Supportive care in a patient with Alstrom syndrome with hyperphenylalaninemia and sleep problems.

Alstrom syndrome is a rare genetic disorder with an autosomal recessive mutation in the ALMS1 gene. The disease's manifestations include ophthalmic problems, hearing loss, obesity, and cardiovascular disorders. In addition, medical cases include other organ complications. However, the overlapping variety of such symptoms with other diseases may delay the diagnosis. In this article, we describe the case of a 7-year-old female patient with Alstrom syndrome, and cardiovascular and hyperphenylalaninemia diseases since birth. Other symptoms included diabetes and ophthalmologic problems with skeletal disability. Blindness and hearing impairment were diagnosed, along with recurrence of respiratory problems at the age of 7 years. The patient's obesity-induced snoring predisposed her to uncontrolled blood glucose. In fact, respiratory tract problems and sleep disorders had occurred as a degraded cycle and left her with a severe disability for years. The similarity of the symptoms with other diseases had misled the physician in diagnosis. However, a polysomnography test (because of complaints of short sleep duration) recognized the source of the patient's sleep disorders and breathing problems. Eventually, we delivered a portable ventilator to the child for continuous positive airway pressure (CPAP) therapy. The child's breathing and oxygenation conditions improved. Using the ventilator and the CPAP system, we discharged her from the hospital without requiring oxygenation, in a stable condition. The procedure could prevent the patient from hypoxia and retinal problem.

Global prevalence of enterobiasis in young children over the past 20 years: a systematic review and meta-analysis.

Parasitic infections are the most common diseases worldwide, and enterobiasis is a common parasitic infection in children. Various studies have reported on the prevalence of Enterobius vermicularis in different regions of the world. However, no study has gathered and analyzed this data systematically. Our systematic review and meta-analysis investigated the overall prevalence of E. vermicularis among children globally. Data were extracted from 4 available databases for studies published from January 2002 to April 2022. The quality of the included studies was scored based on the standard Strengthening the Reporting of Observational Studies in Epidemiology. A random-effect model was chosen to calculate the pooled prevalence and corresponding 95% confidence interval (CI) according to the degree of heterogeneity in the included studies. Thus, 40 publications (42 data sets) that included 3,279 children with enterobiasis met all criteria and were included in the analysis. The meta-analysis showed that heterogeneity among the included studies was high (Q=4,399.35, I2=99.96%; df=41; p<0.001). The pooled global prevalence of enterobiasis among the studied children was 12.9% (95% CI, 8.2%-17.7%). Our systematic review and meta-analysis estimated that, for the past 20 years, 12.9% of children around the world have been infected with E. vermicularis.

Epidemiological data of national Kawasaki disease registry in Iran, 2007-2019.

Introduction: Kawasaki disease(KD) is a vasculitis of childhood that tends to influence the coronary arteries. There is no national data about the prevalence of KD in Iran. This study aimed to perform a national registry in Iran for 13 years. Methods: In this retrospective study, the data for KD extracted from medical records of <19 year-old patients admitted to tertiary hospitals in Iran between 2007 and 2019 were recorded in the national KD registry system. Age, admission date, gender, location, and presence of KD criteria, laboratory and echocardiography findings, and treatment modalities were evaluated. Complete KD was considered if ≥4 clinical criteria of the KD existed and otherwise, incomplete KD was considered. Results: Data from 1,682 KD patients including 999(59.39%) boys and 683(40.61%) girls and male/female ratio of 1.46 were evaluated. The mean age was 3.08 ± 2.49 years and 1465(87%) were living in urban regions. The yearly incidence of the disease was between 2.62 to 3.03 from 2015 to 2019. The highest age-specific incidence was observed in children <1-year-old. Incomplete and resistant KD included 1,321(78.54%) and 9(0.54%) patients, respectively. Abnormal echocardiography was detected in 619(36.80%) patients. Leukocytosis, with dominancy of neutrophils, anemia, thrombocytosis and increased ESR and CRP were the most noticeable laboratory findings. No death due to KD disease was reported. Conclusion: Based on this study, most of the KD cases are presented with atypical presentation in Iran. So, increasing awareness of primary healthcare workers by educating and updating their data is very important in timely diagnosis and management of the disease.

Zinc sulphate for acute bronchiolitis: A double-blind placebo-controlled trial.

The use of adjunctive therapies to achieve rapid recovery from clinical symptoms of acute bronchiolitis would appear necessary. This study was performed to determine the effect of zinc sulphate on treating acute bronchiolitis. In this study, 100 children affected with acute bronchiolitis were investigated. Fifty patients received oral zinc sulphate and 50 patients placebo. Signs and symptoms of the disease were compared between two groups at the time of admission and then 24, 48, 72, 96 and 120 hours after the beginning of treatment. The trend of recovery of clinical signs and symptoms was more favourable in the case group than in the control group in 24, 48, 72, 96 and 120 hours after beginning of the treatment. The significant differences were observed between the two groups in terms of improvements in coughing and wheezing 48 and 72 hours after the beginning of the treatment (P<0.05). Full recovery was observed in 49 (98%) patients receiving zinc sulphate within 72 hours of the beginning of treatment (P=0.0001). The present study showed that administration of zinc sulphate accelerates improvement from clinical signs and symptoms of acute bronchiolitis. Thus we recommend the use of zinc sulphate for the treatment of acute bronchiolitis.

Effect of oral cephalexin in the treatment of BCG lymphadenitis.

Lymphadenitis and abscess formation are the most common side effects of vaccination with Bacille Calmette Guerin (BCG). The lower the child's age at the time of vaccination, the higher the incidence of BCG lymphadenitis tends to be. Although various therapeutic approaches are in use for the treatment of BCG lymphadenitis, there is no consensus on which of them is optimal. This study aimed to determine whether oral cephalexin treatment hastens recovery from BCG lymphadenitis. The study involved 40 children (24 boys and 16 girls) with BCG lymphadenitis who were referred to Qazvin Children's Hospital, Qazvin University of Medical Sciences between December 2008 and the end of September 2009. The patients were randomly assigned to two groups of 20 patients each (12 boys and 8 girls in each group): group A patients did not receive any treatment and served as controls, and group B patients were treated with 50 mg/kg/day cephalexin syrup, administered in four doses, for 10 days. In all patients, clinical examination was normal, except for lymphadenitis. In all patients, BCG vaccination had been performed at birth, and polymerase chain reaction tests were positive for tuberculous bacilli. The recovery period and requirement of fine needle aspiration did not significantly differ between the two groups (P 0.05). This study showed that treatment with cephalexin does not hasten recovery from BCG lymphadenitis.