Factors associated with pulmonary function decline of patients in the cystic fibrosis registry of Turkey: A retrospective cohort study.

BACKGROUND: The decline in pulmonary function is a predictor of disease progression in patients with cystic fibrosis (CF). This study aimed to determine the decline rate of percent predicted forced expiratory volume in 1 s (ppFEV1) based on the data of the CF Registry of Turkey. The secondary aim was to investigate the risk factors related to the decline in ppFEV1. METHODS: A retrospective cohort study of CF patients over 6 years old, with pulmonary function data over at least 2 years of follow-up was extracted from the national CF registry for years 2017-2019. Patients were classified according to disease severity and age groups. Multivariate analysis was used to predict the decline in ppFEV1 and to investigate the associated risk factors. RESULTS: A total of 1722 pulmonary function test results were available from 574 patients over the study period. Mean diagnostic age was older and weight for age, height for age, and body mass index z scores were significantly lower in the group of ppFEV1 < 40, while chronic Pseudomonas aeruginosa (p < .001) and mucoid P. aeruginosa colonization (p < .001) were significantly higher in this group (p < .001). Overall mean annual ppFEV1 decline was -0.97% (95% confidence interval [CI] = -0.02 to -1.92%). The mean change of ppFEV1 was significantly higher in the group with ppFEV1 ≥ 70 compared with the other (ppFEV1 < 40 and ppFEV1: 40-69) two groups (p = .004). Chronic P. aeruginosa colonization (odds ratio [OR] = 1.79 95% CI = 1.26-2.54; p = .01) and initial ppFEV1 ≥ 70 (OR = 2.98 95% CI = 1.06-8.36), p = .038) were associated with significant ppFEV1 decline in the whole cohort. CONCLUSIONS: This data analysis recommends close follow-up of patients with normal initial ppFEV1 levels at baseline; advocates for early interventions for P. aeruginosa; and underlines the importance of nutritional interventions to slow down lung disease progression.

Comparison of clinical features of cystic fibrosis patients eligible but not on CFTR modulators to ineligible for CFTR modulators.

INTRODUCTION: Cystic fibrosis transmembrane conductance regulator (CFTR) modulator drugs target the underlying defect and improve CFTR function. They are a part of standard care in many countries, but not all patients are eligible for these drugs due to age and genotype. Here, we aimed to determine the characteristics of non-eligible patients for CFTR modulators in the CF registry of Turkey (CFRT) to highlight their clinical needs. METHODS: This retrospective cohort study included CF patient data from the CFRT in 2021. The decision of eligibility for the CFTR modulator was determined according to the 'Vertex treatment-Finder' on the Vertex® website. Demographic and clinical characteristics of patients were compared between eligible (group 1) and ineligible (group 2) groups for CFTR modulators. RESULTS: Among the study population (N = 1527), 873 (57.2%) were in group 1 and 654 (42.8%) were in group 2. There was no statistical difference between groups regarding sex, meconium ileus history, diagnoses via newborn screening, FEV1 z-score, CF-associated complications, organ transplant history, and death. Patients in group 2 had a higher incidence of pancreatic insufficiency (87.7% vs. 83.2%, p = .010), lower median height z-scores (-0.87 vs. -0.55, p < .001), lower median body mass index z-scores (-0.65 vs. -0.50, p < .001), longer days receiving antibiotics due to pulmonary exacerbation (0 [interquartile range, IQR: 0-2] vs. 0 [IQR: 0-7], p = 0.001), and more non-invasive ventilation support (2.6% vs. 0.9%, p = 0.008) than patients in group 1. CONCLUSION: The ineligible group had worse clinical outcomes than the eligible group. This highlights their need for life-changing drugs to improve clinical outcomes.

Fecal calprotectin levels used as a noninvasive method for screening for chronic gastritis in pediatric patients. A descriptive study

ABSTRACT BACKGROUND: Gastritis consists of inflammation of the gastric mucosa and is one of the main causes of dyspeptic symptoms in children. OBJECTIVE: To investigate the presence of inflammation by evaluating fecal calprotectin (FC) in children diagnosed with chronic gastritis. DESIGN AND SETTING: Descriptive study in Pediatric Gastroenterology Department of Ondokuz Mayis University Hospital in Turkey. METHODS: Between January 2016 and July 2018, FC levels were compared retrospectively in children with chronic gastritis (histopathology-based diagnosis), patients with inflammatory bowel disease (IBD) and healthy children. RESULTS: A total of 67 chronic gastritis patients (61.2% girls) with a mean age of 13.09 ± 3.5 years were evaluated. The mean FC levels were 153.4 μg/g in the chronic gastritis group, 589.7 μg/g in the IBD group and 43.8 μg/g in the healthy group. These levels were higher in chronic gastritis patients than in healthy individuals (P = 0.001) and higher in IBD patients than in the other two groups (P < 0.001). The FC level in the patients with chronic active gastritis (156.3 μg/g) was higher than in those with chronic inactive gastritis (150.95 μg/g) (P = 0.011). Among the patients with chronic active gastritis, the FC level was significantly higher in Helicobacter pylori-positive individuals than in negative individuals (P = 0.031). CONCLUSION: We confirmed the association between increased FC and chronic gastritis. Elevated FC levels may be seen in patients with chronic active gastritis. In order to be able to use FC as a screening tool for chronic gastritis, further studies in a larger study group are needed.

Efectos de la edad al momento del diagnóstico y cumplimiento de la dieta sobre los parámetros de crecimiento de los pacientes con celiaquía / Effects of age of diagnosis and dietary compliance on growth parameters of patients with celiac disease

Introducción. El objetivo fue evaluar la relación entre edad al diagnóstico y cumplimiento de dieta sin gluten (DSG) y su efecto sobre el crecimiento de niños celiácos y factores que influenciaron el cumplimiento de la DSG. Población y métodos. Se incluyeron pacientes celíacos con seguimiento en nuestro hospital entre enero 2015 a enero 2017. Se los clasificaron según edad al diagnóstico y cumplimiento de la DSG. Se compararon características antropométricas al diagnóstico y durante el seguimiento. Resultados. Participaron 73 pacientes con edad promedio de 10,4 ± 4,5 años; 35 (47,9%), los pacientes de talla baja al diagnóstico; eran mayores (7,8 ± 4,2 años) que los demás (5,1 ± 4,3 años de edad) (p= 0,005). Al diagnóstico, 33 (45,2%) pacientes tenían ≤6 años y 40 (54,8%) tenían >6 años. Los puntajes Z de estatura y peso a la edad >6 años eran significativamente menores que los diagnosticados a ≤6 años, en el diagnóstico (p= 0,01 y 0,04, respectivamente) como en el último control (p= 0,001 y 0,001, respectivamente). Tuvieron cumplimiento riguroso con DSG en 45 (61,6%) pacientes. Al comparar datos antropométricos , el aumento del índice de masa corporal (IMC) y del puntaje Z de peso en el grupo que cumplió la dieta fue significativamente mayor que en el otro grupo.Conclusiones. Demorar el diagnóstico de celiaquía afectó la estatura y peso. El cumplimiento de la DSG mejoró los parámetros de crecimiento, principalmente, el puntaje Z de peso y el IMC.

Introduction. The objective of this study was to evaluate the relation between age at diagnosis and compliance to gluten free diet (GFD) on growth in children with celiac disease and the factors that influenced compliance to GFD. Population and Methods. Celiac disease (CD) patients with villous atrophy followed in our hospital between January 2015 and January 2017, were included. They were classified according to diagnosis age and GFD compliance. Patients' anthropometric characteristics at diagnosis and follow-up were compared. Results. There were 73 patients with 10.4 ± 4.5 years of average age, 35 (47.9%) patients had a short stature at diagnosis, the ages of patients who had short stature (7.8 ± 4.2 years) were higher than those who did not (5.1 ± 4.3 years) (p= 0.005). At diagnosis, 33 (45.2%) patients were aged ≤6 years, 40 (54.8%) were aged >6 years. The height and weight z-scores of patients who were diagnosed at >6 years of age were significantly lower than those who were diagnosed ≤6 years of age both at diagnosis (p= 0.01 and 0.04) and at last control (p= 0.001 and 0.001), respectively. Forty-five (61.6%) patients were fully compliant with GFD. In comparison of anthropometric data in terms of GFD compliance, the increase in BMI and weightz-score in the fully compliant group was found to be significantly higher when compared with the other group. Conclusions. Delay in CD diagnosis negatively affected both the height and weight and other growth parameters. GFD compliance positively affected the patients' all growth parameters, especially weight and BMI z-score.