Determination the blood serum levels of parathyroid hormone, calcitonin and osteocalcin concentrations in Syrian patients with beta-Thalassemia major

Treatment of beta-thalassemia major patients consists of regular blood transfusions and iron chela tor [Desferrioxamine]. This has extended the life expectancy of thalassemic patients and it lets to different endocrine and metabolic complications. The objective of this study is to determine the serum concentrations of some biochemical parameters [parathyroid hormone [PTH], calcitonin and osteocalcin] related to endocrine complications in beta-thalassemia major patients. 215 patients and 80 age- matched controls [6-41 years] entered the study. Hematological data were measured, and the levels of calcium and phosphate were determined. N-MID osteocalcin, intact PTH and calcitonin were determined in serum blood. Student's test was used to evaluate the differences between patient and control groups and P<0.05 was considered statistically significant. Serum concentrations of PTH and N-MID osteocalcin were significantly [P=0.0007 and P=0.0017 respectively] lower in patients than controls. PTH serum levels were clearly under normal range in 11.16% of patients. However, calcium and calcitonin concentrations in patients were not significantly [P=0.101 and P=0.26 respectively] different from those in controls. Our finding are in agreement with other studies and suggest that hypoparathyroidism is one of the endocrine complications, which occurred in beta-thalassemia major patients who undergo blood transfusion regimens. More prospective studies are important to determine the prevalence of other endocrinopathies changes among thalassemic patients due to mixture of reasons other than iron overloads, with much improved regime of therapy

Assessment of adrenal endocrine function in Asian thalassemics.

Adrenal endocrine function was assessed in a cohort of 20 patients, between 10 and 20 years of age, with transfusion dependent beta thalassemia. Cortisol levels were assayed before and after ACTH stimulation with 1 micrograms and 250 micrograms. Adrenal dysfunction was defined as a basal cortisol of greater than 400 nmol/L and/or peak cortisol levels of greater than 500 nmol/L. Overall, 9 patients (45 %) had in vitro evidence of adrenal dysfunction. A statistical significant correlation (r=0.4308; P < 0.05), between wasting and the basal cortisol level, was observed. Similarly, there was correlation between the number of transfusions received and growth failure (r=0.4774;P < 0.05). In comparison to the involvement of other endocrine axes in polytransfused thalassemics, the adrenal endocrine function abnormalities are minor and clinically of little consequence. The observations, albeit, in a small cohort of thalassemics, stress the need for an annual estimation of basal cortisol level, especially in patients with wasting.

Inhibitory effects of some substrate level analogues on salp-activity in B-thalassaemia

Higher levels of SALP-activity was recorded in serum of patients with a-thalassaemia in comparison with normal control Subjects at specific PH and temp. Both kinetic constant, Km and Vmax has been determined. Inhibitory effects of three substrate analogues, L-Phe, Adenosine, and inorganic phosphate were investigated. Types and degree of inhibition were determined together with values of Ki [inhibitory constant] using Dixons plot

Hepatocellular adenoma in a beta-thalassemic woman having secondary iron overload.

An 18-year-old woman had a hepatocellular adenoma of 4 cm across in the right lobe of the liver which was severely hemosiderotic because of beta-thalassemia hemoglobin E disease with numerous blood transfusions. The lesion was an accidental postmortem finding. To our knowledge, this is the second example of liver-cell adenoma occurring in a patient with beta-thalassemia hemoglobin E disease with secondary iron overload. It is suggested that this is an association between hepatocellular adenoma and secondary iron overload of liver cells, a similar event to the relation observed in hepatocellular carcinoma and secondary iron overload of hepatocytes.

Diferenciaçäo entre talassemia beta-heterozigótica e anemia ferropriva / Differentiation between heterozygotic beta-thalassemia and iron deficiency anemia

Várias funcöes discriminantes baseadas nos parâmetros hematimétricos fornecidos pelos contadores eletrônicos de células têm sido desenvolvidas e utilizadas nas diferenciaçäo de várias formas de anemia. A eficiência de alguns desses critérios comumente empregados na distinçäo entre a talassemia beta-heterozigótica e a anemia ferropriva foi testada em 192 indivíduos talassêmicos e em 72 adultos com anemia ferropriva. Foram avaliados: 1) funçäo discriminante de England e Fraser: VCM - (5xHb) - H - 8,4;2) HCM/H; 3) relaçäo de Mentzer: VCM/H; 4) (VCM)² x HCM; e 5) número de hemácias. Os resultados obtidos demonstraram falha na distinçäo entre estas duas entidades, em percentagens variáveis de 9 a 90//, sendo que a equaçäo de England e Fraser demonstrou os resultados mais satisfatórios, enquanto a relaçäo de Mentzer apresentou maiores índices de erro no diagnóstico da anemia ferropriva. Assim, embora algumas dessas fármulas possam ser utilizadas em programa de triagem, em casos individuais a diferenciaçäo entre talassemia beta-heterozigótica e anemia ferropriva deve ser sempre realizada com dosagens de HbA2, ferro sérico, capacidade máxima de transporte de ferro e ferritina