The effect of Lactobacillus reuteri on pulmonary function test and growth of cystic fibrosis patients.

INTRODUCTION: Cystic fibrosis (CF) patients frequently experience gut microbiota dysbiosis. Probiotic supplementation is a potential therapeutic approach to modify gut microbiota and improve CF management through the gut-lung axis. The aim of this study was to investigate the effect of Lactobacillus reuteri supplementation on pulmonary function test, respiratory symptoms and growth in CF patients. METHODS: A randomized, placebo-controlled clinical trial was carried out on 40 children with CF aged from 6 to 20 years. Participants were designated to receive either L. reuteri or placebo daily for 4 months. Pulmonary function tests, weight, height and body mass index (BMI) z-scores were measured pre and post treatment. RESULTS: The median baseline BMI of the patients was 16.28 kg m-2. A significant change in the probiotic group's BMI z-score after the study period was observed (P = 0.034) but not for weight and height z-scores (P > 0.05). After treatment, Pseudomonas aeruginosa grew in sputum cultures of seven in the placebo and one patient in the intervention group (P = 0.03) while at baseline it grew in the sputum of four patients in each group. There was no significant difference in forced expiratory volume in the first second, forced expiratory flow at 25-75% or forced vital capacity change between the two groups after the treatment period (P > 0.05). Additionally, no significant differences were found in pulmonary exacerbations, hospitalization frequencies or COVID-19 infection between the two groups during the study (P > 0.05). CONCLUSION: The results suggest that L. reuteri supplementation may impact the growth of severely malnourished CF patients. Furthermore, it may be concluded that this strain might reduce P. aeruginosa in the sputum culture of CF patients. © 2024 Society of Chemical Industry.

Effects of Vitamin D and E Supplementation on Prevention of Bronchopulmonary Dysplasia (BPD) in Premature Neonates: A Systematic Review and Meta-Analysis.

BACKGROUND: Bronchopulmonary Dysplasia (BPD) has a multifactorial etiology. Vitamin E and vitamin D play an important role in lung development and can potentially be beneficial in the prevention of BPD. OBJECTIVE: The study aimed to compare the risk of BPD occurrence in preterm neonates supplemented with vitamin D or E versus those who did not get supplementation. METHODS: The literature search was conducted for this systematic review by searching the PubMed, Scopus, and Web of Science databases up to December 2022. Randomized controlled trials involved administering vitamin D or E to preterm neonates and examining the occurrence of BPD. We excluded non-English articles, and articles with non-relevant and insufficient data. We used the Critical Appraisal Skills Programme (CASP) checklist to assess the quality of the included studies. We used Egger's test to evaluate the risk of bias among the included studies. Heterogeneity was also assessed through Q-test and I2. We applied the random effect model for analysis. A P-value less than 0.05 was considered as significant. All the statistical analysis in the current study was performed using STATA 14. The Relative Risk (RR) was calculated as the effect size with 95% Confidence Interval (CI). RESULTS: Three eligible studies seeking the role of vitamin D in the prevention of BPD were analysed. Meta-analysis revealed that receiving vitamin D supplementation can significantly reduce the risk of BPD in preterm infants (RR = 0.357, 95% CI: 0.189-0.675, I2 = 0.0%; p = 0.002). Similarly, for assessing the role of Vitamin E in the prevention of BPD, three eligible studies were analysed. Vitamin E supplementation was not found to play a significant role in the reduction of BPD (RR = 0.659, 95%CI = 0.243-1.786, I2 = 38.7%; p = 0.412). CONCLUSION: Vitamin D supplementation could be beneficial in preventing BPD in preterm infants. However, evidence is not enough regarding vitamin E's role in reducing the incidence of BPD in preterm infants.

Vitamin D deficiency in patients with cystic fibrosis: a systematic review and meta-analysis.

AIM: Vitamin D is a prominent modulator of immunity and respiratory function. It plays a vital role in respiratory diseases such as cystic fibrosis (CF). S. However, there is a dearth of information on patients with CF. The purpose of the meta-analysis is to highlight the importance of following the existing guidelines regarding maintenance of Vitamin D serum levels in patients with CF. METHODS: The systematic search was conducted without utilizing any time or language limitations in original database from the beginning until March 2022. The meta-analysis was performed using a random-effects model. Heterogeneity was determined by I2 statistics and Cochrane Q test. RESULTS: Pooled analysis using the random-effects model of the 8 case-control studies with 13 effect sizes revealed that the serum 25-OH-vitamin D in participants with cystic fibrosis was significantly lower than controls in pediatrics and adolescences (WMD: - 3.41 ng/ml, 95% CI - 5.02, - 1.80, p = < 0.001) and adults (WMD: - 2.60 ng/ml, 95% CI - 4.32, - 0.89, p = 0.003). Based on data from 12 studies (21 effect sizes) with a total of 1622 participants, the prevalence of vitamin D levels of 20-30 ng/ml in CF patients was 36% among pediatrics/adolescents and 63% among adults. In addition, 27% of pediatric/adolescent CF patients and 35% of adult CF patients had vitamin D levels of below 20 ng/ml. CONCLUSIONS: As a result, according to the existing guidelines, our results proved the need to pay attention to the level of vitamin D in these patients.

Evaluating the efficacy of inhaled amikacin as an adjunct to intravenous combination therapy (ceftazidime and amikacin) in pediatric cystic fibrosis pulmonary exacerbation.

Background: Pseudomonas aeruginosa is the most common microorganism found in the sputum culture of Cystic fibrosis (CF) patients causing the pulmonary destruction. Aminoglycosides have a low diffuse rate from lipid membranes, and respiratory system secretions. Regarding the burden of pulmonary exacerbation caused by the pseudomonas aeruginosa in cystic fibrosis patients in the long term and the limited number of clinical trials focused on appropriate treatment strategies, the present study evaluated the concurrent inhaled and intravenous aminoglycoside antibiotics for pulmonary exacerbation caused by the pseudomonas aeruginosa as a safe and effective treatment in children. Method: This study was a blinded, randomized clinical trial phase conducted in a tertiary referral pediatric teaching hospital from May 2021 to May 2022. The patients were randomly allocated to receive intravenously administered ceftazidime and Amikacin alone or with inhaled Amikacin. Forced expiratory volume (FEV1), Amikacin via the level, kidney function tests, audiometry, inflammatory markers (erythrocyte sedimentation rate and C-reactive protein), hospital stay, and bacterial eradication rate were compared in two therapy groups. Results: the average FEV1 has increased by 47% in Neb + group compared to Neb- group following treatment. Hospital stay was lower in Neb + group. No renal toxicity or ototoxicity was observed in both therapy groups. Pseudomonas aeruginosa eradication rate Neb- and Neb + groups were 44% and 69%, respectively (p-value = 0.15). Conclusion: Concurrent inhaled and intravenous Amikacin is safe and effective to treat Pseudomonas aeruginosa exacerbation in CF patients. Moreover, co-delivery antibiotics' route treatment increased the eradication rate. Although not statistically significant, never the less, it is clinically relevant. The intervention reduced the length of hospitalization in this group. Clinical Trial Registration: clinicaltrials.gov, identifier [IRCT20120415009475N10].

Role of A Single Nucleotide Polymorphism in Thrombospondine 4 Gene in Premature Myocardial Infarction among Population of Southern Iran.

BACKGROUND: Coronary Artery Diseases (CAD) are the leading cause of Myocardial Infarction (MI). However, their underlying etiology can be found in the interplay between environmental and genetic factors. On the other hand, it has been shown that Extracellular Matrix (ECM) proteins, such as Thrombospondins (TSP), play a crucial regulatory role in vascular pathologies, including atherogenesis. TSPs are extracellular proteins responsible for intercellular and cell-ECM interactions and are involved in regulating functional responses. Recently, a missense mutation in the TSP-4 gene has been reported to potentially increase the risk of CADs. The present study aimed to investigate the role of rs1866389 Guanosine to Cytosine (G/C) Single Nucleotide Polymorphism (SNP) of the TSP-4 gene on the prevalence of premature MI in southern Iran. METHOD: The present case-control study included 100 patients with premature MI and 100 healthy individuals. The DNA extracted from the blood samples of the participants underwent Polymerase Chain Reaction (PCR) for the sequence of the TSP-4 gene. Afterward, the frequency of C (mutated) and G (normal) alleles of the TSP-4 gene was evaluated in the case and control groups. RESULTS: According to our findings, there was no significant intergroup difference in gender, age, and smoking status. However, the case group was significantly higher in the prevalence of Diabetes mellitus (DM), Hyperlipidemia (HLP), and Hypertension (HTN) compared to the control group. Moreover, 22%, 49%, and 29% of the case group had CC, GC, and GG genotypes in the TSP-4 gene, respectively, while the prevalence of CC, GC, and GG genotypes were 10%, 44%, and 46% in the control group. Also, the prevalence of allele C was significantly higher in the case group (47%) compared to the control group (33%, P=0.043), showing its significant association with the increased risk of premature MI (OR = 1.80; 95% CI = 1.01-3.19). CONCLUSIONS: The rs1866389 G/C SNP of the TSP-4 gene significantly increased the risk of premature MI in the population of southern Iran. Thus, such mutated gene can be used as a target for gene therapy or a marker for early detection of individuals at high risk for CADs.

The association of fecal calprotectin and respiratory exacerbation in cystic fibrosis patients.

BACKGROUND: CF patients experience several episodes of pulmonary exacerbations and reduction in their lung function progressively. Lung function is not the only diagnostic index by physicians to decide if CF patients require antibiotic therapy following pulmonary exacerbations. Non-invasive fecal indicators are increasingly being used to assess intestinal inflammation. Calprotectin is the most extensively utilized fecal biomarker in recent CF researches. METHODS: In this longitudinal study, 30 CF patients (1-18 years) without current infectious gastroenteritis were recruited from Mofid Children's Hospital and Masih Daneshvari Hospital, Tehran, Iran. Then, fecal calprotectin levels were evaluated before treatment, two weeks after systemic antibiotic administration, as well as recurrence of pulmonary exacerbation after first post-hospital discharge. RESULTS: The initial fecal calprotectin level in CF patients receiving antibiotics was 651.13 ± 671.04, significantly decreasing two weeks after antibiotic therapy and following recurrence (171.81 ± 224.40, 607.93 ± 549.89, respectively; P < 0.01). Following systemic antibiotic treatment, the patient's respiratory and GI symptoms improved (P < 0.01). CONCLUSION: Our findings revealed that fecal calprotectin modifications are associated with CF pulmonary exacerbations and antibiotic treatment could reduce calprotectin levels. Therefore, the fecal calprotectin level could be considered as a diagnostic tool and an index to follow the response to treatment in CF pulmonary exacerbations.

Corneal opacification, an atypical presentation of cystic fibrosis: a case report and review of the literature.

BACKGROUND: Respiratory and gastrointestinal manifestations are the main causes of mortality and morbidity in cystic fibrosis. Although these symptoms are well recognized, ophthalmic involvement of cystic fibrosis secondary to vitamin A deficiency is uncommon and has been reported very rarely in the medical literature. CASE PRESENTATION: Here, we report a 2.5-year-old Iranian boy who presented with bilateral corneal xerosis and corneal opacity secondary to vitamin A deficiency related to cystic fibrosis malabsorption. CONCLUSION: Malabsorption of fat-soluble vitamins is a common presentation in cystic fibrosis, but corneal opacity secondary to vitamin A deficiency as the initial presentation of cystic fibrosis is a very rare manifestation of fat malabsorption. This highlights the importance of complete systemic examination besides ophthalmic examination in approaching a child with ophthalmic complaint.

To compare nasal mask with binasal prongs in delivering continuous positive airway pressure for reducing need of invasive ventilation: randomized controlled trial.

AIMS: To compare nasal mask with binasal prongs in delivering bubble continuous positive airway pressure (CPAP) for reducing need of invasive ventilation in VLBW infants: randomized controlled trial. METHODS: In this randomized control trial 178 infants were enrolled, 90 were randomized to nasal mask group and 88 to nasal prong group for delivering bubble CPAP. Preterm neonates between 26 and 32 weeks of gestational age requiring CPAP for respiratory distress within 6 h of life were assessed for eligibility and were included if neonates had spontaneous respiratory efforts with respiratory distress in the form of tachypnea (respiratory rate >60/min), intercostal/subcostal retractions, grunting (audible with/without stethoscope), Silverman Anderson score ≥3, increased respiratory efforts or cyanosis. Infants received either nasal mask or binasal prongs for delivery of bubble CPAP. The primary outcome was the need for mechanical ventilation in first 72 h of life. RESULTS: Baseline characteristics were comparable between the two groups. There was significant reduction in incidence of CPAP failure [15 (16.6%) versus 26 (29.5%); RR 0.47 (95% CI 0.23-0.97), p = .04]; nasal trauma (any grade) [14 (15.9%) versus 38 (43.2%); RR 0.26 (95% CI 0.12-0.52), p = .0002]; nasal trauma grade I [12 (13.3%) versus 23 (26.1%); RR 0.43 (95% CI 0.20-0.94), p = .03]; nasal trauma grade II [1 (1.1%) versus 10 (11.3%); RR 0.08 (95% CI 0.01-0.70), p = .02]; nasal trauma severe grade (II and III) [2 (2.2%) versus 15 (17.0%);RR 0.11 (95% CI 0.02-0.49), p = .004] and bronchopulmonary dysplasia [4 (4.4%) versus 12 (13.6%); RR 0.30 (95% CI 0.10-0.95), p = .04] in nasal mask group when compared to nasal prong group. CONCLUSION: Nasal mask leads to significant reduction in need for mechanical ventilation in initial 72 h.

Microbial contamination of home nebulizers in children with cystic fibrosis and clinical implication on the number of pulmonary exacerbations.

BACKGROUND: Early detection of pulmonary contamination in children with cystic fibrosis (CF) is essential since these children are vulnerable to Pseudomonas aeruginosa (P. aeruginosa) colonization. In Iran, home nebulization of antibiotics is a widespread practice in treatment for patients with CF and, to the best our knowledge, no bacteriological surveys have been conducted till date in this regard. METHOD: This observational, cross sectional study was conducted on 61 children with CF at Mofid Children's Hospital, Tehran, from September 2017 to march 2018. The swab sampling was performed from 61 home nebulizers used by children diagnosed with CF. Contemporaneous sputum sample or deep nasopharyngeal swab was taken from each patient for bacterial and fungal testing. Medical records of the patients were reviewed and the number of exacerbations were recorded over the last 12 months prior to the study enrollment. RESULTS: The results of study showed that, 43 (70.5%) nebulizers were contaminated; 31 (50.8%) mouthpieces, 21 (34.4%) reservoirs, and 11 (18%) connecting tubes. The most common organism to be isolated was P. aeruginosa and was recovered from 19 (31%) nebulizers, 16 of them belonged to patients chronically colonized with P. aeruginosa. The remaining three had at least one positive sputum culture for P. aeruginosa in the past 1 year before the study. There was a significant increase in the number of CF exacerbations with an average number of exacerbation being 1.5 ± 1(SD) over last 12 months in children who had pathogenic organisms recovered from their home nebulizers compared with 0.4 ± 0.7(SD) exacerbations per year in whom non-pathogenic organisms were isolated from their nebulizers (P < 0.001). CONCLUSION: The majority of domiciliary nebulizers used by children with CF were contaminated with microorganisms indicating that the nebulizers may serve as potential reservoirs of pathogens for the patients' lung. Perpetuating colonization is a possible concern in the ones recently colonized with P. aeruginosa and, therefore, decontamination of nebulizer requires more attention to prevent ongoing infection. The negative impact of contamination of nebulizer on CF exacerbation requires serious attention and further investigations.

Thoracotomy versus Video-Assisted Thoracoscopy in Pediatric Empyema.

BACKGROUND: To compare the outcomes of video-assisted thoracoscopic surgery (VATS) in comparison to open thoracic surgery in pediatric patients suffering from empyema. METHODS: A prospective study was carried out in 80 patients referred to the Department of Pediatric Surgery between 2015 and 2018. The patients were randomly divided into thoracotomy and VATS groups (groups I and II, respectively). Forty patients were in the thoracotomy group (16 males [40%], 24 females [60%]; average age, 5.77±4.08 years) and 40 patients were in the VATS group (18 males [45%], 22 females [55%]; average age, 6.27±3.67 years). There were no significant differences in age (p=0.61) or sex (p=0.26). Routine preliminary workups for all patients were ordered, and the patients were followed up for 90 days at regular intervals. RESULTS: The average length of hospital stay (16.28±7.83 days vs. 15.83±9.44 days, p=0.04) and the duration of treatment needed for pain relief (10 days vs. 5 days, p=0.004) were longer in the thoracotomy group than in the VATS group. Thoracotomy patients had surgical wound infections in 27.3% of cases, whereas no cases of infection were reported in the VATS group (p=0.04). CONCLUSION: Our results indicate that VATS was not only less invasive than thoracotomy, but also showed promising results, such as an earlier discharge from the hospital and fewer postoperative complications.

Assessing the Prevalence and Treatment of Malnutrition in Hospitalized Children in Mofid Children's Hospital During 2015-2016.

BACKGROUND: Malnutrition in hospitalized patients causes problems in treatment and increases hospitalization duration. The aim of this research was to determine the prevalence of malnutrition in hospitalized children. METHODS: Children aged 1 month to 18 years (n = 1186) who were admitted to medical and surgery wards of Mofid children's hospital from November 2015 to February 2016, entered the study. We measured different anthropometric variables in patients with malnutrition. Also, nutritional counseling was performed and three months follow-up was done. RESULTS: Patient data were registered in questionnaires particularly for children 2 years old and less. 597 children under 2 years of age and 607 children over two years entered the study. The data analysis was done by SPSS version 22.0 (Chicago, IL, USA). The t test inferential method was used in comparing variables. P values less than 0.05 were considered statistically significant. Based on the body mass index (BMI) Z score, and in accordance with the World Health Organization (WHO) cut-off, among children over 2 years, 9% were diagnosed as overweight or obese, 54% were within the normal range and 37% were underweight at time of admission. In the underweight group, 43% were mildly, 21.2% were moderately and 35.8% were severely underweight. Based on the weight for length Z score in patients less than 2 years of age at time of admission, 6% were overweight, 60% were in normal range and 34% were underweight. Among children with malnutrition, 21% had mild, 3.0% had moderate and 10% had severe malnutrition. No significant meaningful relation was found between prevalence of malnutrition and severity of illness. In the moderate to severe undernutrition group, nutritionist counseling was done. Comparison of BMI and weight, before and after admission (the baseline and the follow up visits), was done by means of repeated measurements. Comparison of the patient's weight at time of admission with weight at 1, 2 and 3 months after the first nutritional consultation showed statistically meaningful difference (P value < 0.05). CONCLUSION: Growth indices need to be evaluated in every hospitalized child. Nutritional consultation is useful in children with malnutrition. The main purpose of early diagnosis of malnutrition is to prevent its progression, and also to design a useful, applicable and cost-effective nutritional intervention for malnutrition treatment.

Laryngotracheoesophageal Cleft Type 3 and Severe Laryngotracheomalacia; Delayed Surgical Repair, a Treatment Challenge with an Excellent Outcome.

Laryngotracheoesophageal clefts (LTEC) are rare malformations which involve the upper respiratory and digestive tract. Surgical repair should be undertaken promptly to maintain a secure airway and prevent serious pulmonary aspiration. This paper reports the first case of LTEC type 3 with severe laryngotracheomalacia that was brought to Mofid children's hospital in late infancy with a poor health status. Delayed defect correction was our team strategy for the patient when she had achieved good weight gain. At the age of 22 months in collaboration with the pediatric surgical and otolaryngologist team, the repair of the laryngeal cleft was done with lateral open approach method. She was discharged with tracheostomy and gastrostomy. In the next six months follow up after the surgery tracheostomy decannulation and gastrostomy tube removal were done and the infant is now in regular follow-up.

Neonatal hypertension.

Neonatal hypertension (HT) is a frequently under reported condition and is seen uncommonly in the intensive care unit. Neonatal HT has defined arbitrarily as blood pressure more than 2 standard deviations above the base as per the age or defined as systolic BP more than 95% for infants of similar size, gestational age and postnatal age. It has been diagnosed long back but still is the least studied field in neonatology. There is still lack of universally accepted normotensive data for neonates as per gestational age, weight and post-natal age. Neonatal HT is an important morbidity that needs timely detection and appropriate management, as it can lead to devastating short-term effect on various organs and also poor long-term adverse outcomes. There is no consensus yet about the treatment guidelines and majority of treatment protocols are based on the expert opinion. Neonate with HT should be evaluated in detail starting from antenatal, perinatal, post-natal history, and drug intake by neonate and mother. This review article covers multiple aspects of neonatal hypertension like definition, normotensive data, various etiologies and methods of BP measurement, clinical features, diagnosis and management.