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320 results

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ConceptNode typeBase domainMatched membershipIdentifier
Pyruvate Metabolism, Inborn Errors
Hereditary disorders of pyruvate metabolism. They are difficult to diagnose and describe because pyruvate is a key intermediate in glycolysis, glucon…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160102
Retrognathia
A physical misalignment of the upper (maxilla) and lower (mandibular) jaw bones in which either or both recede relative to the frontal plane of the f…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050178
Rickets, Hypophosphatemic
A disorder characterized by HYPOPHOSPHATEMIA; RICKETS; OSTEOMALACIA; resulting from lack of phosphate reabsorption by the kidneys and possible defect…
Metabolic DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050079
Ring Chromosomes
Aberrant chromosomes with no ends, i.e., circular.
Pathologic ConditionPathologyClinical Genetics [curated_secondary]AMW:DIS:230069
Rothmund-Thomson Syndrome
An autosomal recessive syndrome occurring principally in females, characterized by the presence of reticulated, atrophic, hyperpigmented, telangiecta…
SyndromePathologyClinical Genetics [curated_secondary]AMW:DIS:160051
Rubinstein-Taybi Syndrome
A chromosomal disorder characterized by MENTAL RETARDATION, broad thumbs, webbing of fingers and toes, beaked nose, short upper lip, pouting lower li…
SyndromePathologyClinical Genetics [curated_secondary]AMW:DIS:050024
Sarcoglycanopathies
Deficiencies or mutations in the genes for the SARCOGLYCAN COMPLEX subunits. A variety of phenotypes are associated with these mutations including a …
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050268
Severe Combined Immunodeficiency
Group of rare congenital disorders characterized by impairment of both humoral and cell-mediated immunity, leukopenia, and low or absent antibody lev…
Immune DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160141
Sex Chromosome Aberrations
Abnormal number or structure of the SEX CHROMOSOMES. Some sex chromosome aberrations are associated with SEX CHROMOSOME DISORDERS and SEX CHROMOSOME …
Pathologic ConditionPathologyClinical Genetics [curated_secondary]AMW:DIS:230070
Sex Chromosome Disorders
Clinical conditions caused by an abnormal sex chromosome constitution (SEX CHROMOSOME ABERRATIONS), in which there is extra or missing sex chromosome…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160030
Sex Chromosome Disorders of Sex Development
Congenital conditions of atypical sexual development associated with abnormal sex chromosome constitutions including MONOSOMY; TRISOMY; and MOSAICISM.
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:120079
Sexual Infantilism
The permanent lack of SEXUAL DEVELOPMENT in an individual. This defect is usually observed at an age after expected PUBERTY.
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:120076
Short Rib-Polydactyly Syndrome
A syndrome inherited as an autosomal recessive trait and incompatible with life. The main features are narrow thorax, short ribs, scapular and pelvic…
SyndromePathologyClinical Genetics [curated_secondary]AMW:DIS:050060
Silver-Russell Syndrome
Genetically and clinically heterogeneous disorder characterized by low birth weight, postnatal growth retardation, facial dysmorphism, bilateral body…
SyndromePathologyClinical Genetics [curated_secondary]AMW:DIS:050324
Situs Inversus
A congenital abnormality in which organs in the THORAX and the ABDOMEN are opposite to their normal positions (situs solitus) due to lateral transpos…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160033
Sjogren-Larsson Syndrome
An autosomal recessive neurocutaneous disorder characterized by severe ichthyosis MENTAL RETARDATION; SPASTIC PARAPLEGIA; and congenital ICHTHYOSIS. …
SyndromePathologyClinical Genetics [curated_secondary]AMW:DIS:160049
Skin Diseases, Genetic
Diseases of the skin with a genetic component, usually the result of various inborn errors of metabolism.
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160142
Slipped Capital Femoral Epiphyses
A developmental deformity in which the metaphysis of the FEMUR moves proximally and anteriorly away from FEMUR HEAD (epiphysis) at the upper GROWTH P…
DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050061
Smith-Lemli-Opitz Syndrome
An autosomal recessive disorder of CHOLESTEROL metabolism. It is caused by a deficiency of 7-dehydrocholesterol reductase, the enzyme that converts 7…
SyndromePathologyClinical Genetics [curated_secondary]AMW:DIS:160020
Sotos Syndrome
Congenital or postnatal overgrowth syndrome most often in height and occipitofrontal circumference with variable delayed motor and cognitive developm…
SyndromePathologyClinical Genetics [curated_secondary]AMW:DIS:160021
Steatocystoma Multiplex
A disorder characterized by multiple, wide spread cutaneous cysts that often become inflamed and rupture. It is caused by the same mutations in the g…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160036
Steroid Metabolism, Inborn Errors
Errors in metabolic processing of STEROIDS resulting from inborn genetic mutations that are inherited or acquired in utero.
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160136
Syndactyly
A congenital anomaly of the hand or foot, marked by the webbing between adjacent fingers or toes. Syndactylies are classified as complete or incomple…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050029
Synostosis
A union between adjacent bones or parts of a single bone formed by osseous material, such as ossified connecting cartilage or fibrous tissue. (Dorlan…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050025
Talipes
Deformity in which the foot is misaligned with respect to the TALUS in the ANKLE JOINT. While mostly congenital, as in CLUBFOOT, acquired deformities…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050153
Talipes Cavus
A foot deformity in which the arch of the foot is high and often the heel adducted.
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050157
Tarsal Coalition
Congenital, complete or partial fusion of the TARSAL BONES of the foot. PES PLANUS is usually a feature.
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050032
Tetraploidy
The presence of four sets of chromosomes. It is associated with ABNORMALITIES, MULTIPLE; and MISCARRAGES.
Pathologic ConditionPathologyClinical Genetics [curated_secondary]AMW:DIS:230067
Tetrasomy
The possession of four chromosomes of any one type in an otherwise diploid cell.
Pathologic ConditionPathologyClinical Genetics [curated_secondary]AMW:DIS:230054
Thanatophoric Dysplasia
A severe form of neonatal dwarfism with very short limbs. All cases have died at birth or later in the neonatal period.
Pathologic ConditionPathologyClinical Genetics [curated_secondary]AMW:DIS:050008