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            Clinical Practice Consensus Guidelines 2022: Management of cystic fibrosis‐related diabetes in children and adolescents

Ode, Katie Larson; Ballman, Manfred; Battezzati, Alberto; Brennan, Amanda; Chan, Christine L.; Hameed, Shihab A.; Ismail, Heba M.; Kelly, Andrea; Moran, Antoinette; Rabasa‐Lhoret, Rémi; Saxby, Nichole A; Craig, Maria E.

doi:10.1111/pedi.13453

Cited by 30 publications

(23 citation statements)

References 242 publications

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“…In addition, patients with advanced cystic fibrosis may develop pulmonary hypertension, which is associated with decreased survival . Adults in the US have been reported to have increased risk of comorbidities including cystic fibrosis–related diabetes (29.2%), liver disease with cirrhosis (4.1%), and osteoporosis (7.5%) . People with cystic fibrosis who have at least 1 copy of a CFTR variant with residual function often have later onset of lung disease yet have comparable disease progression with those with minimally functional variants …”

Section: Discussion and Observationsmentioning

confidence: 99%

“…Annual screening for psychosocial health concerns is recommended in children aged 12 years or older . Monitoring for comorbidities includes annual oral glucose tolerance testing (≥10 years) for cystic fibrosis–related diabetes, dual-energy x-ray absorptiometry scanning every 2 to 5 years (>8 years) for bone density, and colonoscopy every 5 years (≥40 years) for colorectal cancer …”

Section: Discussion and Observationsmentioning

confidence: 99%

“…Adults comprise 58.3% of the total US cystic fibrosis population. As this population ages, expanded services will be needed across multiple subspecialities to manage comorbidities and age-related complications including gastrointestinal cancers, diabetes, obesity, and hypertension . Rates of pregnancy in women with cystic fibrosis have increased from 210 in 2011 to 675 in 2021, underscoring the importance of attending to reproductive health .…”

Section: Discussion and Observationsmentioning

confidence: 99%

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Cystic Fibrosis

Ong

Ramsey

2023

JAMA

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ImportanceCystic fibrosis, a genetic disorder defined by variants in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, affects more than 30 000 individuals in the US and approximately 89 000 worldwide. Absent or decreased function of the CFTR protein is associated with multiorgan dysfunction and shortened life expectancy.ObservationsCFTR is an anion channel in the apical membrane of epithelial cells. Loss of function leads to obstructed exocrine glands. Of people with cystic fibrosis in the US, approximately 85.5% have the gene variant F508del. Manifestations of cystic fibrosis in patients with the F508del gene variant begin in infancy with steatorrhea, poor weight gain, and respiratory symptoms (coughing, wheezing). As people with cystic fibrosis age, chronic respiratory bacterial infections cause loss of lung function and bronchiectasis. With the availability of universal newborn screening in multiple countries including the US, many people with cystic fibrosis are asymptomatic at diagnosis. With multidisciplinary care teams that included dietitians, respiratory therapists, and social workers, treatment of cystic fibrosis can slow disease progression. Median survival has improved from 36.3 years (95% CI, 35.1-37.9) in 2006 to 53.1 years (95% CI, 51.6-54.7) in 2021. Pulmonary therapies for patients with cystic fibrosis consist of mucolytics (eg, dornase alfa), anti-inflammatories (eg, azithromycin), and antibiotics (such as tobramycin delivered by a nebulizer). Four small molecular therapies, termed CFTR modulators, that facilitate CFTR production and/or function have received regulatory approval. Examples are ivacaftor and elexacaftor-tezacaftor-ivacaftor. For example, in patients with 1 F508del variant, the combination of ivacaftor, tezacaftor, and elexacaftor improved lung function from −0.2% in the placebo group to 13.6% (difference, 13.8%; 95% CI, 12.1%-15.4%) and decreased the annualized estimated rate of pulmonary exacerbations from 0.98 to 0.37 (rate ratio, 0.37; 95% CI, 0.25-0.55). Improved respiratory function and symptoms have lasted up to 144 weeks in postapproval observational studies. An additional 177 variants are eligible for treatment with the elexacaftor-tezacaftor-ivacaftor combination.ConclusionCystic fibrosis affects approximately 89 000 people worldwide and is associated with a spectrum of disease related to exocrine dysfunction, including chronic respiratory bacterial infections and reduced life expectancy. First-line pulmonary therapies consist of mucolytics, anti-inflammatories, and antibiotics, and approximately 90% of people with cystic fibrosis who are 2 years or older may benefit from a combination of ivacaftor, tezacaftor, and elexacaftor.

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Section: Discussion and Observationsmentioning

confidence: 99%

Section: Discussion and Observationsmentioning

confidence: 99%

Section: Discussion and Observationsmentioning

confidence: 99%

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Cystic Fibrosis

Ong

Ramsey

2023

JAMA

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“…In this prospective case-cohort study, we used register-data of both diagnoses and collected prescriptions of drugs to investigate the association of T1D and the three atopic diseases in both directions. All our study populations were based on Danish citizens born from 01-01-1997 to 31-12-2018, excluding individuals with migration back and forth and children with the diagnosis of cystic brosis associated to a speci c form of diabetes 14 . The period was chosen to ensure valid prescriptions from children (introduced in 1996) and end-of-follow-up was due to available register data.…”

Section: Methodsmentioning

confidence: 99%

No associations between type 1 diabetes and atopic dermatitis, allergic rhinitis, or asthma in childhood: a nationwide Danish case-cohort study

Berg

Svensson

Thyssen

et al. 2023

Preprint

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Studies examining the association between type 1 diabetes (T1D) and atopic diseases, i.e., atopic dermatitis, allergic rhinitis and asthma have yielded conflicting results due to different algorithms for classification, sample size issues and risk of referral bias of exposed groups with frequent contact to prescribers. Using Danish national registries and well-established disease algorithms, we examined the bidirectional association between T1D and atopic diseases in childhood and adolescence using Cox Proportional Hazard regression compared to two different unexposed groups from a population of 1.5 million Danish children born from 1997–2018. We found no associations between T1D and atopic dermatitis, allergic rhinitis, or asthma (defined after age five). However, in multivariate analysis we found an increased risk of persistent wheezing (defined as asthma medication before age five) after T1D with an adjusted Hazard Ratio (aHR) of 1.70 [1.17–2.45]. We also identified an increased risk of developing T1D after persistent wheezing with aHR of 1.24 [1.13–1.36]. This study highlights similar risks of atopic diseases in children with T1D and of T1D in children with atopic disease after age of five years versus healthy controls, however more research is needed to understand the possible early immunological effects of the link between persistent wheezing and T1D.

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“…Thus, six different groups of glucose metabolism alterations are identified in cystic fibrosis children and adolescents [5 ▪▪ ,10] that need to be well identified in order to reduce cystic fibrosis morbidity and mortality. These six classes are based on the fasting, intermediate and 2 h OGTT glucose values and are as follows:…”

Section: Diagnosismentioning

confidence: 99%

Diabetes and prediabetes in children with cystic fibrosis

Schiaffini

Pampanini

2023

Current Opinion in Pediatrics

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Purpose of review Glucose metabolism alterations in cystic fibrosis range from the classic cystic fibrosis-related diabetes (CFRD) to forms of glucose intolerance and prediabetes. The aim of the present work is to review the most up-to-date novelties in terms of CFRD diagnosis and therapy. This review is timely and relevant because it allows an update for the early and correct classification of glucose abnormalities in cystic fibrosis and because it favours an appropriate therapeutic approach. Recent findings Confirm that Oral Glucose Tolerance Test is still the diagnostic gold standard despite the advent of continuous glucose monitoring (CGM) systems; this latter is spreading very rapidly, however, to date, there is still no strong evidence to hypothesize the use of CGM for diagnostic purposes. CGM has indeed proven to be very useful in managing and guiding CFRD therapy. Summary Tailored and personalized insulin therapy is still the recommended therapy for children and adolescents with CFRD, although nutritional intervention and oral hypoglycaemic treatment are equally important and efficacious. Finally CFTR modulators have allowed the increase of the life expectancy of cystic fibrosis patients and have proven effective not only in improving the pulmonary function and the nutritional status but also the glucose control.

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ISPAD Clinical Practice Consensus Guidelines 2022: Management of cystic fibrosis‐related diabetes in children and adolescents

Cited by 30 publications

References 242 publications

Cystic Fibrosis

Cystic Fibrosis

No associations between type 1 diabetes and atopic dermatitis, allergic rhinitis, or asthma in childhood: a nationwide Danish case-cohort study

Diabetes and prediabetes in children with cystic fibrosis

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