Nasal polyposis from cystic fibrosis in children - the experience of a single center.

BACKGROUND: Cystic fibrosis (CF) is the most common monogenic disease, characterized by clinically notable polymorphism. Respiratory disease is the main factor that influences the disease outcome and prognosis of the patient with CF, bacterial infections being responsible for severe exacerbations and rhinosinusitis a difficult complication, besides lung disease. AIM: The aim of the paper was to present a case series of CF-associated nasal polyposis and our management experience, providing new data for nasal and sinus complications. PATIENTS, MATERIALS AND METHODS: Patients attending the National Cystic Fibrosis Center, Timisoara, Romania, were evaluated for nasal polyposis. Besides clinical examination, endoscopy, and computed tomography (CT) was performed for comprehensive evaluation. Patients with persistent symptoms or with complicated sinusitis underwent surgical approach. RESULTS: Fourteen (18.18%) children were diagnosed with nasal polyposis and had surgery, with positive outcomes. One patient received Omalizumab for an associated, uncontrolled asthma with a subsequent substantial effect with the significant polyp reduction and lack of recurrence. CONCLUSIONS: Even if extremely difficult to manage, complicated nasal polyposis CF related might have an improved outcome and better life quality.

Oral Glucose Tolerance Test in Patients with Cystic Fibrosis Compared to the Overweight and Obese: A Different Approach in Understanding the Results.

(1) Background: In cystic fibrosis (CF), the oral glucose tolerance test (OGTT) is recommended from 10 years old annually to screen and diagnose cystic fibrosis-related diabetes (CFRD). Alternative OGTT characteristics (glucose curve shape, time to glucose peak, one-hour glucose value, and three-hour glucose value with the new shape curve) were studied in other populations considered at high risk for diabetes; (2) Methods: The study analyses classical and alternative OGGT characteristics from 44 children (22 CF, 22 obese without CF), mean age: 12.9 ± 2.2 years evaluated in a single-center from Romania. (3) Results: In 59.1% of children with CF, the predominant OGTT pattern was: abnormal glucose metabolism or CFRD, with a monophasic curve shape, a late peak glucose level, and 1 h glucose ≥ 155 mg/dL, showing a very different pattern compared with sex and age-matched obese children. Statistical estimation agreement between the late glucose peak (K = 0.60; p = 0.005), the 1 h glucose ≥ 155 mg/dL during OGTT (K = 0.69, p = 0.001), and the classical method of interpretation was found. (4) Conclusions: Late peak glucose and 1 h glucose level ≥ 155 mg/dL during OGTT can be used for diagnosing the early glucose metabolism alteration in children with CF.

The Relation between Vitamin D Level and Lung Clearance Index in Cystic Fibrosis-A Pilot Study.

BACKGROUND: Life expectancy has increased in cystic fibrosis (CF) patients; however, the rate of mortality is still high, and in a majority of cases, the cause of death is due to respiratory deterioration. Vitamin D plays an important role in immunity and infection prophylaxis, as its deficiency is associated with frequent infections. In CF patients, a deficit of liposoluble vitamins is common, despite daily supplementation. The aim of this study is to evaluate the relation between vitamin D status and lung function expressed by lung clearance index (LCI) in patients with CF. We also assessed the relation of factors such as nutritional status, genotype, and associated comorbidities such as Pseudomonas infection, cystic fibrosis-related diabetes (CFRD), and cystic fibrosis liver disease (CFLD) with vitamin D and LCI. METHODS: A cross-sectional study was conducted at the National Cystic Fibrosis Center by analyzing patients with CF who presented in our center between November 2017 and November 2019. We enrolled in the study patients diagnosed with CF, who were followed up in our CF center and who were able to perform lung function tests. Patients in exacerbation were excluded. RESULTS: A strong negative correlation was found between vitamin D and LCI (r = -0.69, p = 0.000). A lower vitamin D storage was found in patients with CFLD and CFRD. Higher LCI values were found among patients with chronic Pseudomonas infection, with BMI under the 25th percentile, or with associated CFLD. CONCLUSION: In CF patients, vitamin D plays an important role, and its deficit correlates with an impaired LCI. Vitamin D deficit is a risk factor in patients with associated comorbidities such as CFLD and CFRD. Chronic infection with Pseudomonas, the presence of impaired nutritional status, and CFLD are associated with a prolonged LCI.

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

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.

SARS-CoV-2 Seroprevalence in Children from Western Romania, March to June 2021.

Limited data are available regarding severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) seroprevalence in children. In this study, we assessed for the first time the seroprevalence of SARS-CoV-2 in children from Romania. Serum samples of 379 children were investigated for the presence of SARS-CoV-2 total antibodies. Serologic tests were performed using Elecsys Anti-SARS-CoV-2 electrochemiluminiscence immunoassay that targets the nucleocapsid protein of the virus. The overall seroprevalence of SARS-CoV-2 total antibodies was 46.70%. No significant difference was observed between seropositive and seronegative children according to age groups, gender, and area of residence. Our findings revealed a high SARS-CoV-2 seroprevalence in Romanian children at the end of the third COVID-19 pandemic wave. Results suggest that children, regardless of age, gender, or area of residence, are susceptible to infection with SARS-CoV-2. Seroprevalence in children was similar to the seroprevalence reported in the adult population from Western Romania during the same period of time, March to June 2021.

Antibiotherapy in Children with Cystic Fibrosis-An Extensive Review.

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.

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

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.

Lung Ultrasound Is More Sensitive for Hospitalized Consolidated Pneumonia Diagnosis Compared to CXR in Children.

BACKGROUND: Pneumonia is the leading cause of death among children; thus, a correct early diagnosis would be ideal. The imagistic diagnosis still uses chest X-ray (CXR), but lung ultrasound (LUS) proves to be reliable for pneumonia diagnosis. The aim of our study was to evaluate the sensitivity and specificity of LUS compared to CXR in consolidated pneumonia. METHODS: Children with clinical suspicion of bacterial pneumonia were screened by LUS for pneumonia, followed by CXR. The agreement relation between LUS and CXR regarding the detection of consolidation was evaluated by Cohen's kappa test. RESULTS: A total of 128 patients with clinical suspicion of pneumonia were evaluated; 74 of them were confirmed by imagery and biological inflammatory markers. The highest frequency of pneumonia was in the 0-3 years age group (37.83%). Statistical estimation of the agreement between LUS and CXR in detection of the consolidation found an almost perfect agreement, with a Cohen's kappa coefficient of K = 0.89 ± 0.04 SD, p = 0.000. Sensitivity of LUS was superior to CXR in detection of consolidations. CONCLUSION: Lung ultrasound is a reliable method for the detection of pneumonia consolidation in hospitalized children, with sensitivity and specificity superior to CXR. LUS should be used for rapid and safe evaluation of child pneumonia.