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.

Fractional Exhalation Nitric Oxide (FeNO) changes in cystic fibrosis patients induced by compound honey syrup: a pretest-posttest clinical trial.

OBJECTIVE: To evaluate the effect of Persian medicine Syrup 'compound honey syrup (CHS)' on fractional exhalation nitric oxide (FENO) changes in patients with cystic fibrosis (CF). STUDY DESIGN: We conducted a before-after clinical trial on 70 CF patients. All patients received classical treatments for CF along with CHS (including honey, Ginger, cinnamon, saffron, cardamom and galangal), 5-10 cc (depending on the age and weight of patients) in 100 cc of warm boiled water twice a day, 30 min after meals. In this clinical trial, before and 12 weeks after the start of the CHS, FeNO test was evaluated. RESULTS: From 70 patients were enrolled, 44 patients completed this 12-week course of treatment. At the end of the study, changes in FeNO was significantly different before and after treatment (P-value < 0.05). At the end of the study, no dangerous side effects of CHS was reported. CONCLUSIONS: This study revealed that CHS can be effective as a complementary and safe drug in the medication of CF patients.

Congenital Skin Rashes in an IVF Baby Progressed to Multisystem Langerhans Cell Histiocytosis with Lung and Bone Involvement: A Case Report and Literature Review.

Langerhans cell histiocytosis is an uncommon proliferative disorder that may influence many organs; so, the clinical presentations vary. Here we describe an 85-day-old female who was born with In vitro fertilization after 10 years of infertility. She referred to us due to severe pulmonary insufficiency and congenital progressive maculopapular rash with desquamation. There were significant cystic changes in chest imaging studies. Further evaluation demonstrated lytic lesions in cranial, femoral, and humorous bones. The skin biopsy verified the diagnosis of LCH. A combination of Vinblastine, VP16, and Dexamethasone regimen was applied for the patient. In the course of the disease, she encountered multiple bilateral pneumothoraxes but didn't respond to tube thoracostomy and chemotherapy management. The patient died due to respiratory failure raised from complications of lung involvement as a multisystem LCH, 29 days later. Pediatricians should pay much more attention to the cutaneous lesions in the neonatal period especially if there is any risk factor for presenting LCH such as IVF. The lesions should be monitored closely owing to a high correlation between skin lesions and MS LCH.

A dual H-type tracheoesophageal fistula; why not being repaired simultaneously? A case report and review of literature.

BACKGROUND: H-type Tracheoesophageal Fistula (TEF) is a particular type of congenital esophageal anomalies, in which patients present with non-specific symptoms that can result in delayed diagnosis. Here, we report two pediatric cases with a rarer variant called ?dual H-type TEFË®. CASE PRESENTATION: We present two cases of H-type TEF. The first was a 45-day-old boy with feeding problem and cyanosis while feeding, and the second was a three-month-old girl with cough and choking after feeding from the first day of birth. In both cases, two separate TEFs were detected during diagnostic evaluation by flexible bronchoscopy. Both were repaired simultaneously through a cervical incision. The first patient deteriorated 13 days after the surgery, disturbancing in acid-base balance and expired unfortunately. CONCLUSION: Hence, it is necessary to consider the possibility of double TEF in any newly diagnosed H-type TEF.

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].

The usage of a magnet-powered instrument in the extraction of metallic or magnetic aspirated foreign bodies: A case report.

INTRODUCTION AND IMPORTANCE: The unintended passage of foreign bodies into the airway, is a common problem among children which can lead to death. Prompt diagnosis and extraction of the entrapped object is the key of success in these cases. Unfortunately, in addition to various extraction methods, in some cases the bronchoscopy may not be successful, and exploratory thoracotomy is inevitable. CASE PRESENTATION: We describe the story of a 7-year-old preschooler boy who was taken to the emergency department complaining the sudden onset of cough and dyspnea. Initial investigations, showed an entrapped oval-shaped object in the right main bronchus. Several conventional bronchoscopic attempts were failed. We used a handmade magnet-powered instrument to extract the object. CONCLUSION: Using the magnet-powered grasping forceps may be beneficial after repetitive failed attempts in the extraction process of airway foreign bodies. Although there is no specific evidence-based guideline for choosing the best removal technique, we recommend using this technique in round-shaped high weight metallic objects as the first removal technique.

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.

Severe Combined Immunodeficiency: A Case Series and Review from a Tertiary Pediatric Hospital.

Severe combined immunodeficiency syndrome (SCID) is a life-threatening condition leading to early infant death as a result of severe infection, due to impaired cellular and humoral immune systems. Various forms of SCID are classified based on the presence or absence of T cells, B cells and natural killer cells. Patients usually present with recurrent infections and failure to thrive. Definitive treatment is hematopoietic stem cell transplantation. To achieve the best outcome, it should be performed prior to the development of severe infection. In This study, we described 10 patients (6 male and 4 female) with SCID who were admitted to Mofid Children Hospital, Tehran, Iran, from 2006 to 2013. We reviewed patients' clinical manifestation, laboratory data, family history and outcome. The mean age at the time of diagnosis was 131.8 days. One patient had non-consanguineous parents. Seven patients received BCG vaccine before the diagnosis of SCID, three of them showed disseminated BCG infection. One patient presented with invasive pulmonary aspergillosis. Flow cytometric analysis showed T⁻B⁺NK⁻ in three patients, T⁻B⁻NK⁺ in five patients, T⁻B⁻NK⁻ in one patient, and T⁻B⁺NK⁺ in one patient. This study highlights the importance of early diagnosis and patient referral before the occurrence of serious infection.

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.