Cystic fibrosis-related diabetes: an update on pathophysiology, diagnosis, and treatment

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art_ALVES_Cystic_fibrosisrelated_diabetes_an_update_on_pathophysiology_diagnosis_2020.PDF.pdf (271.67 KB)
Citações na Scopus
19
Tipo de produção
article
Data de publicação
2020
DOI
Scopus
Web of Science
PubMed
Título da Revista
ISSN da Revista
Título do Volume
Editora
WALTER DE GRUYTER GMBH
Autores
ALVES, Cresio
ALBUQUERQUE, Cristiano Tulio Maciel
Citação
JOURNAL OF PEDIATRIC ENDOCRINOLOGY & METABOLISM, v.33, n.7, p.835-843, 2020
Projetos de Pesquisa
Unidades Organizacionais
Fascículo
Resumo
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 h of hospital admission. Fasting plasma glucose levels >= 126 mg/dL (7.0 mmol/L) or 2-h postprandial plasma glucose levels 200 mg/dL (11.1 mmol/L) that persist for more than 48 h 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 <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.
Palavras-chave
cystic fibrosis, diabetes, pathophysiology, treatment
URI
https://observatorio.fm.usp.br/handle/OPI/37208
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Coleções
Artigos e Materiais de Revistas Científicas - HC/ICr
Artigos e Materiais de Revistas Científicas - LIM/36
Artigos e Materiais de Revistas Científicas - ODS/03