Prenatally diagnosed autosomal recessive polycystic kidney disease: initial postnatal management.
We report a case of autosomal recessive polycystic kidney disease (ARPKD). A presumptive diagnosis was made after a late-term prenatal ultrasound revealed hypoplastic lungs, massive polycystic kidneys, and oligohydramnios. A full-term baby girl was delivered vaginally. Respiratory distress required intubation. Twelve hours after birth, she underwent bilateral nephrectomy and peritoneal dialysis catheter placement. The average kidney size was 150 g and 9.25 cm. Pathologic examination confirmed ARPKD. Peritoneal dialysis was started on the third day of life. The baby had no gross neurologic deficit. At 6 months of age, she was growing well, and the mother was a candidate to be a living-related kidney donor.
Munding
M
M
Sections of Pediatric Urology and Nephrology, University of Arizona College of Medicine, Tucson, Arizona, USA.
Al-Uzri
A
A
Gralnek
D
D
Riden
D
D
D000328:Adult; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007668:Kidney / Q000473:pathology; D009392:Nephrectomy; D016104:Oligohydramnios / Q000000981:diagnostic imaging*; D009929:Organ Size; D010348:Patient Care Team; D010530:Peritoneal Dialysis; D017044:Polycystic Kidney, Autosomal Recessive / Q000175:diagnosis* / Q000628:therapy; D011247:Pregnancy; D016216:Ultrasonography, Prenatal