{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,2,21]],"date-time":"2025-02-21T17:54:31Z","timestamp":1740160471886,"version":"3.37.3"},"reference-count":36,"publisher":"Walter de Gruyter GmbH","issue":"12","funder":[{"DOI":"10.13039\/501100004006","name":"Bilimsel Ara\u00c5\u0178tirma Projeleri Birimi, Istanbul \u00c3\u0153niversitesi","doi-asserted-by":"publisher","award":["37969"],"award-info":[{"award-number":["37969"]}],"id":[{"id":"10.13039\/501100004006","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,12,15]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec id=\"j_jpem-2023-0325_abs_001\">\n                  <jats:title>Objectives<\/jats:title>\n                  <jats:p>There is growing concern about the low-protein and high-energy diet therapies used in the treatment of inherited amino acid metabolism disorders. We aimed to identify the risk factors for noncommunicable diseases that may arise from nutritional therapies and suggests approaches that may prevent the development of the noncommunicable diseases.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_jpem-2023-0325_abs_002\">\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>The present study evaluates 112 patients, on long-term nutritional therapy for at least the last 2 years with a diagnosis of an inborn error of the amino acid metabolism, and their 28 healthy siblings. The participants are assessed for the development of overweight and metabolic syndrome based on an analysis of anthropometric parameters, body composition and the results of biochemical tests.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_jpem-2023-0325_abs_003\">\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Anthropometric measurements including BMI, weight Z-score, waist circumference and fat mass were not significantly different between patients and controls. Height Z-scores were similar in phenylketonuria patients compared to controls, but lower in urea cycle disorders, organic acidemia and maple syrup urine disease groups. No increased risk of development of overweight or metabolic syndrome was detected in the patient group, while there were findings suggesting malnutrition in patients diagnosed with urea cycle disorders. There was a correlation between patients\u2019 BMI and C3-carnitine levels in organic acidemia patients and leucine levels in maple syrup urine disease patients.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_jpem-2023-0325_abs_004\">\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>All forms of malnutrition can be prevented in patient groups receiving limited nutrients under a dietary management protocol, based on the findings of anthropometric and biochemical evaluations and analyses of body composition.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1515\/jpem-2023-0325","type":"journal-article","created":{"date-parts":[[2023,10,5]],"date-time":"2023-10-05T10:40:51Z","timestamp":1696502451000},"page":"1146-1153","source":"Crossref","is-referenced-by-count":1,"title":["Evaluation of the risk factors for noncommunicable diseases in patients with inborn errors of amino acid metabolism receiving nutrition therapy"],"prefix":"10.1515","volume":"36","author":[{"given":"Mehmet Cihan","family":"Balci","sequence":"first","affiliation":[{"name":"Division of Nutrition and Metabolism , Istanbul Medical Faculty Children\u2019s Hospital, Istanbul University , Istanbul , T\u00fcrkiye s"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Meryem","family":"Karaca","sequence":"additional","affiliation":[{"name":"Division of Nutrition and Metabolism , Istanbul Medical Faculty Children\u2019s Hospital, Istanbul University , Istanbul , T\u00fcrkiye s"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alihan","family":"Yesil","sequence":"additional","affiliation":[{"name":"Division of Nutrition and Metabolism , Istanbul Medical Faculty Children\u2019s Hospital, Istanbul University , Istanbul , T\u00fcrkiye s"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7264-038X","authenticated-orcid":false,"given":"Arzu","family":"Selamioglu","sequence":"additional","affiliation":[{"name":"Division of Nutrition and Metabolism , Istanbul Medical Faculty Children\u2019s Hospital, Istanbul University , Istanbul , T\u00fcrkiye s"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Huseyin Kutay","family":"Korbeyli","sequence":"additional","affiliation":[{"name":"Division of Nutrition and Metabolism , Istanbul Medical Faculty Children\u2019s Hospital, Istanbul University , Istanbul , T\u00fcrkiye s"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Asli","family":"Durmus","sequence":"additional","affiliation":[{"name":"Division of Nutrition and Metabolism , Istanbul Medical Faculty Children\u2019s Hospital, Istanbul University , Istanbul , T\u00fcrkiye s"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Belkis","family":"Ak","sequence":"additional","affiliation":[{"name":"Division of Nutrition and Metabolism , Istanbul Medical Faculty Children\u2019s Hospital, Istanbul University , Istanbul , T\u00fcrkiye s"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6932-3815","authenticated-orcid":false,"given":"Tugba","family":"Kozanoglu","sequence":"additional","affiliation":[{"name":"Division of Nutrition and Metabolism , Istanbul Medical Faculty Children\u2019s Hospital, Istanbul University , Istanbul , T\u00fcrkiye s"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ilknur","family":"Hacioglu","sequence":"additional","affiliation":[{"name":"Division of Nutrition and Metabolism , Istanbul Medical Faculty Children\u2019s Hospital, Istanbul University , Istanbul , T\u00fcrkiye s"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gulden Fatma","family":"Gokcay","sequence":"additional","affiliation":[{"name":"Division of Nutrition and Metabolism , Istanbul Medical Faculty Children\u2019s Hospital, Istanbul University , Istanbul , T\u00fcrkiye s"},{"name":"Department of Rare Diseases , Institute of Child Health, Istanbul University , Istanbul , T\u00fcrkiye"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"374","published-online":{"date-parts":[[2023,10,6]]},"reference":[{"key":"2023112810420455682_j_jpem-2023-0325_ref_001","doi-asserted-by":"crossref","unstructured":"Humphrey, M, Truby, H, Boneh, A. 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