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ConceptNode typeBase domainMatched membershipIdentifier
Craniosynostoses
Premature closure of one or more CRANIAL SUTURES. It often results in plagiocephaly. Craniosynostoses that involve multiple sutures are sometimes ass…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050027
Crigler-Najjar Syndrome
A familial form of congenital hyperbilirubinemia transmitted as an autosomal recessive trait. It is characterized by icterus and brain damage caused …
SyndromePathologyClinical Genetics [curated_secondary]AMW:DIS:160105
Cryopyrin-Associated Periodic Syndromes
A group of rare autosomal dominant diseases, commonly characterized by atypical URTICARIA (hives) with systemic symptoms that develop into end-organ …
SyndromePathologyClinical Genetics [curated_secondary]AMW:DIS:160064
Cutis Laxa
A group of connective tissue diseases in which skin hangs in loose pendulous folds. It is believed to be associated with decreased elastic tissue for…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160143
Cystinosis
A metabolic disease characterized by the defective transport of CYSTINE across the lysosomal membrane due to mutation of a membrane protein cystinosi…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160128
Cytochrome-c Oxidase Deficiency
A disease that results from a congenital defect in ELECTRON TRANSPORT COMPLEX IV. Defects in ELECTRON TRANSPORT COMPLEX IV can be caused by mutations…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160103
Darier Disease
An autosomal dominantly inherited skin disorder characterized by warty malodorous papules that coalesce into plaques. It is caused by mutations in th…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160144
Dentofacial Deformities
An abnormality of the jaws or teeth affecting the contour of the face. Such abnormality could be acquired or congenital.
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050318
Dermatitis, Atopic
A chronic inflammatory genetically determined disease of the skin marked by increased ability to form reagin (IgE), with increased susceptibility to …
Inflammatory DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160145
Developmental Dysplasia of the Hip
Dislocation of the HIP JOINT from an abnormal FEMORAL HEAD to the ACETABULUM relationship. It is most often due to ligamentous laxity, abnormal posit…
Pathologic ConditionPathologyClinical Genetics [curated_secondary]AMW:DIS:050232
DiGeorge Syndrome
Congenital syndrome characterized by a wide spectrum of characteristics including the absence of the THYMUS and PARATHYROID GLANDS resulting in T-cel…
SyndromePathologyClinical Genetics [curated_secondary]AMW:DIS:050309
Dihydropyrimidine Dehydrogenase Deficiency
An autosomal recessive disorder affecting DIHYDROPYRIMIDINE DEHYDROGENASE and causing familial pyrimidinemia. It is characterized by thymine-uracilur…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160135
Disorder of Sex Development, 46,XY
Congenital conditions in individuals with a male karyotype, in which the development of the gonadal or anatomical sex is atypical.
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:120069
Disorders of Sex Development
In gonochoristic organisms, congenital conditions in which development of chromosomal, gonadal, or anatomical sex is atypical. Effects from exposure …
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:120062
Distal Myopathies
A heterogeneous group of genetic disorders characterized by progressive MUSCULAR ATROPHY and MUSCLE WEAKNESS beginning in the hands, the legs, or the…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050265
DNA Repair-Deficiency Disorders
Disorders resulting from defective DNA REPAIR processes or the associated cellular responses to DNA DAMAGE.
Metabolic DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:180016
Donohue Syndrome
Rare autosomal recessive syndrome of extreme insulin resistance due to mutations in the binding domain of INSULIN RECEPTOR. Clinical features include…
SyndromePathologyClinical Genetics [curated_secondary]AMW:DIS:050311
Dwarfism
A genetic or pathological condition that is characterized by short stature and undersize. Abnormal skeletal growth usually results in an adult who is…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050006
Ectodermal Dysplasia
A group of hereditary disorders involving tissues and structures derived from the embryonic ectoderm. They are characterized by the presence of abnor…
Pathologic ConditionPathologyClinical Genetics [curated_secondary]AMW:DIS:160009
Ectodermal Dysplasia 1, Anhidrotic
An X-linked form of ectodermal dysplasia which results from mutations of the gene encoding ECTODYSPLASIN.
Pathologic ConditionPathologyClinical Genetics [curated_secondary]AMW:DIS:160010
Ectodermal Dysplasia 3, Anhidrotic
An autosomal dominant form of ectodermal dysplasia which is due to mutations in the gene for the EDAR RECEPTOR.
Pathologic ConditionPathologyClinical Genetics [curated_secondary]AMW:DIS:160011
Ectodermal Dysplasia, Hypohidrotic, Autosomal Recessive
An autosomal recessive form of ectodermal dysplasia which is due to mutations in the gene for the EDAR RECEPTOR or EDAR-ASSOCIATED DEATH DOMAIN PROTE…
Pathologic ConditionPathologyClinical Genetics [curated_secondary]AMW:DIS:160012
Ectromelia
Gross hypo- or aplasia of one or more long bones of one or more limbs. The concept includes amelia, hemimelia, phocomelia, and sirenomelia.
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050330
Ellis-Van Creveld Syndrome
Dwarfism occurring in association with defective development of skin, hair, and teeth, polydactyly, and defect of the cardiac septum. (Dorland, 27th …
SyndromePathologyClinical Genetics [curated_secondary]AMW:DIS:050043
Enchondromatosis
Benign growths of cartilage in the metaphyses of several bones.
DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050044
Epidermolysis Bullosa
Group of genetically determined disorders characterized by the blistering of skin and mucosae. There are four major forms: acquired, simple, junction…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160037
Epidermolysis Bullosa Dystrophica
Form of epidermolysis bullosa characterized by atrophy of blistered areas, severe scarring, and nail changes. It is most often present at birth or in…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160039
Epidermolysis Bullosa Simplex
A form of epidermolysis bullosa characterized by serous bullae that heal without scarring. Mutations in the genes that encode KERATIN-5 and KERATIN-1…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160041
Epidermolysis Bullosa, Junctional
Form of epidermolysis bullosa having onset at birth or during the neonatal period and transmitted through autosomal recessive inheritance. It is char…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160040
Equinus Deformity
Plantar declination of the foot.
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050155