title: Severe lactic acidosis due to thiamine deficiency in a patient with B-cell leukemia/lymphoma on total parenteral nutrition during high-dose methotrexate therapy.
abstract: An 11-month-old girl with B-cell leukemia/lymphoma developed profound lethargy due to severe lactic acidosis during chemotherapy and total parenteral nutrition (TPN). Initial treatment with NaHCO3 was ineffective. Treatment with a vitamin cocktail (OH-cobalamin, pyridoxine, thiamine, riboflavine, biotin, carnitine) at pharmacologic doses rapidly improved the child's clinical and laboratory status. Lactic acidosis was caused by an impairment of pyruvate dehydrogenase complex, which was due to lack of its necessary cofactor thiamine in the TPN. This case report indicates that lactic acidosis may be a front-line diagnosis in patients on TPN with lethargy and outlines the need for monitoring thiamine supply in TPN.
authors.affiliation: Department of Pediatric Hematology/Oncology, G. Gaslini Children's Hospital, Genova, Italy. johannasvahn@hotmail.com
authors.forename: Johanna
authors.initials: J
authors.lastname: Svahn
authors.forename: Maria Cristina
authors.initials: MC
authors.lastname: Schiaffino
authors.forename: Ubaldo
authors.initials: U
authors.lastname: Caruso
authors.forename: Michaela
authors.initials: M
authors.lastname: Calvillo
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Minniti
authors.forename: Carlo
authors.initials: C
authors.lastname: Dufour
mesh_terms: D000140:Acidosis, Lactic / Q000175:diagnosis / Q000209:etiology*; D005260:Female; D008401:Gas Chromatography-Mass Spectrometry; D006801:Humans; D007223:Infant; D019344:Lactic Acid / Q000097:blood; D015448:Leukemia, B-Cell / Q000150:complications* / Q000628:therapy; D016393:Lymphoma, B-Cell / Q000150:complications* / Q000628:therapy; D008727:Methotrexate / Q000008:administration & dosage*; D010288:Parenteral Nutrition / Q000009:adverse effects*; D011768:Pyruvate Dehydrogenase Complex / Q000378:metabolism; D012894:Sleep Stages; D013832:Thiamine Deficiency / Q000150:complications*
chemical_list: D011768:Pyruvate Dehydrogenase Complex; D019344:Lactic Acid; D008727:Methotrexate