A 17-year-old primigravida complained of decreased fetal movements. She delivered a baby weighing 2000gms at 30 weeks of gestation. The Apgar scores of the baby were 4 and 5 at 1 and 5 minutes respectively. The baby died in an hour. Post- mortem examination revealed multiple, peripheral, radially arranged cysts in the kidney. Most common associated finding with this condition is?
Ans. B. Congenital hepatic fibrosisAutosomal recessive polycystic kidney disease is characterized by pathologic changes in the kidney and/or liver. Hepatic fibrosis with hepatic cysts is known association. In the kidney, epithelial hyperplasia occurs along the collecting duct of the nephron. The hyperplastic cells undergo a functional change from being resorptive to becoming secretory. The fluid secreted from these abnormal cells is rich in epithelial growth factors, which further stimulate epithelial proliferation. The combination of epithelial hyperplasia and fluid secretion results in significant ductal ectasia. Approximately 10-90% of the ducts may be affected, resulting in a wide variability of renal dysfunction. Depending on the number of ducts involved, the kidneys may be massively enlarged.
Holoprosencephaly
Congenital hepatic fibrosis
Ureteral agenesis
Medullary sponge kidney
Miscellaneous