Primary ciliary dyskinesia in six patients with bronchiectasis

INTRODUCTION: Primary ciliary dyskinesia [PCD] is generally considered as a rare autosomal recessive disorder. Previous studies reported various prevalence of PCD among patients with bronchiectasis. MATERIAL AND METHODS: Six PCD patients who were diagnosed during the investigation of 40 patients with bronchiectasis were enrolled in this study. Ultra structural studies for both epithelium and cilia were performed, and the deformities in detailed electron microscopic images confirmed the diagnosis of PCD. RESULTS: Four patients experienced the first symptoms shortly after the birth, 1 by the age of 1 and 1 by the age of 4 years. Except of 1 case that was diagnosed 2 months after the onset of disease, diagnosis delay was longer than 5 years in all patients. Consanguineous marriage was observed in the parents of all patients. Upper respiratory tract infections were documented for all patients. CONCLUSIONS: PCD should be considered as a probable underlying disorder in patients with bronchiectasis. Past medical history of otitis media and history of similar clinical findings in family members should raise suspicion toward PCD.INTRODUCTION: Primary ciliary dyskinesia [PCD] is generally considered as a rare autosomal recessive disorder. Previous studies reported various prevalence of PCD among patients with bronchiectasis. MATERIAL AND METHODS: Six PCD patients who were diagnosed during the investigation of 40 patients with bronchiectasis were enrolled in this study. Ultra structural studies for both epithelium and cilia were performed, and the deformities in detailed electron microscopic images confirmed the diagnosis of PCD. RESULTS: Four patients experienced the first symptoms shortly after the birth, 1 by the age of 1 and 1 by the age of 4 years. Except of 1 case that was diagnosed 2 months after the onset of disease, diagnosis delay was longer than 5 years in all patients. Consanguineous marriage was observed in the parents of all patients. Upper respiratory tract infections were documented for all patients. CONCLUSIONS: PCD should be considered as a probable underlying disorder in patients with bronchiectasis. Past medical history of otitis media and history of similar clinical findings in family members should raise suspicion toward PCD

The Effect of Outpatient Management of Cystic Fibrosis Exacerbation on Pulmonary Function Tests: A Clinical Trial

Background: Cystic fibrosis (CF) is an inherited disease which involves multiple organs including respiratory tract. It results in chronic respiratory signs and symptoms which exacerbate with frequent lung infections. The majority of exacerbations require hospitalization and intravenous antibiotic therapy as part of the management. The aim of this survey was to study the effectiveness of outpatient management of pulmonary exacerbations on pulmonary function tests.Methods: This randomized clinical trial included all CF patients older than 6 years old who presented with a pulmonary exacerbation and were able to perform Spirometric tests. All eligible CF patients first referred to pulmonary function tests center to determine forced expiratory volume in the first second (FEV1), residual volume (RV), total lung capacity (TLC) and airway specific resistance (SRaw). Then, they were treated outpatiently for 2 to 4 weeks and after that, they underwent all the tests again; and the participants’ improvements in pulmonary function tests after outpatient treatment were investigated.Result: In this study, 32 patients were enrolled including 13 women (40.6%) and 19 men (59.4%). Mean and standard deviation (SD) of pulmonary variable, before and after the treatment were investigated. All of them were statistically significant. Sub-analysis of data based on gender was also performed showing that outpatient treatment led to significant improvements in all of the variables except for the percentage of RV in females (p value = 0.08).Conclusion: The findings revealed that outpatient management of CF exacerbations effectively improved values of FEV1, SRaw and TLC along with reducing RV values and air trapping. Thus, applying this method on appropriately selected CF patients, may prevent unnecessary hospitalization and subsequent disadvantages

First Cystic Fibrosis Patient Registry Annual Data Report - Cystic Fibrosis Foundation of Iran

Cystic fibrosis (CF), as a fatal genetic condition, is associated with high morbidity and mortality rates. In Iran, limited studies exist on this disease. This study aims to compare the demographic, clinical and paraclinical data of CF patients. This cross-sectional study was conducted in 2014-2015 on 174 CF patients referred to the Tehran Children Medical Center hospital, which is the main referral center for CF. For each patient, the forced  expiratory volume in one second (FEV1) was measured and the comparative demographic, clinical and laboratory data of patients were recorded. Overall, 59% of studied patients were boys (n=102) and 41% were girls (n=72). The mean patient age (and standard deviations) was 7.1 ±5.7 years, with a range of 10 days to 28 years. In 67% of cases, the disease was diagnosed before their first birthday. The patients in this study were classified based on the FEV1 into mild (62%), moderate (33%) and sever (5%), indicating the degree of pulmonary complications. Cultures of respiratory secretions were positive for Pseudomonas aeruginosa and Staphylococcus aureus, in 23% and 16% of cases, respectively. In total, 61% of patients (n=83) were assigned to receive oral azithromycin for prophylaxis. Gastroesophageal reflux (reflux) was the most common gastrointestinal complication (35%), Regarding the complex nature of CF and the necessity of constant monitoring of patients during the life-span, the comparative demographic, clinical and laboratory analysis of patients and registering and standardization of patients’ data, can be a major step in the better understanding of the disease, and thereby increasing the quality of life and life expectancy in the affected population

The relationship of air pollution and surrogate markers of endothelial dysfunction in a population-based sample of children

<p>Abstract</p> <p>Background</p> <p>This study aimed to assess the relationship of air pollution and plasma surrogate markers of endothelial dysfunction in the pediatric age group.</p> <p>Methods</p> <p>This cross-sectional study was conducted in 2009-2010 among 125 participants aged 10-18 years. They were randomly selected from different areas of Isfahan city, the second large and air-polluted city in Iran. The association of air pollutants' levels with serum thrombomodulin (TM) and tissue factor (TF) was determined after adjustment for age, gender, anthropometric measures, dietary and physical activity habits.</p> <p>Results</p> <p>Data of 118 participants was complete and was analyzed. The mean age was 12.79 (2.35) years. The mean pollution standards index (PSI) value was at moderate level, the mean particular matter measuring up to 10 μm (PM₁₀) was more than twice the normal level. Multiple linear regression analysis showed that TF had significant relationship with all air pollutants except than carbon monoxide, and TM had significant inverse relationship with ozone. The odds ratio of elevated TF was significantly higher in the upper vs. the lowest quartiles of PM₁₀, ozone and PSI. The corresponding figures were in opposite direction for TM.</p> <p>Conclusions</p> <p>The relationship of air pollutants with endothelial dysfunction and pro-coagulant state can be an important factor in the development of atherosclerosis from early life. This finding should be confirmed in future longitudinal studies. Concerns about the harmful effects of air pollution on children's health should be considered a top priority for public health policy; it should be underscored in primordial and primary prevention of chronic diseases.</p

Primary Ciliary Dyskinesia in Six Patients with Bronchiectasis

Introduction: Primary ciliary dyskinesia [PCD] is generally considered as a rare autosomal recessive disorder. Previous studies reported various prevalence of PCD among patients with bronchiectasis. Material and methods: Six PCD patients who were diagnosed during the investigation of 40 patients with bronchiectasis were enrolled in this study. Ultra structural studies for both epithelium and cilia were performed, and the deformities in detailed electron microscopic images confirmed the diagnosis of PCD. Results: Four patients experienced the first symptoms shortly after the birth, 1 by the age of 1 and 1 by the age of 4 years. Except of 1 case that was diagnosed 2 months after the onset of disease, diagnosis delay was longer than 5 years in all patients. Consanguineous marriage was observed in the parents of all patients. Upper respiratory tract infections were documented for all patients. Conclusions: PCD should be considered as a probable underlying disorder in patients with bronchiectasis. Past medical history of otitis media and history of similar clinical findings in family members should raise suspicion toward PCD

Lactose-Free Compared with Lactose-Containing Formula in Dietary Management of Acute Childhood Diarrhea

Objective: Few reports are available on some benefits, such as shortened duration of diarrhea and better weight gain, for lactose-free over lactose-containing formula in acute childhood diarrhea. We evaluated the effects of lactose-free formula in dietary management of acute diarrhea in formula-fed children. Methods: This controlled-clinical trial was conducted on formula-fed children, aged 1 to 24 months, referring with acute non-bloody diarrhea (≤2 weeks). Those who had major systemic illness, severe malnutrition, severe dehydration, severe vomiting, or history of antibiotic therapy were not included. Children were allocated to receive lactose-free formula (intervention, n=37) or lactose-containing formula (control, n=34). Time to diarrhea relief and weight change were compared between the two groups after one week. Findings: During the study, 32 male and 39 female children (7.1±3.7 months) were included. Those who received lactose-free formula had a significantly shorter time to diarrhea relief compared with the controls (1.7±0.7 vs. 2.6±0.7 days, P<0.001). Weight significantly increased in both groups, but there was no difference between the two groups in weight change (37±100 vs. 38±77 gr, P=0.673). Multivariate analysis showed that receiving lactose-free formula significantly predicted time to diarrhea relief (95% CI: 1.5 to 3.9, P<0.001) controlling for baseline characteristics. Conclusion: Early administration of lactose-free formula for formula-fed children presenting with acute diarrhea can result in a more rapid relief of acute diarrhea and thus perhaps less mortality and morbidity. Trials with longer follow-ups are warranted to better evaluate long-term results such as weight change and feeding problems in this regard

The relationship between the burden of care and fear of disease progression in mothers of children with cystic fibrosis

Objectives: Aim of this study was determining the relationship between burden of care and fear of disease progression in mothers of children with cystic fibrosis. Methodology: 142 mothers of children with cystic fibrosis (CF) participated in this correlational study. Data were collected with sequential method. The researchers employed the self-report Zarit burden of care and fear of disease progression questionnaire in parents of children with CF. Mothers answered the questioners separately.&nbsp; Data were analyzed with statistical analysis of variance and independent t-test. Results: According to the results, the mean maternal burden of care was 34.12 ± 69.77, and the mean fear of disease progression was 32.12 ± 92.11. The burden of care had the highest regression effect on fear of disease progression in mothers of children with CF (β = 0.896). With an increase of one unit of standard deviation in the burden of care, an increase of 0.896 units occurred in fear of disease progression in parents of children with CF. Conclusion: Mothers of children with CF experience a moderate burden of care and fear of disease progression. An increase in the burden of care and psychological stress increases the fear of disease progression in these mothers

Associação da poluição atmosférica com parâmetros hematológicos em crianças e adolescentes

OBJETIVO: Avaliar a relação entre poluição atmosférica e parâmetros hematológicos em uma amostra populacional de crianças e adolescentes. MÉTODOS: Este estudo transversal foi realizado em 2009-2010 com estudantes escolhidos aleatoriamente de diversas áreas de Isfahan, a segunda maior e mais poluída cidade iraniana. A associação entre os níveis de poluentes do ar e os de hemoglobina, plaquetas, glóbulos brancos (GB) e glóbulos vermelhos (GV) foi determinada pelas análises linear múltipla e de regressão logística ajustadas para idade, sexo, medidas antropométricas, fatores meteorológicos, e hábitos alimentares e de atividade física. RESULTADOS: Participaram do estudo 134 estudantes (48,5% meninos), com idade média de 13,10±2,21 anos. Com níveis moderados de Pollutant Standards Index (PSI), a média de material particulado (particulate matter) < 10 µm (PM10) foi mais do que o dobro do normal. A análise de regressão linear demonstrou que o PSI e a maioria dos poluentes atmosféricos, especialmente PM10, estiveram negativamente relacionados com a contagem de hemoglobina e GV e positivamente relacionados com a contagem de GB e plaquetas. O odds ratio de uma elevação nos GB aumentou conforme os quartis de PM10, ozônio e PSI aumentavam, embora essas associações fossem significativas somente no quartil superior de PM10 e PSI. Os valores correspondentes de hemoglobina e GV seguiram a direção oposta. CONCLUSÕES: Destaca-se a associação dos poluentes atmosféricos com parâmetros hematológicos e um possível estado pró-inflamatório. A presença dessas associações com PM10 em níveis regulares de PSI enfatiza a necessidade de se reavaliar as políticas ambientais de saúde na faixa etária pediátrica

Association of childhood croup and increased incidence of airway hyperreactivity in adulthood

BACKGROUND: Some evidence suggests that childhood croup could be associated with increased incidence of adulthood bronchial reactivity, but its significance is uncertain. The aim of the present study was to evaluate the long-term outcome of early life croup. PATIENTS AND METHODS: This case–control study was conducted in 2010–2012 in Isfahan, Iran. The case group consisted of 164 adolescents with a history of severe croup in early life and an equal number of healthy controls without any history of croup or other chronic or recurrent respiratory diseases. The two groups were compared according to pulmonary function tests and bronchial reactivity (exercise challenge test). Statistical analyses were performed using the SPSS software package, version 20 (SPSS Inc., Chicago, IL, USA). P < 0.05 was considered significant. RESULTS: Baseline spirometric values (forced expiratory volume in 1st s (FEV1), forced volume capacity (FVC), FEV1/FVC, and forced expiratory flow at 25%–75% (FEF25–75) were similar in case and control groups. A reduction in FEV1 and FEF25–75 after exercise challenge test was seen in 9% and 12.8% of patients, respectively, whereas this was reduced in only 4.2% and 6.1% of the controls (P = 0.034 and P = 0.021, respectively). CONCLUSION: Our findings suggest that childhood croup might be a predisposing factor for bronchial hyperreactivity in adulthood. Longitudinal studies are necessary to confirm the clinical significance of these findings

The Etiology of Bronchiectasis in Iran

BackgroundBronchiectasis is defined by permanent and abnormal widening of the bronchi. Although this process occurs in the context of chronic airway infection and inflammation, since there is no accurate estimation of the etiology of the disease. This study aimed to determine the most important cause of bronchiectasis in Tehran, Iran.Materials and MethodsIn this retrospective cohort study we used the information of 91 patients admitted to two subspecialty lung hospitals in Tehran-Iran, where a wide range of bronchiectasis patients from around the country referred during 2013to 2014 period. Patients referring with the manifestation of chronic productive cough who had not responded to conventional treatment with the evidences of bronchiectasis on high resolution computed tomography were included. Data were analyzed using SPSS-16.0.ResultsThe etiology of bronchiectasis was diagnosed in 73 of 91 patients (80.2%), the most important of which included cystic fibrosis, post infectious, and primary ciliary dyskinesia (PCD). The most common causes of bronchiectasis in the children group (Age ≤ 18 years), were cystic fibrosis (57.1%), allergic bronchopulmonary aspergillusis (14.3%) and PCD (9.5%), respectively. In the adults group (Age >18 years), the most common causes were post infectious (22.6%), PCD (15.7%) and cystic fibrosis (14.3%), respectively.ConclusionMain causes of bronchiectasis in this study were not significantly different from other studies. Special attention should be paid to the probable causes of bronchiectasis in order to effectively execute on-time diagnosis, proper treatment and management of complications