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320 results

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ConceptNode typeBase domainMatched membershipIdentifier
Osteogenesis Imperfecta
COLLAGEN DISEASES characterized by brittle, osteoporotic, and easily fractured bones. It may also present with blue sclerae, loose joints, and imperf…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050054
Osteopetrosis
Excessive formation of dense trabecular bone leading to pathological fractures; OSTEITIS; SPLENOMEGALY with infarct; ANEMIA; and extramedullary hemop…
DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050057
Osteopoikilosis
An asymptomatic, autosomal dominant trait in which pea-sized sclerotic spots, prominent in the metaphyseal area, are accompanied by unique cutaneous …
DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050058
Osteosclerosis
An abnormal hardening or increased density of bone tissue.
Degenerative DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050055
Pachyonychia Congenita
A group of inherited ectodermal dysplasias whose most prominent clinical feature is hypertrophic nail dystrophy resulting in PACHYONYCHIA. Several sp…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160014
Papillon-Lefevre Disease
Rare, autosomal recessive disorder occurring between the first and fifth years of life. It is characterized by palmoplantar keratoderma with periodon…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160151
Paralyses, Familial Periodic
A heterogenous group of inherited disorders characterized by recurring attacks of rapidly progressive flaccid paralysis or myotonia. These conditions…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050291
Paralysis, Hyperkalemic Periodic
An autosomal dominant familial disorder which presents in infancy or childhood and is characterized by episodes of weakness associated with hyperkale…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050293
Pectus Carinatum
A developmental anomaly characterized by abnormal anterior protrusion of the STERNUM and adjacent COSTAL CARTILAGE.
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050063
Pemphigus, Benign Familial
An autosomal dominantly inherited skin disorder characterized by recurrent eruptions of vesicles and BULLAE mainly on the neck, axillae, and groin. M…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160153
Peroxisomal Disorders
A heterogeneous group of inherited metabolic disorders marked by absent or dysfunctional PEROXISOMES. Peroxisomal enzymatic abnormalities may be sing…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160132
Philadelphia Chromosome
An aberrant form of human CHROMOSOME 22 characterized by translocation of the distal end of chromosome 9 from 9q34, to the long arm of chromosome 22 …
Pathologic ConditionPathologyClinical Genetics [curated_secondary]AMW:DIS:230072
Piebaldism
Autosomal dominant, congenital disorder characterized by localized hypomelanosis of the skin and hair. The most familiar feature is a white forelock …
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160057
Plagiocephaly
The condition characterized by uneven or irregular shape of the head often in parallelogram shape with a flat spot on the back or one side of the hea…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050322
Plagiocephaly, Nonsynostotic
A deformity of the SKULL that is not due to bone fusion (SYNOSTOSIS), such as craniosynostoses, and is characterized by an asymmetric skull and face.…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050323
Platybasia
A developmental deformity of the occipital bone and upper end of the cervical spine, in which the latter appears to have pushed the floor of the occi…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050064
Polydactyly
A congenital anomaly of the hand or foot, marked by the presence of supernumerary digits.
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050332
Polyploidy
The chromosomal constitution of a cell containing multiples of the normal number of CHROMOSOMES; includes triploidy (symbol: 3N), tetraploidy (symbol…
Pathologic ConditionPathologyClinical Genetics [curated_secondary]AMW:DIS:230066
Porokeratosis
A heritable disorder of faulty keratinization characterized by the proliferation of abnormal clones of KERATINOCYTES and lesions showing varying atro…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160154
Porphyria, Erythropoietic
An autosomal recessive porphyria that is due to a deficiency of UROPORPHYRINOGEN III SYNTHASE in the BONE MARROW; also known as congenital erythropoi…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160155
Primary Immunodeficiency Diseases
Genetic immunologic deficiency diseases and syndromes due to mutations in genes involved in IMMUNITY generally characterized by an increased suscepti…
Immune DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160138
Progeria
An abnormal congenital condition, associated with defects in the LAMIN TYPE A gene, which is characterized by premature aging in children, where all …
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160070
Prognathism
A condition marked by abnormal protrusion of the mandible. (Dorland, 27th ed)
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050177
Prolidase Deficiency
Rare autosomal recessive disorder of metabolism due to mutations in the prolidase gene. It is characterized by recurrent lower extremity skin ulcers,…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160018
Propionic Acidemia
Autosomal recessive metabolic disorder caused by mutations in PROPIONYL-COA CARBOXYLASE genes that result in dysfunction of branch chain amino acids …
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160079
Prune Belly Syndrome
A syndrome characterized by abdominal wall musculature deficiency, cryptorchism, and urinary tract abnormalities. The syndrome derives its name from …
SyndromePathologyClinical Genetics [curated_secondary]AMW:DIS:160019
Pseudohypoparathyroidism
A hereditary syndrome clinically similar to HYPOPARATHYROIDISM. It is characterized by HYPOCALCEMIA; HYPERPHOSPHATEMIA; and associated skeletal devel…
Metabolic DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050074
Pseudopseudohypoparathyroidism
A form of PSEUDOHYPOPARATHYROIDISM characterized by the same features except for the abnormal response to hormones such as PARATHYROID HORMONE. It is…
Metabolic DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050075
Purine-Pyrimidine Metabolism, Inborn Errors
Dysfunctions in the metabolism of PURINES or PYRIMIDINES resulting from inborn genetic mutations that are inherited or acquired in utero.
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:160134
Pycnodysostosis
Rare autosomal recessive syndrome characterized by delayed closing of CRANIAL SUTURES, short stature, ACRO-OSTEOLYSIS of distal phalanges, dental and…
Congenital DisorderPathologyClinical Genetics [curated_secondary]AMW:DIS:050059