An 18-Year-Old Woman With Hyperammonemia

Sehli, Sharmila; Nugent, Kenneth; Samathanam, Christina; Alalawi, Raed

doi:10.1378/chest.08-1611

Cited by 1 publication

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“…5 Six enzymes are required for the conversion of ammonia to urea and deficiencies in these enzymes or in transporters cause urea cycle disorders and the accumulation of ammonia and precursor metabolites. 6 High-dose cytoreductive treatment might be responsible for the inactivation of glutamine synthetase. In our patient, normal levels of glutamine, arginine, and citrulline and absent argininosuccinic acid argued against the presence of urea cycle disorders, which made heterozygous ornithine transcarbamylase deficiency unlikely.…”

Section: Discussionmentioning

confidence: 99%

Hyperammonemic Encephalopathy After Induction Chemotherapy for Acute Lymphoblastic Leukemia

Jaing¹,

Lin²,

Lin

et al. 2009

Journal of Pediatric Hematology/Oncology

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The syndrome of hyperammonemic encephalopathy occurs in patients who have received high-dose cytoreductive therapy for the treatment of hematologic malignancy. It is characterized by acute alteration in mental status and respiratory alkalosis associated with markedly elevated plasma ammonium levels in the absence of any identifiable cause and frequently results in cerebral edema, coma, and eventually death. Although the etiology of this syndrome is yet to be determined, it seems to be invariably multifactorial in nature. Optimal therapy remains elusive, and the critical step is increased awareness of the syndrome by measurement of plasma ammonia levels in patients with neurologic symptoms, leading to accurate diagnosis and the rapid implementation of therapy.

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Section: Discussionmentioning

confidence: 99%