title: VLCAD deficiency in a patient who recovered from ventricular fibrillation, but died suddenly of a respiratory syncytial virus infection.
abstract: VLCAD deficiency is an autosomal recessive disorder caused by a defect of fatty acid oxidation. The phenotype is classified into three clinical forms on the basis of the onset of symptoms: a severe form with neonatal onset; a milder form with childhood onset; and a late-onset form. The neonatal form is the most common, and has a higher mortality rate than the others. We report the case of a newborn infant with VLCAD deficiency who developed ventricular fibrillation, which was successfully treated by intensive care, but who suddenly died after a respiratory syncytial virus infection. Early institution of i.v. glucose treatment and active immunization with vaccine, such as palivizumab (anti-RSV mAb), may be important to reduce the frequency and severity of life-threatening episodes.
authors: Department of Pediatrics, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
authors: Akiko
authors: A
authors: Yamamoto
authors: Kimitoshi
authors: K
authors: Nakamura
authors: Shirou
authors: S
authors: Matsumoto
authors: Masanori
authors: M
authors: Iwai
authors: Yosuke
authors: Y
authors: Shigematsu
authors: Go
authors: G
authors: Tajima
authors: Miyuki
authors: M
authors: Tsumura
authors: Satoshi
authors: S
authors: Okada
authors: Hiroshi
authors: H
authors: Mitsubuchi
authors: Fumio
authors: F
authors: Endo
mesh_terms: D044942:Acyl-CoA Dehydrogenase, Long-Chain / Q000172:deficiency*; D002675:Child, Preschool; D000080984:Congenital Bone Marrow Failure Syndromes; D017809:Fatal Outcome; D006801:Humans; D008052:Lipid Metabolism, Inborn Errors / Q000150:complications*; D008297:Male; D028361:Mitochondrial Diseases / Q000150:complications*; D009135:Muscular Diseases / Q000150:complications*; D018357:Respiratory Syncytial Virus Infections / Q000150:complications*; D014693:Ventricular Fibrillation / Q000209:etiology*
chemical_list: D044942:Acyl-CoA Dehydrogenase, Long-Chain
keywords: VLCAD deficiency; arrythmia; fatty acid oxidation disorder; neonatal sudden death; respiratory syncytial infection