Autosomal recessive hereditary disorder	Dentin dysplasia
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Atypical dentin dysplasia due to SPARC related modular calcium binding 2 deficiency (disorder)
	Atypical dentin dysplasia due to SMOC2 deficiency
	Dentin dysplasia type 1 with microdontia and shape anomalies Atypical dentin dysplasia due to SPARC related modular calcium binding 2 deficiency
	A rare genetic dentin dysplasia disease with characteristics of extreme microdontia, oligodontia and abnormal tooth shape (including globular teeth, incisal notches and double tooth formation). Short roots with a variable pulp phenotype (including taurodontia and flame-shaped pulp) enamel hypoplasia and anterior open bite may also be associated. Caused by homozygous mutation in the SMOC2 gene on chromosome 6q27.
	atypische dentinedysplasie door SMOC2-deficiëntie
	atypische dentinedysplasie door 'SPARC related modular calcium binding 2'-deficiëntie

Id	783059004
Status	Primitive

Associated morphology	Dysplasia
Finding site	Dentin structure
Occurrence	Congenital
Pathological process	Pathological developmental process

ICD-10 complex map reference set

Target	K00.5
Rule	TRUE
Advice	ALWAYS K00.5
Correlation	SNOMED CT source code to target map code correlation not specified