Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED), also named as autoimmune polyglandular syndrome (APS) type 1, is a rare autosomal recessive disorder caused by mutations in autoimmune regulator (
Background:Hyperphenylalaninemia (HPA) and Phenylkeonuria (PKU) are metabolic errors caused by deficiency of phenylalanine hydroxylase enzyme, which results in increased level of phenylalanine. This increase is toxic to the growing brain. Objectives:The purpose of this study was to compare the intellectual and developmental status in HPA and PKU children with normal population in national screening program.Patients and Methods:In a historical cohort study, 41 PKU patients who had the inclusion criteria and 41 healthy children were evaluated. Wechsler preschool and primary scale of intelligence-3rd edition (WPPI-3) was used in order to assess the intellectual status of children 4 years and older and Ages and stages questionnaire (ASQ) was used to assess the developmental status of children 5 years and younger.Results:In intellectual test comparison, the two groups showed significant difference in Wechsler’s performance intelligence score and some performance subscales (P-value < 0.01). In comparison of developmental status, no significant difference was observed between the two groups (P-value > 0.05).Conclusions:Even with early diagnosis and treatment of PKU patients, these children show some deficiencies intellectually compared to normal children. This study emphasizes on necessity for screening intellectual and developmental status of PKU patients so that effective medical or educational measures can taken in case of deficiencies.
K Ke ey y w wo or rd ds s: : constipation, urinary tract infection, children.A Ad dd dr re es ss s f fo or r c co or rr re es sp po on nd de en nc ce e: : Hazhir Javaherizadeh MD, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran, phone/fax:+98-631-3331031/3335363, e-mail: hazhirja@yahoo.com Original paper/Artykuł oryginalny AbstractA Ai im m: : The aim of this study was to evaluate the effect of sex, age, number of urinary tract infections, and duration of constipation on resolution of urinary tract infection (UTI) following treatment of constipation in Iranian children referred to pediatric gastroenterology and pediatric nephrology clinics. M Ma at te er ri ia al l a an nd d m me et th ho od ds s: : This study was conducted in Mofid Children's Hospital. Duration of the study was from 1 October 2010 to 31 June 2011. In this study every child who visited due to urinary tract infection or constipation was asked about the other condition. Data were analyzed by SPSS ver. 16.0 (Chicago, IL, USA). χ 2 and t-test were used for comparison. R Re es su ul lt ts s: : In this study, 550 children visited the hospital due to urinary tract infection or constipation. Finally 380 patients with constipation and UTI were detected. Among 220 patients with UTI > 2 times, 90 (40.90%) showed UTI resolution after treatment of constipation. Of 160 patients with ≤ 2 UTIs, 85 (53.12%) showed UTI improvement following treatment of constipation (p = 0.018). In children younger than 1 year, treatment of UTI after treatment of constipation was more effective than in other ages (p < 0.001). There was no significant difference between males and females. In children with constipation duration less than 6 months, most cases of urinary tract infection resolved after treatment of constipation (p < 0.001). C Co on nc cl lu us si io on ns s: : The UTI resolution after treatment of constipation was higher in children < 1 year old. If the patient has a history of more than 2 UTIs, improvement of UTI following treatment of constipation will be decreased.
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