المؤلفون: Scully, Kevin J, Sawicki, Gregory, Kremen, Jessica, Putman, Melissa S

المصدر: Journal of the Endocrine Society; Jan2021, Vol. 5 Issue 1, p1-5, 5p

مصطلحات موضوعية: CYSTIC fibrosis, DIABETES

مستخلص: Cystic fibrosis–related diabetes (CFRD) is associated with worsening pulmonary function, lower body mass index, increased infection frequency, and earlier mortality. While the incidence of CFRD is rising, its development in patients under the age of 10 years is uncommon. We present a 9-year-old girl with cystic fibrosis (CF) who presented with a 5-year history of nonprogressive hyperglycemia, demonstrated by abnormal oral glucose tolerance tests, glycated hemoglobin A1c (HbA1c) levels consistently >6.5%, and negative pancreatic autoantibodies. Subsequent genetic testing revealed a pathogenic heterozygous recessive mutation in the GCK gene at c.667G>A (p.Gly223Ser), consistent with a diagnosis of GCK-MODY. Significant dysglycemia in young children with CF should raise suspicion for alternative etiologies of diabetes and warrants further investigation. The clinical impact of underlying monogenic diabetes in patients with CF is unclear, and close follow-up is warranted. This case also offers unique insight on the impact of hyperglycemia in the absence of insulin deficiency on CF-specific outcomes. [ABSTRACT FROM AUTHOR]

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المؤلفون: Marks, Brynn E.^1,2 (AUTHOR) bmarks@childrensnational.org, Kilberg, Marissa J.^3,4 (AUTHOR), Aliaj, Enid⁵ (AUTHOR), Fredkin, Kelsey⁵ (AUTHOR), Hudson, Jessica⁵ (AUTHOR), Riva, Dara⁵ (AUTHOR), Román, Christina⁵ (AUTHOR), Kelly, Andrea^3,4 (AUTHOR), Putman, Melissa S.^6,7,8 (AUTHOR), Kilberg, Marissa^9,10 (AUTHOR)

المصدر: Diabetes Technology & Therapeutics. Nov2021, Vol. 23 Issue 11, p753-759. 7p.

مصطلحات موضوعية: *GLYCEMIC control, *TYPE 1 diabetes, *HYPERGLYCEMIA, *INSULIN pumps, *DIABETES, *DIAGNOSIS, *QUALITY of life, *INSULIN therapy, *RESEARCH, *BLOOD sugar monitoring, *EVALUATION research, *CYSTIC fibrosis, *COMPARATIVE studies, *TECHNOLOGY, *DISEASE complications

مستخلص: Background: Diabetes technologies are associated with improvements in glycemic control and health-related quality of life among people with type 1 diabetes (T1D). Use and perceptions of continuous glucose monitors (CGM) and insulin pumps within the cystic fibrosis (CF) community have not been well studied. Methods: A 30-item online survey addressing cystic fibrosis-related diabetes (CFRD) diagnosis, CGM and insulin pump use, and perceptions of diabetes technologies was sent to a CF community group, including people with CF (pwCF) and parents of children with CF (cwCF). Results: The response rate was 11% (n = 120; 83 pwCF, 35 cwCF). Sixty-one percent of pwCF and 34% of cwCF reported a diagnosis of CFRD. CGM use was reported by 75% (n = 47) of respondents with CFRD but was discontinued by 19% (n = 9), most commonly due to cost and increased worry about glycemia. Insulin pump therapy was reported by 29% (n = 18 of 62) of respondents with CFRD and was discontinued by 28% (n = 5), most commonly due to pain or skin irritation. Overall, 91% agreed or strongly agreed that CGM facilitated CFRD management. Eighty-one percent agreed with at least five of seven positive statements about CGM as compared with 22% for insulin pumps. Potential embarrassment over device wear, concerns about cost, and pain were commonly held negative perceptions of both technologies. Conclusions: As compared with T1D and despite perceived benefits, rates of sustained diabetes technology use are low in the CFRD community. Better insurance coverage to mitigate cost, better patient education, and confirmation that these technologies improve health and patient-reported outcomes may increase uptake. [ABSTRACT FROM AUTHOR]

المؤلفون: Sherwood, Jordan S.¹ (AUTHOR), Jafri, Rabab Z.¹ (AUTHOR), Balliro, Courtney A.¹ (AUTHOR), Zheng, Hui² (AUTHOR), El-Khatib, Firas H.³ (AUTHOR), Damiano, Edward R.³ (AUTHOR), Russell, Steven J.¹ (AUTHOR), Putman, Melissa S.^1,4 (AUTHOR) msputman@partners.org

المصدر: Journal of Cystic Fibrosis. Jan2020, Vol. 19 Issue 1, p159-161. 3p.

مصطلحات موضوعية: *GLYCEMIC control, *PANCREAS, *BIONICS, *DIABETES, *CYSTIC fibrosis

مستخلص: Cystic fibrosis-related diabetes (CFRD) is the most common extrapulmonary manifestation of cystic fibrosis. The current standard of care for CFRD involves treatment with insulin, typically via multiple daily injections. We conducted a small pilot study comparing usual care with automated glycemic control using the bihormonal (insulin and glucagon) and insulin-only configurations of the bionic pancreas. Both configurations of the bionic pancreas achieved good glycemic control, with mean glucose levels <150 mg/dl and minimal hypoglycemia. Subjects reported improved treatment satisfaction and reduced burden of diabetes management with the bionic pancreas. Further investigation of automated glycemic control in the treatment of CFRD is warranted. [ABSTRACT FROM AUTHOR]