| Successful management of refractory intracranial hypertension from acute hyperammonemic encephalopathy in a woman with ornithine transcarbamylase deficiency. | |
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MedLine Citation:
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PMID: 20422467 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Ornithine transcarbamylase deficiency (OTCD) is the most common of the urea cycle disorders and results in an accumulation of ammonia and its metabolites. Excess ammonia in the brain is metabolized to glutamine, which increases intracellular osmolarity and contributes to cytotoxic edema. METHODS: We report a case of a woman heterozygous for OTCD who developed acute hyperammonemic encephalopathy and increased intracranial pressure (ICP). RESULTS: Despite hemodialysis, protein restriction, and administration of pharmacologic nitrogen scavengers, she developed progressive cerebral edema and increased ICP that was refractory to maximal medical management. She underwent a bifrontal decompressive craniectomy resulting in resolution of her intracranial hypertension. CONCLUSION: Aggressive multimodality management of the patient coupled with bifrontal decompressive hemicraniectomy was a life-saving measure, offering the patient a reasonable outcome. At 6 month follow-up she had moderate disability on the Glasgow Outcome Score associated with cognitive difficulties. |
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Authors:
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Linda C Wendell; Amir Khan; Jonathan Raser; Shih-Shan Lang; Neil Malhotra; W Andrew Kofke; Peter LeRoux; Soojin Park; Joshua M Levine |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Neurocritical care Volume: 13 ISSN: 1556-0961 ISO Abbreviation: Neurocrit Care Publication Date: 2010 Aug |
Date Detail:
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Created Date: 2010-07-05 Completed Date: 2010-10-14 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 101156086 Medline TA: Neurocrit Care Country: United States |
Other Details:
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Languages: eng Pagination: 113-7 Citation Subset: IM |
Affiliation:
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Department of Neurology, Hospital of the University of Pennsylvania, Philadelphia, PA 19104, USA. linda.wendell@uphs.upenn.edu |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Acute Disease Adult Brain Diseases / etiology*, radiography Brain Edema / etiology Decompressive Craniectomy / methods* Diet, Protein-Restricted Female Follow-Up Studies Gas Scavengers Heterozygote Humans Hyperammonemia / complications*, etiology* Intracranial Hypertension / etiology*, physiopathology, radiography, therapy Nitrogen / antagonists & inhibitors Ornithine Carbamoyltransferase Deficiency Disease / complications*, genetics Renal Dialysis Retreatment Tomography, X-Ray Computed |
| Chemical | |
Reg. No./Substance:
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7727-37-9/Nitrogen |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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