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syndroom van aplasia cutis congenita met intestinale lymfangiëctasie (aandoening)
syndroom van aplasia cutis congenita met intestinale lymfangiëctasie
syndroom van Bronspiegel-Zelnick
Bronspiegel-Zelnick-syndroom
Aplasia cutis congenita with intestinal lymphangiectasia syndrome
Bronspiegel Zelnick syndrome
An extremely rare association syndrome, described in only two brothers to date (one of which died at 2 months of age), characterized by aplasia cutis congenita of the vertex and generalized edema (as well as hypoproteinemia and lymphopenia) due to intestinal lymphangiectasia. There have been no further descriptions in the literature since 1985.
Id720500008
StatusPrimitive
Associated morphologyaplasia
Finding sitegedeelte van huid
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
referentieset met complexe 'mapping' naar ICD-10
TargetQ84.8
RuleTRUE
AdviceALWAYS Q84.8
CorrelationSNOMED CT source code to target map code correlation not specified
TargetQ82.0
RuleTRUE
AdviceALWAYS Q82.0
CorrelationSNOMED CT source code to target map code correlation not specified