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320 results

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ConceptNode typeBase domainMatched membershipIdentifier
Glycogen Storage Disease Type VIII
An x-linked recessive hepatic glycogen storage disease resulting from lack of expression of phosphorylase-b-kinase activity. Symptoms are relatively …
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160060
Goldenhar Syndrome
Mandibulofacial dysostosis with congenital eyelid dermoids.
SyndromePathologyClinical Genetics [curated_secondary]AMW:DIS:050020
Gonadal Dysgenesis
A number of syndromes with defective gonadal developments such as streak GONADS and dysgenetic testes or ovaries. The spectrum of gonadal and sexual …
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:120074
Gonadal Dysgenesis, 46,XX
The 46,XX gonadal dysgenesis may be sporadic or familial. Familial XX gonadal dysgenesis is transmitted as an autosomal recessive trait and its locus…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:120065
Gonadal Dysgenesis, 46,XY
Defects in the SEX DETERMINATION PROCESS in 46, XY individuals that result in abnormal gonadal development and deficiencies in TESTOSTERONE and subse…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:120072
Gonadal Dysgenesis, Mixed
A type of defective gonadal development in patients with a wide spectrum of chromosomal mosaic variants. Their karyotypes are of partial sex chromoso…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:120075
Hajdu-Cheney Syndrome
Rare, autosomal dominant syndrome characterized by ACRO-OSTEOLYSIS, generalized OSTEOPOROSIS, and skull deformations.
SyndromePathologyClinical Genetics [curated_secondary]AMW:DIS:050005
Hand Deformities, Congenital
Alterations or deviations from normal shape or size which result in a disfigurement of the hand occurring at or before birth.
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050170
Hemimegalencephaly
Rare MALFORMATIONS OF CORTICAL DEVELOPMENT, GROUP I characterized by the enlargement of one side of the brain. It is associated with seizures, partia…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050316
Hemochromatosis
A disorder of iron metabolism characterized by a triad of HEMOSIDEROSIS; LIVER CIRRHOSIS; and DIABETES MELLITUS. It is caused by massive iron deposit…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160130
Hereditary Autoinflammatory Diseases
Hereditary inflammation conditions, characterized by recurrent episodes of systemic inflammation. Common symptoms include recurrent fever, rash, arth…
Inflammatory DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160063
Hereditary Complement Deficiency Diseases
Genetic disorders due to mutations in genes involved in COMPLEMENT SYSTEM PROTEINS. They are often classified into distinct pathway of complement act…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160139
Hernias, Diaphragmatic, Congenital
Protrusion of abdominal structures into the THORAX as a result of embryologic defects in the DIAPHRAGM often present in the neonatal period. It can b…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160031
Hip Dislocation, Congenital
Congenital dislocation of the hip generally includes subluxation of the femoral head, acetabular dysplasia, and complete dislocation of the femoral h…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050325
Holocarboxylase Synthetase Deficiency
The neonatal form of MULTIPLE CARBOXYLASE DEFICIENCY that is caused by a defect or deficiency in holocarboxylase synthetase. HLCS is the enzyme that …
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160078
Holoprosencephaly
Anterior midline brain, cranial, and facial malformations resulting from the failure of the embryonic prosencephalon to undergo segmentation and clea…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050312
Homozygous Familial Hypercholesterolemia
A rare inherited genetic disorder, one form of HYPERLIPOPROTEINEMIA TYPE II, characterized by high level of LOW-DENSITY LIPOPROTEIN (LDL) which if no…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160112
Hyaline Fibromatosis Syndrome
Autosomal recessive disorder characterized by HYALINE deposition in the skin, bone, gastrointestinal tract, muscles and glands; multiple subcutaneous…
SyndromePathologyClinical Genetics [curated_secondary]AMW:DIS:160147
Hyperandrogenism
A condition caused by the excessive secretion of ANDROGENS from the ADRENAL CORTEX; the OVARIES; or the TESTES. The clinical significance in males is…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:120066
Hyperbilirubinemia, Hereditary
Inborn errors of bilirubin metabolism resulting in excessive amounts of bilirubin in the circulating blood, either because of increased bilirubin pro…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160104
Hyperhomocysteinemia
Condition in which the plasma levels of homocysteine and related metabolites are elevated (>13.9 μmol/l). Hyperhomocysteinemia can be familial or acq…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160074
Hyperkeratosis, Epidermolytic
A form of congenital ichthyosis inherited as an autosomal dominant trait and characterized by ERYTHRODERMA and severe hyperkeratosis. It is manifeste…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160044
Hyperlipidemia, Familial Combined
A type of familial lipid metabolism disorder characterized by a variable pattern of elevated plasma CHOLESTEROL and/or TRIGLYCERIDES. Multiple genes …
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160109
Hyperlipoproteinemia Type I
An inherited condition due to a deficiency of either LIPOPROTEIN LIPASE or APOLIPOPROTEIN C-II (a lipase-activating protein). The lack of lipase acti…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160110
Hyperlipoproteinemia Type II
A group of familial disorders characterized by elevated circulating cholesterol contained in either LOW-DENSITY LIPOPROTEINS alone or also in VERY-LO…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160111
Hyperlipoproteinemia Type III
An autosomal recessively inherited disorder characterized by the accumulation of intermediate-density lipoprotein (IDL or broad-beta-lipoprotein). ID…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160113
Hyperlipoproteinemia Type IV
A hypertriglyceridemia disorder, often with autosomal dominant inheritance. It is characterized by the persistent elevations of plasma TRIGLYCERIDES,…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160114
Hyperlipoproteinemia Type V
A severe type of hyperlipidemia, sometimes familial, that is characterized by the elevation of both plasma CHYLOMICRONS and TRIGLYCERIDES contained i…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160115
Hyperostosis Frontalis Interna
Thickening of the inner table of the frontal bone, which may be associated with hypertrichosis and obesity. It most commonly affects women near menop…
DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050051
Hyperostosis, Cortical, Congenital
A disease of young infants characterized by soft tissue swellings over the affected bones, fever, and irritability, and marked by periods of remissio…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050050