ODCs

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4 OMIM references -
3 associated genes
12 signs/symptoms

PROTEIN INTERACTIONS: 1

1 OMIM reference -
1 associated gene
9 signs/symptoms

Bilateral striopallidodentate calcinosis

Legg-CalvÃ©-Perthes disease


INTERACTOME ASSOCIATIONS (click on a score value to see the evidence)	PDGFB	(0.68)	COL2A1

Citations in the biomedical literature:

Bilateral striopallidodentate calcinosis		Legg-CalvÃ©-Perthes disease
	Synonym(s): - BSPDC - Cerebrovascular ferrocalcinosis - Idiopathic basal ganglia calcification		Synonym(s): - Aseptic necrosis of the capital femoral epiphysis - Osteochondritis of the capital femoral epiphysis - Osteochondrosis of the capital femoral epiphysis - Perthes disease

	Classification (Orphanet): - Rare genetic disease - Rare neurologic disease		Classification (Orphanet): - Rare bone disease - Rare genetic disease

	Classification (ICD10): - Diseases of the nervous system -		Classification (ICD10): - Diseases of the musculoskeletal system and connective tissue -

	Epidemiological data: Class of prevalence: unknown Average age onset: adulthood Average age of death: - Type of inheritance: autosomal dominant		Epidemiological data: Class of prevalence: unknown Average age onset: childhood Average age of death: normal Type of inheritance: autosomal dominant

	External references: 4 OMIM references - No MeSH references		External references: 1 OMIM reference - No MeSH references

Bilateral striopallidodentate calcinosis		Legg-CalvÃ©-Perthes disease
	Very frequent - Agyria / micro / pachy / macrogyria / lissencephaly / gyration / neuronal migration defect - Autosomal recessive inheritance - Dilated cerebral ventricles without hydrocephaly - Hepatomegaly / liver enlargement (excluding storage disease) - Intracranial / cerebral calcifications - Intrauterine growth retardation - Microcephaly - Purpura / petichiae - Seizures / epilepsy / absences / spasms / status epilepticus - Thrombocytopenia / thrombopenia Frequent - Corneal clouding / opacity / vascularisation - Structural anomalies of the liver and the biliary tract		Very frequent - Abnormal dentition / dental position / implantation / unerupted / dental ankylosis - Articular / joint pain / arthralgia - Cartilage destruction / chondrolysis - Delayed bone age - Joint / articular deformation - Muscle hypotrophy / atrophy / dystrophy / agenesis / amyotrophy - Osteonecrosis / bone infarction - Polygenic / multifactorial inheritance - Short stature / dwarfism / nanism