Association between sputum culture results and pulmonary changes in children with cystic fibrosis.

Background and Objectives: Despite the significant improvement in the prognosis of cystic fibrosis (CF), it is still regarded as the most common life-shortening genetic disease in Caucasian populations. This disease is the most important cause of chronic lung disease and exocrine pancreatic insufficiency in infancy and childhood. The aim of our study was to assess the potential association between bacterial colonization detected by sputum cultures and pulmonary structural and functional changes in Iranian children with CF. Materials and Methods: In this cross-sectional study, 76 CF children ≥6 years old registered in the CF Foundation of Children's Medical Center Hospital, Tehran, Iran, who underwent high resolution CT scan (HRCT), pulmonary function test, and sputum cultures within a month of each other during the study period were included. For each patient, demographic characteristics (age and sex), results of sputum cultures, forced expiratory volume in 1st second (FEV1), and chest HRCT findings based on the Bhalla scoring system were recorded in a check list. Results: Sixty seven percent of the patients had positive sputum cultures, with the most commonly isolated microorganism being Pseudomonas aeruginosa (mucoid strain). Based on categorization of Bhalla scores, none of the patients had severe pulmonary involvement. FEV1 was mainly >70%. There was a statistically significant correlation between colonization with mucoid P. aeruginosa and lower Bhalla scores in children aged 14-16 years (P=0.001). Colonization with mucoid P. aeruginosa was also significantly associated with patient's age (P=0.020) and FEV-1 (P=0.001). Conclusion: Severity of lung involvement in CF children is clearly dependent to mucoid P. aeruginosa colonization in airways and this notorious bacterium is the most prevalent one in Iranian CF children. Prompt identification and eradication by proper nebulized and systemic antibiotics can have valueless effects on patients' quality of life and prevent lifelong destructive complications such as bronchiectasis. Timely lung CT scan wisely advised by expert CF treatment team can meticulously detect injuries and it seems to act more efficacious than -still helpful-clinical scores and pulmonary function tests.

Comparative Efficacy of Transcutaneous Functional Electrical Stimulation With or Without Biofeedback Therapy on Functional Non-retentive Fecal Incontinence in Children: A Randomized Clinical Trial.

BACKGROUND: We compared the effects of transcutaneous functional electrical stimulation (TFES) and biofeedback therapy with TFES alone in a cohort of children with functional non-retentive fecal incontinence (FNRFI). METHODS: This prospective, single-center randomized clinical trial was performed on 40 children with FNRFI. Patients were randomly allocated into two equal treatment groups. Group A (n = 20) underwent TFES + biofeedback therapy, and group B (n = 20) received TFES alone. All patients were assessed with a pediatric fecal incontinence (FI) score questionnaire, a visual pain score, and a bowel habit diary both before and at the end of treatment sessions and also at 6 months of follow-up. In addition, a FI quality-of-life (QoL) questionnaire was recorded for all patients before and 6 months after the treatment. RESULTS: FI significantly improved in 13/20 (65%) patients in group A and 11/20 (55%) patients in group B (P < 0.05). A significant reduction in FI score was seen in each group at the end of treatment sessions and maintained at 6 months of follow-up (P < 0.05). A significant improvement in FI-QoL scores was seen in both groups at 6 months of follow-up in which there was no significant difference in terms of FI-QoL scores improvement between both groups after treatment. CONCLUSIONS: The use of electrical stimulation in combination with other treatment methods improves symptoms in patients with FNRFI who are refractory to conventional treatment.

Clinical and Paraclinical Screening for Celiac Disease in Children with Intractable Epilepsy.

BACKGROUND: Celiac disease is the inflammatory entropy caused by hypersensitivity to gluten, which occurs in susceptible individuals. Some studies have suggested a link between celiac disease and epilepsy in children. Our aim was to screen for clinical and paraclinical features of celiac disease in children with intractable epilepsy. METHODS: This was a cross-sectional study. Children aged 2 to 18 years with refractory epilepsy that referred to the pediatric neurology clinic within one year (2018-2019) were enrolled. Demographic and clinical characteristics of patients, especially clinical manifestations of celiac disease, were recorded in a questionnaire. A venous blood sample was sent to determine the total IgA, anti-tTG (IgA), and anti-endomysial antibody (IgA). Endoscopy was performed in cases where the celiac serological test was positive. RESULTS: Seventy children with idiopathic drug-resistant epilepsy (44 boys) were evaluated. The height-for-age index was 49.2% and the weight-for-age index was 38.2% less than normal. Constipation (48.6%), anorexia (25.7%), and abdominal pain (21.4%) were the most common gastrointestinal symptoms. Celiac serological tests were negative in all children. Therefore, endoscopy and bowel biopsy were not performed in any case. CONCLUSION: Celiac disease was not found in any patient with intractable epilepsy. Gastrointestinal symptoms and growth disorders in this group may be related to the underlying disease or medications and not to celiac disease.

Effects of Interferential Electrical Stimulation Plus Pelvic Floor Muscles Exercises on Functional Constipation in Children: A Randomized Clinical Trial.

OBJECTIVES: Functional constipation is a common condition in children. We assessed the effectiveness of combined interferential (IF) electrical stimulation and pelvic floor muscle (PFM) exercises on functional constipation in children. METHODS: We conducted a single-center, double-blind randomized clinical trial study during 2014-2017 in Tehran, Iran. Ninety children, aged 5-13 years, who fulfilled Rome III criteria were enrolled and randomly assigned into two treatment groups. Case group (n=45) underwent IF electrical stimulation and PFM exercises, whereas the control group (n=45) received PFM exercises plus sham stimulation. A complete bowel habit diary (with concerning data on the frequency of defecation per week, stool form, and the number of fecal soiling episodes), a constipation score questionnaire, and a visual pain score were recorded before, after the treatment and 6 months later for all participants. In addition, children in both groups were assessed with a constipation-related quality-of-life questionnaire before, after the end of treatment sessions, and 6 months after the treatment. RESULTS: Treatment success was achieved for 88.4% of children in the case group compared with 43.2% of children in the control group after the treatment (P<0.003). The median constipation score was reduced in both groups, with the cases having significantly lower scores after the treatment (4 vs. 8, P<0.000). Stool form normalized in 75.6% of the cases and 45.5% of the controls after the treatment (P<0.01). CONCLUSIONS: Our results showed that using IF electrical stimulation as an adjuvant therapy to the medical and rehabilitation programs significantly boosts the effects of treatment among these patients.

Prevalence of Acanthosis nigricans and Related Factors in Iranian Obese Children.

INTRODUCTION: Recognition of Acanthosis nigricans (AN) provides important opportunities for screening of obesity syndrome, dyslipidemia, hypertension and insulin resistance with diabetes mellitus 2. Considering the high prevalence of obesity among Iranian children, we designed this study to estimate the prevalence of AN and related laboratory factors in Iranian obese children. MATERIALS AND METHODS: Seventy-one obese children were enrolled in this study. Diagnosis of AN was done by clinical examination. Body mass index (BMI), fasting blood sugar, total cholesterol, triglycerides (TG), alanine aminotransferase, aspartate aminotransferase (AST), alkaline phosphatase, high- and low-density lipoprotein cholesterol, insulin, thyroid-stimulating hormone, free thyroxin (fT4), calcium, phosphorus and 25-hydroxyvitamin D were measured with routine techniques. Collected data were compared between cases with AN and without AN. Independent t-test was used for comparison of variables. RESULTS: Twenty-five of children were female (35.2%). Forty-eight children (67.6%) had AN. In 20 cases (28.2%), homeostasis model assessment-insulin resistance (HOMA-IR) was <2.5 and in 51 (71.8%), HOMA-IR was more than 2.5. Mean BMI, insulin, HOMA-IR, TG and AST levels were significantly higher in cases with AN. CONCLUSION: Obese children with AN are at risk of developing diabetes. Hence early identification of this feature and precise evaluation of children is recommended.

Assessment of Vitamin D Status and Response to Vitamin D3 in Obese and Non-Obese Iranian Children.

BACKGROUND: Obesity seems to be a critical issue nowadays because of its high prevalence and its adverse effects on health. There is some evidence indicating the relationship between obesity and lower serum 25-hydroxyvitamin D [25(OH)D] concentration. The aim of the present study was to examine serum 25(OH)D status of obese and non-obese Iranian children and compare their therapeutic response with identical oral vitamin D3 treatment. METHODS: In a non-randomized clinical trial, serum 25(OH)D level of 45 obese and 45 non-obese Iranian children aged 2-14 years was measured. Those with serum 25(OH)D status <30 ng/ml (73 cases) were treated with one pearl of vitamin D3 (50 000 International Units) once a week for 6 weeks. Serum vitamin D was measured once more 2 weeks after treatment. RESULTS: The frequency of hypovitaminosis D was 43/45 (95.6%) in obese and 30/45 (66.7%) in non-obese children at baseline (p < 0.001). After treatment of 73 cases (43 obese, 30 non-obese), the above percentages were decreased to 24/43 (55.8%) and 1/30 (3.3%), respectively (p < 0.001). CONCLUSION: Our study demonstrated a high frequency of vitamin D deficiency among Iranian children, particularly the obese ones. Moreover, low therapeutic response in the obese group is witnessed.

Apparent life-threatening events in neonatal period: clinical manifestations and diagnostic challenges in a pediatric referral center.

OBJECTIVE: Apparent Life-Threatening Events (ALTEs) is an episode that is frightening to the observer and is characterized by some combination of apnea, color change, altered muscle tone, choking, and gagging. This study was designed to evaluate and follow up neonates who presented with clinical manifestation of an ALTE in a year. METHODS: In this prospective observational study, all of the neonates with episode of ALTE who were admitted to the Children's Medical Center (CMC) in Tehran, from June 15(th) 2010 to May 14(th) 2011 were enrolled in the study. Data from patients consisting of history, physical examinations, and paraclinical findings were recorded in a checklist and all followed up 3 to 6 months after discharge. FINDINGS: During the study period 18 neonates were admitted due to ALTE episode(s) with mean age of 15±13 days. Nine (50%) neonates had previous attacks of ALTE. The most frequent complaint was cyanosis in 12 (67%) and apnea in 8 (44%) patients. In 10 (56%) the event lasted less than one minute, 13 (72%) were awake, 17 (95%) in supine position and 13 (72%) on their parent's lap. Primary antagonistic impression on admission was sepsis in 11 (61%) and concomitant seizure in 5 (28%). The most common final diagnosis according to repeated physical examinations, result of paraclinical investigations and follow up was sepsis 4 (22%) and aspiration 9 (50%). ALTE recurred in none of the neonates during follow up. CONCLUSION: The rate of ALTE seems to be higher than in this study owing to high incidence of recurrent ALTE. Although most of these attacks regress spontaneously, more attention should be paid for the underlying diseases.