INTRODUCTION Metabolic bone disease represents a major cause of morbidity in patients with thalassaemia major. The aim of our study was to assess the prevalence and underlying contributory factors of osteopenia/osteoporosis in a randomly selected population of adult patients with thalassaemia major. PATIENTS AND METHODS The study population was selected using the random sampling method from t...

BACKGROUND Fatimid Foundation with its centers serves as a charitable organization for millions of blood disease carriers in Pakistan. This retrospective survival study is an analysis of the Thalassaemia patients registered in Multan centre who are followed up to nine years to assess the gender risk of death. METHODS Data on 120 patients of Thalassaemia during 1994-2002 was analyzed. The stan...

Thirty-seven patients with SCD were studied: 24 were diagnosed as homozygous Hb S on the basis of their haematological findings, and alpha:non-alpha globin chain ratios were found to be balanced in all. Thirteen patients were thought to have alpha or beta thalassaemia interaction with Hb S on the basis of low MCV and MCH, family history and/or presence of Hb A on electrophoresis. Six of them ha...

OBJECTIVE To investigate the frequency of metabolic syndrome and its components in subjects with β-thalassaemia minor. SUBJECTS AND METHODS A total of 194 subjects, i.e. 92 subjects with β-thalassaemia minor (study group) and 102 subjects without β-thalassaemia minor (control group), were enrolled into this case-control study. Haemoglobin electrophoresis was performed on all patients. The wai...

The effectiveness of the one tube method of osmotic fragility with three buffered solutions (0.32% saline, 0.36% saline, and tyrode) as a screening test for beta-thalassaemia trait was evaluated in several groups of subjects from Greece, Yugoslavia, and Thailand. The results clearly demonstrated that 0.36% saline is the most sensitive and effective solution since it could detect 96 to 100% of h...

OBJECTIVE To get preliminary data regarding the prevention of thalassaemia major in future generations. METHODS This Knowledge Attitude Practices study was conducted at Pakistan Institute of Medical Sciences, Islamabad, Pakistan, from January to June 2016, using non-probability purposive sampling. Parents of children undergoing transfusion were interviewed. Questionnaires were used to collect...

Haemochromatotic cardiomyopathy is the main cause of morbidity and mortality in patients with beta thalassaemia major. Once congestive heart failure develops most patients die in a few months. Congestive heart failure was reversed and echocardiographic findings were restored to normal in a 24 year old woman with beta thalassaemia who resumed treatment with chelation therapy (desferrioxamine).

Although DNA analysis is needed for characterization of the mutations that cause β-thalassaemia, measurement of the Hb A(2) is essential for the routine identification of people who are carriers of β-thalassaemia. The methods of quantitating Hb A(2) are described together with pitfalls in undertaking these laboratory tests with particular emphasis on automated high-performance liquid chromato...

Two cases of spinal cord compression resulting from extramedullary haemopoiesis in patients with thalassaemia of intermediate clinical severity are reported. The association between mild thalassaemia with long survival and the risk of spinal cord compression from extramedullary haemopoietic deposits is emphasised.

Madam, Beta thalassaemia major is a common genetic disorder in the country and hepatitis B and C viruses are prevalent in the blood donors population. therefore, patients with beta thalassaemia major who receive blood transfusions regularly to maintain optimal Hb level frequently suffer from the hepatitis infection. An earlier study has shown 51% prevalence of hepatitis C infection among them. ...