title: A patient with propionic acidemia managed with continuous insulin infusion and total parenteral nutrition.
abstract: An infant newly diagnosed with propionic acidemic coma was managed successfully with total parenteral nutrition (TPN) and continuous infusion of insulin. The urinary excretion of 3-hydroxypropionic acid was reduced to 3% of the admission value in 4 days, gradually decreasing to 1.5% in 16 days. The treatment did not prevent a prolonged episode of thrombocytopenia. The infant tolerated TPN well, except for continued hyper-lactic acidemia (2 to 4 times normal). Metabolic acidosis and mild hyperammonemia recurred only when the patient had sepsis secondary to Candida albicans and Staphylococcus aureus infection.
authors.affiliation: Department of Pediatrics, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
authors.forename: A
authors.initials: A
authors.lastname: Kalloghlian
authors.forename: H
authors.initials: H
authors.lastname: Gleispach
authors.forename: P T
authors.initials: PT
authors.lastname: Ozand
mesh_terms: D000592:Amino Acid Metabolism, Inborn Errors / Q000097:blood / Q000628:therapy*; D001928:Brain Diseases, Metabolic / Q000097:blood / Q000628:therapy*; D003128:Coma / Q000097:blood / Q000628:therapy*; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007223:Infant; D007262:Infusions, Intravenous; D007328:Insulin / Q000008:administration & dosage*; D010289:Parenteral Nutrition, Total; D011422:Propionates / Q000097:blood*
chemical_list: D007328:Insulin; D011422:Propionates; C029658:propionic acid