questions 7

Adult Polycystic Kidney

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Topic updated on 03/08/17 4:23pm

Snapshot
    ADPKD
  • A 32-year-old man has diastolic hypertension and hematuria. There is positive family history of hypertension in his father, who is on renal dialysis, and his paternal grandmother who died of a stroke. He complains of discomfort in the abdomen. His serum creatinine is normal. An ultrasound of the kidneys reveals numerous black spots in the kidney parenchyma.
Introduction
  • Autosomal dominant disease (90% chromosome 16 mutation) 
  • Patients do not have cysts at birth
  • Kidney function is usually retained until the third or fourth decade of life
  • Associated with
    • cysts in the liver ( 30% ) 
    • berry aneurysms of the circle of Willis ( 10-15%)   
    • mitral valve prolapse (MVP)
    • diverticulosis
Presentation
  • Symptoms
    • hypertension
    • hematuria
    • abdominal discomfort
    • progressive renal failure
  • Physical exam 
    • bilateral flank masses
Evaluation
  • Ultrasound reveals numerous bilateral black spots in the kidney parenchyma
Treatment
  • Long term hemodialysis
  • Kidney transplantation


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Qbank (7 Questions)

TAG
(M3.RL.22) A 40-year-old woman presents to your office with a history of "bad kidneys in the family." She states that she has recently moved to the area and that she would like to establish you as her primary care physician. She brings a previous imaging study with her that she had completed prior to moving to town, which is demonstrated in Figure F. What is the most common complication of this pathology found outside of the kidneys? Topic Review Topic
FIGURES: A   B   C   D   E   F

1. Figure A
2. Figure B
3. Figure C
4. Figure D
5. Figure E

PREFERRED RESPONSE ▶
TAG
(M3.RL.24) A 42-year-old man presents to his physician with dark urine and intermittent flank pain. He has no significant past medical history. Vital signs are as follows: Temp 37C, HR 78, BP 180/105, RR 13, and O2 saturation 99% on room air. Physical examination is significant for bilateral palpable flank masses. Urinary analysis is positive for hemoglobin. Which of the following diagnostic modalities should be used to screen members of this patient's family to assess if they are affected by the same condition? Topic Review Topic

1. Abdominal CT
2. Renal ultrasound
3. Renal biopsy
4. Voiding cystourethrogram
5. Genetic sequencing

PREFERRED RESPONSE ▶
TAG
(M2.RL.45) A 45-year-old man presents to general medical clinic with flank pain and dark urine. He has no significant past medical history. Vital signs are temperature 37 degrees Celsius, heart rate 75 beats per minute, blood pressure 185/105, respiratory rate 12, and oxygen saturation 99% on room air. Physical examination is significant for bilateral palpable flank masses. Urinary analysis is positive for heme. MRI shows the following in Figure A. Given this patient's underlying disease, he is at greater risk for which of the following neurologic complications? Topic Review Topic
FIGURES: A   B   C   D   E   F

1. Figure B
2. Figure C
3. Figure D
4. Figure E
5. Figure F

PREFERRED RESPONSE ▶
TAG
(M2.RL.56) A 45-year-old man with a history of hypertension presents to his primary doctor complaining of bloody urine. He is worried that he may have the same disease as his mother. His vitals are as follows: T 97.5 HR 85 BP 145/82 RR 12 SpO2 100% on room air. His physical exam is unremarkable. A urine analysis is notable for 20-30 RBCs. His renal ultrasound is shown in Figure A. What is the probability that his daughter will also have this disease if his wife is unaffected? Topic Review Topic
FIGURES: A          

1. 0%
2. 25%
3. 50%
4. 75%
5. 100%

PREFERRED RESPONSE ▶
TAG
(M2.RL.62) A 39-year-old male is rushed to the emergency department after he developed a sudden-onset severe headache with ensuing nausea, vomiting, vision changes, and loss of consciousness. Past medical history is unattainable. He reports that the headache is worse than any he has experienced before. Noncontrast CT of the head is significant for an intracranial hemorrhage. Follow-up cerebral angiography is performed and shows a ruptured anterior communicating artery aneurysm. Which of the following has the strongest association with this patient's current presentation? Topic Review Topic

1. History of multiple hemangioblastomas of the retina and spine as well as pheochromocytoma
2. Brain MRI showing a butterfly glioma with a central necrotic core
3. Abdominal CT suggestive of renal cell carcinoma
4. Kidney ultrasound showing numerous bilateral renal cysts
5. History of renal transplantation at 8 years of age

PREFERRED RESPONSE ▶
TAG
(M2.RL.75) A 47-year-old male with a history of migraines presents to the emergency department with 10/10 pain caused by a severe headache. He states the headache started suddenly while he was mowing the lawn and didn't appear to have any precipitating factors. A CT scan is immediately obtained which demonstrates the findings shown in Figure F, the patient is admitted to the hospital, and the appropriate management is initiated. The inpatient team pursues a workup to identify the underlying cause of his presentation and obtains a renal ultrasound which shows multiple bilateral cysts. Which figure correctly represents the mode of inheritance of this patient's underlying condition? Topic Review Topic
FIGURES: A   B   C   D   E   F

1. Figure A
2. Figure B
3. Figure C
4. Figure D
5. Figure E

PREFERRED RESPONSE ▶
TAG
(M2.RL.172) A 32-year-old female presents to general medical clinic with a chief complaint of blood in her urine. She has no pain urinating and denies any fevers. Vital signs include Temp 37 C, HR 70, BP 185/95, RR 12, and O2 sat 99% on room air. Her urinary analysis is positive for heme but negative for white cells. Serum creatinine is 3.5. Later in the patient's course, she is treated with a transplant. A representative image of her organ is shown in Figure A. Which of the following is true regarding this illness? Topic Review Topic
FIGURES: A          

1. This disease is associated with gastroesophageal reflux disease
2. In the autosomal dominant form of the disease, patients most often present in childhood
3. In the autosomal recessive form of the disease, patients most often present in adulthood
4. Cysts in the spleen often accompany this disease
5. This disease is often accompanied by a valvular abnormality characterized by a mid systolic click

PREFERRED RESPONSE ▶


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