Pre and post-COVID 19 infection pulmonary functions in children with chronic respiratory disease: A case series.

As functional respiratory impairment following COVID-19 infection (COVID-19) is increasingly reported in adult, data regarding children especially with pre-existing chronic respiratory disease (PCRD) remain scarce. We retrospectively assessed clinical presentation, duration of symptoms related to COVID-19 from paediatric patients with PCRD and compared their pre/post COVID-19-I spirometry values. Data from 12 patients were analysed. Timing between COVID-19 diagnosis and subsequent functional evaluation ranged from 26 to 209 days (mean 77). The PCRD in these patients included asthma, cystic fibrosis, bronchiolitis obliterans and bronchomalacia. During COVID-19, all clinical presentations were mild. One patient displayed persistent post-COVID-19 symptoms for 8 weeks after infection. Two patients presented significant deterioration of post-COVID-19 spirometric values with a return to pre-COVID-19 values in subsequent measures. We concluded that children with PCRD are not at increased risk for severe COVID disease and that most of them have no or only transient pulmonary functional impairment 1 to 7 months after COVID-19.

Hormonal Contraception Effects on Pulmonary Function in Adolescents with Cystic Fibrosis.

STUDY OBJECTIVE: Estrogens are suspected to have a negative effect on pulmonary function in women with cystic fibrosis (CF). The aim of our study was to investigate, in a CF adolescent population, the effect of hormonal contraception (HC) on lung function by assessing the forced expiratory volume in 1 second (FEV1), the number of exacerbations of pulmonary condition, and antibiotic use. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: We conducted a cohort retrospective chart review of girls from age 13 to 18 years old who were followed in the CF clinic of a university hospital center. Wilcoxon rank sum test with continuity correction, 2-sample t test, conditional test of Poisson rates, and χ2 test were conducted to identify differences in results between adolescents with or without use of HC for the following outcomes: FEV1, use of antibiotics by nebulizer, and hospital admission for exacerbations of pulmonary condition. RESULTS: Among 127 adolescents, 64/127 (50.4%) took HC; 12/127 (9%) continuously had been taking HC over 3 years. For girls taking HC for more than 3 years, FEV1 at 18 years old was significantly higher than for girls who had never taken HC (85.17% vs 71.05%; P = .043). However, there was no difference in the number of hospital admissions for exacerbation of pulmonary condition between these 2 groups (P = .057). There was no difference between HC vs non-HC users in the percent of patients taking antibiotics by nebulizer over the 6 years of follow-up. CONCLUSION: Our study suggests that in adolescents with CF, HC has no deleterious effects on the FEV1. Further prospective studies could be done to confirm these results.

Association between fat-soluble nutrient status and auditory and visual related potentials in newly diagnosed non-screened infants with cystic fibrosis: A case-control study.

Nutritional deficiencies often precede the diagnosis of cystic fibrosis (CF) in infants, and occur at a stage where the rapidly developing brain is more vulnerable to insult. We aim to compare fat-soluble nutrient status of newly diagnosed non-screened infants with CF to that of healthy infants, and explore the association with neurodevelopment evaluated by electroencephalography (EEG). Our results show that CF infants had lower levels of all fat-soluble vitamins and docosahexaenoic acid (DHA) compared to controls. The auditory evoked potential responses were higher in CF compared to controls whereas the visual components did not differ between groups. DHA levels were correlated with auditory evoked potential responses. Although resting state frequency power was similar between groups, we observed a negative correlation between DHA levels and low frequencies. This study emphasizes the need for long-term neurodevelopmental follow-up of CF infants and pursuing intervention strategies in the future.

Restricting indications for sinonasal computed tomography in children with cystic fibrosis.

OBJECTIVES To evaluate whether a low rate of exposure to sinonasal computed tomographic (CT) scans can be achieved when strict criteria are applied for their use in children with cystic fibrosis (CF) and to emphasize the importance of limiting radiation exposure in the context of the current longer life expectancy in this group of patients. DESIGN Retrospective chart review. SETTING Tertiary care children's hospital. PATIENTS The study included 277 children who were regularly followed up in the CF clinic in the last 11 years (mean duration of follow up, 7.87 years), 33 of whom underwent sinonasal CT. MAIN OUTCOME MEASURES Indications used for scanning, health professional (ear, nose, and throat specialist or pulmonologist) ordering the test, eventual modifications of ongoing treatment according to CT results, and time lapse between CT scanning and surgery. RESULTS Of 277 children with CF, 33 (12%) underwent a total of 39 sinonasal CT scans during the follow-up period (0.018 scans per patient per year of follow-up). Twenty-nine of the CT scans (74% of all cases, 90% of CT scans ordered by the ear, nose, and throat surgeon) were performed in the preoperative context and demonstrated the extent of the polypoid disease whenever present (26 cases [90%]) and the cause of nasal obstruction (20 cases [69%]). The mean period between the scanning and the surgery was 57 days (range, 0.10-173 days). Computed tomographic scans that were not meant for preoperative planning were performed in 10 cases (26%). The indications were disease evaluation (10%), ruling out a mucocele (5%); pre-lung transplantation status (5%), ruling out an intraorbital complication (3%); and headache investigation (3%). The results of the scans did not modify the management of the disease in those patients. CONCLUSIONS With the use of stringent criteria, it is possible to achieve a low rate of exposure to sinonasal CT scans in the population of children with CF. The main indication should be the preoperative planning regarding anatomy, extent of disease, and sites of nasal obstruction. The use of CT scans for disease evaluation does not seem to appreciably modify the treatment course and could be avoided.

13C-labeled mixed triglyceride breath test (13C MTG-BT) in healthy children and children with cystic fibrosis (CF) under pancreatic enzyme replacement therapy (PERT): a pilot study.

The MTG-BT estimates the hydrolysis of triacyl-glycerols by pancreatic lipase, and appears attractive for monitoring exogenous lipase requirements in patients with exocrine pancreatic insufficiency. To assess the test's discrimination capacity and repeatability, 9 CF patients with PERT and 10 healthy children underwent the (13)C-MTG-BT twice, at a 2- to 4-week interval. The test distinguished well between patients with severe exocrine pancreatic insufficiency (SEPI) and healthy subjects. However, within-subject variability for postprandial per thousand(13)C-enrichment and postprandial % dose recovery (PDR) was high in both groups. Therefore, the (13)C-MTG-BT seems useful to distinguish between SEPI and normal exocrine pancreatic function, but requires further development to improve its repeatability.

Prophylaxis against respiratory syncytial virus in young children with cystic fibrosis.

INTRODUCTION: In cystic fibrosis (CF) patients, respiratory syncytial virus (RSV) infection is associated with significant morbidity. Although passive prophylaxis with palivizumab lowers hospitalization rate for RSV infection in populations at risk of severe infection, its use is not recommended in infants with CF disease. OBJECTIVE: To determine the effect of palivizumab prophylaxis on hospitalization for acute respiratory illness in young children with CF during the first RSV season following the diagnosis of CF. METHODS: In this retrospective study, medical records of patients diagnosed with CF between the years 1997 and 2005 inclusively and on whom the diagnosis was made before 18 months of age were reviewed. Collected data included age at diagnosis, palivizumab prophylaxis, occurrence of hospitalization for acute respiratory tract illness during the RSV season and identification of RSV infection. RESULTS: A diagnosis of CF was made in 76 young children and data collected from 75 children. Of those, 40 did not receive RSV prophylaxis while 35 received palivizumab injection monthly during the RSV season. Among non-recipient children, 7 out of 40 were hospitalized for acute respiratory illness during the RSV season. Of these seven patients, RSV detection was positive in nasopharyngeal secretions in three patients, negative in one patient and not requested in the others. Among palivizumab recipients, 3 out of 35 children were hospitalized for acute respiratory illness (P > 0.05 compared to non-recipients group). In these three palivizumab recipients, RSV detection was negative in nasopharyngeal secretions. Palivizumab recipients experienced fewer hospital days per patient for acute respiratory illness (mean +/- SD: 0.8 +/- 3.07 days) as compared to non-recipients (mean +/- SD: 1.73 +/- 4.27 days) but this difference did not reach statistical significance. CONCLUSION: CF infants may benefit from RSV immunoprophylaxis with palivizumab.

Comparative efficacy of two doses of nebulized colistimethate in the eradication of Pseudomonas aeruginosa in children with cystic fibrosis.

BACKGROUND: Cystic fibrosis (CF) affects the respiratory and digestive systems. It evolves toward deterioration of pulmonary function through colonization with Pseudomonas aeruginosa. There is no consensus with respect to its eradication. Nebulized colistimethate is used for eradication treatment, but the optimal dose and duration is yet to be determined. OBJECTIVES: To compare the efficacy of two doses of nebulized colistimethate (30 mg versus 75 mg twice daily) for the eradication of P aeruginosa in children with CF and intermittent colonization. METHODS: A cohort study with both historical (30 mg) and prospective (75 mg) arms was conducted from 1999 to 2003. Medical records were used to collect data. RESULTS: Eighty-one patients were recruited in the retrospective group, for a total of 111 treatment courses. Twenty patients were recruited in the prospective group, for a total of 20 events. There was no statistically significant difference in the rate of eradication of P aeruginosa at days 28 and 90, neither when comparing the doses of colistimethate nor duration of treatment. There was a statistically significant difference (P=0.004) between days 1 and 90 in all analyzed subgroups (regardless of dose or duration of treatment) for forced vital capacity only. In the group of patients in whom eradication was achieved at day 28 (after receiving a three-week treatment course of colistimethate), 50% of patients developed a new infection 5.75 months later, on average, regardless of the dose administered. In the group of patients who achieved eradication at day 90 (after receiving a 15-week treatment course of colistimethate), 50% of the 14 patients developed a new infection after an average period of 7.3 months (P=0.28). CONCLUSIONS: There is no difference in the efficacy between a 30 mg dose and a 75 mg dose of colistimethate for P aeruginosa eradication in children with CF and intermittent colonization.

Epidemiology of liver disease in cystic fibrosis: a longitudinal study.

BACKGROUND/AIMS: To describe the prevalence of liver disease in a cohort of 241 cystic fibrosis (CF) patients. METHODS: 241 CF patients were followed-up every 3 months with clinical and biological assessment, and every year with ultrasonography of the liver. The presence of liver disease was studied using a multivariate Cox's regression analysis including variables such as history of meconium ileus, pulmonary function, pancreatic insufficiency and CFTR gene mutations. RESULTS: The prevalence of liver disease was 18, 29, and 41% after 2, 5 and 12 years, respectively, and did not increase thereafter. In multivariate analysis, the probability of liver disease was independently associated with history of meconium ileus (P = 0.001) and pancreatic insufficiency (P = 0.004). CFTR mutations and severity of pulmonary disease were not associated with liver disease. Cirrhosis occurred in 19 (7.8%) patients at a median age of 10 years, and liver transplantation was required in five patients. CONCLUSIONS: This study shows that CF related-liver disease occurs mainly in the first decade of life with a prevalence of 41% of patients at 12 years of age. A history of meconium ileus and pancreatic insufficiency are predictive of liver disease. Preventive treatment with ursodesoxycholic acid could be considered in patients with meconium ileus.

Surveillance for cystic fibrosis-associated hepatobiliary disease: early ultrasound changes and predisposing factors.

OBJECTIVE: To investigate routine ultrasonography (US) as an early marker and to identify risk factors for the development of cirrhosis and portal hypertension (PHT) in cystic fibrosis (CF). STUDY DESIGN: A cohort of 106 children with CF aged 5.9+/-2.3 years were followed for 10.4+/-0.2 years in a CF clinic. RESULTS: At enrollment, the US was normal, but biochemical and/or clinical disease was present in 10%. By the end of the study, 19 had developed US changes, eight with evidence of PHT. At the time of the initial US change, only 36.4% of those had, at the end of the study, either a heterogeneous or a nodular parenchyma, and only 50% of those with PHT had biochemical and/or clinical disease. Of the 30 patients treated with ursodeoxycholic acid for biochemical and/or clinical disease with (n=15) and without (n=15) associated US changes, PHT developed in six of the former and two of the latter. Univariate analysis and logistic regression showed that children with more severe disease in terms of forced expiratory volume in one second were at somewhat greater risk (P<.06) of PHT developing. CONCLUSION: US was an early marker of liver disease and more severe CF disease, a predictor of progressive liver disease. A controlled trial should be done to assess isolated US-detected disease as an indication for UDCA.