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320 results

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ConceptNode typeBase domainMatched membershipIdentifier
LEOPARD Syndrome
An autosomal dominant disorder with an acronym of its seven features (LENTIGO; ELECTROCARDIOGRAM abnormalities; ocular HYPERTELORISM; PULMONARY STENO…
SyndromePathologyClinical Genetics [curated_secondary]AMW:DIS:050313
Leukocyte-Adhesion Deficiency Syndrome
Rare, autosomal recessive disorder caused by deficiency of the beta 2 integrin receptors (RECEPTORS, LEUKOCYTE-ADHESION) comprising the CD11/CD18 fam…
SyndromePathologyClinical Genetics [curated_secondary]AMW:DIS:160140
Leukokeratosis, Hereditary Mucosal
An autosomal dominant disorder that is manifested by thickened spongiform ORAL MUCOSA with a white opalescent tint. Other MUCOSAL TISSUE may also be …
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160152
Limb Deformities, Congenital
Congenital structural deformities of the upper and lower extremities collectively or unspecified.
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050327
Lingual Goiter
Pathological enlargement of the LINGUAL THYROID, ectopic thyroid tissue at the base of the TONGUE. It may cause upper AIRWAY OBSTRUCTION; DYSPHAGIA; …
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160055
Lingual Thyroid
A condition characterized by the presence of rudimentary THYROID tissue at the base of the TONGUE. It is due to failed embryonic development and migr…
Metabolic DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160054
Lipid Metabolism, Inborn Errors
Errors in the metabolism of LIPIDS resulting from inborn genetic MUTATIONS that are heritable.
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160108
Lipidoses
Conditions characterized by abnormal lipid deposition due to disturbance in lipid metabolism, such as hereditary diseases involving lysosomal enzymes…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160121
Lipodystrophy, Congenital Generalized
Congenital disorders, usually autosomal recessive, characterized by severe generalized lack of ADIPOSE TISSUE, extreme INSULIN RESISTANCE, and HYPERT…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160124
Lipodystrophy, Familial Partial
Inherited conditions characterized by the partial loss of ADIPOSE TISSUE, either confined to the extremities with normal or increased fat deposits on…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160069
Loeys-Dietz Syndrome
An autosomal dominant aneurysm with multisystem abnormalities caused by increased TGF-BETA signaling due to mutations in type I or II of TGF-BETA REC…
SyndromePathologyClinical Genetics [curated_secondary]AMW:DIS:050314
Lower Extremity Deformities, Congenital
Congenital structural abnormalities of the LOWER EXTREMITY.
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050331
Lysosomal Storage Diseases
Inborn errors of metabolism characterized by defects in specific lysosomal hydrolases and resulting in intracellular accumulation of unmetabolized su…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160126
Mandibulofacial Dysostosis
A hereditary disorder occurring in two forms: the complete form (Franceschetti's syndrome) is characterized by a slant of the palpebral fissures, COL…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050019
Mannosidase Deficiency Diseases
Diseases caused by the loss of one or more enzymes involved in the hydrolysis of mannoside linkages (MANNOSIDASES). The defects in enzyme activity ar…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160093
Marfan Syndrome
An autosomal dominant disorder of CONNECTIVE TISSUE with abnormal features in the heart, the eye, and the skeleton. Cardiovascular manifestations inc…
SyndromePathologyClinical Genetics [curated_secondary]AMW:DIS:050036
Megalencephaly
A congenital abnormality in which the occipitofrontal circumference is greater than two standard deviations above the mean for a given age. It is ass…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050315
Melorheostosis
A form of osteosclerosis extending in a linear track mainly through one of the long bones of the upper and lower limbs.
DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050056
Metabolism, Inborn Errors
Errors in metabolic processes resulting from inborn genetic mutations that are inherited or acquired in utero.
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160071
Metal Metabolism, Inborn Errors
Dysfunctions in the metabolism of metals resulting from inborn genetic mutations that are inherited or acquired in utero.
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160129
Microcephaly
A congenital abnormality in which the CEREBRUM is underdeveloped, the fontanels close prematurely, and, as a result, the head is small. (Desk Referen…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050319
Micrognathism
Abnormally small jaw.
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050175
Micronuclei, Chromosome-Defective
Defective nuclei produced during the TELOPHASE of MITOSIS or MEIOSIS by lagging CHROMOSOMES or chromosome fragments derived from spontaneous or exper…
Pathologic ConditionPathologyClinical Genetics [curated_secondary]AMW:DIS:230063
Microsatellite Instability
The occurrence of highly polymorphic mono- and dinucleotide MICROSATELLITE REPEATS in somatic cells. It is a form of genome instability associated wi…
Pathologic ConditionPathologyClinical Genetics [curated_secondary]AMW:DIS:230135
Mineralocorticoid Excess Syndrome, Apparent
A hereditary disease characterized by childhood onset HYPERTENSION, hypokalemic alkalosis, and low RENIN and ALDOSTERONE secretion. It results from a…
SyndromePathologyClinical Genetics [curated_secondary]AMW:DIS:160137
Mitochondrial Diseases
Diseases caused by abnormal function of the MITOCHONDRIA. They may be caused by mutations, acquired or inherited, in mitochondrial DNA or in nuclear …
Metabolic DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:180047
Mitochondrial Encephalomyopathies
A heterogenous group of disorders characterized by alterations of mitochondrial metabolism that result in muscle and nervous system dysfunction. Thes…
Metabolic DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050253
Mitochondrial Myopathies
A group of muscle diseases associated with abnormal mitochondria function.
Metabolic DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050252
Monilethrix
Rare autosomal dominant disorder of the hair shaft. The clinical features of the disease include HYPOTRICHOSIS, dry, and/or brittle hair, with varyin…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160016
Monosomy
The condition in which one chromosome of a pair is missing. In a normally diploid cell it is represented symbolically as 2N-1.
Pathologic ConditionPathologyClinical Genetics [curated_secondary]AMW:DIS:230052