{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,21]],"date-time":"2026-05-21T10:11:11Z","timestamp":1779358271946,"version":"3.51.4"},"reference-count":56,"publisher":"Walter de Gruyter GmbH","issue":"7","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,7,28]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Cystic fibrosis (CF) is a highly prevalent autosomal recessive disorder that is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene (7q31.2), which encodes the CFTR chloride-anion channel that is expressed in several tissues. Life expectancy has increased significantly over the past few decades due to therapeutic advances and early diagnosis through neonatal screening. However, new complications have been identified, including CF-related diabetes (CFRD). The earliest detectable glycemic abnormality is postprandial hyperglycemia that progresses into fasting hyperglycemia. CFRD is associated with a decline in lung function, impairments in weight gain and growth, pubertal development, and increased morbidity and mortality. Annual screening with oral glucose tolerance test is recommended beginning at the age of 10, and screenings are recommended for any age group during the first 48\u00a0h of hospital admission. Fasting plasma glucose levels \u2265126\u00a0mg\/dL (7.0\u00a0mmol\/L) or 2-h postprandial plasma glucose levels \u2265200\u00a0mg\/dL (11.1\u00a0mmol\/L) that persist for more than 48\u00a0h are diagnostic criteria for CFRD. Under stable health condition, the diagnosis is made when laboratory abnormalities in accordance with the American Diabetes Association criteria are detected for the first time; however, levels of HbA1c &lt;6.5% do not rule out the diagnosis. Treatment for CFRD includes insulin replacement and a hypercaloric and hyperproteic diet that does not restrict carbohydrates, fats or salt, and diabetes self-management education. The most important CFRD complications are nutritional and pulmonary disease deterioration, though the microvascular complications of diabetes have already been described.<\/jats:p>","DOI":"10.1515\/jpem-2019-0484","type":"journal-article","created":{"date-parts":[[2020,7,13]],"date-time":"2020-07-13T12:25:43Z","timestamp":1594643143000},"page":"835-843","source":"Crossref","is-referenced-by-count":36,"title":["Cystic fibrosis-related diabetes: an update on pathophysiology, diagnosis, and treatment"],"prefix":"10.1515","volume":"33","author":[{"given":"Cr\u00e9sio","family":"Alves","sequence":"first","affiliation":[{"name":"Pediatric Endocrinology Unit, Hospital Universitario Prof. Edgard Santos, Faculty of Medicine , Federal University of Bahia , Salvador , Bahia , Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thais","family":"Della-Manna","sequence":"additional","affiliation":[{"name":"Pediatric Endocrinology Unit, Instituto da Crian\u00e7a, Hospital das Cl\u00ednicas, Faculty of Medicine , University of S\u00e3o Paulo (ICr-HC-FMUSP) , S\u00e3o Paulo , Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cristiano Tulio Maciel","family":"Albuquerque","sequence":"additional","affiliation":[{"name":"Pediatric Endocrinology , Hospital Infantil Jo\u00e3o Paulo II \u2013 Funda\u00e7\u00e3o Hospitalar do Estado de Minas Gerais (HIJPII\/MG \u2013 FHEMIG) , Belo Horizonte , Minas Gerais , Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"374","published-online":{"date-parts":[[2020,7,10]]},"reference":[{"key":"2023040101122173297_j_jpem-2019-0484_ref_001_w2aab3b7c15b1b6b1ab2b1b1Aa","doi-asserted-by":"crossref","unstructured":"Bell, SC, Mall, MA, Gutierrez, H, Macek, M, Madge, S, Davies, JC, et al. 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