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partiële pancreatische agenesie (aandoening)
partiële pancreatische agenesie
partiële agenese van alvleesklier
Partial pancreatic agenesis
Partial agenesis of pancreas
Partial agenesis of the pancreas is an absence of a critical mass of pancreatic tissue. It is a rare disorder with only around 50 cases being reported in the literature so far. The severity of the disease depends on the amount of functional pancreatic tissue present. Pancreatic agenesis is commonly associated with other malformations, in particular pancreaticobiliary duct anomalies or more rarely, polysplenia. In the majority of cases, patients are diagnosed after reporting abdominal pain. Agenesis of the dorsal pancreas usually manifests as diabetes. Pancreatic agenesis has been associated with mutations in the PDX1 gene (13q12.1), which encodes the insulin promoter factor-1 (IPF-1) transcription factor.
Id719044008
StatusDefined
Associated morphologyaplasia
Finding sitestructuur van pancreas
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
referentieset met complexe 'mapping' naar ICD-10
TargetQ45.0
RuleTRUE
AdviceALWAYS Q45.0
CorrelationSNOMED CT source code to target map code correlation not specified