This double-blind clinical trial was conducted in Thailand to assess the impact of iron treatment on the IQ and educational attainment of 1358 9-11-y-old children. The children were classified into one of three groups: iron replete, iron depleted, and iron-deficient anemic. The Raven Progressive Matrices was used to measure IQ. A Thai language and a math test were administered to assess school attainment. A 50-mg/d tablet of ferrous sulphate was given for 2 wk and a 100 mg/d tablet, for 14 wk. An anthelminthic drug was given on the day of the blood test before treatment and 3 mo after the intervention started. There is evidence of a positive association between iron status and IQ and a language school achievement test but there is no support for the internal validity of the hypothesis that this association is causal.

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Summary:Bone marrow transplantation is the only therapeutic option that can eliminate thalassemic disease. Early results indicated that children in class 3 Lucarelli had a much worse outcome because of high nonrejection mortality and high rejection rate. We therefore tried to investigate a nonmyeloablative stem cell transplantation (NST) approach for such a disease in order to reduce mortality and rejection. We report here the case of successful NST in a 10-year-old girl who had class 3 Lucarelli ␤-thalassemia major. The conditioning regimen consisted of busulfan, fludarabine, antilymphocyte globulin and total lymphoid irradiation. Her GVHD prophylaxis included mycophenolate mofetil and cyclosporin. The patient had full donor engraftment without acute and chronic GVHD. She is now alive and well and remains disease-free 1 year after transplant.

A bleeding syndrome due to severe prothrombin complex deficiency is reported in 93 infants. Most were breast fed (98 per cent), aged 2 weeks to 1 year and there were no serious preceding or associated diseases. Hemorrhagic diathesis, pallor and mild hepatomegaly were the major manifestations. The incidence of intracr anial bleeding was strikingly high (63 per cent) particularly with subdural and subarachnoid hemorrhage. Acute onset, short course and rapid clinical and laboratory improvement after vitamin K therapy were observed. Mortality rate was 35 per cent but has been reduced to 17 per cent since 1969. The location of bleeding, prompt diagnosis and early treatment are the major factors affecting prognosis. Severe prothrombin complex deficiency due to vitamin K deficiency accounted for the pathogenesis of bleeding. Possible causes of vitamin K deficiency were discussed but definite conclusions could not be drawn.

Haemophiliac treatment in less developed countries is limited to locally prepared fresh frozen plasma, cryoprecipitate, cryo-removed plasma and lyophilized products as replacement therapy. Factor concentrate is seldom used because of the high price. The present study reports the survival analysis of 164 patients comprising 138 haemophilia A and 26 haemophilia B cases from 134 families registered at the International Haemophilia Training Centre-Bangkok, Faculty of Medicine, Ramathibodi Hospital, Mahidol University from 1971 to 2000. The duration of follow-up ranged from 1 to 27 years and 8 months with a median of 9 years and 1 month. They received treatment on demand of bleedings, and 85 patients received additional home treatment for early bleedings. The proportion of death was 15.2%. The Kaplan-Meier survival curves revealed the median (95% CI) survival time of patients with severe and moderate degrees of 35 years and 6 months (21.4-49.7), and 38 years and 1 month (28.8-47.3), respectively. To compare the progressive achievement of haemophilia care services, the treatment period was divided into three decades: 1971-1980, 1981-1990 and 1991-2000. The patients with unaided proper walking increased from 62.8% in the first decade to 84.5% in the third decade. However, one-third of the patients developed one to four chronic haemarthrosis determined by clinical evaluation, especially patients with severe degree. Moreover, the estimated probability of a survival time beyond 13 years of age among patients with severe degree increased from 0.85 in the first decade to 0.94 and 1 in the second and third decades, respectively. Thus, established haemophilia care, even with limited resources, has significantly decreased the risk of death and increased the survival time among patients with haemophilia.

A double-blind clinical trial was conducted among 9- to 11-year-old children in sixteen schools in the Chon Buri province of Thailand to assess the effects of an iron supplement combined with an anthelminthic agent (i.e. albendazole). In addition to the albendazole, Fe or placebo tablets were distributed to 2268 children enrolled in grades three to five without knowledge of the Fe status of the children. Criteria for case inclusion were: (a) absence of A E Bart's or haemoglobin (Hb) H disease, (b) absence of abnormal Hb EE, and (c) age, 108–144 months. The results showed a significant improvement in the Fe status of the children after 16 weeks of treatment. The increments were: Hb from 124 to 128 g/l, serum ferritin from 34.54 to 104.72 μg/l, transferrin saturation from 24.09 to 35.05%; free erythrocyte protoporphyrin decreased from 444.7 to 281.4 μg/l erythrocytes. These changes were significantly greater than in the control group that received only the anthelminthic agent. However, the administration of albendazole only also resulted in significant changes in the same Fe indicators.

There is no difference in the frequency of this genetic variation between Asian and North American Caucasian populations. Determination of the TPMT genotype by PCR method before antileukemic therapy is practical and may have clinical relevance. This knowledge could be applied towards organ transplant recipients who require these medications for immunosuppression.

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