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Titolo:
Management of acute metabolic decompensation in maple syrup urine disease:A multi-center study
Autore:
Yoshino, M; Aoki, K; Akeda, H; Hashimoto, K; Ikeda, T; Inoue, F; Ito, M; Kawamura, M; Kohno, Y; Koga, Y; Kuroda, Y; Maesaka, H; Murakami-Soda, H; Sugiyama, N; Suzuki, Y; Yano, S; Yoshioka, A;
Indirizzi:
Kurume Univ, Sch Med, Dept Pediat & Child Hlth, Kurume, Fukuoka 8300011, Japan Kurume Univ Kurume Fukuoka Japan 8300011 , Kurume, Fukuoka 8300011, Japan Kagawa Nutr Univ, Sakado, Saitama, Japan Kagawa Nutr Univ Sakado Saitama Japan Nutr Univ, Sakado, Saitama, Japan Naha Hosp, Dept Pediat, Naha, Japan Naha Hosp Naha JapanNaha Hosp, Dept Pediat, Naha, Japan Nara Med Univ, Dept Pediat, Nara, Japan Nara Med Univ Nara JapanNara Med Univ, Dept Pediat, Nara, Japan Kumamoto Municipal Hosp, Dept Pediat, Kumamoto, Japan Kumamoto Municipal Hosp Kumamoto Japan sp, Dept Pediat, Kumamoto, Japan Kyoto Prefectural Univ Med, Dept Pediat, Kyoto 602, Japan Kyoto Prefectural Univ Med Kyoto Japan 602 Dept Pediat, Kyoto 602, Japan Univ Tokushima, Sch Med, Dept Pediat, Tokushima 770, Japan Univ TokushimaTokushima Japan 770 ed, Dept Pediat, Tokushima 770, Japan Kawamura Childrens Clin, Nagoya, Aichi, Japan Kawamura Childrens Clin Nagoya Aichi Japan ns Clin, Nagoya, Aichi, Japan Nagara Natl Hosp, Dept Pediat, Gifu, Japan Nagara Natl Hosp Gifu JapanNagara Natl Hosp, Dept Pediat, Gifu, Japan Kanagawa Childrens Med Ctr, Dept Pediat, Kanagawa, Japan Kanagawa Childrens Med Ctr Kanagawa Japan Dept Pediat, Kanagawa, Japan Nagoya City Univ, Sch Med, Dept Pediat, Nagoya, Aichi 467, Japan Nagoya City Univ Nagoya Aichi Japan 467 Pediat, Nagoya, Aichi 467, Japan Gifu Univ, Sch Med, Dept Pediat, Gifu 500, Japan Gifu Univ Gifu Japan 500 ifu Univ, Sch Med, Dept Pediat, Gifu 500, Japan
Titolo Testata:
PEDIATRICS INTERNATIONAL
fascicolo: 2, volume: 41, anno: 1999,
pagine: 132 - 137
SICI:
1328-8067(199904)41:2<132:MOAMDI>2.0.ZU;2-A
Fonte:
ISI
Lingua:
ENG
Soggetto:
BRANCHED-CHAIN AMINO; PERITONEAL-DIALYSIS; INBORN-ERRORS;
Keywords:
maple syrup urine disease; metabolic decompensation; outcome;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
21
Recensione:
Indirizzi per estratti:
Indirizzo: Yoshino, M Kurume Univ, Sch Med, Dept Pediat & Child Hlth, 67 Asahi Machi,Kurume, Fukuoka 8300011, Japan Kurume Univ 67 Asahi Machi Kurume Fukuoka Japan 8300011 , Japan
Citazione:
M. Yoshino et al., "Management of acute metabolic decompensation in maple syrup urine disease:A multi-center study", PEDIATR INT, 41(2), 1999, pp. 132-137

Abstract

Background: Therapeutic modalities in acute metabolic decompensation in maple syrup urine disease (MSUD) are variable, and outcomes of each therapeutic measure have been known only individually. Factors that affect neurological outcome are not clear. Methods: A questionnaire was sent throughout Japan to each pediatrician treating any of the 42 MSUD patients. Results: Necessary information was available for 13 patients through the questionnaire, and through a publication for one patient. In nine of the 14 patients episodes of metabolic decompensation developed in the neonatal period. In the other five, the onset of disease was delayed until infancy or later. In the nine patients with neonatal onset, a pretreatment level of plasma leucine greater than 40 mg/100 mL or a duration of altered level of alertness longer than 10 days was associated with a poor neurological outcome. The therapeutic measures employed included intravenous infusion of glucoseand electrolyte solution or hypertonic glucose and electrolyte solution, exchange transfusion, peritoneal dialysis, a large dose of thiamine and intravenous hyperalimentation. All patients had survivied the episodes and werealive at the time of the survey. Five of the nine patients with neonatal onset have developed neurological sequelae to varying degrees. Episodes of metabolic decompensation in infancy or therafter did not affect, or only minimally affected, the neurological outcome. Conclusion: Therapeutic goals. to improve neurological outcome are to shorten the duration of the altered level of consciousness, and to minimize thepeak plasma leucine level as much as possible.

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Documento generato il 04/07/20 alle ore 17:18:34