OBJECTIVE To validate continuous glucose monitoring (CGM) in children and adolescents with cystic fibrosis. RESEARCH DESIGN AND METHODS Paired oral glucose tolerance tests (OGTTs) and CGM monitoring was undertaken in 102 children and adolescents with cystic fibrosis (age 9.5–19.0 years) at baseline (CGM1) and after 12 months (CGM2). CGM validity was assessed by reliability, reproducibility, and repeatability. RESULTS CGM was reliable with a Bland-Altman agreement between CGM and OGTT of 0.81 mmol/l (95% CI for bias ± 2.90 mmol/l) and good correlation between the two (r = 0.74–0.9; P < 0.01). CGM was reproducible with no significant differences in the coefficient of variation of the CGM assessment between visits and repeatable with a mean difference between CGM1 and CGM2 of 0.09 mmol/l (95% CI for difference ± 0.46 mmol/l) and a discriminant ratio of 13.0 and 15.1, respectively. CONCLUSIONS In this cohort of children and adolescents with cystic fibrosis, CGM performed on two occasions over a 12-month period was reliable, reproducible, and repeatable.

Validation of Continuous Glucose Monitoring in Children and Adolescents with Cystic Fibrosis Related Diabetes a Prospective Cohort Study

Cystic fibrosis-related diabetes(CFRD)is the most common comorbidity in people with cystic fibrosisThese guidelines are the result of a joint effort between the Cystic Fibrosis Foundation,the American Diabetes Association,and the Pediatric Endocrine SocietyThe guidelines include recommendations for screening,diagnosis,and medical management of CFRDThis report focuses on aspects of the clinical care unique to CFRD

Clinical care guidelines for cystic fibrosis-related diabetes

Background: Cystic fibrosis (CF) lung disease determines the outcome of this condition. For lung evaluation processes, computed tomography (CT) is the gold standard, but also causes irradiation. Lately, lung ultrasound (LUS) has proven to be reliable for the diagnosis of consolidations, atelectasis, and/or bronchiectasis. The aim of our study was to evaluate the value of a newly conceived LUS score by comparing it to the modified Bhalla CT score. A further aim was to evaluate the correlation between the score and the lung clearance index (LCI). Methods: Patients with CF were screened by LUS, followed by a CT scan. Spearman’s test was used for correlations. Results: A total of 98 patients with CF were screened, and 57 were included in the study; their mean age was 11.8 ± 5.5 (mean ± SD) years. The mean LUS score was 5.88 ± 5.4 SD. The LUS CF score had a very strong correlation with the CT score of rs = 0.87 (p = 0.000). LUS showed a good sensibility for detecting atelectasis (Se = 83.7%) and consolidations (Se = 94.4%). A lower Se (77.7%) and Sp (9%) were found for cylindrical bronchiectasis. Conclusion: Our study shows that LUS and the lung CF score are parameters that can be used with a complementary role in the diagnosis and monitoring of CF lung disease in children.

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Lung Ultrasound in Children with Cystic Fibrosis in Comparison with Chest Computed Tomography: A Feasibility Study

Chronic endometritis (CE) is a local mucosal infectious and inflammatory disorder characterized by unusual filtration of CD138(+) endometrial stromal plasmacytes. CE is attracting attention due to its potential association with infertility of unknown etiology, repeated implantation failure, recurrent pregnancy loss, and several maternal/neonatal complications. Due to the variance in study design among researchers, universal diagnostic criteria remain to be established for the clinical diagnosis and management of CE. This review article aims to summarize current knowledge and provide insights into unsolved questions on CE to establish clinical guidelines for the disease from the viewpoint of human reproduction.

https://www.mdpi.com/2075-4418/12/11/2711/pdf?version=1667644361

Challenges in Clinical Diagnosis and Management of Chronic Endometritis

Background High-resolution computed tomography (HRCT) is the gold standard for the evaluation of cystic fibrosis (CF) lung disease; however, lung ultrasound (LUS) is being increasingly used for the assessment of lung in these patients due to its lower cost, availability, and lack of irradiation. We aimed to determine the diagnostic performance of LUS for the evaluation of CF pulmonary exacerbation. Methods This cross-sectional study included patients with CF pulmonary exacerbation admitted to Masih Daneshvari Hospital, Tehran, Iran, from March 21, 2020 to March 20, 2021. Age, gender, and body mass index (BMI) of the patients were recorded. All patients underwent chest X-ray (CXR), HRCT, and LUS on admission. Pleural thickening, atelectasis, air bronchogram, B-line, and consolidation were noted in LUS and then compared with the corresponding findings in CXR and HRCT. Taking HRCT findings as reference, sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and diagnostic accuracy (DA) of LUS and CXR for the detection of each pulmonary abnormality were determined. Results Of the 30 patients included in this study, with a mean age of 19.62 ± 5.53 years, 14 (46.7%) were male. Of the 15 patients aged 2-20 years, BMI was below the 5th percentile in 10 (66.7%), within the 5-10 percentiles in 1 (6.7%), 10-25 percentiles in 3 (20%), and 25-50 percentiles in 1 (6.7%). The mean BMI for 15 patients > 20 years was 18.03 ± 2.53 kg/m2. LUS had better diagnostic performance compared to CXR for the detection of air bronchogram, consolidation, and pleural thickening (area under the receiver operating characteristic curve [AUROC]: 0.966 vs. 0.483, 0.900 vs. 0.575, and 0.656 vs. 0.531, respectively). Also, LUS was 100% and 96.7% specific for the diagnosis of pleural effusion and atelectasis, respectively. Conclusions LUS appears to be superior to CXR and comparable with HRCT for the evaluation of CF pulmonary exacerbation, especially in terms of air bronchogram and consolidation detection. LUS can be used to lengthen the HRCT evaluation intervals in this regard or utilized along with HRCT for better evaluation of CF pulmonary exacerbation.

Lung ultrasound for the diagnosis of cystic fibrosis pulmonary exacerbation.

Background: An important percentage of CF patients associates structural lung damages associated with impaired lung function. Lung ultrasound (LUS) can detect structural features like emphysema, consolidation, interstitial inflammation or bronchiectasis while the most accurate parameter of the lung function in cystic fibrosis is the lung clearance index(LCI), therefore it would be important to evaluate the relation between the two parameters. Aim: To evaluate the relation between LUS score and lung function expressed by LCI among children with CF. Methods: Forty-two children with CF (mean age 13.93± 5.1years) who underwent the annual lung function assessment of LCI, were also evaluated by LUS. Specific characteristics on ultrasound were used for LUS score: presence of B-lines for interstitial damage cylindrical bronchiectasis and air trapping, lake sign for detection of varicose bronchiectasis or cystic bronchiectasis, parenchymal structure for consolidation. Of these, 15 patients (53.3% girls) performed a reproductible LCI. The relation between LCI as a constant and the LUS score was evaluated with Pearson correlation test. Results: At LUS, air trapping was the most frequent feature detected in 79.6% of patients and the median value of LCI was 16.53+-7.6. A median LUS score of 4.67+-3, consistent for moderate morphological lung impairment was found. LCI showed a strong positive correlation with the LUS score, with coefficient of 0.82, p Conclusion: In our study an important correlation, statistically significant, between lung damage evaluated by a LUS score and the lung clearance index was demonstrated. LUS score might be used for a primary evaluation of the CF lung damages.

Lung clearance index and lung ultrasound in cystic fibrosis children

Purpose The aim of this cross-sectional study was to identify the major factors influencing pulmonary function in CF patients from western side of Romania. Patients and Methods The study enrolled 51 patients with CF between the ages of 6 and 27.8 years who were monitored at regular visits to the National Cystic Fibrosis Centre and Pius Branzeu County Hospital in Timisoara, Romania, over a period of 2 years. The relationships between lung function, as measured by forced expiratory volume in 1 s (FEV1), with patient age, sex, body mass index (BMI), pancreatic insufficiency (PI), microbial infection, CF-related diabetes (CFRD), CF-associated liver disease (CFLD), and physical activity <30 min/day, were evaluated by multivariate regression analysis. Results The results showed that FEV1 was 0.32% higher for each increase in percentile of BMI (95% confidence interval: 0.034-0.619). In addition, mean FEV1 was 1.52% lower with every year rise of age. PI and female sex increased the risk of impaired lung function (FEV1 <60%). The factors most closely correlated with pulmonary function in pediatric CF patients were current age (negative) and BMI (positive). Conclusion The findings of this study, in combination with what is known from other studies in CF, suggest that a better nutritional status and infection prophylaxis should be part of a comprehensive clinical management strategy for pediatric CF in Romania.

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Factors Influencing Lung Function in Patients with Cystic Fibrosis in Western Romania.

In cystic fibrosis (CF), the respiratory disease is the main factor that influences the outcome and the prognosis of patients, bacterial infections being responsible for severe exacerbations. The etiology is often multi-microbial and with resistant strains. The aim of this paper is to present current existing antibiotherapy solutions for CF-associated infections in order to offer a reliable support for individual, targeted, and specific treatment. The inclusion criteria were studies about antibiotherapy in CF pediatric patients. Studies involving adult patients or those with only in vitro results were excluded. The information sources were all articles published until December 2021, in PubMed and ScienceDirect. A total of 74 studies were included, with a total number of 26,979 patients aged between 0–18 years. We approached each pathogen individual, with their specific treatment, comparing treatment solutions proposed by different studies. Preservation of lung function is the main goal of therapy in CF, because once parenchyma is lost, it cannot be recovered. Early personalized intervention and prevention of infection with reputable germs is of paramount importance, even if is an asymmetrical challenge. This research received no external funding.

https://www.mdpi.com/2227-9067/9/8/1258/pdf?version=1660994327

Antibiotherapy in Children with Cystic Fibrosis—An Extensive Review

Abstract Cystic fibrosis related diabetes (CFRD) is a redoubtable complication associated to cystic fibrosis, with an increasing frequency, directly proportional to children life expectancy. Although this complication has similar features with DM type 1 and some with type 2, the evolution and even the response to insulin therapy is different. It is also possible that other factors to influence the CFRD clinical expression and subsequently the disease evolution. Since its 1t diagnosis was associated with more frequent pulmonary exacerbations and with the deterioration of the respiratory status, therefore CFRD must be early and correctly diagnosed and managed. The aim of this paper is to present an overview of the recent updates and recommendations regarding this important CF complication.

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Mihaela Dediu

Papers

Lung clearance index and lung ultrasound in cystic fibrosis children

Lung Ultrasound in Children with Cystic Fibrosis in Comparison with Chest Computed Tomography: A Feasibility Study

Factors Influencing Lung Function in Patients with Cystic Fibrosis in Western Romania.

Antibiotherapy in Children with Cystic Fibrosis—An Extensive Review

Cystic fibrosis related diabetes