macrophage activation syndrome (MAS) is life-threatening complication of rheumatic diseases and is most frequent seen in systemic juvenile idiopathic arthritis (sJIA). Prompt recognition and immediate therapy is life saving.

DiGeorge syndrome (DGS) which is also known as velocardiofacial syndrome is caused by a submicroscopic chromosome deletion of band 22q11. It is associated with a disturbed development of the pharyngeal arches. In this report we describe two unrelated male children with clinical features consistent with 22q11.2 microdeletion syndrome characterized by cardiac defect, recurrent respiratory infections and developmental deficiency. Definitive diagnosis is made by Fluorescence In Situ Hybridization analysis (FISH). Children underwent surgical correction of congenital heart defects. During surgery thymic aplasia was confirmed in both children, postoperative course proceeded without major complications. Our report suggests that the criteria in searching for microdeletion 22q11.2 should be expanded and applied in patients with conotruncal and non-conotruncal congenital heart defects and at least one typical feature of this syndrome.

Systemic onset Juvenile Idiopathic Arthritis (SoJIA) is rare pediatric disease, it accounts for 10% of children with Juvenile Idiopathic Arthritis. The onset of disese can be vary nonspecific and may suggest bacterial or viral infection, malignancy or other rheumatic disease. It is highly characterised by its extra-articular systemic illness features and, in some ways, it resembles a fever of unknown origin. Diagnosis is mostly clinical by using ILAR criteria (International League of Associations for Rheumatology).

Common variable immunodeficiency (CVID) or acquired hypogammaglobulinemia is the type of primary immunodeficiency. Deregulation of the immune system, leading to hypogammaglobulinemia, defective activation and proliferation of T cells and dendritic cells, and malfunction of the cytokines are observed in CVID. The clinical picture of CVID varies, any organ or system can be affected, therefore the diagnosis is often difficult and delayed and sometimes is not always possible. This article describes a twelve years old boy with all the clinical signs of immunodeficiency, as confi rmed by laboratory. The main treatment consists of life-long immunoglobulin substitution in intravenous or subcutaneous form.

Introduction: The prevalence of pediatric obesity is increasing. Finding the most effective preventive measures for the development of obesity in each country requires accurate epidemiological data on the number of obese children and adolescents, and their habits regarding nutrition and activity. The objective of this study was evaluate diet and physical activity in primary school students in relation to the occurrence of obesity, to determine the prevalence of overweight, mark the basic causes of this phenomenon and to establish measures for treatment and prevention.Methods: pupils 1-8. grades of primary schools were surveyed in written forms in terms of nutrition and physical activity, and measured height and weight, body mass index (BMI-body mass index) was calculated by whichwas estimated the level of nourishment: BMI> p (percentile) 5-malnutrition, p 5-85 proper body weight, p 85-95 over-nutrition, p> 95 obesity.Results: The study comprised 2329 pupils from 10 primary schools in the Canton of Sarajevo. Number of respondents by age and gender was balanced: I-IV 1077, V-VIII 1252; M-1226 and -1103 W. Obese and overweight was 22.46%, 62.53% of normal weight and 15 underweight, 01%. Most children eat a sandwich from school 34.63%, and food from the bakery 23.36% and 23.64% a sandwich from home. Still-dense juices are mostly drunk, even 22.34% of the students, a maximum of 52.8% water. Daily candy had taken 53.21% of all primary school students. 33.80% of the students were active on physical activity lessons and daily only 28.27%.Conclusions: The overweight problem in relation to the way of nutrition and physical activity is evident. The most important factors in] uencing the development of obesity undernutrition of children in school, the high frequency of intake of sweets and thick juice, an inadequate level ofphysical activity and sedanteran way of life.

A modern diagnostic and therapeutical approach to paediatric cardiology enables early application of foetal echocardiography in order of achieving diagnosis of congenital heart anomalies in utero. The aim of this study is to evaluate the percentage of prenatal diagnosis of congenital heart anomalies. This study has been conducted on 73 patients at Paediatric clinic of Clinical Centre of Sarajevo in a period from January 2000 until December 2004 with diagnosis of heart malformations. Among them 14 were preterm newborns, 40 boys. Diagnosis of cardiac anomalies with left to right shunt was done in 56.1%, obstructive 13.7%, cyanotic 1.36% and complex in 28.7% patients. The prenatal diagnosis was established in 4 patients (5.5%) by ultrasound examination which is very low in comparison to other European countries. There is a need for making prenatal diagnosis of congenital heart anomalies in foetus as early as it can be done in order to treat cardiac anomalies in utero, to decrease the number of congenital heart anomalies and to reduce the cost of cardiosurgical and postsurgical treatment.