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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