National Journal of Health Sciences
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Genetic Haemoglobin Disorders in Pakistan
No full textGenetic haemoglobin disorders are broadly divided into thalassaemia syndromes and abnormal haemoglobins. Thalassaemias are a heterogeneous group of inherited disorders of haemoglobin synthesis in which one or more globin chains are either not produced at all (ßo) or are produced at a reduced rate (ß+). These are classified according to the type of globin chain(s) affected. The abnormal haemoglobins are produced in normal amount but are structurally abnormal and therefore lack the usual qualities of haemoglobin. Some abnormal haemoglobins like Hb-E, in addition to being structurally abnormal are produced in reduced amount [1]
An Insight in Understanding the Symptomatology of ß-Thalassaemia Major - II
No full textMost of the symptoms of ß-thalassaemia major are considered to be due to anaemia and massive iron overload in the body [1]. Despite of regular blood transfusion and iron chelation, delayed growth, risk of infection & thrombosis, asthenia, and exercise intolerance remain a major reason of debility for patients, and a major concern for treating physicians [1]. Treatment with Hydroxyurea (HU) in ß-thalassaemia major and intermedia has ameliorated the need of blood transfusion [2-6]. One consistent finding in these patients is subjective improvement (as reported by almost mothers of these kids) in quality of life, vitality, better exercise tolerance, improved food intake and energy level within a couple of weeks of exposure to the drug despite of minimal or no change in haemoglobin level [2-6]. This improvement persisted during the course of treatment even in partial responders(who were maintaining an Hb of 6-7 g/dl). Recently, additional benefit of HU on iron chelation/reduction in serum ferritin has been reported [7, 8]
Bortezomib-Induced Cardiac Dysfunction in a Patient with Plasma Cell Leukemia–A Remote Complication
No full textABSTRACT: Bortezomib is an important proteasome inhibitor widely used in plasma cell neoplasms. An acute cardiac dysfunction is not a frequent side effect of bortezomib, but it is mentioned in literature search via multiple case reports. We have discussed here, another case of a young male diagnosed with plasma cell leukemia on treatment, presenting with sudden onset dyspnea due to an acute myocardial toxicity which subsequently upon thorough evaluation turned out to be an association with cyclical bortezomib administration