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Diagnsis of TAR syndrome usually are made at birth because of the characteristic physical appearace combined with thocmbocytopenia. The Two essential features of TAR syndrome are hypomegakaryocytic thrombocytopenia and bilateral radial aplasia. The rest of the phenotype varies widely and can manifest with abnormalities involving skeletal, skin, gasterointestinal and cardiac systems. Considering to limited cases of syndrome and variability of phenotypic abnormalities exess two essential features, all cases of this syndrome are reportable. We reported a patient with TAR syndrome with any related malformations.

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Background & Objective: Preterm infants have less iron storage compared with the term one. Due to rapid growth they need more iron during infancy. This study was designed to evaluate the effect of early iron supplementation on hematologic indices and incidence of iron deficiency anemia in preterm infants. Materials & Methods: This experimental study was done on 20-days-old preterm breast-feed infants referred to the Gorgan-North of Iran primary health care services for vaccination, without any underline disease or growth retardation during 2005. They were divided to two groups (n=15 in each) and a questionnaire was completed for each case. Iron drop (2mg/kg) was given in the interventional group. Iron deficiency anemia was assesed in all sample T-student test and chi-square were used to analyze the independent variables and comparing the hematologic indices, after entering in SPSS-13 software and testing the normal distribution with komologrof-smearnoff test. Relative risk index (RR) was used to compare the two groups. Results: The relative risk of iron deficiency anemia in the interventional group was 0.4 folds [RR=0.4,CI %95 for RR=(0.091,1.749)]. No significant difference was seen between the two groups. Conclusion: The finding of this study showed that iron drop supplementation for preterm infant prevent the iron defeciency anemia, although this observation was not significant.

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Background and Objective: Thalassemias is the most common inherited disease in the world. This study was designed to assess general health of patients with beta-thalassemia major in Gorgan, northern Iran. Materials and Methods: This descriptive study was performed on 163 patients with beta-thalassemia major whom referred to Thalghani hospital in Gorgan, northern Iran. Socio-Psychosocial status of patients was assessed by using general health questionnaire-28 (GHQ-28). Results: Socio-Psychosocial status of 44.2%, 46% and 9.8% of patients was normal, suspected and suffered from psychosocial disorders, respectively. Social dysfunction (12.9%) and depression (10.4%) were the most prevalent and anxiety was the less prevalent (4.9%) in the patients. There were significant correlations between psychosocial status and mother’s ocupation (P<0.05), patients level of education (P<0.05) and history of associated diseases (P<0.05). Conclusion: Social dysfunction and depression were the two major general health complications in beta-thalassemia patients in this region.

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There is not comprehensive information available about the control, complications and treatment methods of factor VII deficiency. Online registry system called seven treatment evaluation registries (STER) has been created which investigated the disease as an international multi-center prospective observational project in order to provide information about the effect and immunity of available therapies. In this report, five patients with factor VII deficiency were diagnosed and registered in Golestan Province, Northern Iran, during 2010-11. All treatment protocol, demographic charectristic of patients were collected and registered in www.targetseven.org on the basis of STER´s protocol.

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Background and Objective: Deferoxamin is the current “gold standard” chelator in comparison with new chelators. Combined therapy of Deferiprone and deferoxamin reduces the cardiac iron overload in patients with major talassemia. This study was done to evaluate the effect of defriprone-deferoxamine on heart function in patients with major thalassemia. Methods: In this historical cohort study, 8 patients with major beta thalassemia treated by subcutaneous deferoxamine were randomly selected and LVEF (the rate of blood that exited from heart in each beat) and serum ferritin were measeared. The patients were treated by deferiprone (50-100 mg/kg/day) compained with dferoxamine (30-50 mg/kg as 3 times in a week). In the end of each year, LVEF and serum ferritin of patients were measured. Results: The ferritin level changed from 3243.12 in the first year to 2672.75 mg/kg at the end of third year. The mean of LVEF changed from 71.12% to 64.62 %. The correlation of serum ferritin and LVEF only at the end of third year was significant (P<0.05). Conclusion: Combined therapy of deferiprone-deferoxamine during 3 years reduces ferritin and LVEF in patients with major thalassemia.