Diagnosis Of Cystic Fibrosis Related Diabetes Research Articles

7522 Effects of Trikafta On Bone Mineral Density And Body Composition In Patients With Cystic Fibrosis

Abstract Disclosure: N. Mon: None. S.S. Krishnasamy: None. G. Bakeerathan: None. L. Healy: None. E. Favier: None. S. Foushee: None. Background: Cystic fibrosis related bone disease (CFBD) is a common complication of adult Cystic fibrosis (CF) patients due to calcium, vitamin D and K malabsorption from exocrine pancreatic insufficiency, increased inflammatory cytokines, glucocorticoid therapy, delayed puberty, physical inactivity, and hypogonadism. Newer CF transmembrane conductance regulator (CFTR) modulator therapy elexacaftor/tezacaftor/ivacaftor (Trikafta) has proven benefit in pulmonary and nutritional outcomes. There is limited data on effects of Trikafta on bone health and body composition. The aim is to examine the effects of Trikafta on bone mineral density (BMD) at the lumbar spine (LS) and femoral neck (FN), and body composition in terms of fat free mass index (FFMI) and fat mass index (FMI) in our CF patients. Method: We conducted a cross sectional and retrospective review of 38 adults with CF (Ages 20-59, 28 males and 10 females) in our CF clinic. Body Mass Index (BMI), Body composition measured by bioelectric impedance analysis, BMD of LS and FN by Dual-energy X-ray absorptiometry, Vitamin D level, and forced expiratory volume in one second (FEV1 % predicted) in patients taking Trikafta were analyzed and compared with control CF group who were not on Trikafta. Results: 38 subjects on Trikafta, aged 33.29 ± 10.36 years with FEV1 % predicted 65.11± 28.14% were studied. Serum 25-hydroxyvitamin D level was 35.92 ± 18.52 ng/dL. Of the 38 study patients, 23 (60.53%) had a diagnosis of CF related diabetes (CFRD). Among the Trikafta cohort, 18 (50%) had low BMD with Z-scores ≤ -2.0 and 7 (19.44%) of them had Z score ≤ -1.0 and > -2.0. Compared with the control group, LS BMD 0.93 ± 0.16 g/cm2 and FN BMD 0.73± 0.13g/cm2 (p >0.05) were not improved significantly. BMI of study population 23.09 ± 4.21 kg/m2 (p <0.05) and FMI 6.11 ± 4.25 kg/m2 (p <0.05) increased significantly; however, there was a decrease in FFMI 9.60 ± 1.44 kg/m2 (p <0.05). Conclusion: Treatment with Trikafta in CF patients was associated with increases in BMI and fat mass index but decrease in fat free mass index. There was no statistically significant difference in BMD at LS and FN in our study cohort. This study has limitations in terms of gender differences within the study cohort, duration of Trikafta use in relation to bone mass, and also extent of glucocorticoid use among the study subjects. Larger prospective clinical trials are needed to study efficacy of Trikafta on CFBD. Presentation: 6/3/2024

Metabolomics Panel Associated with Cystic Fibrosis-Related Diabetes toward Biomarker Discovery.

The most prevalent comorbidity among cystic fibrosis (CF) patients is cystic fibrosis-related diabetes (CFRD). CFRD has been linked to one of the worse clinical outcomes and a higher mortality. Improved clinical results have been related to earlier diagnosis and treatment of CFRD. Therefore, the present study aimed to investigate the metabolome of human serum of patients with CFRD. This might aid in identifying novel biomarkers linked with the pathophysiology of CFRD and its diagnosis. The liquid chromatography-high-resolution mass spectrometry (LC-HRMS) metabolomics approach was utilized for serum samples from patients with CF (n = 36) and healthy controls (n = 36). Nine patients in the CF group had CFRD, and 27 were non-CFRD patients (nCFRD). A total of 2328 metabolites were significantly altered in CF compared with the healthy control. Among those, 799 significantly dysregulated metabolites were identified between CFRD and nCFRD. Arachidonic acid (AA), ascorbate, and aldarate metabolism were the most common metabolic pathways dysregulated in CF. l-Homocysteic acid (l-HCA) levels were significantly reduced in CF and CFRD compared to the control and nCFRD, respectively. In addition, gamma-glutamylglycine and l-5-hydroxytryptophan (5-HTP) had the highest discrimination between CFRD and nCFRD with AUC (0.716 and 0.683, respectively). These biomarkers might serve as diagnostic biomarkers and aid in understanding potential metabolic changes linked to CF and CFRD.

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.

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.

Clinical Observations in Patients With Cystic Fibrosis-Related Diabetes and Self-Reported Ototoxicity Symptoms.

Persons with cystic fibrosis (PwCF) are at high risk for ototoxicity due to the routine use of intravenous aminoglycoside (IV-AG) antibiotics in respiratory infection management. Additionally, factors that contribute to ototoxicity-related symptom development and severity in PwCF are unknown. Given the increased risk of ototoxicity in people with diabetes, we explored the association between cystic fibrosis-related diabetes (CFRD) and self-reported ototoxicity symptoms (tinnitus and vestibular problems) in PwCF treated with aminoglycosides. PwCF (N = 39; 25 females, 14 males; Mage = 30.1 years, SD = 10.3) were recruited from the Cystic Fibrosis Care Center at Oregon Health & Science University. Patients completed the validated questionnaires to ascertain their experiences with ototoxicity-related symptoms of tinnitus and balance function. The diagnosis of CFRD, including oral glucose tolerance testing (OGTT), insulin treatment, hemoglobin A1c, and cumulative IV-AG treatment history, was obtained through a medical chart review. Participants were classified into three groups based on their medical diagnoses via OGTT: normal glucose tolerance (NGT; control; n = 16), abnormal glucose tolerance (AGT; n = 9), and CFRD (n = 14). Participants in each group were further classified based on survey outcomes for ototoxicity-related symptoms. There was a trend toward a higher proportion of patients with CFRD reporting tinnitus compared to the AGT and NGT groups, but did not meet statistical significance (X2 = 2.24, p = .13). Approximately, 43% of patients with CFRD reported experiencing clinically significant tinnitus lasting > 3 min compared to 11% in the AGT group and 13% in the NGT group (X2 = 3.751, p = .05). Cumulative IV-AG exposure tended to be higher in CFRD compared to other groups. High balance function was generally reported in all groups. Patients with CFRD have greater ototoxicity-related symptoms. Further investigation of the relationship between CF-related comorbidities and the risk of developing ototoxicity-related symptoms is warranted to improve the detection and management of ototoxicity in PwCF.

EPS4.03 An audit of diagnosing cystic fibrosis-related diabetes using newly developed diagnostic criteria using flash glucose monitoring system (Freestyle Libre®) at a regional adult cystic fibrosis centre

Objectives: In 2022, the West Midlands Adult Cystic Fibrosis Centre began using Freestyle Libre flash glucose monitoring system (FGM) as a first line method for cystic fibrosis related diabetes (CFRD) screening, as an alternative to oral glucose tolerance testing (OGTT). There are no published criteria for the diagnosis of CFRD using FGM. Using the WHO criteria, the Brompton’s paediatric clinical guideline and clinical judgement we devised diagnostic criteria to standardise the diagnosis of CFRD using FGM. Diagnostic criteria - CFRD >2 readings >11.1, impaired glucose tolerance (IGT) >2 readings 7.8–11 or <3.5; over a 2 week period. The aim of this study was to compare the number of patients diagnosed with CFRD and IGT using our new FGM diagnostic criteria compared to using our previous non standardised practice of using clinical judgement. We aimed to assess if there was any difference in the number of patients being diagnosed with CFRD or IGT using our new criteria.

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.

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

P181 The effects of linagliptin in patients with a diagnosis of Cystic Fibrosis-Related Diabetes

P181 The effects of linagliptin in patients with a diagnosis of Cystic Fibrosis-Related Diabetes

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.

Cystic fibrosis related diabetes (CFRD) prognosis

Cystic fibrosis related diabetes (CFRD) occurs in at least 40–50% of adults with CF. With other forms of diabetes, microvascular and macrovascular disease are the major causes of morbidity and mortality. Macrovascular disease is rare in CF. While microvascular disease does occur in this population, there are CF-specific diabetes complications that have a more important impact on prognosis. The additional diagnosis of diabetes in CF is associated with decreased lung function, poor nutritional status, and an overall increase in mortality from lung disease. These negative findings start even before the clinical diagnosis of CFRD, during the period when patients experience abnormal glucose tolerance related to insulin insufficiency. The main mechanisms by which CFRD negatively affects prognosis are thought to be a combination of 1) protein catabolism, decreased lean body mass and undernutrition resulting from insulin insufficiency, and 2) an increased pro-inflammatory and pro-infectious state related to intermittent hyperglycemia. With the introduction of CFTR modulators, the care of CF patients has been revolutionized and many aspects of CF health such as BMI and lung function are improving. The impact of these drugs on the adverse prognosis related to the diagnosis of diabetes in CF, as well as the potential to delay or prevent onset of CFRD remain to be determined.

Marginal Association of Fasting Blood Glucose With the Risk of Cystic Fibrosis Related Diabetes

The criteria for diagnosis of cystic fibrosis-related diabetes (CFRD) are a fasting blood glucose ≥7.0 mmol/L and/or a glucose of 11.1 mmol/L following an oral glucose tolerance test (OGTT). Yet, no studies have compared the contributions of fasting versus post-prandial glucose elevations to the diagnosis of incident CFRD.

Nutritional status between 5‐10 years is associated with cystic fibrosis‐related diabetes in adolescence

Cystic fibrosis-related diabetes (CFRD) is a risk factor for adverse clinical outcomes including poor nutritional status, deterioration in lung functions, and increased mortality. The association between nutritional status between 5 and 10 years of age and later diagnosis of CFRD is not known. A retrospective chart review was performed for our patients with CF between 10 and 18 years. Data was collected at age 5 and 10 years. Comparison made between patients with and without CFRD. Two groups were comparable for age and sex. At age 5, groups had no differences in weight, height, and body mass index. At age 10, the CFRD group had a lower body mass index (40.2 ± 24.7 vs. 61.5 ± 22.5 percentile, p = 0.02). Spirometry was similar between groups at 5 and 10 years. Patients with CFRD had lower growth velocity (5 ± 0.9 vs. 5.7 ± 0.9 cm/year, p = 0.03) and reduced weight gain rate (2.2 ± 0.9 vs. 3.2 ± 1.2 kg/year, p = 0.03) compared to patients without CFRD between 5 and 10 years. Patients with a weight gain less than 2.5 kg/year between 5 and 10 years were nine times more likely to develop CFRD in adolescence (Unadjusted Odds Ratio: 8.9; 95% CI:1.4, 47.2; p = 0.01). Patients who later developed CFRD had significantly lower weight gain rate and height growth between 5 and 10 years of age than those without diabetes. Close monitoring of nutritional status in before age 10 years may help identify CF patients at-risk of developing CFRD.

Perceptions of Diabetes Technology Use in Cystic Fibrosis-Related Diabetes Management.

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.

Continuous Glucose Monitoring as a Valuable Tool in the Early Detection of Diabetes Related to Cystic Fibrosis.

Aims: We evaluated the impact of cystic fibrosis-related diabetes (CFRD) on lung disease and nutritional status.Study Design: The retrospective cohort study evaluated the subjects' medical records from 2004 to 2019. All participants older than 10 years diagnosed by a 30-minutely sampled OGTT formed OGTT-CFRD subgroup. The participants diagnosed with continuous glucose monitoring (CGM) (at least two peaks above 11.1 mmol/l and more than 10% of recorded time above 7.8 mmol/l) formed a CFRD-CGM subgroup. The participants without CFRD formed a non-CFRD group. The longitudinal follow-up was made 2 years before and 3 years after insulin therapy initiation.Results: Of 144 participants included, aged 10–55 years (44% males), 28 (19.4%) had CFRD. The HbA1c was significantly lower in the CGM-CFRD in comparison to the OGTT-CFRD subgroup (5.9 ± 0.62 and 7.3 ± 1.7% respectfully; p = 0.04). Subjects with CFRD were malnourished in comparison to non-CFRD, with significant improvements with insulin replacement therapy in regard to BMI Z-score (−1.4 ± 1.3 vs. −0.5 ± 1.2%, p = 0.04) and pulmonary exacerbation score (p = 0.02). In OGTT-CFRD subgroup there is an increase in FEV1 (62.7 ± 26.3 to 65.1 ± 21.7%, p = 0.7) and decrease in FVC (from 76.4 ± 24.2 to 71.2 ± 20%, p = 0.003) from diagnosis to second year of follow-up. In CGM-CFRD subgroup there was a decrease in FEV1 (from 58.2 ± 28.2 to 52.8 ± 25.9%, p = 0.2) and FVC-values (from 72.4 ± 26.5 to 67.4 ± 29.1%, p = 0.08).Chronic Pseudomonas aeruginosa infection was more prevalent in the CFRD group (p = 0.003).Conclusion: Continuous glucose monitoring is a useful tool for insight of glucose impairment and diagnosis of CFRD. Early recognition of CFRD and therapeutic intervention has favorable effects on clinical course of the disease.

Cystic Fibrosis - Related Diabetes

Cystic fibrosis (CF) is life-shortening autosomal recessive disease, caused by mutations in the cystic fibrosis transmembrane conductance regulator protein. The most common of CF complications is cystic fibrosis-related diabetes (CFRD). The pathophysiology of CFRD is complex. The best test for screening and diagnosis of CFRD is the oral glucose tolerance test (OGTT). Insulin therapy is a treatment of choice in CFDR pharmacotherapy. An inseparable element of CFRD therapy is also physical activity and diet.

HOMA indices as screening tests for cystic fibrosis-related diabetes

BackgroundWe assessed the diagnostic performances of homeostasis model assessment indices (HOMA) of β-cell function (HOMA-%β) and of insulin resistance (HOMA-IR) for cystic fibrosis related diabetes (CFRD) screening. MethodsData were collected from a prospective cohort of 228 patients with CF (117 adults and 111 children). Fasting insulin and glucose levels were measured to calculate HOMA-%β and HOMA-IR. HOMA-%β <100 indicated insulin secretion deficiency and HOMA-IR >1 insulin resistance. Both were used to calculate sensitivity, specificity, and positive and negative predictive values (PPV and NPV). Two-hour oral glucose tolerance tests (2h-OGTT) defined CFRD. Analyses were conducted separately for children and adults. Performances of HOMA-%β and HOMA-IR were calculated at inclusion, for each year of follow-up and for pooled data over the follow-up period. ResultsSensitivity, specificity, NPV and PPV were respectively: 88%, 45%, 98% and 11% for HOMA-%β and 42%, 48%, 91% and 6% for HOMA-IR in the pooled data of children; and 83%, 18%, 90% and 10% for HOMA-%β, and 39%, 80%, 92% and 18% for HOMA-IR in the pooled data of adults. Combining HOMA-%β and HOMA-IR did not improve performances. ConclusionWithin both age groups, HOMA-%β <100 provided good sensitivity and NPV. HOMA-IR >1 had low sensitivity. Calculation of the HOMA-%β could be an interesting first-line screening approach to exclude CFRD and thus avoid unnecessary OGTT in patients for whom value is ≥100. However, HOMA-%β<100 does not support the diagnosis of CFRD and should be complemented by OGTT.

Continuous glucose monitoring indices predict poor FEV1 recovery following cystic fibrosis pulmonary exacerbations

BackgroundLittle is known about the effect of dysglycemia during cystic fibrosis pulmonary exacerbation (PEx) on recovery of FEV1 percentage predicted (ppFEV1) MethodsContinuous glucose monitoring (CGM) was commenced at the time of admission to hospital for PEx and continued for 6 weeks. The CGM indices, percentage of time glucose greater than 7.8 mmol/L (%T>7.8) and mean glucose were evaluated as predictors of absolute ppFEV1 change following treatment of PEx. ResultsOf the 20 participants who completed the study 13 (65%) had cystic fibrosis related diabetes (CFRD). The mean of both CGM indices were highest during the first week of pulmonary exacerbation and continued to decline over the first 4 weeks at which point they plateaued. Using multivariate regression models, factors which were predictive of maximum attained ppFEV1 change over 6 weeks were %T>7.8, mean glucose, HbA1c and preadmission ppFEV1 change from baseline. These relationships were independent of a diagnosis of CFRD, which was not associated with ppFEV1 recovery. In a longitudinal model of ppFEV1 change at weeks 1, 2 and 6, the CGM index %T>7.8 approached significance as a predictive variable. ConclusionsHyperglycemia during PEx in adult CF patients is associated with poorer ppFEV1 recovery. Conversely, there was no association observed between CFRD diagnosis and ppFEV1 improvement, suggesting that optimization of glycemic control in CFRD patients may positively influence recovery of lung function. Further clinical trials are required to evaluate the merits of intensive glycemic control in CFRD during PEx.

P15 Uso del monitoreo continuo de glucosa en pacientes con diabetes asociada a fibrosis quística y con curva indeterminada de tolerancia a la glucosa. Resultados preliminares

Introducción: la diabetes relacionada con fibrosis quística (DRFQ) es la enfermedad asociada más frecuente en personas con FQ, relacionada con peor estado nutricional, enfermedad pulmonar inflamatoria grave y mayor mortalidad. La detección precoz y tratamiento de DRFQ mejoran el pronóstico general. El monitoreo continuo de glucosa tipo flash (MCG) ha sido validado en pacientes tratados con insulina, aunque no para diagnosticar diabetes.Objetivos: evaluar las características clínicas y bioquímicas de una cohorte de pacientes FQ y analizar la utilidad del MCG para diagnóstico y control evolutivo de DRFQ.Materiales y métodos: se presenta el corte transversal inicial de estudio longitudinal abierto desde 2018, que incluye 15 pacientes con FQ sin exacerbación infecciosa, mayores de 10 años, con o sin DRFQ (9 evaluados con MCG). Se obtuvieron: antropometría, espirometría, glucemia, PTOG, HbA1c y glucosa intersticial de 7 días. Se calcularon estadísticos con pruebas T y X2, correlación y regresión.

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.