title: Infectious complications of propionic acidemia in Saudia Arabia.
abstract: A retrospective study of 38 patients with propionic acidemia indicates a high frequency of infections; affecting 80% of such patients. The Saudi Arabian population studied is a product of consanguineous marriages, and presents with a severe phenotype. Most microorganisms implicated are unusual, which suggests an underlying immune deficiency. These frequent infections occur despite aggressive treatment with appropriate diets, carnitine and during acute episodes of the disease with metronidazole, which suggests a global effect of the disease on T and B lymphocytes as well as on the bone marrow cells. Any patient with propionic acidemia should be closely followed up for an intercurrent infection in association with acute metabolic decompensation.
authors.affiliation: Department of Pediatrics, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
authors.forename: M
authors.initials: M
authors.lastname: Al Essa
authors.forename: Z
authors.initials: Z
authors.lastname: Rahbeeni
authors.forename: S
authors.initials: S
authors.lastname: Jumaah
authors.forename: S
authors.initials: S
authors.lastname: Joshi
authors.forename: E
authors.initials: E
authors.lastname: Al Jishi
authors.forename: M S
authors.initials: MS
authors.lastname: Rashed
authors.forename: M
authors.initials: M
authors.lastname: Al Amoudi
authors.forename: P T
authors.initials: PT
authors.lastname: Ozand
mesh_terms: D000138:Acidosis / Q000150:complications* / Q000276:immunology / Q000382:microbiology; D006801:Humans; D007223:Infant; D007239:Infections / Q000150:complications*; D007532:Isoleucine / Q000378:metabolism; D008661:Metabolism, Inborn Errors / Q000150:complications* / Q000276:immunology / Q000382:microbiology; D012189:Retrospective Studies; D012529:Saudi Arabia
chemical_list: D007532:Isoleucine