|||||||||||
renohepaticopancreatische dysplasie (aandoening)
renohepaticopancreatische dysplasie
Ivemark II-syndroom
Renal hepatic pancreatic dysplasia
Renohepaticopancreatic dysplasia
Ivemark II syndrome
A rare genetic developmental defect during embryogenesis syndrome with the triad of pancreatic fibrosis (and cysts, with a reduction of parenchymal tissue), renal dysplasia (with peripheral cortical cysts, primitive collecting ducts, glomerular cysts and metaplastic cartilage) and hepatic dysgenesis (enlarged portal areas containing numerous elongated binary profiles with a tendency to perilobular fibrosis). Situs abnormalities, skeletal anomalies and anencephaly have also been associated. Patients that survive the neonatal period present renal insufficiency, chronic jaundice and insulin-dependant diabetes.
Id763891005
StatusPrimitive
Associated morphologyfibrose
Finding sitestructuur van pancreas
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
Associated morphologypolycysteuze verandering
Finding sitestructuur van nier
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
referentieset met complexe 'mapping' naar ICD-10
TargetQ61.4
RuleTRUE
AdviceALWAYS Q61.4 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified
TargetQ45.3
RuleTRUE
AdviceALWAYS Q45.3
CorrelationSNOMED CT source code to target map code correlation not specified
TargetQ44.7
RuleTRUE
AdviceALWAYS Q44.7
CorrelationSNOMED CT source code to target map code correlation not specified