Autosomal recessive hereditary disorder	Congenital anomaly in number of teeth	Developmental hereditary disorder	Hereditary disorder of tooth	Hereditary metabolic disease	Metabolic and genetic disorder affecting the liver	Multiple system malformation syndrome
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Steroid dehydrogenase deficiency and dental anomaly syndrome (disorder)
	Steroid dehydrogenase deficiency and dental anomaly syndrome
	Lyngstadaas syndrome
	An autosomal recessive liver disease, which was associated with numerical dental aberrations in a consanguineous Arabia Saudi family. This association suggests that the same gene is involved in both defects. General hypomineralization and enamel hypoplasia found in this family is thought to be secondary to malabsorption due to liver disease.
	syndroom van steroïdedehydrogenasedeficiëntie en dentale anomalieën
	syndroom van Lyngstadaas

Id	723583009
Status	Primitive

Associated morphology	Congenital abnormal number
Finding site	Tooth structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Finding site	Liver structure
Occurrence	Congenital
Pathological process	Pathological developmental process

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	K76.8
Rule	TRUE
Advice	ALWAYS K76.8 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified

Target	K00.9
Rule	TRUE
Advice	ALWAYS K00.9
Correlation	SNOMED CT source code to target map code correlation not specified