Distribution and protective role of hlab40 in Iranian patients with kawasaki disease; a report from southern Iran

Kawasaki disease [KD] clinically presents as a systemic vasculitis syndrome with significant cardiovascular involvement. With different incidence among different ethnic groups, the role of certain human leukocyte antigens and their products has been considered as a crucial predisposing factor in the immune responses in this disease. We determined the distribution of human leukocyte antigens type B for 90 Iranian patients with Kawasaki disease in order to evaluate a possible association between these antigens and this disease in our area. We used the polymerase chain reaction [PCR] sequence specific primers [PCR-SSP] technique for antigen typing. Distribution of these antigens for 89 healthy Iranians used as control. While 7 [3.9%] of our patients were positive for human leukocyte antigen type B 40, there were 18 [10.1%] subjects from the control group who had this antigen with statistically significant difference between patients and control group [CI= 95%, RR=1.15 and P= 0.02]. Data were analyzed by Pearson chi-square test and Fisher's exact test. SPSS version 15 was used for statistical analysis and a P value less than 0.05 considered statistically significant. The presence of higher frequency of allele type-B40 in the control group may represent a protective role for this antigen with resultant decreased susceptibility to KD in our area

Clinical and genetic features in patients with cystic fibrosis in southwestern Iran

Cystic fibrosis [CF] is a common autosomal recessive genetic disease caused by a mutation in the CF transmembrane conductance regulatory [CFTR] gene. This study attempted to identify the most common CFTR mutations and any correlations between certain mutations and the clinical presentation of the disease in CF patients in southwestern Iran. Twenty nine common CFTR gene mutations were examined in 45 CF patients. Chronic cough, intestinal obstruction, dehydration, heat exhaustion and steatorrhea were the most common early clinical symptoms among our patients. The most common mutation was deltaF508, with an allele frequency of 21%. The homozygous deltaF508 mutation was observed in eight patients [18%], and three patients [7%] were deltaF508 carriers. The 2183AA>G mutation was observed in four patients, one of whom was also a deltaF508 carrier. The R1162X mutation was detected in two patients. The G542X, R334W and N1303K mutations were detected each in one patient, the first of whom was also a deltaF508 carrier. Out of 45 patients, 27 [60%] had none of the CFTR gene mutations we tested for. The most frequent mutations in southwestern Iranian patients with CF should be identified by sequencing the entire CFTR gene in order to optimize the design of a diagnostic kit for common regional mutations

Specific IgE to common food allergens in children with atopic dermatitis

Atopic dermatitis is a major public health problem, often starting in early childhood and sometimes followed by other allergic diseases. Although hypersensitivity to foods is assumed to play an essential role in the development of atopic dermatitis in some patients, little is known about common food allergens in Iranian children with atopic dermatitis. This study was designed to identify probable food allergens in Iranian children with atopic dermatitis and find the relationship between food sensitization and the severity of atopic dermatitis. This study included 90 children aged 2-48 months with atopic dermatitis. Skin prick tests for cow's milk, hen's egg, almond, potato and soybean were done. Serum specific IgE to 20 food allergens was also screened. Among children with atopic dermatitis, the frequency of food sensitization was 40% by skin prick test and 51% by food-specific IgE. Children with atopic dermatitis were most commonly sensitized to cow's milk [31%], hen's egg [17.7%], tree nuts [17.7%], wheat [12.2%], potato [11.1%], tomato [8.8%] and peanut [8.8%]. In 42 children with moderate to severe eczema, sensitivity to food allergens was 78.5% by skin prick test and 88% by serum specific IgE evaluation. Our results showed that cow's milk, hen's egg and tree nuts were the most common food allergens in Iranian children with atopic dermatitis. Sensitization to foods was much higher in patients with moderate to severe atopic dermatitis. Determining specific IgE in children with atopic dermatitis can be helpful in managing these patients

The Relationship Between Serum 25 Hydroxy Vitamin D Levels and Asthma in Children

PURPOSE: Asthma and other allergic disorders have increased over the past decades in nearly all nations. Many studies have suggested the role of vitamin D deficiency in both T-helper1 and T-helper2 diseases; however, the association between vitamin D, allergy, and asthma remains uncertain. In this study, the associations of 25-hydroxy vitamin D3 levels with asthma and with the severity of asthma were evaluated. METHODS: This cross-sectional study was conducted on 50 asthmatic children and 50 healthy controls aged 6-18 years. Serum 25-hydroxy vitamin D3 levels were determined and compared between the two groups. The relationship between serum vitamin D levels and pulmonary function test outcomes and eosinophil counts were examined in asthmatic patients. RESULTS: Univariate analysis of the relationship between asthma and vitamin D showed that decreased vitamin D levels were associated with significantly increased odds of asthmatic state (P=0.002). In a multivariate analysis after adjustment for age, body mass index, and sex, the relationship between vitamin D and asthma increased. In asthmatic patients, 25-hydroxy vitamin D levels had direct and significant correlations with both predicted FEV1 (R2=0.318; P=0.024) and FEV1/FVC (R2=0.315; P=0.026). There were no associations between vitamin D level and eosinophil counts, duration of disease, and the number of hospitalization or unscheduled visits in the previous year (P>0.05). CONCLUSIONS: These results showed that serum 25-hydroxy vitamin D levels were inversely associated with asthma, and there was a direct and significant relationship between vitamin D levels and pulmonary function test outcomes in asthmatic children. An interventional study in asthmatic patients with low serum vitamin D concentration may establish a causal relationship between asthma and vitamin D.

Antigen-specific antibody response in juvenile-onset SLE patients following routine immunization with tetanus toxoid

Infection is now the most common cause of morbidity in Systemic Lupus Erythematosus [SLE]. There is lack of information regarding the specific antibody formation in response to vaccines in young SLE patients. To determine the efficacy of anti-tetanus antibody response in young patients with SLE. Forty SLE patients with mean age of 14.1 years [range: 7-21] and 60 age and sex matched normal controls were enrolled in this study over a period of one year. Diagnosis was made according to the ACR criteria and disease activity was determined based on SLE Disease Activity Index [SLEDAI]. All patients and controls had received the complete schedule of tetanus vaccinations consisting of three primary doses and two boosters by the age of six. Serum immunoglobulins and anti-tetanus antibody titers were determined by Nephelometry and ELISA. Anti-tetanus antibody levels greater than 0.1 IU/ml have been suggested as protective. In all of the patients and controls anti-tetanus antibody titer was > 0.1 IU/ml. IgG, IgA, and IgM levels were in the normal range for their age. Mean disease activity score was 4.9 [range: 0-16]. There was no association between SLEDAI score and anti-tetanus antibody response. School age onset and immunosuppressive therapy does not seem to interfere with development of consistent immunity to tetanus vaccine in young SLE patients

evaluation of new classification of allergic rhinitis in patients referred to a clinic in the city of Shiraz

Allergic rhinitis is the most common cause of allergic diseases. The recent guidelines of Allergic Rhinitis and its Impact on Asthma [ARIA] group, classified it to intermittent [less than 4 days per week and for less than 4 weeks] and persistent [more than 4 days per week or lasting more than 4 weeks regardless of the number of days per week]. The present study evaluates the patients with ARIA classification. The patients referred to Motahhari Clinic [Shiraz, Iran] were evaluated. Age, sex, seasonal or perennial, intermittent or persistent type, duration, sneezer or blocker, diurnal change and presence of allergic conjunctivitis were recorded. The patients who had one or more symptoms of; impairment of sleep, daily activity or work in school or troublesome symptoms had moderate to severe disease and those who did not have these problems have mild disease. The information of 96 patients [46 male and 50 female] were analysed [mean age +/- SD: 24.45 +/- 10.37 years]. The mean duration of disease was 4.26 +/- 4.12 years, was more in female [P<0.05]. Seasonal allergic rhinitis was presentxin 49 [52%] patients; 30 male, 19 female [P=0.007], perennial in 15 [16%], 1 male, 14 female and mixed type in 29 [30%]; 11 male, 18 female. Mild intermittent rhinitis was diagnosed in only 2 [2%] patients, 1 male and 1 female, mild persistent rhinitis in 23 [24%]; male 11, female 12; moderate/severe intermittent rhinitis in 4 [4%]; 3 male, 1 female; and moderate/severe persistent rhinitis in 56 [58%]; 22 male, 34 female [P<0.05]. Fifty-seven percent were more sneezer and 36% were more blocker type, sneezers suffered more in days and others more at nights. Others were both sneezer and blocker. Sixty percent had allergic conjunctivitis too, 76% of them were moderate/severe persistent, and 85% were in seasonal or mixed groups. Eleven patients [12%], in persistent group, had history of asthma. Family history of allergic rhinitis and asthma was found in 53% and 25% of patients, respectively. According to new guidelines for classification of allergic rhinitis, most of the patients are moderate to severe types. The classification of allergic rhinitis to seasonal and perennial disease is not proportional to intermittent and persistent types of classification

Case Report: Churg-Strauss Syndrome in an 8-year-old girl

Churg-Strauss Syndrome [CSS] is a small vessel granulomatous vasculitis that characteristically affects the middle age group and is extremely rare in children. We describe an 8-year-old girl with a prolonged history of cough and eosinophila that initially was labelled as pulmonary tuberculosis, but after complementary assays, associated with skin biopsy, eosinophilic fibrinoid vasculitis, CSS was eventually diagnosed. In this case, due to her young age and more prevalence of infectious lung diseases in our area, there was a few months delay for correct diagnosis and treatment. After meeting the CSS criteria the appropriate treatment was started and her condition improved

Evaluation of asthmatic patients referred to Jahrom Hospital and Clinic

Asthma is the most common chronic respiratory disease of children in the world. Serial studies in the world have showed an increased prevalence of bronchial asthma. In this study, the children younger than 12 years old referred to Jahrom hospital and clinic due to asthma were selected. We issued 100 questionnaires, according to International Study of Asthma and Allergies in Childhood [ISAAC] criteria and were completed by the physicians. The ratio of male to female was 1 to 9. The patients who were under the age of 4, 3 and 1 year were 82%, 60% and 15% respectively. Passive smoking was present in 56% of the patients, and 22% had pets at home like cat, dog or bird. Home dampness was present in 33%. Ninety percent of patients had used breast feeding during the first year of life. Seventy percent of patients had family history of asthma. Food allergy was present and could trigger asthma in 15%. The result of ISSAC questionnaire showed that during the last year wheezing was present in 10%, 6% had 1-3 attacks and 4% had 4-12 attacks. Sleep disturbance by wheezing had occurred in 5% but cough in 16%. Thirteen percent of patients had wheezing after exercise. In Jahrom town the climate is warm and dry. In this town asthma in children is more common among the children who are younger than 4 years old. The risk factor like smoking at home, pets and home dampness should be eliminated from their environment