Cystic Fibrosis-related Diabetes Screening Research Articles

Feasibility and accuracy of at-home glucose tolerance tests for cystic fibrosis related diabetes screening

BackgroundAdult people living with Cystic Fibrosis (CF) undergo annual screening for CF-related diabetes. These tests represent a burden and can lead to undesirable effects resulting in low adherence. The objectives of this study were to 1) compare gold-standard in-hospital oral glucose tolerance testing (OGTT) with at-home options, and 2) evaluate acceptability of at-home options. MethodsA total of 34 adults living with CF undertook 3 types of OGTTs in standardized conditions within two weeks: 1) in a hospital using a 75 g glucose beverage, 2) at home with the same glucose beverage, and 3) at home using a standardized quantity of candy. Glucose levels were measured prior to the OGTT, after 1 and 2 hours. Concordance of glucose measurement, side effects and general appreciation were assessed across the three options. ResultsMean blood glucose was comparable among the three tests. Glucose tolerance categorization (normal, impaired glucose tolerance, or diabetes) was concordant with the hospital reference test in 59 % of participants for the glucose beverage and 75 % for the candies. Side effects were mild with all types of OGTTs, and 94 % of participants preferred the home options. Among the at-home OGTTs, the glucose beverage was preferred to the candy option. ConclusionsHome-based OGTT could be an alternative to gold standard hospital-based OGTT testing, improving adherence to annual testing and reducing costs. However, the discrepancy between various OGTT testing methods could lead to diagnosis dilemma. This approach should be tested on a larger sample size.

The Role of Continuous Glucose Monitoring in Detecting Early Dysglycemia and Clinical Outcomes in Patients with Cystic Fibrosis.

Cystic fibrosis-related diabetes (CFRD) is the most common comorbidity in patients with cystic fibrosis (CF). CFRD has been correlated with important clinical outcomes, including poor nutrition, reduced pulmonary function, and earlier mortality. However, clinical decline due to abnormalities of blood glucose (dysglycemia) begins early in CF, before the diagnosis of CFRD by the gold-standard oral glucose tolerance test (OGTT). Continuous glucose monitoring (CGM) has been validated in patients with CF and has been recognized as a valuable tool in detecting early glucose abnormalities in patients with CF. Several CGM parameters have been used to predict CFRD in some but not all studies, and there is no consensus regarding CGM use for diagnostic purposes. Thus, it remains a complementary test to OGTT in CFRD diagnosis. The aim of this review is to provide an update on the pathophysiological mechanisms of CFRD, recent advances in the use of CGM for CFRD screening, and the association between CGM measures and CF-related clinical outcomes.

Cystic fibrosis-related diabetes screening at a large pediatric center.

Cystic Fibrosis Foundation guidelines recommend annual diabetes screening by oral glucose tolerance test (OGTT) in pediatric patients with cystic fibrosis (CF) starting at the age of 10 years. Adherence to these guidelines proves to be challenging, and the nationwide screening rates are still considered suboptimal. The aim of this study was to assess and improve the screening rates at our large pediatric center. A 4-year retrospective audit of OGTT completion among pediatric patients with CF of age ≥10 years who are not yet diagnosed with diabetes was conducted. A collaborative working group was formed to identify the barriers to screening and formulate a quality improvement plan, which was monitored and evaluated for a 9-month period. Diabetes screening rates determined by OGTT completion at our center showed a gradual decline during the COVID-19 pandemic from 2019 to 2022. Following the implementation of the quality improvement plan during the summer of 2023, there was a marked increase in OGTT ordering compliance by providers as well as test completion by patients. Notably, the fractional OGTT completion rate rose from 45% during the preintervention phase (January-April 2023) to 70% during the postintervention phase (May-September 2023). Diabetes screening in pediatric patients with CF can be effectively improved by refining practices related to patient experience, care coordination, and laboratory testing strategies.

Novel time-saving OGTT sparing HbA1c-HOMA2 based algorithm for the diagnosis of cystic fibrosis-related diabetes

AimsThe diagnosis of cystic fibrosis-related diabetes (CFRD) faces several challenges. We propose a novel screening algorithm to alleviate the burden of cystic fibrosis (CF). MethodsThrough a retrospective cross-sectional single-centre study, HbA1c and HOMA2 indices were assessed in multiple models as alternative diagnostic tools from OGTT data. We sought to establish specific thresholds for CFRD screening with oral glucose tolerance test (OGTT) as gold standard. We evaluated various straightforward or sequential approaches, in terms of diagnostic accuracy while also quantify the potential reduction in OGTTs through these different methods. ResultsHOMA indices were recovered in 72 patients. We devised a composite index that combines HbA1c and HOMA-B: Diabetes Predicting Index in cystic fibrosis (DIPIc) = (HbA1c(%) × 3.455) – (HOMA-B(%) × 0.020) – 19.294. This index yields the highest screening accuracy according to receiver-operating characteristics curves. Using a stepwise algorithm that incorporates DIPIc decreases the requirement for annual OGTTs. A CFRD exclusion cutoff less than −1.7445 (sensitivity 98 %), in conjunction with a CFRD diagnostic threshold greater than 0.4543 (specificity 98 %) allows for 71 % OGTT sparing. ConclusionThe composite index DIPIc is a suitable, less invasive screening method for CFRD, which enables to avoid many OGTTs.

1264-P: Trends in Incidence and Prevalence of CFRD from the U.S. Cystic Fibrosis Foundation Patient Registry (CFFPR)

Intro: The US CFFPR includes data from ~30,000 individuals with cystic fibrosis (CF). We aimed to examine trends in incidence and prevalence alongside screening rates of cystic fibrosis related diabetes (CFRD) from 2003-2018. Methods: Prevalence of CFRD was defined as the percent of patients with CFRD in the registry in a given year. Annual incidence of CFRD was calculated as the number of new diagnoses per 1,000 person-years. Annual screening rates were defined as the percent of individuals without CFRD who underwent an oral glucose tolerance test (OGTT). Individuals <10 years were excluded, and data were grouped by age decade. Results: (Table 1) CFRD screening rates increased from 2003-2018, particularly in 10-18 yr olds. Although screening rates increased in adults as well, overall rates remain low. In 10-18 yr olds, the incidence of CFRD did not increase, and incident cases in adults decreased. The prevalence of CFRD is gradually increasing in adults. Conclusions: Despite increased screening, the incidence of CFRD is stable or declining. CFRD prevalence is steadily rising in older adults. Screening rates and trends in CFRD incidence support a need for revised and potentially targeted screening algorithms. With increasing longevity in people with CF and rising prevalence of CFRD, there is great need for more specialists trained to care for a growing population of adults with CFRD. Disclosure C.L.Kohler: None. T.B.Vigers: None. C.Sakamoto: None. A.Moran: Advisory Panel; Abata Therapeutics, Dompé, Other Relationship; Novo Nordisk, Research Support; Abbott Diabetes. S.Sagel: None. C.L.Chan: None.

P304 Oral glucose tolerance test in diagnosis of cystic fibrosis-related diabetes in the era of continuous glucose monitoring

Oral glucose tolerance testing (OGTT) is the recommended screening for cystic fibrosis-related diabetes (CFRD). Increasing evidence for benefit of early detection of CFRD has led to utilisation of continuous glucose monitoring (CGM). One-hour plasma glucose (1-h PG) during the oral glucose tolerance test (OGTT) is known to be an accurate predictor of type 2 diabetes.

A provider survey of cystic fibrosis related diabetes screening and management practices at North American CF centers.

Cystic Fibrosis Foundation (CFF) Guidelines recommend annual screening for cystic fibrosis related diabetes (CFRD) with an oral glucose tolerance test (OGTT). However, screening rates remain consistently low. We conducted surveys of 1) US CF center directors and 2) Endocrinologists affiliated with the CFF-sponsored EnVision program to characterize CFRD screening practices, describe provider perceived barriers to screening, and identify strategies for improving screening. The surveys queried OGTT protocols, alternate screening strategies, and perceived barriers to screening. CF center characteristics and procedures for coordinating OGTTs were compared between centers achieving ≥50% versus <50% OGTT completion. Endocrinologists received additional questions regarding OGTT interpretation and management. The survey response rate was 18% (51/290) from CF Centers and 63% (25/40) from Endocrinologists. The majority (57%) of CF centers utilized 2 OGTT timepoints (0,120 min). The majority (72%) of Endocrinologists utilized 3 timepoints (0,60,120 min). Four percent of CF centers and 8% of Endocrinologists utilized other timepoints. Forty-nine percent of CF centers reported ≥50% OGTT completion in the past year. Completion of ≥50% OGTT was 5 times more likely when patient reminders were consistently provided (p = 0.017). Both CF Centers and Endocrinologists employed alternative screening strategies including HbA1c (64%, 92%), fasting plasma glucose (49%, 67%), continuous glucose monitoring (30%, 58%), and home fingerstick monitoring (55%, 50%). OGTT is the gold standard screening method for CFRD, but completion rates remain suboptimal, practice variation exists, and many providers utilize alternate screening strategies. Systematic reminders may improve completion rates. Studies to improve our approach to CFRD screening are urgently needed.

Canadian Cystic Fibrosis–related Diabetes Clinical Practice Survey: Analysis of Current Practices and Gaps in Clinical Care

ObjectivesOur aim in this study was to identify challenges and gaps in Canadian practices in screening, diagnosis, and treatment of cystic fibrosis–related diabetes (CFRD), with the goal of informing a Canadian-specific guideline for CFRD. MethodsWe conducted an online survey of health-care professionals (97 physicians and 44 allied health professionals) who care for people living with CF (pwCF) and/or CFRD (pwCFRD). ResultsMost pediatric centres followed <10 pwCFRD and adult centres followed >10 pwCFRD. Children with CFRD are usually followed at a separate diabetes clinic, whereas adults with CFRD may be followed by respirologists, nurse practitioners, or endocrinologists in a CF clinic or in a separate diabetes clinic. Less than 25% of pwCF had access to an endocrinologist with a special interest or expertise in CFRD. Many centres perform screening oral glucose tolerance testing with fasting and 2-hour time points. Respondents, especially those working with adults, also indicate use of additional tests for screening not currently recommended in CFRD guidelines. Pediatric practitioners tend to only use insulin to manage CFRD, whereas adult practitioners are more likely to use repaglinide as an alternative to insulin. ConclusionsAccess to specialized CFRD care may be a challenge for pwCFRD in Canada. There appears to be wide heterogeneity of CFRD care organization, screening, and treatment among health-care providers caring for pwCF and/or pwCFRD across Canada. Practitioners working with adult pwCF are less likely to adhere to current clinical practice guidelines than practitioners working with children.

ODP235 Quality Improvement Survey to Identify Barriers to Oral Glucose Tolerance Test in Patients with Cystic Fibrosis

Abstract Background Cystic Fibrosis (CF) is the most common autosomal recessive disorder in Caucasians and can involve multiple organ systems. Cystic Fibrosis Related Diabetes (CFRD) is the most common complication which affects at least 40-50% of the adult patients with CF. Untreated CFRD adversely affects pulmonary function, nutritional status, and is associated with overall poor outcomes. Early diagnosis and treatment with insulin improve nutritional status, pulmonary function and is associated with reduced mortality. An annual two-hour Oral Glucose Tolerance Test (OGTT) is recommended for all patients with CF beginning at 10 years of age. Unfortunately, OGTT screening rates remain very low in patients with CF. The aim of this study was to identify patient barriers to OGTT screening and list them in order of relative importance from patient perspective. Methods A survey was designed to identify patient barriers associated with completing the screening OGTT in the CF population. Data collection included patient demographics and pertinent clinical information. All patients &amp;gt;18 years of age and who receive care at the Cystic Fibrosis center at our institute received a survey. Survey was sent through email, postal mail, or during CF center visits. Patients who did not initially complete survey received a reminder email, phone call, and hand survey during CF appointment. Participants were excluded if they had a diagnosis of CFRD or were currently pregnant. There was no intervention planned during this study. Results 49 patients without CFRD and 19 patients with CFRD responded to survey. Only 12.2% patients were appropriately screened by OGTT in last 1 year. The survey demonstrated that the top 3 barriers to obtaining OGTT in descending order were "I don't think I have diabetes, I don't need this"; "I don't like the taste of glucola"; and "I don't think OGTT is important for assessing my health". Conclusions The prevalence of CFRD continues to increase as the median life expectancy increases in patients with CF and is associated with worse outcomes. Early diagnosis and treatment with insulin are crucial. Unfortunately, screening for CFRD remains low in this population. Our study highlights the patient perceived barriers for screening OGTT. The top 3 barriers noted in study show the importance of patient education and awareness about CFRD. Further research is needed to identify specific interventions that will improve CFRD screening and lead to earlier diagnosis and treatment. Presentation: No date and time listed

Characterization of glucose metabolism in youth with vs. without cystic fibrosis liver disease: A pilot study

BackgroundDiabetes and liver disease are life-threatening complications of cystic fibrosis (CF). CF-liver disease is a risk factor for CF related diabetes (CFRD) development, but the underlying mechanisms linking the two co-morbidities are not known. The objective of this pilot study was to characterize glucose metabolism in youth with CF with and without liver disease. MethodsIn this two-center cross-sectional study, 20 youth with CF with and without liver disease underwent a 3-hour oral glucose tolerance test. Subjects were categorized by liver disease (LD) status [no LD, mild LD, severe LD] and diabetes status. Measures of glucose excursion, islet cell secretory responses, insulin sensitivity and clearance were obtained. ResultsParticipants with severe LD had the highest fasting, peak, and glucose area under the curve over 3 h (AUC3h) among individuals with CFRD (interaction p < 0.05). In parallel with glycemic changes, prandial β-cell secretory response (AUC C-peptide 3h) was lower in those with severe LD compared to mild or no LD (p < 0.01). There was a trend of higher HOMA-IR in those with severe LD (p = 0.1) as well as lower fasting insulin clearance in those with mild and severe LD compared to no LD (p = 0.06) and lower prandial insulin clearance in severe LD among those with CFRD (interaction p = 0.1). ConclusionIn this small cohort, subjects with severe LD tended to have more impaired glycemia, insulin secretion, insulin sensitivity and clearance. Larger studies are imperative to define the pathogenesis to inform clinical care guidelines in terms of CFRD screening, diagnosis, and treatment options.

Cystic fibrosis-related diabetes: Prevalence, screening, and diagnosis

Cystic fibrosis-related diabetes (CFRD) is the most common comorbidity in patients with cystic fibrosis (CF). Prevalence of CFRD increases with age and is greater with severe mutations. Other risk factors associated with CFRD are female sex, pancreatic insufficiency, liver disease, need for gastrostomy tube feedings, history of bronchopulmonary aspergillosis, and poor pulmonary function. CFRD is related to worse clinical outcomes and increased mortality. Early diagnosis and treatment have been shown to improve clinical outcomes. Screening for CFRD is recommended with an annual oral glucose tolerance test (OGTT) starting at age 10 years. Diagnosis of CFRD is made by standard American Diabetes Association (ADA) criteria during baseline health. CFRD can also be diagnosed in individuals with CF during acute illness, while on enteral feeds, and after transplant. In this review we will discuss the epidemiology of CFRD and provide an overview of the advantages and pitfalls of current screening and diagnostic tests for CFRD.

Gestational and pregestational diabetes in pregnant women with cystic fibrosis

As cystic fibrosis transmembrane regulator (CFTR) modulator therapies offer greater longevity and improved health quality, women living with cystic fibrosis (CF) are increasingly pursuing pregnancy. Maternal risks for pregnant women with CF largely depend on a woman’s baseline pulmonary and pancreatic function, and the majority of CF pregnancies will successfully end in live births.Diabetes, either gestational or pre-existing cystic fibrosis-related diabetes (CFRD), is highly prevalent in women with CF, affecting 18 to 62% of pregnancies in recent CF center reports. In addition to the rising incidence of CFRD with age, gestational diabetes is also more common in women with CF due to lower insulin secretion, higher insulin resistance, and increased hepatic glucose production as compared to pregnant women without CF. Diabetes occurring during pregnancy has important implications for maternal and fetal health. It is well established in women without CF that glycemic control is directly associated with risks of fetal malformation, neonatal-perinatal mortality, cesarean delivery and need for neonatal intensive care. Small studies in women with CF suggest that pregnancies affected by diabetes have an increased risk of preterm delivery, lower gestational age, and lower fetal birth weight compared to those without diabetes.Women with CF preparing for pregnancy should be counseled on the risks of diabetes and should undergo routine screening for CFRD with oral glucose tolerance testing (OGTT) if not already completed in the past six months. Glycemic control in those with pre-gestational CFRD should be optimized prior to conception. Insulin is preferred for the management of diabetes in pregnant women with CF via multiple daily injections or insulin pump therapy, and continuous glucose monitors (CGM) can be useful in mitigating hypoglycemia risks. Women with CF face many unique challenges impacting diabetes care during pregnancy and would benefit from support by a multidisciplinary care team, including nutrition and endocrinology, to ensure healthy pregnancies.

Characterization of Intermittently Scanned Glucose Monitoring Profiles in Patients at Risk for Cystic Fibrosis-Related Diabetes

Oral glucose tolerance testing (OGTT) screening for Cystic Fibrosis-Related Diabetes (CFRD) is limited by poor sensitivity, specificity, repeatability and compliance. Intermittently scanned continuous glucose monitoring (isCGM) may be an alternative.

Glycated Hemoglobin as a First-line Screening Test for Cystic Fibrosis‒Related Diabetes and Impaired Glucose Tolerance in Children With Cystic Fibrosis: A Validation Study

ObjectivesOur aims in this study were to document the screening rate for cystic fibrosis‒related diabetes (CFRD) in children followed at a cystic fibrosis (CF) clinic in Canada and to evaluate the accuracy of various glycated hemoglobin (A1C) cutoffs to screen for CFRD and impaired glucose tolerance (IGT) in a pediatric CF population. MethodsThe CFRD screening rate was calculated over a follow-up period of up to 8 years among children who attended the CF clinic between 1993 and 2018. Test performance of A1C at various thresholds ranging from 5.5% to 6.2% was compared with the oral glucose tolerance test (OGTT) as the reference method. Children with CF aged ≥10 years with an OGTT performed within 120 days of A1C measurement were included in the analysis. ResultsThe overall CFRD screening rate was 53.0%. A total of 256 children were included for the A1C performance analysis, of whom 8.6% had an OGTT-confirmed CFRD diagnosis. An A1C threshold of 5.8% demonstrated an optimal balance between sensitivity (90.9%) and specificity (60.7%) for CFRD screening, leading to a potential reduction of 56.3% of the annual required OGTTs. A1C demonstrated poor accuracy for identifying children with IGT. ConclusionsAn A1C threshold ≥5.8% allows for identification of children requiring further CFRD investigations, which may reduce the clinical burden of children with CF without compromising the ability of early CFRD diagnosis.

Influence of pre-diabetic and pancreatic exocrine states on pulmonary and nutritional status in adults with Cystic Fibrosis

BackgroundIn 1992, a landmark study demonstrated clinical deterioration in respiratory function and nutritional status prior to the onset of cystic fibrosis-related diabetes (CFRD). We re-evaluated this outcome. MethodsThe Montreal Cystic Fibrosis Cohort is a prospective CFRD screening study. We performed a 6-year retrospective analysis of nutritional parameters and FEV1 (%) in subjects who developed incident CFRD and in controls who maintained normoglycemia (NG). In the former group, data was collected over 6 years prior to diabetes onset. ResultsSubjects (n = 86) had a mean age of 31.7 ± 8.1 years, BMI of 23.0 ± 4.0 kg/m2, and FEV1% of 70.1 ± 24.2%. Eighty-one percent had pancreatic insufficiency (PI). Patients were grouped as follows: NG+PS (pancreatic sufficient) (n = 16), NG+PI (pancreatic insufficient) (n = 21), CFRD+PS (n = 3) and CFRD+PI (n = 46).At their most recent screen NG+PS subjects had significantly greater BMI, as compared to NG+PI and CFRD+PI groups (26.2 ± 3.6 kg/m2 vs 22.6 ± 4.2 kg/m2 vs 22.1 ± 3.5 kg/m2, p = 0.0016). FEV1 was significantly greater in the NG+PS group (91.5 ± 16.8% vs 67.8 ± 25.3% vs 63.5 ± 22.2%, p = 0.0002). The rates of change in weight, BMI, fat mass (%), and FEV1 prior to the most recent visit (NG+PS, NG+PI groups) or to the diagnosis of de novo CFRD were similar between groups. ConclusionIn a contemporary context, CFRD onset is not preceded by deterioration in BMI, fat mass, or pulmonary function. Low BMI and FEV1 are more closely associated with PI than a pre-diabetic state.

EPS2.04 The role of Flash Glucose Monitoring System at annual review during the COVID-19 restrictions, within Cystic Fibrosis Diabetes Service at Blackpool Adult Cystic Fibrosis Centre, UK

Objectives: The gold standard of care for diagnosing Cystic Fibrosis-Related Diabetes (CFRD), as recommended by CF Trust and American Diabetes Association guidelines (Bridges et al., 2018), is to use the Oral Glucose Tolerance Test (OGTT). Flash Glucose Monitoring System (FGMS) was introduced for CF patients, shielding due to COVID-19, as part of their CF annual review (A/R) alongside chest status, weight and HbA1c. Methods: • A/R information sheet outlining reason for using FGMS and sharing data agreement via Libre View. • Video consultation with Dietitian/CF Diabetes Nurse to discuss and support insertion of Libre sensor and link to sharing data. • Patients completed 2 weeks of daily scanning and food diary. • 2 weekly CFRD MDT data analysis, using the Brompton CGM criteria for CFRD diagnosis (see table 1). [Table presented] Results: • Good uptake of CFRD screening process. • 13 patients screened with FGMS (see table 2). • Monitoring of glucose against timely food diary, using Libre View App. • FGMS has identified patients with impaired glucose handling that may have been missed on OGTT. • Patients able to observe effect of food on blood glucose levels. [Table presented] Conclusion: Using the FGMS has enhanced patient's compliance with A/R screening for CFRD. The FGMS has identified early diagnosis of patients with CFRD and patients with delayed insulin response leading to effective insulin treatment and appropriate dietary changes. Feedback to the patient following A/R has been quicker and more efficient due to video consultations and the sharing of glucose results and food diary via the App and Libre View sharing agreement. The role of FGMS at annual review allows closer analysis of glucose trends which otherwise would have been missed on the OGTT screening.

P244 Simplification of Cystic Fibrosis-Related Diabetes screening by the use of a home-based oral glucose tolerance test: a pilot study to evaluate feasibility, validity and patient perception (AtHome)

P244 Simplification of Cystic Fibrosis-Related Diabetes screening by the use of a home-based oral glucose tolerance test: a pilot study to evaluate feasibility, validity and patient perception (AtHome)

Use of continuous glucose monitoring in diagnosis and management of cystic fibrosis-related diabetes in children

Background: Cystic fibrosis related diabetes (CFRD) is the most common comorbidity in children with cystic fibrosis (CF). Routine screening for CFRD is the oral glucose tolerance test (OGTT) where blood glucose levels are measured at 0 and 120 min. Studies have found that patients with CFRD often have an initial postprandial raised blood glucose level at 60 min while still achieving a normal 120-min blood glucose level from an OGTT, resulting in delayed diagnosis and treatment of CFRD. The aim of this study was to assess whether the use of continuous glucose monitoring (CGM) could aid in early diagnosis and treatment in children with CFRD. Methods: We included five patients diagnosed with CFRD. All the patients had a raised 1-h OGTT blood glucose level (>11.0 mmol/L), and consented for CGM for two weeks. Treatment was commenced initially as bolus fast-acting insulin (Novorapid), which targeted the initial postprandial rise in blood glucose level. If the CGM showed continued overnight hyperglycemia, basal long-acting insulin (Lantus) was later added to the treatment regimen. Results: All the patients showed improvement in their forced expiratory volume at 1s and weights following insulin treatment. Conclusions: Abnormalities of glucose metabolism have an adverse impact on morbidity and mortality in patients with CF. Early detection of glucose abnormalities using CGM and early targeted intervention is an important factor to consider in improving lung function and nutritional status. Further studies are warranted on the use of CGM in the early detection and management of CFRD in children.

Cystic fibrosis related diabetes: Pathophysiology, screening and diagnosis

Cystic fibrosis related diabetes (CFRD) is a distinct form of diabetes that is associated with significantly increased morbidity and mortality in the CF population. The primary etiology is relative insulin insufficiency secondary to destruction of pancreatic islets, and to other factors that affect the function of the remaining beta cells. The prevalence of CFRD increases markedly with age and more than half of CF adults develop the disease. As the initial presentation of CFRD may be clinically silent, annual screening is recommended starting at least by the age of 10 with an oral glucose tolerance test (OGTT). The importance of an early diagnosis is based on the association of diabetes and abnormal glucose tolerance with worse health outcomes in patients with CF, and evidence that early treatment of diabetes improves prognosis. This review presents a concise overview of the current understanding of CFRD pathogenesis, natural history, screening and diagnosis.

The association of pediatric cystic fibrosis-related diabetes screening on clinical outcomes by center: A CF patient registry study

BackgroundCystic fibrosis related diabetes (CFRD) has been associated with pulmonary function decline, nutritional status decline and increased mortality. In 2010, the CFRD Clinical Care Guideline were updated, recommending all patients with CF begin CFRD screening at 10 years old. This study uses CF Foundation Patient Registry to examine the impact of screening practices at centers across the United States from 2008 to 2015. We examined the association of screening practices and CFRD diagnosis at individual centers and trends in ppFEV1 and BMI percentile prior to and after diagnosis. MethodsThe cohort was defined as patients with CF and without CFRD who turned 10 years old from 2009 to 2015. Centers were classified based on their CFRD screening rates. Kaplan-Meier curves summarized the distribution of age at CFRD diagnosis. Among patients diagnosed with CFRD, we examined differences in ppFEV1 and BMI percentile two years prior to diagnosis and two years post-diagnosis by clinic screening rate. ResultsOf 3553 patients, 445 (13%) were diagnosed with CFRD. The average age of diagnosis was 13 years old. The screening rate of the patients' clinic was significantly associated with time to diagnosis (pvalue=0.0001). Among patients diagnosed with CFRD, clinics with lower screening rates of CFRD had steeper rates of pulmonary decline two years prior to diagnosis. ConclusionCenters that screen for CFRD more tend to diagnose a larger percent of patients with CFRD and at a younger age. Additionally, patients at centers with lower screening rates have faster rates of pulmonary decline prior to CFRD diagnosis.