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CONTENTS Intro: Hyperammonemia is a serial killer Clinical presentation & diagnosis Causes Investigation & monitoring Management [1] Neurocritical care of hyperammonemia [2] Reduced enteral ammonia absorption [3] Basic nutritional manipulations [4] Discontinue potentially offensive medications [5] Consider treatment for infectious etiologies [6] Consider zinc supplementation [7] Consider carnitine supplementation [8] Consider additional vitamins [9] Consider L-arginine administration [10] Support renal clearance & consider CRRT [11] Sodium phenylacetate plus sodium benzoate (Ammonul) hyperammonemia is a serial killer hyperammonemia is a serial killer Nonhepatic hyperammonemia behaves like a serial killer

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Only a single multicenter, randomized, double-blind, controlled trial found positive changes in Aberrant Behavior Checklist-Community (ABC), Autism Treatment Evaluation Checklist (ATEC), and Clinical Global Impression-Improvement (CGI) scores [137]

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Primary carnitine deficiency (Carnitine uptake defect, CUD) Primary carnitine deficiency (OMIM #212140) is an autosomal recessive disorder of the carnitine cycle that results in defective fatty acid oxidation [3, 12, 82]