Correlation between benign joint hypermobility syndrome and primary focal hyperhidrosis in children: a novel concept.

BACKGROUND: Benign joint hypermobility syndrome (BJHS) is one of the most common hereditary connective tissue disorders in children in which autonomic nervous system involvement has been reported. This study aimed to evaluate the frequency of primary focal hyperhidrosis in children with BJHS. METHODS: This observational-analytical study was conducted in a case-control setting on children aged 3 to 15 years in 2018 at Mofid Children's Hospital, Tehran, Iran. Benign joint hypermobility syndrome was diagnosed according to the Brighton criteria; then, the patients referred to a dermatologist for evaluation of hyperhidrosis. RESULTS: In total, 130 eligible patients with confirmed BJHS and 160 age- and sex-matched healthy subjects were enrolled in this study. Primary focal hyperhidrosis (PFH) was seen in 56.2 and 16.3% of the cases and controls, respectively, indicating a significant difference (P < 0.05). The severity of hyperhidrosis did not differ between the two groups. CONCLUSION: Although the results of the study showed a significant correlation between BJHS and PFH, more comprehensive studies are needed to confirm these findings.

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.

Serum level of specific IgG antibody for aspergillus and its association with severity of asthma in asthmatic children.

Aspergillosis is one of the frequent causes of exacerbation of asthma depending on the geographical regions. The specific serum IgG level for aspergillus is a major diagnostic criterion in aspergillosis.Ninety-six asthmatic patients, with mean age of 5.4 ± 3.0 years who were referred to the asthma clinic of the Mofid Children's Hospital, were enrolled in this study. Serum specific IgG for aspergillus was measured and its association with severity of asthma was evaluated.Nineteen asthmatic patients (10 females and 9 males) had aspergillus IgG antibody. Among them, severe persistent asthma and moderate persistent asthma were detected in 5 and 13 cases, respectively, whereas only one patient suffered from mildpersistent asthma. A total of 36.5% of the 96 patients had a history of atopy, while 26% had allergic rhinitis. There was an association between the severity of asthma and the presence of aspergillus IgG antibody. Moreover, the positivity for aspergillus IgG antibody was higher in older patients.Our results indicated an association between aspergillus antibody level and severity of asthma. It could be recommended that the IgG titer for aspergillus is measured in pediatric patients with asthma, whereas co-morbidity of aspergillosis and asthma increases the risk of asthma exacerbation.

Thyroid nodules in childhood: a single institute experience.

OBJECTIVE: Thyroid nodules are rare in children. Multiple diagnostic modalities are used to evaluate the thyroid mass. The aim of this study was to determine results of management of thyroid nodules in children with special attention to the role of fine needle aspiration biopsy (FNAB) in diagnosis. METHODS: Thirty-two children who underwent surgery for thyroid nodules in Mofid Children's Hospital within 10 years (1996 to 2005) were retrospectively studied. From clinical records we obtained data about demographic characteristics, clinical manifestations, ultrasonography (USG) findings, and FNAB results, pathological reports, surgical therapy and complications. Data was analyzed statistically for association with thyroid cancer. FINDINGS: Twenty-five patients (78.1%) were girls, and 7 (21.9%) boys. Mean age was 10.9 (range 8 to 14) years. 24 (75%) patients had benign and 8 (25%) malignant tumors. 18 (56.25%) nodules were located in the right lobe. Statistical analysis revealed sensitivity, specificity, accuracy, and positive and negative predictive values as follows: 80%, 65%, 63%, 25%, and 86% for USG; 35%, 41%, 40%, 18%, and 66% for RNS; 91%, 94%, 90%, 74%, and 96% for FNAB respectively. CONCLUSION: Clinical judgment as determined by serial physical findings with USG continues to be the most important factor in the management of thyroid nodules in children. FNAB is the most accurate method of investigation and its accuracy is improved by USG guidance.