Premium
VLCAD deficiency in a patient who recovered from ventricular fibrillation, but died suddenly of a respiratory syncytial virus infection
Author(s) -
Yamamoto Akiko,
Nakamura Kimitoshi,
Matsumoto Shirou,
Iwai Masanori,
Shigematsu Yosuke,
Tajima Go,
Tsumura Miyuki,
Okada Satoshi,
Mitsubuchi Hiroshi,
Endo Fumio
Publication year - 2013
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/ped.12111
Subject(s) - medicine , palivizumab , respiratory system , virus , pediatrics , immunization , ventricular fibrillation , immunology , antibody
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.