Diagnostic of Early Onset Polycystic Kidney Disease in Neonates.

ABSTRACT

Polycystic kidney disease represented by autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD) have a major impact of mortality in children. We conducted a study of a premature infant with an estimated gestation date of 32 weeks with a presumptive prenatal diagnosis of right polycystic kidney. A 28-year-old primigravida with pre-eclampsia was admitted at the gynecology unit of Clinical Emergency County Hospital of Craiova. The clinical examination revealed a large abdominal distention due probably to the right polycystic kidney, suspected on prenatal ultrasound and radiography. The preterm neonate undergone right nephrectomy 5 days after birth. Histopathology of the kidney was performed in the Pathology Department of the Emergency County Hospital of Craiova and in the Center for Microscopic Morphology and Immunology of U.M.F. of Craiova. Microscopy revealed dilated cysts lined by simple cuboidal or flattened epithelium, and islets of remnant kidney parenchyma separated by edematous stroma. Immunohistochemistry for CD34 revealed incomplete blood arcades which did not seem to be in contact with all the tubular elements of the parenchyma, when compared to a control age-matched kidney. The patient had a favorable postoperative evolution, she was clinically stable on discharge from the hospital with a follow-up strategy including genetic testing.