Managing Autosomal Dominant Polycystic Kidney Disease: Key Considerations - Internal Medicine

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How to manage polycystic kidney disease?


Dear Director Wu,
My father is 70 years old.
Two years ago, he experienced sudden hypertension and dizziness.
After blood tests, he was found to have hematuria, with a creatinine level of 1.3 and a GFR (CG) of 56, indicating stage 3 chronic kidney disease (CKD).
An abdominal ultrasound revealed cysts on both kidneys, with one on the left and three on the right.
The doctor diagnosed him with Autosomal Dominant Polycystic Kidney Disease (ADPKD).
In our family, my father's sister started dialysis at 50 and passed away at 70, while his brother died at 56 due to renal failure.
Currently, my father undergoes blood tests every three months and abdominal ultrasounds every six months.
I would like to ask the following questions:
1.
The cysts are approximately 10 cm in size.
Recently, the cyst on the left kidney has been causing severe pain due to pressure on the kidney, occurring every two days for about 10 minutes.
The doctor prescribed Acetaminophen for pain relief.
Will taking this medication increase the burden on the kidneys? Does the cyst need to be drained through laparoscopic surgery at the hospital?
2.
Polycystic kidney disease often leads to hypertension, which is a major enemy of the kidneys.
Recently, with the colder weather, my father's blood pressure has increased, with a systolic pressure of 160 mmHg and diastolic pressure of 90 mmHg, causing significant dizziness.
The doctor prescribed Norvasc for blood pressure management, with one tablet taken in the morning and evening, which reduced the systolic pressure to 130 mmHg, but the diastolic pressure remains at 90 mmHg.
Additionally, he has developed very itchy red spots on his calves, possibly indicating an allergy to dihydropyridine compounds.
Should he continue taking this blood pressure medication or switch to another?
Thank you for your assistance.

peter, 30~39 year old female. Ask Date: 2008/12/15

Dr. Wu Jiyu reply Internal Medicine


1.
The left-sided cyst is compressing the kidney; consider referring to urology for "percutaneous renal cyst aspiration and sclerotherapy." If there is a recurrence, consider "laparoscopic cyst decortication" for management.
2.
Consider treating with "Norvasc + ACEI" or "Norvasc + ARB," one tablet each in the morning, as ACEIs (Monopril/Ramipril/Captopril, etc.) or ARBs (Cozaar/Diovan/Irbesartan/Micardis, etc.) are antihypertensive medications that protect the kidneys.
It is not recommended to take Norvasc twice daily.

Reply Date: 2008/12/15

More Info


Managing Autosomal Dominant Polycystic Kidney Disease (ADPKD) involves a comprehensive approach that addresses both the renal complications and associated systemic issues, particularly hypertension. Given your father's situation, it is crucial to consider the following key aspects:

Pain Management and Cyst Management
1. Pain Management with Acetaminophen: Acetaminophen is generally considered safe for patients with kidney disease when used at recommended doses. However, it is essential to monitor the total daily intake to avoid potential liver toxicity, especially if there are underlying liver issues. In your father's case, since he is experiencing significant pain due to the cysts, using acetaminophen as prescribed should not significantly increase the burden on his kidneys. However, if the pain persists or worsens, further evaluation may be necessary.

2. Cyst Management: The presence of large cysts (approximately 10 cm) causing pain may require intervention. Options include:
- Percutaneous Cyst Aspiration: This is a minimally invasive procedure where a needle is inserted into the cyst to drain the fluid. This can provide temporary relief from pain.

- Laparoscopic Surgery: If cysts are recurrent or significantly symptomatic, surgical intervention may be warranted. This could involve cystectomy or other procedures to alleviate pressure on the kidney.


Hypertension Management
Hypertension is a common complication of ADPKD and can exacerbate kidney damage. Your father's blood pressure readings indicate that he is experiencing significant hypertension, particularly with a systolic pressure of 160 mmHg. Here are some considerations:
1. Medication Review: Norvasc (amlodipine) is a calcium channel blocker effective for hypertension, but if your father is experiencing allergic reactions (itchy red spots), it may be necessary to switch to another antihypertensive class. Alternatives include:
- ACE Inhibitors (e.g., lisinopril): These not only lower blood pressure but also provide renal protection, particularly beneficial in patients with ADPKD.

- Angiotensin II Receptor Blockers (ARBs): Similar to ACE inhibitors, ARBs can help manage blood pressure and protect kidney function.

2. Monitoring and Follow-Up: Regular monitoring of blood pressure is crucial. If the current regimen does not adequately control blood pressure or if side effects persist, a consultation with a nephrologist or a hypertension specialist may be beneficial.


Family History and Genetic Considerations
Given the family history of ADPKD, it is important to consider genetic counseling and testing for other family members. Early detection through imaging (ultrasound or MRI) can help identify asymptomatic individuals who may be at risk for developing significant kidney disease later in life.


Lifestyle Modifications
Encouraging lifestyle changes can also play a significant role in managing ADPKD:
- Dietary Adjustments: A diet low in sodium can help manage blood pressure. Adequate hydration is also essential, but fluid intake should be tailored to individual kidney function.

- Regular Exercise: Engaging in regular physical activity can help control blood pressure and improve overall health.

- Avoiding Nephrotoxic Substances: It is crucial to avoid medications and substances that can harm kidney function, including non-steroidal anti-inflammatory drugs (NSAIDs) and certain antibiotics.


Conclusion
In summary, managing ADPKD requires a multifaceted approach that includes effective pain management, careful monitoring and treatment of hypertension, and consideration of genetic factors. Regular follow-ups with healthcare providers specializing in nephrology are essential to optimize care and address any emerging complications. Your father's situation is complex, and ongoing communication with his healthcare team will be vital in navigating his treatment plan effectively.

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