title: Acute management of propionic acidemia.
abstract: Propionic acidemia or aciduria is an intoxication-type disorder of organic metabolism. Patients deteriorate in times of increased metabolic demand and subsequent catabolism. Metabolic decompensation can manifest with lethargy, vomiting, coma and death if not appropriately treated. On January 28-30, 2011 in Washington, D.C., Children's National Medical Center hosted a group of clinicians, scientists and parental group representatives to design recommendations for acute management of individuals with propionic acidemia. Although many of the recommendations are geared toward the previously undiagnosed neonate, the recommendations for a severely metabolically decompensated individual are applicable to any known patient as well. Initial management is critical for prevention of morbidity and mortality. The following manuscript provides recommendations for initial treatment and evaluation, a discussion of issues concerning transport to a metabolic center (if patient presents to a non-metabolic center), acceleration of management and preparation for discharge.
authors: Children's National Medical Center, Washington, DC 20010, USA. kchapman@childrensnational.org
authors: Kimberly A
authors: KA
authors: Chapman
authors: Andrea
authors: A
authors: Gropman
authors: Erin
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authors: MacLeod
authors: Kathy
authors: K
authors: Stagni
authors: Marshall L
authors: ML
authors: Summar
authors: Keiko
authors: K
authors: Ueda
authors: Nicholas
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authors: Ah Mew
authors: Jill
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authors: Franks
authors: Eddie
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authors: Island
authors: Dietrich
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authors: Matern
authors: Loren
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authors: Pena
authors: Brittany
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authors: Smith
authors: V Reid
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authors: Sutton
authors: Tiina
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authors: Urv
authors: Charles
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authors: Venditti
authors: Anupam
authors: A
authors: Chakrapani
mesh_terms: D006287:Health Planning Guidelines; D006801:Humans; D056693:Propionic Acidemia / Q000178:diet therapy / Q000628:therapy*