aandoening van spijsverteringsstelsel specifiek voor foetus of neonaat	autosomaal recessieve ernstige gecombineerde immunodeficiëntie	ernstige gecombineerde immunodeficiëntie met laag aantal T- en B-cellen	hereditaire aandoening van cardiovasculair systeem	hereditaire aandoening van spijsverteringsstelsel	veno-occlusieve ziekte van lever
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syndroom van hepatische veno-occlusieve ziekte en immunodeficiëntie (aandoening)
	syndroom van hepatische veno-occlusieve ziekte en immunodeficiëntie
	syndroom van hepatische veno-occlusieve ziekte en immuundeficiëntie VODI-syndroom
	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.

Id	724361001
Status	Primitive

Finding site	structuur van immuunsysteem
Occurrence	congenitaal
Pathological process	afwijkend immuunproces

Associated morphology	obstructie
Finding site	structuur van vena hepatica

DHD Diagnosis thesaurus reference set

RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set

Target	K76.5
Term	'Veno-occlusive disease' [VOD] van lever

Target	D81.8
Term	Overige gespecificeerde gecombineerde immunodeficiënties

SNOMED CT to Orphanet simple map

79124

SNOMED CT to ICD-10 extended map

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