Practice questions renal cysts

Practice questions I
Renal cysts
© Jun Wang, MD, PhD

1. Use this case for next four questions. A 39-year-old man presents with vague right flank pain for 6 months. He has a history of intermittent headache and hypertension. Physical examination reveal a blood pressure at 160/110 mmHg and bulging abdomen. His vital signs are otherwise normal. No other abnormalities are seen. No mass can be palpated. CBC and liver, renal functions tests are within normal range. Abdominal sonographic examination reveals multiple cystic changes in enlarged kidneys and liver. Kidney biopsies reveal cysts lined by flattened epithelium in a background of fibrosis. Scattered normal appearing glomeruli are seen. No cytological atypia nor necrosis are seen. What is the most likely diagnosis?
A. Autosomal dominant polycystic kidney disease
B. Autosomal recessive polycystic kidney disease
C. Cystic renal cell carcinoma
D. Medullary sponge kidney disease
E. Simple cyst

2. A 39-year-old man presents with vague right flank pain for 6 months. He has a history of intermittent headache and hypertension. Physical examination reveal a blood pressure at 160/110 mmHg and bulging abdomen. His vital signs are otherwise normal. No other abnormalities are seen. No mass can be palpated. CBC and liver, renal functions tests are within normal range. Abdominal sonographic examination reveals multiple cystic changes in enlarged kidneys and liver. Kidney biopsies reveal cysts lined by flattened epithelium in a background of fibrosis. Scattered normal appearing glomeruli are seen. No cytological atypia nor necrosis are seen. What genetic abnormality is the most likely seen in this patient?
A. NPHP-2
B. PKD1
C. PKHD1
D. TFE3
E. VHL

3. A 39-year-old man presents with vague right flank pain for 6 months. He has a history of intermittent headache and hypertension. Physical examination reveal a blood pressure at 160/110 mmHg and bulging abdomen. His vital signs are otherwise normal. No other abnormalities are seen. No mass can be palpated. CBC and liver, renal functions tests are within normal range. Abdominal sonographic examination reveals multiple cystic changes in enlarged kidneys and liver. Kidney biopsies reveal cysts lined by flattened epithelium in a background of fibrosis. Scattered normal appearing glomeruli are seen. No cytological atypia nor necrosis are seen. What clinical condition will the most likely develop in this patient?
A. End stage renal disease
B. Hepatocellular carcinoma
C. Liver fibrosis
D. Renal cell carcinoma
E. Respiratory failure

4. A 39-year-old man presents with vague right flank pain for 6 months. He has a history of intermittent headache and hypertension. Physical examination reveal a blood pressure at 160/110 mmHg and bulging abdomen. His vital signs are otherwise normal. No other abnormalities are seen. No mass can be palpated. CBC and liver, renal functions tests are within normal range. Abdominal sonographic examination reveals multiple cystic changes in enlarged kidneys and liver. Kidney biopsies reveal cysts lined by flattened epithelium in a background of fibrosis. Scattered normal appearing glomeruli are seen. No cytological atypia nor necrosis are seen. What findings would most likely revealed by liver biopsy?
A. Congenital portal fibrosis with increased bile ducts at periphery
B. Cysts lined by normal appearing biliary epithelium
C. Hepatocytes with cytoplasmic clumps of cytokeratin complexed with other proteins
D. Neutrophilic portal infiltrate
E. Plasmacytic portal infiltrate


5. Use this case for next four questions. A male newborn presents with severe respiratory distress and gasping breathing. He was born to non-consanguineous parents with an gestational age of 35 weeks. Oligohydramnion was noted prenatally. Physical examination reveals an arterial systolic pressure at 122 mmHg (normal 67-84 mmHg). He has a recessed chin, flattened ears and nose, and club feet. His abdomen is markedly distended. Laboratory test results includes a BUN at 97 mg/dl (normal 5-17 mg/dl), creatinine at 6.7mg/dl (normal 0.2-1.0). Sonographic examination reveal marked enlarged kidneys with cystic changes. He was treated with mechanical ventilation, furosemide and hydrochlorothiazide, but his conditions deteriorate and he died the second day. Autopsy finding of the kidneys included markedly enlarged kidney with dilated ducts perpendicularly to the kidney surface. What is the most likely diagnosis?
A. Autosomal dominant polycystic kidney disease
B. Autosomal recessive polycystic kidney disease
C. Cystic renal cell carcinoma
D. Medullary sponge kidney disease
E. Simple cyst

6. A male newborn presents with severe respiratory distress and gasping breathing. He was born to non-consanguineous parents with an gestational age of 35 weeks. Oligohydramnion was noted prenatally. Physical examination reveals an arterial systolic pressure at 122 mmHg (normal 67-84 mmHg). He has a recessed chin, flattened ears and nose, and club feet. His abdomen is markedly distended. Laboratory test results includes a BUN at 97 mg/dl (normal 5-17 mg/dl), creatine at 6.7mg/dl (normal 0.2-1.0). Sonographic examination reveal marked enlarged kidneys with cystic changes. He was treated with mechanical ventilation, furosemide and hydrochlorothiazide, but his conditions deteriorate and he died the seconde day. Autopsy finding of the kidneys included markedly enlarged kidney with dilated ducts perpendicularly to the kidney surface. What genetic abnormality is the most likely seen in this patient?
A. NPHP-2
B. PKD1
C. PKHD1
D. TFE3
E. VHL

7. A male newborn presents with severe respiratory distress and gasping breathing. He was born to non-consanguineous parents with an gestational age of 35 weeks. Oligohydramnion was noted prenatally. Physical examination reveals an arterial systolic pressure at 122 mmHg (normal 67-84 mmHg). He has a recessed chin, flattened ears and nose, and club feet. His abdomen is markedly distended. Laboratory test results includes a BUN at 97 mg/dl (normal 5-17 mg/dl), creatine at 6.7mg/dl (normal 0.2-1.0). Sonographic examination reveal marked enlarged kidneys with cystic changes. He was treated with mechanical ventilation, furosemide and hydrochlorothiazide, but his conditions deteriorate and he died the seconde day. Autopsy finding of the kidneys included markedly enlarged kidney with dilated ducts perpendicularly to the kidney surface. What is the underlying cause of his respiratory distress?
A. Congestive heart failure
B. Hypervolemia
C. Pneumonia
D. Pulmonary hypoplasia
E. Uremia

8. A male newborn presents with severe respiratory distress and gasping breathing. He was born to non-consanguineous parents with an gestational age of 35 weeks. Oligohydramnion was noted prenatally. Physical examination reveals an arterial systolic pressure at 122 mmHg (normal 67-84 mmHg). He has a recessed chin, flattened ears and nose, and club feet. His abdomen is markedly distended. Laboratory test results includes a BUN at 97 mg/dl (normal 5-17 mg/dl), creatine at 6.7mg/dl (normal 0.2-1.0). Sonographic examination reveal marked enlarged kidneys with cystic changes. He was treated with mechanical ventilation, furosemide and hydrochlorothiazide, but his conditions deteriorate and he died the seconde day. Autopsy finding of the kidneys included markedly enlarged kidney with dilated ducts perpendicularly to the kidney surface. What is the most likely finding in his liver?
A. Benign hepatocytes with ground glass appearing cytoplasm
B. Fibrotic bands and nodular islands of hepatocytes
C. Lymphocytic infiltration at portal area
D. Portal fibrosis with bile duct proliferation
E. Vacuolar cytoplasmic changes of hepatocytes


9. Use this case for next four questions. A 10-year-old girl presents with chronic fatigue for 6 months. She has history of polyuria and polydipsia.  Her history is unremarkable, except her mother has certain type of kidney disease. Her heights and weight are in the 3rd percentile. She appears to be slightly pale. Laboratory tests results include hemoglobin at 8.1 g/dl (normal 10.9–13.3 g/dl). Her CBC is otherwise unremarkable. Her BUN is 28 mg/dl (normal 7-17 mg/dl), creatinine is 2.9 mg/dl (normal 0.3-0.7 mg/dl). Sonographic examinations reveal slightly smaller than normal kidneys, but her liver and spleen are mildly enlarged. Renal biopsies reveal small cysts lined by flat cells, primarily located at the junction of cortex and medulla, in a background of atrophic tubules, thickened basement membranes, and interstitial fibrosis. Scattered lymphocytes and plasma cells are seen. There is no evidence of atypia or necrosis. What is the most likely diagnosis?
A. Autosomal dominant polycystic kidney disease
B. Autosomal recessive polycystic kidney disease
C. Cystic renal cell carcinoma
D. Medullary sponge kidney disease
E. Nephronophthisis

10. A 10-year-old girl presents with chronic fatigue for 6 months. She has history of polyuria and polydipsia. Her history is unremarkable, except her mother has certain type of kidney disease. Her heights and weight are in the 3rd percentile. She appears to be slightly pale. Laboratory tests results include hemoglobin at 8.1 g/dl (normal 10.9–13.3 g/dl). Her CBC is otherwise unremarkable. Her BUN is 28 mg/dl (normal 7-17 mg/dl), creatinine is 2.9 mg/dl (normal 0.3-0.7 mg/dl). Sonographic examinations reveal slightly smaller than normal kidneys, but her liver and spleen are mildly enlarged. Renal biopsies reveal small cysts lined by flat cells, primarily located at the junction of cortex and medulla, in a background of atrophic tubules, thickened basement membranes, and interstitial fibrosis. Scattered lymphocytes and plasma cells are seen. There is no evidence of atypia or necrosis. Abnormality of what gene is most likely associated with her condition?
A. NPHP
B. PKD1
C. PKHD1
D. TFE3
E. VHL

11. A 10-year-old girl presents with chronic fatigue for 6 months. She has history of polyuria and polydipsia. Her history is unremarkable, except her mother has certain type of kidney disease. Her heights and weight are in the 3rd percentile. She appears to be slightly pale. Laboratory tests results include hemoglobin at 8.1 g/dl (normal 10.9–13.3 g/dl). Her CBC is otherwise unremarkable. Her BUN is 28 mg/dl (normal 7-17 mg/dl), creatinine is 2.9 mg/dl (normal 0.3-0.7 mg/dl). Sonographic examinations reveal slightly smaller than normal kidneys, but her liver and spleen are mildly enlarged. Renal biopsies reveal small cysts lined by flat cells, primarily located at the junction of cortex and medulla, in a background of atrophic tubules, thickened basement membranes, and interstitial fibrosis. Scattered lymphocytes and plasma cells are seen. There is no evidence of atypia or necrosis. What is most likely complication to her after a few years?
A. End stage renal disease
B. Liver failure
C. Portal hypertension
D. Renal cell carcinoma
E. Wilms tumor

12. A 10-year-old girl presents with chronic fatigue for 6 months. She has history of polyuria and polydipsia. Her history is unremarkable, except her mother has certain type of kidney disease. Her heights and weight are in the 3rd percentile. She appears to be slightly pale. Laboratory tests results include hemoglobin at 8.1 g/dl (normal 10.9–13.3 g/dl). Her CBC is otherwise unremarkable. Her BUN is 28 mg/dl (normal 7-17 mg/dl), creatinine is 2.9 mg/dl (normal 0.3-0.7 mg/dl). Sonographic examinations reveal slightly smaller than normal kidneys, but her liver and spleen are mildly enlarged. Renal biopsies reveal small cysts lined by flat cells, primarily located at the junction of cortex and medulla, in a background of atrophic tubules, thickened basement membranes, and interstitial fibrosis. Scattered lymphocytes and plasma cells are seen. There is no evidence of atypia or necrosis. What is most likely associated with her elevated BUN and creatinine?
A. Glomerular inflammation and regeneration
B. Impaired renal tubular function
C. Recurrent bacterial infection
D. Reduced insulin production
E. Urinary tract obstruction


13. Use this case for next two questions. A 27-year-old man presents with recurrent hematuria and kidney stones. His past medical history and physical examination are unremarkable. Urinalysis reveal markedly increased red blood cells and neutrophils. No atypical urothelial cells are identified. CT reveals normal-sized kidneys with medullary tubular dilation and scattered calcifications. The cortex is unremarkable. What is the most likely diagnosis?
A. Autosomal dominant polycystic kidney disease
B. Autosomal recessive polycystic kidney disease
C. Cystic renal cell carcinoma
D. Medullary sponge kidney disease
E. Nephronophthisis

14. A 27-year-old man presents with recurrent hematuria and kidney stones. His past medical history and physical examination are unremarkable. Urinalysis reveal markedly increased red blood cells and neutrophils. No atypical urothelial cells are identified. CT reveals normal-sized kidneys with medullary tubular dilation and scattered calcifications. Abnormality of which part of nephron is associated with his conditions?
A. Cortical and medullary collecting ducts
B. Distal convoluted tubules
C. Loop of Henle
D. Medullary collecting ducts
E. Proximal convoluted tubules


15. Use this case for next two questions. A 2-year-old girl presents with right abdominal mass for 6 months. She has a history of recurrent urinary tract infection since 6-month-old. Her blood pressure is 150/90 mmHg (normal 86-106/42-63 mmHg). Physical examination confirms a non-tender mass at the mid right abdomen. No other abnormality is seen. Sonographic examination  reveal enlarged right kidney but is inconclusive about whether there are cystic changes. The liver, pancreas, and spleen are unremarkable. Microscopic examination of biopsy reveals multiple cysts lined by flat cells in a background of fibrotic stroma. A few glomeruli are seen. There is no cytological atypia nor necrosis. What additional test would most likely yield helpful information for making diagnosis?
A. Abdominal X-ray
B. Dimercaptosuccinic acid renal scanning
C. Retrograde pyelography
D. Ureteropyeloscopy
E. Urine cytology
 
16. A 2-year-old girl presents with right abdominal mass for 6 months. She has a history of recurrent urinary tract infection since 6-month-old. Her blood pressure is 150/90 mmHg (normal 86-106/42-63 mmHg). Physical examination confirms a non-tender mass at the mid right abdomen. No other abnormality is seen. Sonographic examination  reveal enlarged right kidney but is inconclusive about whether there are cystic changes. The liver, pancreas, and spleen are unremarkable. Microscopic examination of biopsy reveals multiple cysts lined by flat cells in a background of fibrotic stroma. A few glomeruli are seen. There is no cytological atypia nor necrosis. Right side nephrectomy was performed and reveal a cystic appearing kidney with no grossly recognizable cortical medulla differentiation. Microscopic examination reveals multiple cysts lined by flat cells in a background of fibrotic stroma. A few glomeruli are seen. There is no cytological atypia nor necrosis. What is the most likely diagnosis?
A. Autosomal recessive polycystic kidney disease
B. Kidney hypoplasia
C. Medullary sponge kidney disease
D. Multicystic renal dysplasia
E. Nephronophthisis


17. Use this case for next three questions. A 62-year-old man presents with right flank discomfort for 6 months. He has a history of type II diabetes and diabetic nephropathy associated renal failure for 20 years. He is currently on peritoneal dialysis therapy. His past medical history is otherwise unremarkable. Physical examination is unremarkable. His CBC reveal a hemoglobin at 7.3 g/dL (normal 13-18 g/dL). Renal function test results include BUN at 92 mg/dL (normal 8-18 mg/dL), creatinine at 14 mg/dL (normal 0.6-1.2 mg/dL). His other laboratory results are within normal range. Sonographic examination reveals slightly enlarged kidneys with several cystic lesions in each kidney, measuring up to 2.5 cm in greatest dimension. His liver, pancreas and spleen are unremarkable. Biopsy reveal cysts in both cortex and medulla lined by flattened epithelium. Oxalate crystals are seen. There is no evidence of cytological atypia. What is the most likely diagnosis?
A. Acquired cystic renal disease
B. Autosomal dominant polycystic kidney disease
C. Cystic renal cell carcinoma
D. Multicystic renal dysplasia
E. Nephronophthisis

18. A 62-year-old man presents with right flank discomfort for 6 months. He has a history of type II diabetes and diabetic nephropathy associated renal failure for 20 years. He is currently on peritoneal dialysis therapy. His past medical history is otherwise unremarkable. Physical examination is unremarkable. His CBC reveal a hemoglobin at 7.3 g/dL (normal 13-18 g/dL). Renal function test results include BUN at 92 mg/dL (normal 8-18 mg/dL), creatinine at 14 mg/dL (normal 0.6-1.2 mg/dL). His other laboratory results are within normal range. Sonographic examination reveals slightly enlarged kidneys with several cystic lesions in each kidney, measuring up to 2.5 cm in greatest dimension. His liver, pancreas and spleen are unremarkable. Biopsy reveal cysts in both cortex and medulla lined by flattened epithelium. Oxalate crystals are seen. There is no evidence of cytological atypia. What is the most likely causing these cystic changes?
A. Congenital cilia malfunction in the renal tubule epithelium
B. Defects of collecting duct differentiation
C. Recurrent infection associated renal parenchyma damage
D. Side effects of peritoneal dialysis
E. Uremic status

19. A 62-year-old man presents with right flank discomfort for 6 months. He has a history of type II diabetes and diabetic nephropathy associated renal failure for 20 years. He is currently on peritoneal dialysis therapy. His past medical history is otherwise unremarkable. Physical examination is unremarkable. His CBC reveal a hemoglobin at 7.3 g/dL (normal 13-18 g/dL). Renal function test results include BUN at 92 mg/dL (normal 8-18 mg/dL), creatinine at 14 mg/dL (normal 0.6-1.2 mg/dL). His other laboratory results are within normal range. Sonographic examination reveals slightly enlarged kidneys with several cystic lesions in each kidney, measuring up to 2.5 cm in greatest dimension. His liver, pancreas and spleen are unremarkable.

He developed right flank pain and hematuria a year later. CT reveal a 1.5 cm solid mass at the right kidney. Biopsy reveal kidney parenchyma with multiple cysts lined by markedly atypical cells. Oxalate crystals are seen. What is the most likely diagnosis?
A. Acquired cystic renal disease
B. Autosomal dominant polycystic kidney disease
C. Cystic renal cell carcinoma
D. Multicystic renal dysplasia
E. Nephronophthisis


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