HLA-DRB1*01:01, but not HLA-DRB1:1503 or HLA-DRB1*11, is associated with decreased inhibitor risk in Iranian hemophilia A patients.

BACKGROUND/OBJECTIVE: Hemophilia A is a genetic disorder through which patients suffer from recurrent bleeding. This can be caused by a defect in human plasma coagulation factor VIII. High incidence of FVIII inhibitors in some severe hemophilia A patients after FVIII therapy is a considerable complication. Determination of good predictive factors can improve the safety of this treatment. HLA-II have been shown as a predictive element for inhibitor development. The goal of this study is to determine the association between HLA-DRB1*15:03, HLA-DRB1*11 and HLA-DRB1*01:01 alleles and FVIII inhibitors in severe hemophilia A patients in Iran. MATERIALS/METHODS: HLA-DRB1 genotyping was performed using Multiplex sequences Specific Primers (PCR-SSP) in two groups of severe hemophilia A patients comprising 51 and 50 individuals with and without FVIII inhibitors respectively. The levels of inhibitor were determined through Nijmegen-modified Bethesda assay. HLA-DRB1 allele frequencies were compared between groups by using multiple logistic regression models. RESULTS: HLA-DRB1*01:01 allele frequency was significantly higher in patients without inhibitor ORadj: 2.7 (95%CI: 1.08, 6.97; P = 0.034). There wasn't any statistically significant difference in HLA-DRB1*11 allele frequency between groups ORadj: 0.7 (95%CI: 0.27, 1.82; P = 0.47). There was no connection between HLA-DRB1*15:03 and inhibitor development ORadj: 0.94 (95%CI: 0.38, 2.35; P = 0.94). CONCLUSION: An association between HLA-DRB1*01:01 and paucity of FVIII inhibitor showed that this allele has probably a protective effect in severe hemophilia A patients in Iran. Determination of the predictive and protective alleles are beneficial in pre-treatment activities and decrease the risk of unsuccessful therapy with FVIII in each population.

Sonographical and serological survey of human cystic echinococcosis and analysis of risk factors associated with seroconversion in rural communities of Kerman, Iran.

Cystic echinococcosis (CE) caused by larval stage of Echinococcus granulosus, is an endemic zoonosis in Iran particularly in rural regions. The objective of this study was to determine the prevalence of CE among rural communities in Kerman using ultrasonography (US) and serology. Kerman Province, in southeastern Iran, is the largest province, with 2.9 million inhabitants. A sample of 1140 individuals (200 males and 940 females) was selected by randomized cluster sampling in 2006-2008. After acquiring informed consent for each participant a questionnaire was filled, complete abdominal US in supine position was carried out and 5 ml blood was collected for ELISA test. Two hydatid cases (0.2%) were detected by ultrasound. Serological results showed 7.3% seropositivity, and females (8.3%) were significantly more positive than males (2.1%). There were significant difference between CE seropositivity and sex, age and occupation. Residents of desert regions (Shahdad, Andouhjerd and Golbaf) were 2.5 times more likely to be seropositive than mountainous regions with better socioeconomic status (OR = 2.5; 95% CI = 1.09-5.95). Dog ownership does not appear to be a significant risk factor for CE in the region. Only about 10% of households own dogs, usually only one dog. However, the stray dog population of Kerman province is estimated at 145 000-480 000 (3.5-11.5 times the owned dog population). Infection in humans and animals would appear to come mostly from infected stray dogs. Management of stray dog population could make major progress in control of hydatid disease. In addition, proper washing of vegetables decreased probability of infection by 53% (OR = 0.47; 95% CI = 0.26-0.84). The serological study showed that many people, especially women, had been exposed to Echinococcus eggs and had seroconverted but were not infected.