Elejalde syndrome - A neuroectodermal melanolysosomal disease: A case report.

Background: Elejalde syndrome is a rare neuroectodermal melanolysosomal disease with an autosomal recessive heredity. Patients usually present with silvery-gray hair, neurological abormalities, diffuse skin hypopigmentation and suntanned skin color. Case Presentation: A 3 1/2-year-old boy presented with hemiplegia since the day before admission. Durig hospital admission, he experienced episodes of status epilepticus and loss of consciousness and underwent mechanical ventilation. The patient had silvery-gray hair, consequently the pathologic evaluation of the hair shaft, revealed enlarged irregularly spaced melanin clumps characteristic for silvery-gray hair syndrome. No immunologic dysfunction was detected due to immunological evaluations, subsequently Elejalde syndrome was confirmed. Conclusion: This study adds one new case to the known cases of Elejalde syndrome and confirms that Elejalde patients may not exhibit neurological symptoms until an older age.

Prevalence of Asthma Symptoms in Children and Adolescents in Karaj, Iran: A Report from the Global Asthma Network Phase I Study.

  This study is a part of the Global Asthma Network (GAN) phase I project to assess asthma symptoms in children, adolescents, and their parents in Karaj, Iran. The present cross-sectional study was conducted in 2019-2020 in Karaj, Iran, in alignment with the goals of the GAN study, including assessing asthma prevalence, severity, and risk factors. In this study, 1500 students were selected using a multistage stratified cluster sampling method from 40 public and private schools in Karaj. The entire population of children aged 6-7 years or adolescents aged 13-14 years in a given school and their parents was considered the sample unit. The GAN core questionnaires were completed for students and parents. The results showed that the response rate was 89.6%. A total of 1326 children and adolescents, 572 children aged 6-7 years, and 754 aged 13-14 years and their parents were enrolled in the study. The prevalence of ever- and current wheezing was 24% and 13.8% among 6-7-year-olds, and 18.8% and 12.3% among 13-14-year-olds, respectively. In children aged 6-7 years, parental wheezing significantly increased the chances of children wheezing (odds ratio: 3.27; 95% confidence interval: 1.70, 6.310). The current study's findings showed that the prevalence of asthma symptoms among children and adolescents and their parents in Karaj, Iran, was mainly higher than the findings of studies conducted in other cities in Iran.