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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.
Id724361001
StatusPrimitive
Finding sitestructuur van immuunsysteem
Occurrencecongenitaal
Pathological processafwijkend immuunproces
Associated morphologyobstructie
Finding sitestructuur van vena hepatica
referentieset met complexe 'mapping' naar ICD-10
TargetK76.5
RuleTRUE
AdviceALWAYS K76.5 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified
TargetD81.8
RuleTRUE
AdviceALWAYS D81.8
CorrelationSNOMED CT source code to target map code correlation not specified