Cystic fibrosis-related diabetes: practice challenges

Spence, Caroline

doi:10.7748/paed2005.03.17.2.23.c968

Cited by 5 publications

(10 citation statements)

References 18 publications

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“…It is classified by the American Academy of Diabetes under 'other types of diabetes', listed as being 'caused by exocrine pancreatic lesion' . (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12) Although insulin deficiency is the principal mechanism, insulin resistance is also part of the etiopathogenesis of CFRD. (13,14) Insulinopenia Chloride channel alterations lead to hyperviscosity of pancreatic duct secretion, causing obstructive lesions, fat cell infiltration and progressive islet fibrosis, as well as reduced secretion of insulin, glucagon, and pancreatic polypeptide.…”

Section: Pathophysiologymentioning

confidence: 99%

“…Multidisciplinary support is fundamental to accepting this new problem and its subsequent limitations. (2,30,31) Since patients with CFRD are typically teenagers or young adults, a psychosocial approach can be more difficult. They often stop monitoring glycemia and do not take their insulin regularly.…”

Section: Psychosocial Effectsmentioning

confidence: 99%

“…Cystic fibrosis (CF) is an autosomal recessive genetic disease, with an incidence of 1:2500 live births in Caucasian populations. (1)(2)(3) It is estimated that there are 2000 CF patients in Brazil. (4) It is a complex, severe disease that involves various organic systems.…”

Section: Introductionmentioning

confidence: 99%

“…To date, more than 1000 CF gene mutations have been identified. (2,4) Mutation ∆F508 is the most common, identified in 60-70% of patients and correlated with exocrine pancreatic insufficiency. (6) These mutations alter the encoding of a transmembrane protein known as the cystic fibrosis transmembrane regulator (CFTR), resulting in chloride channel dysfunction.…”

Section: Introductionmentioning

confidence: 99%

“…(6) Increased longevity has led to a greater incidence of extra-pulmonary complications, chief among which cystic fibrosis-related diabetes (CFRD). (1,2) It is estimated that CFRD affects approximately 15-30% of adults with CF. (3,8) The objective of this study was to review recent knowledge on CFRD, especially regarding the physiopathology, clinical profile, morbidities, diagnosis and treatment regimen.…”

Section: Introductionmentioning

confidence: 99%

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Cystic fibrosis-related diabetes (CFRD) is the principal extra-pulmonary complication of cystic fibrosis, occurring in 15-30% of adult cystic fibrosis patients. The number of cystic fibrosis patients who develop diabetes is increasing in parallel with increases in life expectancy. The aim of this study was to review the physiopathology, clinical presentation, diagnosis and treatment of CFRD. A bibliographic search of the Medline and Latin American and Caribbean Health Sciences Literature databases was made. Articles were selected from among those published in the last twenty years. Insulin deficiency, caused by reduced beta-cell mass, is the main etiologic mechanism, although insulin resistance also plays a role. Presenting features of type 1 and type 2 diabetes, CFRD typically affects individuals of approximately 20 years of age. It can also be accompanied by fasting, non-fasting or intermittent hyperglycemia. Glucose intolerance is associated with worsening of nutritional status, increased morbidity, decreased survival and reduced pulmonary function. Microvascular complications are always present, although macrovascular complications are rarely seen. An oral glucose tolerance test is recommended annually for patients > or = 10 years of age and for any patients presenting unexplained weight loss or symptoms of diabetes. Patients hospitalized with severe diseases should also be screened. If fasting hyperglycemia persists for more than 48 h, insulin therapy is recommended. Insulin administration remains the treatment of choice for diabetes and fasting hyperglycemia. Calories should not be restricted, and patients with CFRD should be managed by a multidisciplinary team.

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