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'limb-body wall'-complex (aandoening)
'limb-body wall'-complex
LBWC
Limb body wall complex
LBWC (limb body wall complex) syndrome
Limb body wall complex (LBWC) is a syndrome with features of severe multiple congenital anomalies. Clinical manifestations vary widely and include limb defects and visceral malformations, spinal abnormalities, absent diaphragm, bowel atresia and renal agenesis. Karyotypes have been reported as normal and no correlations with gender, parental age and teratogenic agents have been found. The principal theories are an extrinsic origin by early amniotic rupture, or a vascular origin due to an early vascular accident during embryological development. Single cases of familial occurrence have been documented. LBWC is fatal, with death occurring antenatally or early in the neonatal period.
Id716106000
StatusPrimitive
DHD Diagnosethesaurus-referentieset
referentieset met complexe 'mapping' naar ICD-10
TargetQ87.8
RuleTRUE
AdviceALWAYS Q87.8
CorrelationSNOMED CT source code to target map code correlation not specified