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Primary pigmented nodular adrenocortical disease (PPNAD) is a form of bilateral adrenocortical hyperplasia that is often associated with adrenocorticotrophin hormone (ACTH) independent Cushing syndrome (see this term) and is characterized by small to normal sized adrenal glands containing multiple small cortical ...
Primary pigmented nodular adrenocortical disease
c1864851
2,300
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=189439
2021-01-23T17:01:28
{"gard": ["10906"], "mesh": ["C566472"], "omim": ["610475", "610489", "614190", "615830"], "icd-10": ["E24.8"], "synonyms": ["PPNAD", "Primary pigmented nodular adrenal dysplasia"]}
Breus' mole Other namesOva tuberculosa,[citation needed] massive mole SpecialtyObstetrics Breus' mole is a massive, subchorionic, tuberous hematoma, formed out of maternal blood in the uterus in pregnancy. It was first described by Karl Breus in 1892.[1][2][3] ## Contents * 1 Cause and pathogenesis * 2...
Breus' mole
c1390676
2,301
wikipedia
https://en.wikipedia.org/wiki/Breus%27_mole
2021-01-18T19:07:13
{"umls": ["C1390676"], "wikidata": ["Q28439873"]}
Hermansky–Pudlak syndrome Other namesAlbinism with hemorrhagic diathesis and pigmented reticuloendothelial cells, Delta storage pool disease Hermansky–Pudlak syndrome is inherited via autosomal recessive manner SpecialtyEndocrinology Heřmanský–Pudlák syndrome (often written Hermansky–Pudlak syndrome or...
Hermansky–Pudlak syndrome
c0079504
2,302
wikipedia
https://en.wikipedia.org/wiki/Hermansky%E2%80%93Pudlak_syndrome
2021-01-18T18:32:22
{"gard": ["6643"], "mesh": ["D022861"], "umls": ["C0079504"], "orphanet": ["231537", "79430", "231531", "280663"], "wikidata": ["Q1506216"]}
Benign familial neonatal-infantile seizures (BFNIS) is a benign familial epilepsy syndrome with an intermediate phenotype between benign familial neonatal seizures (BFNS) and benign familial infantile seizures (BFIS; see these terms). So far, this syndrome has been described in multiple members of 10 families. Ag...
Benign familial neonatal-infantile seizures
c0220669
2,303
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=140927
2021-01-23T19:01:58
{"gard": ["1518"], "mesh": ["D020936"], "omim": ["607745"], "umls": ["C0220669"], "icd-10": ["G40.4"], "synonyms": ["BFNIS", "Benign neonatal-infantile epilepsy"]}
Myelodysplastic syndrome with excess blasts is a rare type of myelodysplastic syndrome (MDS). In this type of MDS, the number of very early forms of blood cells (blasts) are increased in the bone marrow and/or blood. There is also a low numbers of at least one type of blood cell. The early forms of cell types in the ...
Myelodysplastic Syndrome With Excess Blasts
c0002894
2,304
gard
https://rarediseases.info.nih.gov/diseases/13578/myelodysplastic-syndrome-with-excess-blasts
2021-01-18T17:58:51
{"mesh": ["D000754"], "icd-10": ["D46-2"], "orphanet": ["86839"], "synonyms": ["Refractory anemia with excess blasts", "RAEB"]}
This article is written like a personal reflection, personal essay, or argumentative essay that states a Wikipedia editor's personal feelings or presents an original argument about a topic. Please help improve it by rewriting it in an encyclopedic style. (July 2008) (Learn how and when to remove this template message...
Epilepsia partialis continua
c0085543
2,305
wikipedia
https://en.wikipedia.org/wiki/Epilepsia_partialis_continua
2021-01-18T18:29:14
{"mesh": ["D017036"], "umls": ["C0085543"], "icd-9": ["345.71", "345.7"], "icd-10": ["G40.5"], "wikidata": ["Q4898733"]}
Xanthoma striatum palmare SpecialtyDermatology Xanthoma striatum palmare is a cutaneous condition characterized by xanthomas of the palmar creases which are almost diagnostic for dysbetalipoproteinemia.[1] Xanthomas consist of accumulations of lipids within macrophages deposited within the dermis of the s...
Xanthoma striatum palmare
c4476834
2,306
wikipedia
https://en.wikipedia.org/wiki/Xanthoma_striatum_palmare
2021-01-18T18:43:31
{"wikidata": ["Q8043040"]}
This article's tone or style may not reflect the encyclopedic tone used on Wikipedia. See Wikipedia's guide to writing better articles for suggestions. (September 2013) (Learn how and when to remove this template message) Aboulomania (from Greek a– 'without', and boulē 'will')[1] is a mental disorder in whi...
Aboulomania
None
2,307
wikipedia
https://en.wikipedia.org/wiki/Aboulomania
2021-01-18T18:31:37
{"wikidata": ["Q4668562"]}
medical condition Acneiform eruption SpecialtyDermatology Acneiform eruptions are a group of dermatoses including acne vulgaris, rosacea, folliculitis, and perioral dermatitis.[1] Restated, acneiform eruptions are follicular eruptions characterized by papules and pustules resembling acne.[2] The hybrid te...
Acneiform eruption
c0175167
2,308
wikipedia
https://en.wikipedia.org/wiki/Acneiform_eruption
2021-01-18T18:28:02
{"mesh": ["D017486"], "umls": ["C0175167"], "icd-10": ["L70.8"], "wikidata": ["Q2365426"]}
Splenogonadal fusion-limb defects-micrognatia syndrome is a rare dysostosis syndrome characterized by abnormal fusion of the spleen with the gonad (or more rarely with remnants of the mesonephros), limb abnormalities (consisting of amelia or severe reduction defects leading to upper and/or lower rudimentary limbs...
Splenogonadal fusion-limb defects-micrognathia syndrome
c1866745
2,309
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=2063
2021-01-23T17:08:19
{"gard": ["4963"], "mesh": ["C537318"], "omim": ["183300"], "umls": ["C1866745"], "icd-10": ["Q87.8"], "synonyms": ["SGFLD syndrome"]}
A number sign (#) is used with this entry because of evidence that trimethylaminuria, sometimes referred to as fish-odor syndrome, is caused by homozygous or compound heterozygous mutation in the gene encoding flavin-containing monooxygenase-3 (FMO3; 136132) on chromosome 1q24. Another inborn error of metabolism acc...
TRIMETHYLAMINURIA
c0342739
2,310
omim
https://www.omim.org/entry/602079
2019-09-22T16:14:07
{"doid": ["0080361"], "mesh": ["C536561"], "omim": ["602079"], "icd-10": ["E72.52"], "orphanet": ["468726"], "synonyms": ["Alternative titles", "FISH-ODOR SYNDROME"], "genereviews": ["NBK1103"]}
Potassium-aggravated myotonia Other namesPAM[1] This condition is inherited in an autosomal dominant manner Potassium-aggravated myotonia is a rare genetic disorder that affects skeletal muscle. [2]Beginning in childhood or adolescence, people with this condition experience bouts of sustained muscle tensing ...
Potassium-aggravated myotonia
c2931826
2,311
wikipedia
https://en.wikipedia.org/wiki/Potassium-aggravated_myotonia
2021-01-18T18:30:51
{"gard": ["4459"], "mesh": ["C538353"], "umls": ["C2931826"], "orphanet": ["612"], "wikidata": ["Q7234683"]}
MEDNIK syndrome, previously known as Erythrokeratodermia Variabilis type 3 (EKV3), is characterized by intellectual deficit, enteropathy, sensorineural hearing loss, peripheral neuropathy, lamellar and erythrodermic ichthyosis, and keratodermia (MEDNIK stands for Mental retardation, Enteropathy, Deafness, periphe...
MEDNIK syndrome
c1836330
2,312
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=171851
2021-01-23T17:48:31
{"mesh": ["C563739"], "omim": ["609313"], "umls": ["C1836330"], "synonyms": ["Intellectual disability-enteropathy-deafness-peripheral neuropathy-ichthyosis-keratodermia syndrome", "Intellectual disability-enteropathy-hearing loss-peripheral neuropathy-ichthyosis-keratodermia syndrome"]}
A number sign (#) is used with this entry because of evidence that Teebi hypertelorism syndrome (TBHS) is caused by heterozygous mutation in the SPECC1L gene (614140) on chromosome 22q11. Clinical Features Teebi (1987) described a 4-generation Arab family in which many individuals showed striking hypertelorism with...
HYPERTELORISM, TEEBI TYPE
c0796179
2,313
omim
https://www.omim.org/entry/145420
2019-09-22T16:39:51
{"mesh": ["C536951"], "omim": ["145420"], "orphanet": ["1519"], "synonyms": ["Alternative titles", "Teebi hypertelorism syndrome", "Teebi syndrome", "BRACHYCEPHALOFRONTONASAL DYSPLASIA", "Brachycephalofrontonasal dysplasia", "Craniofrontonasal dysplasia, Teebi type"]}
Loeys-Dietz syndrome is a disorder that affects the connective tissue in many parts of the body. Connective tissue provides strength and flexibility to structures such as bones, ligaments, muscles, and blood vessels. There are five types of Loeys-Dietz syndrome, labelled types I through V, which are distinguished by...
Loeys-Dietz syndrome
c4551955
2,314
medlineplus
https://medlineplus.gov/genetics/condition/loeys-dietz-syndrome/
2021-01-27T08:24:59
{"gard": ["10788"], "mesh": ["D055947"], "omim": ["609192", "610168", "613795", "614816", "615582"], "synonyms": []}
## Summary ### Clinical characteristics. LTBP4-related cutis laxa is characterized by cutis laxa, early childhood-onset pulmonary emphysema, peripheral pulmonary artery stenosis, and other evidence of a generalized connective disorder such as inguinal hernias and hollow visceral diverticula (e.g., intestine, bladde...
LTBP4-Related Cutis Laxa
None
2,315
gene_reviews
https://www.ncbi.nlm.nih.gov/books/NBK343782/
2021-01-18T21:15:27
{"synonyms": ["Autosomal Recessive Cutis Laxa Type 1C (ARCL1C)", "Urban-Rifkin-Davis Syndrome (URDS)"]}
This article is an orphan, as no other articles link to it. Please introduce links to this page from related articles; try the Find link tool for suggestions. (October 2018) This article includes a list of references, related reading or external links, but its sources remain unclear because it lacks inline citat...
Dysphrenia
None
2,316
wikipedia
https://en.wikipedia.org/wiki/Dysphrenia
2021-01-18T19:04:04
{"wikidata": ["Q3710022"]}
A number sign (#) is used with this entry because of evidence that rolandic epilepsy with paroxysmal exercise-induced dystonia and writer's cramp (EPRPDC) is caused by compound heterozygous mutation in the TBC1D24 gene (613577) on chromosome 16p13. Description Rolandic epilepsy with paroxysmal exercise-induced ...
EPILEPSY, ROLANDIC, WITH PAROXYSMAL EXERCISE-INDUCED DYSTONIA AND WRITER'S CRAMP
c1842531
2,317
omim
https://www.omim.org/entry/608105
2019-09-22T16:08:15
{"mesh": ["C535499"], "omim": ["608105"], "orphanet": ["163727"], "synonyms": ["Alternative titles", "RE-PED-WC"]}
A group of spinocerebellar ataxias (SCAs) characterized by ataxia with other neurological signs, including oculomotor disturbances, cognitive deficits, pyramidal and extrapyramidal dysfunction, bulbar, spinal and peripheral nervous system involvement. ## Epidemiology The overall prevalence of SCAs is 1/33,000-1/50,...
Autosomal dominant cerebellar ataxia type I
None
2,318
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=94145
2021-01-23T18:26:54
{"icd-10": ["G11.8"], "synonyms": ["ADCA1", "ADCAI", "Autosomal dominant cerebellar ataxia type 1", "Cerebellar plus syndrome"]}
A rare genetic gynecological tumor characterized by early onset breast cancer in association with a germline mutation. Tumors arising in carriers of BRCA1 and BRCA2 mutations differ morphologically and genetically from each other, as well as from sporadic breast cancers. Most BRCA1-associated tumors are invasive duct...
Hereditary breast cancer
c0346153
2,319
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=227535
2021-01-23T18:59:35
{"mesh": ["C562840"], "omim": ["114480", "604370", "612555", "613399"], "umls": ["C0346153"], "icd-10": ["C50.0", "C50.1", "C50.2", "C50.3", "C50.4", "C50.5", "C50.6", "C50.8"], "synonyms": ["Familial breast cancer", "Familial breast carcinoma", "Hereditary breast carcinoma"]}
The most common type of cancers affecting the animal's nose are carcinomas and sarcomas, both of which are locally invasive. The most common sites for metastasis are the lymph nodes and the lungs, but can also include other organs. ## Contents * 1 Signs and symptoms * 2 Diagnosis * 3 Treatment * 4 Reference...
Nose cancer in cats and dogs
None
2,320
wikipedia
https://en.wikipedia.org/wiki/Nose_cancer_in_cats_and_dogs
2021-01-18T18:28:07
{"wikidata": ["Q7061746"]}
A number sign (#) is used with this entry because of evidence that autosomal dominant osteopetrosis-1 (OPTA1) is caused by heterozygous mutation in the LRP5 gene (603506) on chromosome 11q13. Description The osteopetroses are a heterogeneous group of genetic disorders characterized by increased bone density due to ...
OSTEOPETROSIS, AUTOSOMAL DOMINANT 1
c1843330
2,321
omim
https://www.omim.org/entry/607634
2019-09-22T16:08:56
{"doid": ["0110937"], "mesh": ["C536056"], "omim": ["607634"], "orphanet": ["2783"], "synonyms": ["Alternative titles", "OSTEOPETROSIS, AUTOSOMAL DOMINANT, TYPE I"]}
Factor V Leiden thrombophilia SpecialtyHematology Factor V Leiden (rs6025 or F5 p.R506Q[1]) is a variant (mutated form) of human factor V (one of several substances that helps blood clot), which causes an increase in blood clotting (hypercoagulability). Due to this mutation, protein C, an anticoagulant protein...
Factor V Leiden
c0584960
2,322
wikipedia
https://en.wikipedia.org/wiki/Factor_V_Leiden
2021-01-18T18:28:22
{"gard": ["6403"], "mesh": ["C095381"], "icd-9": ["289.81"], "icd-10": ["D68.5"], "wikidata": ["Q185986"]}
Type III hypersensitivity Immune complex SpecialtyImmunology Type III hypersensitivity occurs when there is accumulation of immune complexes (antigen-antibody complexes) that have not been adequately cleared by innate immune cells, giving rise to an inflammatory response and attraction of leukocytes. Suc...
Type III hypersensitivity
c0020951
2,323
wikipedia
https://en.wikipedia.org/wiki/Type_III_hypersensitivity
2021-01-18T18:33:58
{"mesh": ["D007105"], "umls": ["C0020951"], "wikidata": ["Q5898315"]}
Bloom syndrome is an inherited disorder characterized by short stature, a skin rash that develops after exposure to the sun, and a greatly increased risk of cancer. People with Bloom syndrome are usually smaller than 97 percent of the population in both height and weight from birth, and they rarely exceed 5 feet...
Bloom syndrome
c0005859
2,324
medlineplus
https://medlineplus.gov/genetics/condition/bloom-syndrome/
2021-01-27T08:25:18
{"gard": ["915"], "mesh": ["D001816"], "omim": ["210900"], "synonyms": []}
For a discussion of genetic heterogeneity of nonsyndromic hypotrichosis, see 605389. Clinical Features Naz et al. (2010) reported a 4-generation Pakistani family in which 4 individuals, 2 males and 2 females, had hypotrichosis. All 4 individuals had brown, thin, sparse hair on scalp, arms, and legs. Eyebrows and ey...
HYPOTRICHOSIS 9
c1854310
2,325
omim
https://www.omim.org/entry/614237
2019-09-22T15:55:57
{"doid": ["0110706"], "mesh": ["C537160"], "omim": ["614237"], "orphanet": ["55654"]}
A severe form of oculocutaneous albinism type 1 (OCA1) characterized by complete absence of melanin and manifesting as white hair and skin, blue, fully translucent irises, nystagmus and misrouting of the optic nerves. ## Epidemiology The worldwide prevalence of OCA1 is estimated at 1/40,000. OCA type 1A (OCA1A) is ...
Oculocutaneous albinism type 1A
c0268494
2,326
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=79431
2021-01-23T18:28:01
{"mesh": ["C537728"], "omim": ["203100"], "icd-10": ["E70.3"], "synonyms": ["OCA1A", "Tyrosinase-negative oculocutaneous albinism"]}
Abortion in Andorra is banned except in cases where it is necessary to save the life of a pregnant woman.[1][2] In Andorra, a woman who performs an abortion on herself or gives consent to another person to perform an abortion is subject to up two and a half years imprisonment. A person who performs an abortion with ...
Abortion in Andorra
None
2,327
wikipedia
https://en.wikipedia.org/wiki/Abortion_in_Andorra
2021-01-18T18:37:52
{"wikidata": ["Q4668439"]}
Not to be confused with Biceps femoris tendon rupture. Distal biceps tendon rupture, with proximal retraction of the muscle. Panoramic ultrasonography of a proximal biceps tendon rupture. Top image shows the contralateral normal side, and lower image shows a retracted muscle, with a hematoma filling out the pro...
Biceps tendon rupture
c0347952
2,328
wikipedia
https://en.wikipedia.org/wiki/Biceps_tendon_rupture
2021-01-18T18:53:51
{"wikidata": ["Q879257"]}
Not to be confused with Dupuytren fracture. Disease with gradual bending of the fingers due to scar tissue build-up within the palms Dupuytren's contracture Other namesDupuytren's disease, Morbus Dupuytren, Viking disease, and Celtic hand,[1] contraction of palmar fascia, palmar fascial fibromatosis, palmar fibro...
Dupuytren's contracture
c0013312
2,329
wikipedia
https://en.wikipedia.org/wiki/Dupuytren%27s_contracture
2021-01-18T18:53:57
{"mesh": ["D004387"], "umls": ["C0013312"], "icd-9": ["728.6"], "icd-10": ["M72.0"], "wikidata": ["Q1330254"]}
Colour anomaly, sometimes referred to as partial colour blindness, is an inherited condition in which people have full trichromatic colour vision, but do not make the same colour matches as the majority of the human population. It is much more common than dichromacy or other forms of colour blindness, affecting a...
Color anomaly
c4023316
2,330
wikipedia
https://en.wikipedia.org/wiki/Color_anomaly
2021-01-18T18:42:14
{"umls": ["C4023316"], "wikidata": ["Q26739327"]}
Actinic cheilitis Other namesAtinic cheilosis,[1] Atinic keratosis of lip,[2] Solar cheilosis[2],Sailor's lip,[3] Farmer's lip[4] Picture of Actinic Cheilitis. This is also known as sailor's lip or farmer's lip. SpecialtyDermatology Actinic cheilitis is cheilitis (lip inflammation) caused by long term ...
Actinic cheilitis
c0267026
2,331
wikipedia
https://en.wikipedia.org/wiki/Actinic_cheilitis
2021-01-18T18:30:06
{"gard": ["9619"], "mesh": ["C535669"], "icd-9": ["692.74", "692.82", "692.72"], "icd-10": ["L56.8"], "wikidata": ["Q2514487"]}
Bobble-head doll syndrome SpecialtyNeurology Bobble-head doll syndrome is a rare neurological movement disorder in which patients, usually children around age 3, begin to bob their head and shoulders forward and back, or sometimes side-to-side, involuntarily, in a manner reminiscent of a bobblehead doll. T...
Bobble-head doll syndrome
c2931137
2,332
wikipedia
https://en.wikipedia.org/wiki/Bobble-head_doll_syndrome
2021-01-18T18:28:35
{"gard": ["9731"], "mesh": ["C536241"], "umls": ["C2931137"], "icd-9": ["348"], "icd-10": ["G93.0"], "wikidata": ["Q1451507"]}
A rare, indolent primary cutaneous B-cell lymphoma characterized by a solitary or grouped erythematous plaques or tumors, preferentially located on the head, neck or trunk region, and composed of centroblasts and centrocytes arranged in a follicular, diffuse, or mixed growth pattern. The lesions are smooth and ty...
Primary cutaneous follicle center lymphoma
c1333171
2,333
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=178540
2021-01-23T17:20:11
{"umls": ["C1333171", "C1631066"], "icd-10": ["C82.6"], "synonyms": ["PCFCL"]}
For general learning disability, see Intellectual disability. Range of neurodevelopmental conditions "Slow learner" redirects here. For the collection of short stories by Thomas Pynchon, see Slow Learner. Learning disability Other namesLearning difficulties,[1][2] Developmental academic disorder,[3][4] Nonverbal...
Learning disability
c0751265
2,334
wikipedia
https://en.wikipedia.org/wiki/Learning_disability
2021-01-18T18:48:33
{"mesh": ["D007859"], "umls": ["C0751265", "C0023186"], "icd-9": ["315.0", "315.3"], "wikidata": ["Q860740"]}
A number sign (#) is used with this entry because of evidence that autosomal recessive primary microcephaly-24 (MCPH24) is caused by homozygous mutation in the NUP37 gene (609264) on chromosome 12q23. One such family has been reported. For a general phenotypic description and a discussion of genetic heterogeneit...
MICROCEPHALY 24, PRIMARY, AUTOSOMAL RECESSIVE
None
2,335
omim
https://www.omim.org/entry/618179
2019-09-22T15:43:16
{"omim": ["618179"], "orphanet": ["93213"], "synonyms": ["Familial idiopathic steroid-resistant nephrotic syndrome with focal segmental glomerulosclerosis"]}
Gaucher disease (GD) is a lysosomal storage disorder encompassing three main forms (types 1, 2 and 3), a fetal form and a variant with cardiac involvement (Gaucher disease - ophthalmoplegia - cardiovascular calcification or Gaucher-like disease). ## Epidemiology The prevalence is approximately 1/100,000. The annual...
Gaucher disease
c0017205
2,336
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=355
2021-01-23T18:58:58
{"gard": ["8233"], "mesh": ["D005776"], "omim": ["230800", "230900", "231000", "231005", "608013", "610539"], "umls": ["C0017205"], "icd-10": ["E75.2"], "synonyms": ["Acid beta-glucosidase deficiency", "Glucocerebrosidase deficiency"]}
Blue diaper syndrome Other namesOther Names: Hypercalcemia, familial, with nephrocalcinosis and indicanuria Blue diaper syndrome has an autosomal recessive pattern of inheritance. Medicationnone Blue diaper syndrome is a rare, autosomal recessive metabolic disorder characterized in infants by bluish urine-...
Blue diaper syndrome
c0268478
2,337
wikipedia
https://en.wikipedia.org/wiki/Blue_diaper_syndrome
2021-01-18T18:34:59
{"gard": ["5939"], "mesh": ["C536239"], "umls": ["C0268478"], "orphanet": ["94086"], "wikidata": ["Q503458"]}
Speech delay, also known as alalia, refers to a delay in the development or use of the mechanisms that produce speech.[1] Speech – as distinct from language – is the actual process of making sounds, using such organs and structures as the lungs, vocal cords, mouth, tongue, teeth, etc. Language delay refers to a delay...
Speech delay
c0241210
2,338
wikipedia
https://en.wikipedia.org/wiki/Speech_delay
2021-01-18T18:53:29
{"mesh": ["D007805"], "umls": ["C0241210"], "wikidata": ["Q2301465"]}
B-cell lymphoma Micrograph showing a large B cell lymphoma. Field stain. SpecialtyHematology, oncology The B-cell lymphomas are types of lymphoma affecting B cells. Lymphomas are "blood cancers" in the lymph nodes. They develop more frequently in older adults and in immunocompromised individuals. B-cell...
B-cell lymphoma
c0079731
2,339
wikipedia
https://en.wikipedia.org/wiki/B-cell_lymphoma
2021-01-18T18:37:09
{"gard": ["5877"], "mesh": ["D016393"], "umls": ["C1332362"], "icd-10": ["C85.1"], "wikidata": ["Q4833719"]}
Pes anserine bursitis Pes anserine is on the lower right side of image (Pes anserine bursa lies beneath) SpecialtyOrthopedic Pes anserine bursitis is an inflammatory condition of the medial (inner) knee at the anserine bursa, a sub muscular bursa, just below the pes anserinus. ## Contents * 1 Patholo...
Pes anserine bursitis
None
2,340
wikipedia
https://en.wikipedia.org/wiki/Pes_anserine_bursitis
2021-01-18T18:32:28
{"umls": ["CL1378544"], "wikidata": ["Q7171312"]}
Oguchi disease is an autosomal recessive retinal disorder characterized by congenital stationary night blindness (see this term) and the Mizuo-Nakamura phenomenon. ## Epidemiology Oguchi disease is a very rare condition with approximately 50 cases described in the literature to date. It was originally discovere...
Oguchi disease
c1306122
2,341
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=75382
2021-01-23T18:17:31
{"gard": ["10118"], "mesh": ["C537743"], "omim": ["258100", "613411"], "umls": ["C1306122"], "icd-10": ["H53.6"], "synonyms": ["Congenital stationary night blindness, Oguchi type", "Oguchi syndrome"]}
Inflammation of the paranasal sinuses due to fungal infection Fungal sinusitis Aspergillus is responsible in 90% of cases of fungal sinusitis SpecialtyPulmonology SymptomsFacial pain[1] TypesInvasive, Non-invasive[1] Diagnostic methodCT scan, MRI[1] TreatmentSurgical(Management depends on which type)[1] ...
Fungal sinusitis
c1142077
2,342
wikipedia
https://en.wikipedia.org/wiki/Fungal_sinusitis
2021-01-18T18:47:27
{"umls": ["C1142077"], "icd-10": ["J32.9"], "wikidata": ["Q23808336"]}
Most common form of cutaneous mastocytosis Urticaria pigmentosa Other namesGeneralized eruption of cutaneous mastocytosis (childhood type) The back of a child with urticaria pigmentosa SpecialtyMedical genetics Urticaria pigmentosa (also known as generalized eruption of cutaneous mastocytosis (childho...
Urticaria pigmentosa
c0042111
2,343
wikipedia
https://en.wikipedia.org/wiki/Urticaria_pigmentosa
2021-01-18T18:47:07
{"gard": ["12093"], "mesh": ["D014582"], "umls": ["C0042111"], "icd-9": ["757.33"], "orphanet": ["79457"], "wikidata": ["Q3886247"]}
A very rare genetic multisystemic disorder characterized by pituitary dysfunction, ataxia, peripheral neuropathy, spastic paraplegia, and chorioretinal dystrophy. *[v]: View this template *[t]: Discuss this template *[e]: Edit this template *[c.]: circa *[AA]: Adrenergic agonist *[AD]: Acetaldehyde d...
Laurence-Moon syndrome
c0023138
2,344
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=2377
2021-01-23T18:15:10
{"gard": ["12635"], "mesh": ["D007849"], "omim": ["245800"], "umls": ["C0023138"], "icd-10": ["Q87.8"]}
Intestinal pseudo-obstruction is a digestive disorder in which the intestinal walls are unable to contract normally (called hypomotility); the condition resembles a true obstruction, but no actual blockage exists. Signs and symptoms may include abdominal pain; vomiting; diarrhea; constipation; malabsorption of nutrie...
Intestinal pseudo-obstruction
c1864996
2,345
gard
https://rarediseases.info.nih.gov/diseases/6789/intestinal-pseudo-obstruction
2021-01-18T17:59:45
{"mesh": ["C566502"], "omim": ["609629"], "synonyms": ["Intestinal pseudoobstruction", "Hollow visceral myopathy"]}
Tumor necrosis factor receptor-associated periodic syndrome (commonly known as TRAPS) is a condition characterized by recurrent episodes of fever. These fevers typically last about 3 weeks but can last from a few days to a few months. The frequency of the episodes varies greatly among affected individuals; fevers can...
Tumor necrosis factor receptor-associated periodic syndrome
c1275126
2,346
medlineplus
https://medlineplus.gov/genetics/condition/tumor-necrosis-factor-receptor-associated-periodic-syndrome/
2021-01-27T08:25:18
{"gard": ["8457"], "mesh": ["C536657"], "omim": ["142680"], "synonyms": []}
Hereditary angioedema (HAE) is a genetic disease characterized by the occurrence of transitory and recurrent subcutaneous and/or submucosal edemas resulting in swelling and/or abdominal pain. ## Epidemiology Prevalence has been estimated at 1/100,000. ## Clinical description Onset may occur at any age but is most...
Hereditary angioedema
c0019243
2,347
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=91378
2021-01-23T19:04:33
{"gard": ["5979"], "mesh": ["D054179"], "omim": ["106100", "610618"], "umls": ["C0019243"], "icd-10": ["D84.1"], "synonyms": ["Familial angioneurotic edema", "HAE", "Hereditary angioneurotic edema", "Hereditary bradykinine-induced angioedema", "Hereditary non histamine-induced angioedema"]}
A number sign (#) is used with this entry because of evidence that Leber congenital amaurosis-19 (LCA19) is caused by homozygous mutation in the USP45 gene (618439) on chromosome 6q16. One such patient has been reported. Description Leber congenital amaurosis-19 (LCA19) is characterized by reduced vision in early c...
LEBER CONGENITAL AMAUROSIS 19
None
2,348
omim
https://www.omim.org/entry/618513
2019-09-22T15:41:36
{"omim": ["618513"]}
Stimmler et al. (1970) described 2 sisters born in 1963 and 1964 with microcephaly at birth, low birth weight, severe mental retardation and dwarfism, small teeth, and diabetes mellitus. Excessive quantities of alanine were found in the urine. Alanine, pyruvate, and lactate were elevated in the blood. Pyruvate was th...
ALANINURIA WITH MICROCEPHALY, DWARFISM, ENAMEL HYPOPLASIA, AND DIABETES MELLITUS
c1859965
2,349
omim
https://www.omim.org/entry/202900
2019-09-22T16:31:22
{"mesh": ["C565968"], "omim": ["202900"], "orphanet": ["3199"], "synonyms": ["Alternative titles", "STIMMLER SYNDROME"]}
Lacquer dermatitis SpecialtyDermatology Lacquer dermatitis (also known as "Lacquer sensitivity") is a cutaneous condition characterized by a contact dermatitis to various lacquers.[1] ## See also[edit] * Toxicodendron dermatitis * List of cutaneous conditions ## References[edit] 1. ^ Rapini, Ronald P...
Lacquer dermatitis
None
2,350
wikipedia
https://en.wikipedia.org/wiki/Lacquer_dermatitis
2021-01-18T18:57:12
{"wikidata": ["Q6468835"]}
A number sign (#) is used with this entry because Smith-McCort dysplasia-1 (SMC1) is caused by homozygous or compound heterozygous mutation in the DYM gene (607461) on chromosome 18q21. Mutations in the same gene cause Dyggve-Melchior-Clausen disease (DMC; 223800), which is radiologically identical but has the a...
SMITH-MCCORT DYSPLASIA 1
c1846431
2,351
omim
https://www.omim.org/entry/607326
2019-09-22T16:09:25
{"doid": ["0060247"], "mesh": ["C564589"], "omim": ["607326"], "orphanet": ["178355"], "synonyms": ["Alternative titles", "SMC"]}
Buschke–Ollendorff sign Other namesDermatofibrosis lenticularis disseminata[1] Buschke–Ollendorff syndrome has an autosomal dominant pattern of inheritance. SymptomsOsteopoikilosis, bone pain[2] CausesMutations in the LEMD3 gene.[2] Diagnostic methodX-ray, ultrasound[3] TreatmentSurgery for hearing loss(o...
Buschke–Ollendorff syndrome
c0265514
2,352
wikipedia
https://en.wikipedia.org/wiki/Buschke%E2%80%93Ollendorff_syndrome
2021-01-18T18:37:30
{"gard": ["1044"], "mesh": ["C537415"], "orphanet": ["1306"], "wikidata": ["Q5001316"]}
A rare, syndromic intellectual disability characterized by hypotonia, developmetal delay, absent or severly delayed speech development, intellectual disability, obstructive sleep apnea, mild dysmorphic facial features and behavioral abnormalities. Epilepsy, ataxia and nystagmus have also been reported. *[v]: V...
AHDC1-related intellectual disability-obstructive sleep apnea-mild dysmorphism syndrome
c4014419
2,353
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=412069
2021-01-23T18:04:51
{"omim": ["615829"], "icd-10": ["Q87.8"], "synonyms": ["Xia-Gibbs syndrome"]}
For a clinical description of atopic dermatitis (ATOD) and an overview of linkage studies, see ATOD1 (603165). Mapping Using a nonparametric affected relative-pair method in 109 atopic dermatitis pedigrees, Bradley et al. (2002) conducted a genomewide linkage analysis with 367 microsatellite markers and found linka...
DERMATITIS, ATOPIC, 9
c3150764
2,354
omim
https://www.omim.org/entry/613519
2019-09-22T15:58:29
{"omim": ["613519"]}
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: "Pascual-Castroviejo syndrome type 1" – news · newspapers · books · scholar · JSTOR (April 2018) (Learn how and when to ...
Pascual-Castroviejo syndrome type 1
c1859252
2,355
wikipedia
https://en.wikipedia.org/wiki/Pascual-Castroviejo_syndrome_type_1
2021-01-18T18:32:45
{"gard": ["1210"], "mesh": ["C565862"], "umls": ["C1859252"], "orphanet": ["1394"], "wikidata": ["Q55629168"]}
Autosomal dominant cerebellar ataxia, deafness, and narcolepsy (ADCADN) is a nervous system disorder with signs and symptoms that usually begin in mid-adulthood and gradually get worse. People with ADCADN have difficulty coordinating movements (ataxia) and mild to moderate hearing loss caused by abnormalities of...
Autosomal dominant cerebellar ataxia, deafness, and narcolepsy
c3807295
2,356
medlineplus
https://medlineplus.gov/genetics/condition/autosomal-dominant-cerebellar-ataxia-deafness-and-narcolepsy/
2021-01-27T08:24:53
{"gard": ["12372"], "omim": ["604121"], "synonyms": []}
ACAD9 deficiency is a condition that varies in severity and can cause muscle weakness (myopathy), heart problems, and intellectual disability. Nearly all affected individuals have a buildup of a chemical called lactic acid in the body (lactic acidosis). Additional signs and symptoms that affect other body systems occ...
ACAD9 deficiency
c1970173
2,357
medlineplus
https://medlineplus.gov/genetics/condition/acad9-deficiency/
2021-01-27T08:25:28
{"gard": ["3908"], "mesh": ["C567006"], "omim": ["611126"], "synonyms": []}
A rare systemic or rheumatologic disease characterized by peripheral osteolysis (especially carpal and tarsal bones), interphalangeal joint erosions, subcutaneous fibrocollagenous nodules, facial dysmorphism, and a wide range of associated manifestations. ## Epidemiology Multicentric osteolysis-nodulosis-arthropath...
Multicentric osteolysis-nodulosis-arthropathy spectrum
c1850155
2,358
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=371428
2021-01-23T18:29:49
{"mesh": ["C536051"], "omim": ["259600", "277950"], "icd-10": ["M89.5"], "synonyms": ["MONA spectrum", "NAO syndrome", "Nodulosis-arthropathy-osteolysis syndrome", "Torg-Winchester syndrome"]}
17q12 duplication is a chromosomal change in which a small piece of chromosome 17 is copied (duplicated) abnormally in each cell. The duplication occurs on the long (q) arm of the chromosome at a position designated q12. Signs and symptoms related to 17q12 duplications vary significantly, even among members of the s...
17q12 duplication
c3281137
2,359
medlineplus
https://medlineplus.gov/genetics/condition/17q12-duplication/
2021-01-27T08:25:41
{"omim": ["614526"], "synonyms": []}
Injury caused by video games This article includes a list of general references, but it remains largely unverified because it lacks sufficient corresponding inline citations. Please help to improve this article by introducing more precise citations. (August 2018) (Learn how and when to remove this template message) ...
Nintendo thumb
None
2,360
wikipedia
https://en.wikipedia.org/wiki/Nintendo_thumb
2021-01-18T19:01:56
{"wikidata": ["Q7039177"]}
Alagille syndrome is a genetic syndrome that can affect the liver and other parts of the body. The liver problems result from having fewer small bile ducts than normal in the liver. This leads to bile building-up inside the liver, which in turn causes liver scarring and damage. Signs and symptoms of Alagille syndrome...
Alagille syndrome
c0085280
2,361
gard
https://rarediseases.info.nih.gov/diseases/804/alagille-syndrome
2021-01-18T18:02:13
{"mesh": ["D016738"], "omim": ["118450"], "orphanet": ["52"], "synonyms": ["Hepatic ductular hypoplasia", "Watson Alagille syndrome", "Alagille-Watson syndrome", "Cholestasis with peripheral pulmonary stenosis", "Arteriohepatic dysplasia", "Paucity of interlobular bile ducts", "Cardiovertebral syndrome", "Watson-Miller...
A rare soft tissue tumor characterized by a solitary mass-forming fibrous proliferation that usually occurs in the subcutaneous tissue, composed of uniform fibroblastic/myofibroblastic cells displaying a loose growth pattern. Upper extremities, trunk, and head and neck are most frequently affected. The lesion typical...
Nodular fasciitis
c0410005
2,362
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=477742
2021-01-23T17:52:28
{"synonyms": ["Pseudosarcomatous fasciitis", "Pseudosarcomatous fibromatosis"]}
Binswanger's disease Other namesSubcortical arteriosclerotic encephalopathy SpecialtyNeurology Binswanger's disease, also known as subcortical leukoencephalopathy and subcortical arteriosclerotic encephalopathy (SAE),[1] is a form of small vessel vascular dementia caused by damage to the white brain matter.[...
Binswanger's disease
c0270786
2,363
wikipedia
https://en.wikipedia.org/wiki/Binswanger%27s_disease
2021-01-18T18:43:20
{"gard": ["5925"], "mesh": ["D015140"], "icd-9": ["290.12"], "icd-10": ["I67.3"], "wikidata": ["Q1399293"]}
A severe form of lissencephaly with cerebellar hypoplasia, characterized by a microcephaly of at least - 3 SD and a thick cortex associated with complete absence of the corpus callosum. *[v]: View this template *[t]: Discuss this template *[e]: Edit this template *[c.]: circa *[AA]: Adrenergic agonist ...
Lissencephaly with cerebellar hypoplasia type F
c4274989
2,364
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=100016
2021-01-23T17:36:48
{"icd-10": ["Q04.3"]}
This article includes a list of general references, but it remains largely unverified because it lacks sufficient corresponding inline citations. Please help to improve this article by introducing more precise citations. (December 2014) (Learn how and when to remove this template message) Medical fetishism refer...
Medical fetishism
None
2,365
wikipedia
https://en.wikipedia.org/wiki/Medical_fetishism
2021-01-18T18:29:57
{"wikidata": ["Q1413869"]}
This article is about oral exposure to zinc. For inhalation toxicity, see Metal fume fever. Zinc toxicity Zinc SpecialtyEmergency medicine Zinc toxicity is a medical condition involving an overdose on, or toxic overexposure to, zinc. Such toxicity levels have been seen to occur at ingestion of greater t...
Zinc toxicity
None
2,366
wikipedia
https://en.wikipedia.org/wiki/Zinc_toxicity
2021-01-18T19:05:39
{"icd-9": ["985.8"], "icd-10": ["T56.5"], "wikidata": ["Q10724674"]}
Fibrous dysplasia is a skeletal disorder that is characterized by the replacement of normal bone with fibrous bone tissue. It may involve one bone (monostotic) or multiple bones (polyostotic). Fibrous dysplasia can affect any bone in the body. The most common sites are the bones in the skull and face, the long bo...
Fibrous dysplasia
c0259779
2,367
gard
https://rarediseases.info.nih.gov/diseases/6444/fibrous-dysplasia
2021-01-18T18:00:29
{"umls": ["C0259779"], "orphanet": ["249"], "synonyms": ["Fibrous dysplasia of bone"]}
An X-linked intellectual disability syndrome with neuromuscular involvement characterized by infantile hypotonia, muscular hypoplasia, spastic paraparesis with dystonic/athetoic movements, and severe cognitive deficiency. ## Epidemiology At least 132 families with 320 affected individuals have been reported in the ...
Allan-Herndon-Dudley syndrome
c0795889
2,368
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=59
2021-01-23T18:05:21
{"gard": ["5617"], "mesh": ["C537047"], "omim": ["300523"], "umls": ["C0795889"], "icd-10": ["G31.8"], "synonyms": ["AHDS", "MCT8 deficiency", "Monocarboxylate transporter 8 deficiency", "X-linked intellectual disability-hypotonia syndrome"]}
Group of disorders Epstein–Barr virus-associated lymphoproliferative diseases Other namesEBV-associated lymphoproliferative diseases SpecialtyHematology, oncology, Infectious disease, virology CausesEpstein–Barr virus Epstein–Barr virus-associated lymphoproliferative diseases (also termed EBV-associated l...
Epstein–Barr virus-associated lymphoproliferative diseases
None
2,369
wikipedia
https://en.wikipedia.org/wiki/Epstein%E2%80%93Barr_virus-associated_lymphoproliferative_diseases
2021-01-18T18:33:57
{"wikidata": ["Q60791668"]}
Uveal melanoma (155720) is the most common primary intraocular malignancy. Monosomy 3, which is an unusual finding in most tumors, is present in approximately 50% of uveal melanomas and is significantly correlated with metastatic disease. To obtain positional information on putative tumor suppressor genes on chromoso...
MELANOMA, UVEAL, SUSCEPTIBILITY TO, 1
c0346388
2,370
omim
https://www.omim.org/entry/606660
2019-09-22T16:10:14
{"doid": ["6039"], "omim": ["606660"], "orphanet": ["39044"], "synonyms": ["Alternative titles", "UVM1"]}
Pelvis justo major (also called "Giant Pelvis") is a rare condition of the adult female pelvis where the pelvis flairs above the Iliopectineal line.[1] It is 1.5 or more times larger than an average pelvis in every direction and is at least 42 cm (16.5 inches) biiliac width. Even though this condition is classified a...
Pelvis justo major
c0265721
2,371
wikipedia
https://en.wikipedia.org/wiki/Pelvis_justo_major
2021-01-18T19:03:11
{"umls": ["C0265721"], "icd-10": ["Q74.2"], "wikidata": ["Q7161813"]}
A rare non-syndromic syndactyly characterized by mesoaxial reduction of fingers, complete syndactyly of the 3rd and 4th fingers with synostoses of the corresponding metacarpals and associated single phalanges, malformed thumbs, and hypoplasia and clinodactyly of the 5th finger. Preaxial webbing of toes with terminal ...
Mesoaxial synostotic syndactyly with phalangeal reduction
c1836206
2,372
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=157801
2021-01-23T17:39:47
{"gard": ["10590"], "mesh": ["C563721"], "omim": ["609432"], "umls": ["C1836206"], "icd-10": ["Q70.0", "Q70.2"], "synonyms": ["MSSD", "Syndactyly type 9", "Syndactyly, Malik-Percin type"]}
Yellow nail syndrome Other namesprimary lymphedema Yellow nail syndrome: This patient has a 20-year history of severe lymphedema of her legs; thick, ridged, yellowish, hypercurved thumbnails (top right); similarly affected, yellow-green to brown toenails (bottom right); and bilateral, chylous pleural effusions. A...
Yellow nail syndrome
c0221348
2,373
wikipedia
https://en.wikipedia.org/wiki/Yellow_nail_syndrome
2021-01-18T19:05:13
{"gard": ["184"], "mesh": ["D056684"], "umls": ["C0221348"], "orphanet": ["662"], "wikidata": ["Q1786851"]}
Lymphatic disease SpecialtyLymphologist Lymphatic disease is a class of disorders which directly affect the components of the lymphatic system. Examples include Castleman's disease[1] and lymphedema.[2] ## Contents * 1 Types * 2 References * 3 External links ## Types[edit] Diseases and disorder ...
Lymphatic disease
c0024228
2,374
wikipedia
https://en.wikipedia.org/wiki/Lymphatic_disease
2021-01-18T18:41:02
{"mesh": ["D008206"], "umls": ["C0024228"], "wikidata": ["Q6708237"]}
Not to be confused with Chondroma. Chordoma MRI of extensive clival chordoma in 17-year-old male patient, axial view. Tumor in the nasopharynx extending from nasal cavity to brainstem posteriorly is clearly visible. SpecialtyOncology Chordoma is a rare slow-growing neoplasm thought to arise from cellula...
Chordoma
c0008487
2,375
wikipedia
https://en.wikipedia.org/wiki/Chordoma
2021-01-18T18:37:09
{"gard": ["1303"], "mesh": ["D002817"], "umls": ["C0008487"], "orphanet": ["178"], "wikidata": ["Q1076389"]}
A number sign (#) is used with this entry because of evidence that Crouzon syndrome is caused by heterozygous mutation in the gene encoding fibroblast growth factor receptor-2 (FGFR2; 176943) on chromosome 10q26. See also Crouzon syndrome with acanthosis nigricans (CAN; 612247), a distinct disorder caused by a s...
CROUZON SYNDROME
c2931196
2,376
omim
https://www.omim.org/entry/123500
2019-09-22T16:42:43
{"doid": ["2339"], "mesh": ["D003394"], "omim": ["123500"], "orphanet": ["207"], "synonyms": ["Crouzon craniofacial dysostosis", "Alternative titles", "CRANIOFACIAL DYSOSTOSIS, TYPE I", "CROUZON CRANIOFACIAL DYSOSTOSIS"], "genereviews": ["NBK1455"]}
A number sign (#) is used with this entry because of evidence that the Simpson-Golabi-Behmel syndrome type 1 (SGBS1) is caused by mutation in the gene encoding glypican-3 (GPC3; 300037) on chromosome Xq26. Some evidence suggests that disruption of the GPC4 gene (300168), which is adjacent to the GPC3 gene, may also ...
SIMPSON-GOLABI-BEHMEL SYNDROME, TYPE 1
c0796154
2,377
omim
https://www.omim.org/entry/312870
2019-09-22T16:17:16
{"doid": ["0060248"], "mesh": ["C537340"], "omim": ["312870"], "orphanet": ["373"], "synonyms": ["Alternative titles", "SGBS", "BULLDOG SYNDROME", "DYSPLASIA GIGANTISM SYNDROME, X-LINKED", "GOLABI-ROSEN SYNDROME", "SIMPSON DYSMORPHIA SYNDROME"], "genereviews": ["NBK1219", "NBK1294"]}
A number sign (#) is used with this entry because this form of platelet-type bleeding disorder (BDPLT11) can be caused by compound heterozygous mutation in the GP6 gene (605546) on chromosome 19q13. Description Platelet-type bleeding disorder-11 is an autosomal recessive mild to moderate bleeding disorder caused by...
BLEEDING DISORDER, PLATELET-TYPE, 11
c3280120
2,378
omim
https://www.omim.org/entry/614201
2019-09-22T15:56:11
{"doid": ["0111057"], "omim": ["614201"], "orphanet": ["98885", "73271"], "synonyms": ["Alternative titles", "GP VI DEFICIENCY", "GLYCOPROTEIN VI DEFICIENCY"]}
3q29 microdeletion syndrome Other names3qter deletion, Monosomy 3q29 Chromosome 3 is associated with this condition 3q29 microdeletion syndrome is a rare genetic disorder resulting from the deletion of a segment of chromosome 3. This syndrome was first described in 2005.[1][2] ## Contents * 1 Presentatio...
3q29 microdeletion syndrome
c2674949
2,379
wikipedia
https://en.wikipedia.org/wiki/3q29_microdeletion_syndrome
2021-01-18T18:51:46
{"gard": ["11974"], "mesh": ["C567184"], "umls": ["C2674949"], "orphanet": ["65286"], "wikidata": ["Q4636618"]}
T-B- severe combined immunodeficiency (SCID; see this term) is a group of rare monogenic primary immunodeficiency disorders characterized by a lack of functional peripheral T and B lymphocytes, resulting in recurrent early-onset severe respiratory viral, bacterial or fungal infections, diarrhea and failure to thr...
T-B- severe combined immunodeficiency
None
2,380
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=317419
2021-01-23T17:54:36
{"icd-10": ["D81.1"], "synonyms": ["T-B- SCID"]}
A number sign (#) is used with this entry because congenital dyserythropoietic anemia type IV (CDAN4) is caused by heterozygous mutation in the KLF1 gene (600599), which encodes a transcriptional activator, on chromosome 19p13. Description Congenital dyserythropoietic anemia type IV is an autosomal dominant inherit...
ANEMIA, CONGENITAL DYSERYTHROPOIETIC, TYPE IV
c3150926
2,381
omim
https://www.omim.org/entry/613673
2019-09-22T15:58:02
{"doid": ["1338"], "omim": ["613673"], "orphanet": ["293825"], "synonyms": ["CDA type 4", "CDA due to KLF1 mutation", "CDA, TYPE IV", "CDA type IV", "Congenital dyserythropoietic anemia type 4", "CDA IV", "Alternative titles", "CDAN4", "Congenital dyserythropoietic anemia due to KLF1 mutation"]}
Pierquin et al. (1991) reported the cases of 2 unrelated children with similar clinical features, particularly facial dysmorphism and multiple joint dislocations, suggesting the diagnosis of Larsen syndrome (150250). Both carried an inherited unbalanced translocation resulting in partial trisomy 1q and partial monoso...
LARSEN-LIKE SYNDROME
c1837884
2,382
omim
https://www.omim.org/entry/608545
2019-09-22T16:07:40
{"mesh": ["C563914"], "omim": ["608545"], "orphanet": ["2370"], "synonyms": ["Alternative titles", "LRSL"]}
Dyschromatosis universalis hereditaria SpecialtyDermatology Dyschromatosis universalis hereditaria is a rare genodermatosis characterized by reticulate hyper- and hypo- pigmentated macules in a generalized distribution.[1]:856 Both autosomal dominant and recessive inheritance have been reported with the disor...
Dyschromatosis universalis hereditaria
c1306229
2,383
wikipedia
https://en.wikipedia.org/wiki/Dyschromatosis_universalis_hereditaria
2021-01-18T18:53:31
{"gard": ["1996"], "mesh": ["C535730"], "umls": ["C1306229"], "orphanet": ["241"], "wikidata": ["Q5319369"]}
Partial or complete wasting away of a part of the body For the American thrash metal band, see Atrophy (band). Atrophy Mouse (right) with spinal muscular atrophy SpecialtyPathology Atrophy is the partial or complete wasting away of a part of the body. Causes of atrophy include mutations (which can dest...
Atrophy
c0333641
2,384
wikipedia
https://en.wikipedia.org/wiki/Atrophy
2021-01-18T18:32:03
{"mesh": ["D001284"], "wikidata": ["Q194520"]}
Hemoglobin Constant Spring is a variant of hemoglobin in which a mutation in the alpha globin gene produces an alpha globin chain that is abnormally long. It is the most common nondeletional alpha-thalassemia mutation associated with hemoglobin H disease.[1] The quantity of hemoglobin in the cells is low because ...
Hemoglobin Constant Spring
c3891114
2,385
wikipedia
https://en.wikipedia.org/wiki/Hemoglobin_Constant_Spring
2021-01-18T18:59:43
{"wikidata": ["Q28209257"]}
## Summary ### Clinical characteristics. CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is characterized by mid-adult onset of recurrent ischemic stroke, cognitive decline progressing to dementia, a history of migraine with aura, mood disturbance, apathy, and di...
CADASIL
c0751587
2,386
gene_reviews
https://www.ncbi.nlm.nih.gov/books/NBK1500/
2021-01-18T21:38:49
{"mesh": ["D046589"], "synonyms": ["Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy"]}
Virus-associated trichodysplasia spinulosa is a rare infectious skin disease characterized by the development of follicular papules with keratin spicules in various parts of the body, predominantly in the face (e.g. nose, eyebrows, auricles), that is due to polyomavirus infection in immunocompromized patients. *[v...
Virus-associated trichodysplasia spinulosa
c3267126
2,387
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=228379
2021-01-23T17:22:26
{"umls": ["C3267126"], "synonyms": ["Cyclosporine-induced folliculodystrophy", "Pilomatrix dysplasia", "TS", "Trichodysplasia spinulosa", "VATS"]}
A number sign (#) is used with this entry because of evidence that inflammatory bowel disease-25 (IBD25) is caused by homozygous mutation in the IL10RB gene (123889) on chromosome 21q22. Another form of early-onset inflammatory bowel disease, IBD28 (613148), is caused by mutation in the IL10RA gene (146933) encoding...
INFLAMMATORY BOWEL DISEASE 25, AUTOSOMAL RECESSIVE
c2675508
2,388
omim
https://www.omim.org/entry/612567
2019-09-22T16:01:12
{"doid": ["0110909"], "mesh": ["C567251"], "omim": ["612567"], "orphanet": ["238569"], "synonyms": ["Alternative titles", "IL10-related early-onset inflammatory bowel disease", "IL10-related early-onset IBD", "INFLAMMATORY BOWEL DISEASE, EARLY-ONSET, AUTOSOMAL RECESSIVE"]}
Erythrokeratoderma variabilis progressiva (EKVP) is a type of erythrokeratoderma characterized by the association of hyperkeratosis and erythema in persistent, although sometimes variable, circumscribed lesions. Progressive symmetric erythrokeratoderma (PSEK) and erythrokeratoderma variabilis (EKV) are probably no lo...
Erythrokeratoderma variabilis progressiva
None
2,389
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=308166
2021-01-23T18:37:44
{"gard": ["10923"], "icd-10": ["Q82.8"]}
Odynophagia Pronunciation * /oʊˌdɪnəˈfeɪdʒ(i)ə/ SpecialtyGastroenterology Odynophagia is pain when swallowing.[1][2] The pain may be felt in the mouth or throat and can occur with or without difficulty swallowing.[3] The pain may be described as an ache, burning sensation, or occasionally a stabbing ...
Odynophagia
c0221150
2,390
wikipedia
https://en.wikipedia.org/wiki/Odynophagia
2021-01-18T18:56:36
{"icd-9": ["787.20"], "icd-10": ["R13"], "wikidata": ["Q2455868"]}
Autoimmune endocrine disease Graves' disease Other namesToxic diffuse goiter, Flajani–Basedow–Graves disease The classic finding of exophthalmos and lid retraction in Graves' disease SpecialtyEndocrinology SymptomsEnlarged thyroid, irritability, muscle weakness, sleeping problems, fast heartbeat, weigh...
Graves' disease
c0154138
2,391
wikipedia
https://en.wikipedia.org/wiki/Graves%27_disease
2021-01-18T18:59:04
{"gard": ["6549"], "umls": ["C0154138"], "wikidata": ["Q16483"]}
In a brother and sister born to nonconsanguineous parents, Davee et al. (1992) described renal hypoplasia, mullerian duct hypoplasia, and strikingly similar facial features. Facies consisted of frontal bossing, hypertelorism, strabismus, short nose, and mild micrognathia. Both sibs had severe growth and developme...
RENAL AND MULLERIAN DUCT HYPOPLASIA
c1849439
2,392
omim
https://www.omim.org/entry/266810
2019-09-22T16:22:47
{"mesh": ["C564853"], "omim": ["266810"]}
Spinal muscular atrophy with respiratory distress type 2 is a rare, genetic, motor neuron disease characterized by progressive early respiratory failure associated with diaphragm paralysis, distal muscular weakness, joint contractures, and axial hypotonia with preserved antigravity limb movements. Phenotype overlaps ...
Spinal muscular atrophy with respiratory distress type 2
None
2,393
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=404521
2021-01-23T16:52:33
{"icd-10": ["G12.2"], "synonyms": ["Diaphragmatic spinal muscular atrophy type 2", "SMARD2", "Severe infantile axonal neuropathy with respiratory failure type 2", "X-linked spinal muscular atrophy with respiratory distress"]}
Pitt-Hopkins-like syndrome is a rare, genetic, syndromic intellectual disability disorder characterized by severe intellectual disability, lack of speech with normal, or mildly delayed, motor development, episodic breathing abnormalities, early-onset seizures and facial dysmorphism which only includes a wide mouth. A...
Pitt-Hopkins-like syndrome
c2750246
2,394
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=221150
2021-01-23T17:07:26
{"gard": ["11967"], "mesh": ["C567657"], "omim": ["610042", "614325"]}
Vitreous hemorrhage Slit lamp photograph showing retinal detachment with visible vitreous hemorrhage. SpecialtyOphthalmology Vitreous hemorrhage is the extravasation, or leakage, of blood into the areas in and around the vitreous humor of the eye.[1] The vitreous humor is the clear gel that fills the space b...
Vitreous hemorrhage
c0042909
2,395
wikipedia
https://en.wikipedia.org/wiki/Vitreous_hemorrhage
2021-01-18T18:56:44
{"mesh": ["D014823"], "umls": ["C0042909"], "icd-9": ["379.23"], "icd-10": ["H43.1"], "wikidata": ["Q1529650"]}
A rare head and neck tumor characterized by a malignant epithelial neoplasm with evidence of squamous differentiation, most commonly located in the supraglottis or glottis. The tumor can spread directly to adjacent structures or metastasize via lymphatic and blood vessels to regional lymph nodes, or lung, liver, ...
Squamous cell carcinoma of the larynx
c1168401
2,396
orphanet
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=494550
2021-01-23T16:57:17
{"mesh": ["D000077195"], "omim": ["275355"]}
## Summary ### Clinical characteristics. The FLNB disorders include a spectrum of phenotypes ranging from mild to severe. At the mild end are spondylocarpotarsal synostosis (SCT) syndrome and Larsen syndrome; at the severe end are the phenotypic continuum of atelosteogenesis types I (AOI) and III (AOIII) and Piepko...
FLNB Disorders
None
2,397
gene_reviews
https://www.ncbi.nlm.nih.gov/books/NBK2534/
2021-01-18T21:27:24
{"synonyms": []}
Wandering spleen Other namesPelvic spleen, displaced spleen, drifting spleen, splenoptosis, floating spleen, splenic ptosis A picture of an enlarged spleen taken using medical ultrasonography. SpecialtyAngiology Wandering spleen (or pelvic spleen) is a rare medical disease caused by the loss or weakening o...
Wandering spleen
c0272414
2,398
wikipedia
https://en.wikipedia.org/wiki/Wandering_spleen
2021-01-18T18:31:05
{"gard": ["328"], "mesh": ["D050805"], "icd-9": ["289.59"], "wikidata": ["Q7967227"]}
A number sign (#) is used with this entry because of evidence that susceptibility to cutaneous malignant melanoma-6 (CMM6) is conferred by variation in the XRCC3 gene (600675) on chromosome 14q32. Description Malignant melanoma is a neoplasm of pigment-producing cells called melanocytes that occurs most often i...
MELANOMA, CUTANEOUS MALIGNANT, SUSCEPTIBILITY TO, 6
c2314896
2,399
omim
https://www.omim.org/entry/613972
2019-09-22T15:56:59
{"omim": ["613972"], "orphanet": ["618"]}