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What's UP Doc? Acute porphyria and kidney health

  • Jul 15, 2022
  • Acute Porphyrias, What's UP Doc
  • Dr. Herbert Bonkovsky


What's UP Doc? Is a monthly column where we feature a patient question along with a response from a member of the UPA Scientific Advisory Board.

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I heard a doctor in a presentation say that people with porphyria should “look after their kidneys.” How do you do that?

This week our response was provided by Dr. Herbert Bonkovsky.

Persons with acute hepatic porphyrias are at increased risk to develop chronic kidney disease. One likely cause of this is increased levels of ALA, which occur in some patients with AHPs.

Among other factors that can lead to chronic kidney damage are systemic arterial hypertension [high blood pressure], which should be looked for at least twice per year, such as during periodic visits with primary care providers. Many persons now have devices for monitoring BP. The idea is not more than 130/80 mmHg. Diabetes mellitus is another and major risk factor for development of chronic kidney disease, so avoiding obesity and avoiding foods with high glycemic indices are best. Avoidance of or prompt treatment of urinary tract infections is also important. Some medications can damage the kidneys and are better avoided if possible. For example amino glycoside antibiotics, chronic use of NSAIDs, chronic use of high doses of acetaminophen, which some patients take for chronic pain syndromes. Givosiran is known to lead to some adverse renal effects, so any patients chronically receiving givosiran should have urinalysis, urine protein and creatinine, and CMP with estimation of GFR performed at least every 6 months. If evidence of progressive increase in serum creatinine or urinary protein occurs, or decrease in eGFR, greater than 10% below baseline, additional evaluation should be undertaken by a medical kidney specialist [nephrologist].


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Meet Dr. Herbert Bonkovsky

"With rare diseases, you could call every patient a clinical trial of one because each one is unique. It is important to listen to them and try to address their symptoms."

Dr. Herbert L. Bonkovsky