A 31-year-old woman experiences abdominal pain 1 week after noticing blood in her urine. She has had three episodes of urinary tract infection during the past year. There are no remarkable findings on physical examination. Urinalysis shows 2+ hematuria, 1+ proteinuria, hypercalciuria, and no glucose or ketones. Serum creatinine is 1.0 g/dL. Microscopic examination of the urine shows numerous RBCs and oxalate crystals. An abdominal CT scan with contrast shows linear striations radiating into the renal papillae, along with small cystic collections of contrast material in dilated collecting ducts. She is advised to increase her daily intake of fluids, and her condition improves. Which of the following renal cystic diseases is most likely to be associated with these findings?
Correct Answer: Medullary sponge kidney
Description: The congenital disorder known as medullary sponge kidney (MSK) is present to some degree in 1% of adults. In MSK, cystic dilation of 1 to 5 mm is present in the inner medullary and papillary collecting ducts. MSK is bilateral in 70% of cases. Not all papillae are equally affected, although calculi are often present in dilated collecting ducts. Patients usually develop kidney stones, infection, or recurrent hematuria in the third or fourth decade. More than 50% of patients have stones. Autosomal dominant polycystic kidney disease (ADPKD) produces much larger cysts that involve the entire kidney, eventually leading to massive renomegaly. Autosomal recessive polycystic kidney disease (ARPKD) is rare and leads to bilateral, symmetric renal enlargement manifested in utero, with renal failure evident at birth. Multi-cystic renal dysplasia may occur sporadically or as part of various genetic syndromes, such as Meckel-Gruber syndrome, in fetuses and newborns.
Category:
Pathology
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