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dc.contributor.authorBalcl, Mehmet Cihan
dc.contributor.authorGökçay, Gülden Fatma
dc.contributor.authorKaraca, Meryem
dc.contributor.authorKozanoǧlu, Tuǧba
dc.date.accessioned2023-02-21T10:38:10Z
dc.date.available2023-02-21T10:38:10Z
dc.date.issued2023
dc.identifier.citationKozanoǧlu T., Balcl M. C., Karaca M., Gökçay G. F., "Leucine tolerance in children with MSUD is not correlated with plasma leucine levels at diagnosis.", Journal of pediatric endocrinology & metabolism : JPEM, cilt.36, sa.2, ss.167-173, 2023
dc.identifier.issn0334-018X
dc.identifier.othervv_1032021
dc.identifier.otherav_487ec738-97ec-4140-8646-0ad97f4643d4
dc.identifier.urihttp://hdl.handle.net/20.500.12627/188577
dc.identifier.urihttps://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=85144338752&origin=inward
dc.identifier.urihttps://doi.org/10.1515/jpem-2022-0230
dc.description.abstract© 2022 Maple syrup urine disease (MSUD) is an inborn metabolic disease. The nutritional treatment with restricted intake of branched chain amino acids and prevention of leucine toxicity are crucially important for a favorable outcome. The aim of this study is to analyze the relation of blood leucine levels at diagnosis with future leucine tolerances, to determine whether any prediction about the future leucine tolerances or plasma leucine levels is possible by evaluating blood leucine levels at diagnosis. The study group consisted of 45 MSUD patients. Leucine levels at diagnosis were compared with age at diagnosis, leucine tolerances, maximum leucine levels/ages, and average blood leucine levels. The mean plasma leucine level at diagnosis was 2,355.47 ± 1,251.7 μmol/L (ref: 55-164 μmol/L). The median age at diagnosis was 17 days. Leucine tolerances per kg body weight declined until the age of 8 years and stabilized subsequently. The average age of maximum leucine level during follow-up was 3.14 ± 1.92 years, and the mean maximum lifetime plasma leucine level on follow-up was 1,452.13 ± 621.38 μmol/L. The leucine levels at diagnosis did not have any significant relationship with lifetime leucine tolerances, maximum plasma leucine levels or mean plasma leucine levels. The plasma leucine levels at diagnosis did not have a predictive value for later leucine tolerances or plasma leucine levels. The maximum lifetime leucine level is likely to happen within the first 3 years of life, underlining the importance of good metabolic control and compliance to dietary treatment at early ages.
dc.language.isoeng
dc.subjectSağlık Bilimleri
dc.subjectPediatri, Perinatoloji ve Çocuk Sağlığı
dc.subjectEndokrinoloji, Diyabet ve Metabolizma
dc.subjectEndokrinoloji
dc.subjectYaşam Bilimleri
dc.subjectİç Hastalıkları
dc.subjectEndokrinoloji ve Metabolizma Hastalıkları
dc.subjectÇocuk Sağlığı ve Hastalıkları
dc.subjectDahili Tıp Bilimleri
dc.subjectTıp
dc.subjectPEDİATRİ
dc.subjectENDOKRİNOLOJİ VE METABOLİZMA
dc.subjectKlinik Tıp
dc.subjectKlinik Tıp (MED)
dc.titleLeucine tolerance in children with MSUD is not correlated with plasma leucine levels at diagnosis.
dc.typeMakale
dc.relation.journalJournal of pediatric endocrinology & metabolism : JPEM
dc.contributor.departmentİstanbul Tıp Fakültesi , ,
dc.identifier.volume36
dc.identifier.issue2
dc.identifier.startpage167
dc.identifier.endpage173
dc.contributor.firstauthorID4232025


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