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Hyperammonemia Physiology and Management: A Nephrology-Centered Review
Patrick Hofmann1, Danielle L Saly2
1Nephrology Division, Department of Internal Medicine, Massachusetts General Hospital, Boston, MA; Harvard Medical School, Boston, MA; Harvard T.H. Chan School of Public Health, Boston, MA.
Abstract:
Hyperammonemia is a rare but life-threatening disorder that arises from hepatic and diverse nonhepatic etiologies and demands prompt physiologic evaluation and intervention to prevent neurologic injury. This review highlights the physiological roles of the intestine, liver, skeletal muscle, brain, and kidneys in ammonia homeostasis, with emphasis on acid-base and potassium regulation. We summarize major causes of hyperammonemia, diagnostic strategies, and nondialytic therapies, review principles guiding kidney replacement therapy selection and dosing, and identify key uncertainties that warrant future study in adult populations.
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