Partial splenic embolization in patients with thalassemia major.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to A Karamerou.
Explore the source record for details and available documents.
The phagocytic and bactericidal activity of polymorphonuclear leucocytes (PMNL) obtained from 50 non-splenectomised patients with homozygous beta-thalassaemia was studied. Polymorphonuclear leucocytes suspended in the serum of patients from whom they were derived ingested fewer Escherichia coli, 72.2 +/- 21.8 (mean +/- s.d.) per 50 PMNLs compared with 144.5 +/- 36.8 bacteria phagocytosed by PMNLs from healthy volunteers and suspended in normal serum (P less than 0.01). Killing of ingested bacteria by the PMNLs from patients was also significantly reduced. These abnormalities are in part serum-associated and are due to the presence of heat-labile inhibitor(s) in the patients' serum. When PMNLs from patients were suspended in patients' heat-inactivated serum, phagocytosis increased to 99.2 +/- 29.2 (P less than 0.01). Similar improvement was noted in PMNL bactericidal activity. These abnormalities provide additional information that helps to explain the increased susceptibility to bacterial infections of patients with homozygous beta-thalassaemia.
Thyroid function was evaluated in 20 patients aged 8-30 years who were suffering from homozygous beta-thalassaemia. All patients have been receiving frequent blood transfusions and treated for the resulting transfusional iron overload with intramuscular injections of Desferrioxamine. Total thyroxine (T4), T3-uptake, total triiodothyronine (T3), and reverse triiodothyronine (rT3) were measured. In addition a standard TRH stimulation test was performed and blood samples were checked for the presence of thyroid antibodies. It was found that total T4 was significantly lower in the patients than in the controls. Total T3 and rT3 levels were similar in both patients and controls and all patients were negative for thyroid antibodies. T3 uptake in the patients was also statistically different from the controls resulting in significantly lower free thyroxine index (FT1). Basal TSH values were not different from the controls but the TSH increase following TRH stimulation was significantly higher in the patients suggesting, together with the low total T4 and FTI, a state of compensated hypothyroidism.
The distribution of phenotypes and gene frequencies of the third component of complement (C3) were studied in 106 beta-thalassemic patients and in 112 carriers of the beta-thalassemia trait. A statistically significant association was found between the C3F gene and homozygous beta-thalassemia. It can be suggested that this association may be related with the high incidence of infections encountered in these patients.