&lt;p&gt;Cystic fibrosis-related diabetes: optimizing care with a multidisciplinary approach&lt;/p&gt;

Frost, F.; Dyce, P.; Ochota, A.; Pandya, S.; Clarke, Tom; Walshaw, Martin; Nazareth, D.

doi:10.2147/dmso.s180597

Cited by 14 publications

(12 citation statements)

References 72 publications

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“…Patients with CFRD have a greater incidence of lung disease and a higher frequency of pulmonary exacerbation than non‐CFRD CF. In addition, diabetes increases mortality in CF 3 . Therefore, early diagnosis and control of blood glucose levels of CF are important.…”

Section: Discussionmentioning

confidence: 99%

Maturity‐onset diabetes of the young: Different diabetes in an infant with cystic fibrosis

Hangül

Erdoğan

Hatipoğlu

et al. 2020

Pediatric Pulmonology

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Cystic fibrosis (CF) is one of the most common autosomal recessive and multisystemic diseases. CF affects many systems. One of these systems is the endocrine and exocrine functions of the pancreas, causing cystic fibrosis‐related diabetes, which is extremely complex and has unique pathogenesis. Maturity‐onset diabetes of the young (MODY) is a rare type of diabetes with autosomal dominant inheritance and is not expected in patients with CF. In this study, we present MODY due to a novel glucokinase gene mutation, which is an unexpected form of diabetes in patients with CF. This is previously unreported in the literature.

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Section: Discussionmentioning

confidence: 99%

Maturity‐onset diabetes of the young: Different diabetes in an infant with cystic fibrosis

Hangül

Erdoğan

Hatipoğlu

et al. 2020

Pediatric Pulmonology

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“…FEV1 in CF patients with diabetes is markedly reduced in all age groups compared to CF patients without diabetes. Simultaneous liver-pancreas transplantation is associated with an improved BMI in the posttransplant course [56,57].…”

Section: The Treatment Of Portal Hypertension Should Includementioning

confidence: 99%

Liver Changes in the Course of Cystic Fibrosis

Więcek¹

2020

Cystic Fibrosis - Heterogeneity and Personalized Treatment

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Liver damage observed in cystic fibrosis is a complicated process comprised of fibrogenesis, inflammation, remodeling, apoptosis, and cholestasis. Complexity of processes which take place in the liver and bile ducts in the course of this disease are not clearly defined. Changes in the liver are observed in 5-20% of patients with diagnosed cystic fibrosis; however they can increase mortality, shorten time of life, and deteriorate quality of life. The aim of the chapter was to review the risk factors, clinical symptoms, diagnostic methods, and treatment of liver changes in the course of cystic fibrosis.

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“…15 Furthermore, it is recommended that people with CFRD should have multi-disciplinary team follow-up (preferably four times per year), be offered diabetes structured education programmes and that HbA1c should be monitored with a target of <7% (<53 mmol/mol). 3,5 Although macrovascular disease is currently rare, 16 microvascular complications are increasingly being recognised as complications of CFRD, particularly with increased duration of diabetes and poorer glycaemic control. 17 Retinopathy, nephropathy and neuropathy are all recognised complications 2,8 and prevalence of retinopathy has been reported from small studies as being equivalent to rates observed in type 1 diabetes.…”

Section: Retinopathy and Microalbuminuria Are Common Microvascular Comentioning

confidence: 99%

Retinopathy and microalbuminuria are common microvascular complications in cystic fibrosis-related diabetes

Kempegowda

Sunsoa

Chandan

et al. 2020

Therapeutic Advances in Endocrinology

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Aims: To study the prevalence of microvascular complications and renal changes associated with cystic fibrosis-related diabetes (CFRD). Methods: This retrospective cohort study was conducted at the West Midlands Adult Cystic Fibrosis centre, United Kingdom. Data regarding age, sex, microalbuminuria, retinopathy neuropathy, and biochemical results were collected for all people with CFRD who had an annual review from 1 January 2018 to 31 December 2018 at the centre. Descriptive statistics were analysed using STATAv15.1. Results: A total of 189 patients were included, of which 56.6% were male and median age (interquartile range) was 33 (27–39) years; 79.4% (150/189) had their annual review in 2018. Those with a biochemically impaired renal function numbered 7.2% (13/180) and 22.7% (32/141) had microalbuminuria; 17.2% (10/58) had diabetes related retinopathy. No one in our cohort had diabetic ulcers; however, 10.3% (13/126) had absent foot pulses. Conclusion: We found a higher prevalence of microalbuminuria compared with retinopathy in a large cohort of cystic fibrosis adults. This study demonstrates the need for regular specialist follow-up to facilitate early identification of such complications and a long-term prospective cohort to understand underlying mechanisms.

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<p>Cystic fibrosis-related diabetes: optimizing care with a multidisciplinary approach</p>

Cited by 14 publications

References 72 publications

Maturity‐onset diabetes of the young: Different diabetes in an infant with cystic fibrosis

Maturity‐onset diabetes of the young: Different diabetes in an infant with cystic fibrosis

Liver Changes in the Course of Cystic Fibrosis

Retinopathy and microalbuminuria are common microvascular complications in cystic fibrosis-related diabetes

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