikidata": ["Q7449060"]}
A rare, genetic, neurological disorder characterized by horizontal gaze palsy, sensorineural deafness, central hypoventilation, developmental delay, and intellectual disability, described in persons of Athabascan American Indian heritage. Swallowing dysfunction, vocal cord paralysis, facial paresis, seizures, interna...
Athabaskan brainstem dysgenesis syndrome
c1832215
2,998
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=69739
2021-01-23T18:59:54
{"gard": ["8333"], "mesh": ["C535397"], "omim": ["601536"], "umls": ["C1832215"], "synonyms": ["ABSD", "Athabascan brainstem dysgenesis syndrome", "Navajo brainstem syndrome"]}
Klebe et al. (1970), using mouse-human hybrid somatic cells in culture, found that Es-2 esterase activity was depressed. Human chromosomes are selectively lost from the hybrid cells. Depression of esterase activity was present when human chromosome 10 was present and the activity returned to normal when chromosome 10...
ESTERASE ES-2, REGULATOR FOR
c1851475
2,999
omim
https://www.omim.org/entry/133300
2019-09-22T16:41:28
{"omim": ["133300"]}