Question of the Week # 14

Q14) A 46 y/o woman who is a school bus driver by occupation presents to your office for regular follow up. She has a history of  ADPKD. Her blood pressure is well controlled at 120/70 on enalapril. She has no other problems. She denies any headache. There is no family history of intracranial or subarachnoid hemorrhage. However, she is concerned that her head might explode because her sister who also has ADPKD was recently diagnosed of having a berry aneurysm. She wants to be screened for berry aneurysm as soon as possible. Her physical examination is benign and does not reveal any focal neurological deficits. Which of the following suggests the necessity for screening in her case?

A. Family history of berry aneurysm
B. Polycystic kidneys
C. School bus driving
D. Cysts in the liver
E. No screening necessary in her case

Copy Rights: Archer

16 Responses

  1. A.

  2. A

  3. E: The sensitivity of renal ultrasonography for the detection of ADPKD is 100% for subjects 30 years or older with a positive family history. Diagnostic criteria require two or more cysts in one kidney and at least one cyst in the contralateral kidney in young subjects, but four or more in subjects older than 60 years because of the increased frequency of benign simple cysts. Most often, the diagnosis is made from a positive family history and imaging studies showing large kidneys with multiple bilateral cysts and possibly liver cysts. Before the age of 30 years, CT scan or T2-weighted MRI is more sensitive for detecting presymptomatic disease because the sensitivity of ultrasound falls to 95% for ADPKD type 1 and <70% for ADPKD type 2.Genetic counseling is essential for those being screened. It is recommended that screening for asymptomatic intracranial aneurysms should be restricted to patients with a personal or family history of intracranial hemorrhage. Intervention should be limited to aneurysms larger than 10 mm. Someone with this disease has a 5% chance of getting brain aneurysms.

  4. E. No screening necessary as she doesn’t have family history of intracranial hemorrage.

  5. Answer. C

    High risk jobs ( pilot, bus driver etc) is one of the indications to screen for berry aneurysm in asymptomatic ADPKD patients. Family hx of berry aneurysm (Ans. A) alone does not warrant screening for berry aneurysm in asymptomatic ADPKD patients. Asymptomatic ADPKD patients must be screened if there is a family history of “Ruptured” berry aneurysm ( history of SAH in the family etc)
    E. is not the answer because this patient is a school bus driver by occupation and needs to be screened.

    Key Concepts :

    Screening for Berry Aneurysms:

    – MRA of head – recommended screening test to detect berry aneurysms.

    – Screen only if
    – family history of subarachnoid hemorrhage ( Family hx of a ruptured berry aneurysm) not just a family history of berry aneurysm.
    – Patients with with high risk jobs (pilots/ bus-drivers) – an event during such a job is a risk to other’s safety as well.
    – Patients with symptoms suggestive of a berry aneurysm
    ( severe headache, focal neurological deficits)

  6. thanks

  7. If she does have a berry aneurysm on mra and its large but she refuses treatment, are you an obligated reporter if she wishes to continue as a bus driver?

  8. Although outside the realm of Step 3, one interesting thing to note is that cyst formation in the kidney is likely associated with cAMP transmission from ADH release. So ADH suppression may aid in the prevention of and possibly the regression of cyst formation in the kidney. Considering how common this disease is, this may be another reason to advise people to drink more water. 😀

    Just thought it was an interesting example of penetrance by behavior.

  9. very good question? JUst like general knowledge—-not medical knowledge.

  10. Dr Red –Have u ever seen case of ADPKD with berry anu?
    is this case for primary care physician or neurologist???

  11. According to the Washington manual, “patients with a family history of cerebral aneurisms or with symptoms attributable to a cerebral aneurysm shiuld undergo evaluation with brain MRI/MRA.”

  12. so whats the correct answer?

  13. As per Uptodate :

    Routine screening is recommended only for high-risk patients, such as those with a previous rupture, a positive family history of a brain hemorrhage or stroke, warning symptoms (eg, unusual headaches), or a person with a high-risk occupation (eg, airline pilot), in whom a loss of consciousness would place the patient or others at extreme risk.

  14. B

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