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Idiopathic Hyperammonemia That Developed During Initial Treatment With Steroid in a Patient With Newly Diagnosed Leukemia

Kobayashi, Shogo MD*; Ito, Masaki MD*; Sano, Hideki MD; Mochizuki, Kazuhiro MD; Akaihata, Mitsuko MD*; Waragai, Tomoko MD*; Ohara, Yoshihiro MD*; Hosoya, Mitsuaki MD*; Kikuta, Atsushi MD

Journal of Pediatric Hematology/Oncology: August 2015 - Volume 37 - Issue 6 - p e361–e363
doi: 10.1097/MPH.0000000000000255
Online Articles: Clinical and Laboratory Observations

Idiopathic hyperammonemia (IHA) has been described as a complication of intensive chemotherapy for the treatment of hematologic malignancy but has subsequently been found in patients undergoing bone marrow transplantation and in those with solid tumors treated with 5-fluorouracil. Although IHA is a rare complication, it is sometimes associated with high mortality in hematologic malignancies. Here we report the case of a 15-year-old boy in whom hyperammonemia developed during the initial treatment with prednisolone for newly diagnosed acute lymphoblastic leukemia and who survived after early detection and oral lactulose therapy. To the best of our knowledge, this is the first report of IHA that was not induced by intensive chemotherapy, stem cell transplantation, or asparaginase therapy in a patient with newly diagnosed leukemia, but developed during an initial treatment with a steroid. Early detection of IHA by measuring the plasma ammonia level in patients with neurological symptoms may improve the outcome.

Departments of *Pediatrics

Pediatric Oncology, Fukushima Medical University School of Medicine, Fukushima, Japan

The authors declare no conflict of interest.

Reprints: Shogo Kobayashi, MD, PhD, Department of Pediatrics, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima City, Fukushima 960-1295, Japan (e-mail:

Received June 22, 2014

Accepted August 8, 2014

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