Glomerulonephritis in beta-thalassemia Hb-E disease: clinical manifestations, histopathologic studies and outcome.

We presented 8 patients with beta-thal/Hb E with glomerular diseases. Diverse glomerular lesions were seen, but diffuse endocapillary glomerulonephritis was the most common. The clinical manifestations of acute glomerulonephritis in beta-thal/Hb E differed from typical cases in the older age group, female preponderance, longer duration of edema, less hypertension, marked proteinuria, hypoalbuminemia and hypertriglyceridemia and also a longer period of recovery but their outcome was still favorable despite many risk factors of renal injury. Renal biopsy was necessary in doubtful cases to detect the correct diagnosis and give proper management. The association and mechanism of glomerulonephritis in these patients require further prospective study.

High-dose intravenous immunoglobulin in the management of immune hemolysis in patients with thalassemic disease: factors which determine refractoriness.

We report our experience with high dose intravenous immunoglobulin (IVIg) in 3 thalassemic patients who had evidence of possible immune hemolysis. In 2 patients who had serious sepsis, their responses to IVIg were only partial and transient. The other patient who had marked splenomegaly had no evidence of response to IVIg. Both serious infections and large spleen may hamper the effect of IVIg and should be considered before IVIg is to be used in thalassemia.

Red cell and plasma calcium, copper and zinc in beta-thalassemia/hemoglobin E.

Beta-thalassemia/Hb E is a genetic disease prevalent in Thailand. This study has used atomic absorption spectroscopy to evaluate red cell and plasma calcium, copper and zinc in patients with beta-thalassemia/Hb E, both splenectomized and non-splenectomized. The levels of these trace elements in both red cells and plasma were different between the non-thalassemic controls and the disease patients. The most prominent result was that calcium concentration in red cells increased significantly in thalassemia subjects, particularly in splenectomized cases. These results might reflect the abnormal trace element metabolism and defects in the calcium transport system of the red cell membrane in thalassemia.

Investigation of the causes of suboptimal haemoglobin response to iron supplementation.

The investigation concerned 572 children, 6-11 years of age, who were divided into three groups: urban control, rural control and rural study. During the first phase of supplementation, each child received 120 mg of elemental iron (as ferrous sulphate) with 0.50 mg of folic acid daily for 60 school days (five days a week) which resulted in a suboptimal haemoglobin concentration. During the second phase, all groups continued to take iron-folate tablets for 60 school days while the children in the study group also received protein, vitamins and minerals. Final blood examinations showed no significant difference in mean haemoglobin concentrations between the rural study and rural control groups. Only 1.5% of all children had haemoglobin below 12 g/dl. It can therefore be concluded that strict supervision of iron tablet intake and long-term supplementation with iron are essential for optimal haemoglobin response.

A prophylactic supplementation of iron and folate in pregnancy.

The supplementation of iron and folic acid were studied in 567 pregnant women with 18 and 26 weeks of gestation. Sixty mg and 180 mg of iron were given daily to pregnant women of group I and group II respectively while 180 mg of iron and 5 mg folic acid were given to group III. The Hb values increased significantly in group II and III after supplementation for 1 1/2 months, however if supplementation was extended for 3 months, highly significant increase in Hb levels were observed in all these groups. These findings indicated that in supplementation for a shorter period, i.e. 1 1/2 months at least 180 mg of iron was needed, and only 60 mg of iron was sufficient to increase Hb levels for a supplementation of 3 months. Vitamin B12 deficiency was not detected in pregnant women both before and after supplementation with iron and iron plus folate for 3 months. It was suggested therefore that perhaps it was not necessary to supplement vitamin B12 to Thai pregnant women. In this study 15% of pregnant women had low serum folate with normal red cell folate level, and a greater number of women with low serum folate concentrations were observed after supplementation with iron alone for 3 months. However, increased serum folate and red cell folate levels after supplementation with 5 mg folic acid indicated that some pregnant women needed folate supplementation in preventing folic acid deficiency during pregnancy.

Heterogeneity of beta thalassaemia in Thailand.

Beta thalassaemia in Thailand is heterogeneous. Clinical, genetical, haematological and globin chain biosynthetic studies were performed in seven beta-thalassaemia families. The results showed different gene combinations. These were alpha-thalassaemia/homozygous beta 0-thalassaemia, questionable double heterozygosity between a beta-thalassaemia and a silent beta-thalassaemia genes with low Hb F of unexplained cause, silent beta-thalassaemia/beta+ - or beta 0-thalassaemia, high Hb A2 high Hb F-beta-thalassaemia - a new mutant, mild beta+-thalassaemia/Hb E, and beta 0-thalassaemia/heterocellular HPFH or delta beta-thalassaemia associated with alpha-thalassaemia. Most of variability of clinical and haematological findings in these families is due to heterogeneity of the beta-thalassaemia and related genes.