Autosomal recessive SCID (severe combined immunodeficiency disease)	Cardiovascular system hereditary disorder	Digestive system hereditary disorder	Severe combined immunodeficiency with low T- and B-cell numbers	Veno-occlusive disease of the liver
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Hepatic veno-occlusive disease with immunodeficiency syndrome (disorder)
	Hepatic veno-occlusive disease with immunodeficiency syndrome
	VODI syndrome VODI (veno-occlusive disease, immunodeficiency) syndrome
	Syndrome that is characterized by the association of severe hypogammaglobulinemia, combined T and B cell immunodeficiency, absent lymph node germinal centers, absent tissue plasma cells and hepatic veno-occlusive disease. Mutations in the gene coding PML nuclear body protein Sp110 on chromosome 2q37 were found to be responsible for this association. Transmission is autosomal recessive.
	syndroom van hepatische veno-occlusieve ziekte en immunodeficiëntie
	syndroom van hepatische veno-occlusieve ziekte en immuundeficiëntie VODI-syndroom

Id	724361001
Status	Primitive

Finding site	Structure of immune system
Occurrence	Congenital
Pathological process	Abnormal immune process

Associated morphology	Obstruction
Finding site	Structure of hepatic vein

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	K76.5
Rule	TRUE
Advice	ALWAYS K76.5 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified

Target	D81.8
Rule	TRUE
Advice	ALWAYS D81.8
Correlation	SNOMED CT source code to target map code correlation not specified