Renohepaticopancreatic dysplasia: diagnostic dilemma.
We present the case of an eighteen day old baby boy hospitalized with an abdominal mass, renal insufficiency and jaundice. Multiple radiographic, radionuclear and surgical interventions were required to diagnose renohepaticopancreatic dysplasia, also known as Ivemark II syndrome. In spite of aggressive intensive care support, the patient developed multisystemic organ failure and died. Clinical presentation and autopsy findings are presented.
Fernández-Sein
Alicia
A
Department of Pediatrics, Medical Sciences Campus, University of Puerto Rico, San Juan, Puerto Rico. afernandez@rcm.upr.edu
Correa-Rivas
María S
MS
Pratts
Keila
K
Lugo-Vicente
Humberto
H
D058186:Acute Kidney Injury / Q000150:complications* / Q000209:etiology / Q000473:pathology; D001344:Autopsy; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005355:Fibrosis / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D007565:Jaundice / Q000209:etiology / Q000473:pathology; D007668:Kidney / Q000473:pathology; D008099:Liver / Q000473:pathology; D017093:Liver Failure / Q000150:complications* / Q000209:etiology / Q000473:pathology; D008297:Male; D009102:Multiple Organ Failure / Q000209:etiology / Q000473:pathology; D010179:Pancreas / Q000473:pathology; D010181:Pancreatic Cyst / Q000150:complications* / Q000473:pathology; D013577:Syndrome