Sarcopenia is a loss of muscle mass and function in the elderly that reduces mobility, diminishes quality of life, and can lead to fall-related injuries, which require costly hospitalization and ex...

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Sarcopenia: Aging-Related Loss of Muscle Mass and Function.

The hypercoagulable state in thalassemia.

Thalassemias and the hemoglobinopathies such as Hemoglobins S, C and E, are now a global problem. They have spread through migration from their native areas in the Mediterranean, Africa and Asia and are now endemic throughout Europe, the Americas and Australia. Comprehensive control programs in recent years have succeeded in limiting the numbers of new births and prolonging life in affected individuals. Such programs have been successful in a minority of countries and have little global impact. Over 300,000 infants with major syndromes are born every year and the majority die undiagnosed, untreated or under-treated. Countries may be divided into three general categories according to the services available: A. Endemic Mediterranean countries. In these long-established prevention programs have succeeded in achieving 80%-100% prevention. Specialized clinics able to provide optimum treatment. B. Areas of the developed, industrialized world where prevalence is increasing because of migration. These countries have the means to provide adequate control but have problems in reaching immigrant groups with different cultural background. C. Countries of the developing world where the provision of services is hampered by economic difficulties, other health priorities due to high infant mortality from infectious diseases, and religious/cultural constraints.

Global Epidemiology of Hemoglobin Disorders

Cet article etudie successivement la frequence de la thalassemie α, de la thalassemie β, des variants genetiques de la chaine α et de la chaine β, de l'hemoglobine constant spring et de l'HbE

Hemoglobinopathies in Southeast Asia

Hemoglobin H disease: not necessarily a benign disorder

Hydroxyurea increases hemoglobin F levels and improves the effectiveness of erythropoiesis in beta-thalassemia/hemoglobin E disease.

Genetic factors determining the difference in severity of anaemia in beta-thalassaemia/HbE disease were studied in 90 patients who had haemoglobin levels, at steady state, ranging from 4.2 to 12.6 g/dl. Co-inheritance of alpha-thalassaemia 2 and haemoglobin Constant Spring could significantly decrease the severity of the disease. Inheritance of a beta-thalassaemia chromosome with Xmn I cleavage site at position -158 of the G gamma-globin gene which was linked to the haplotype -+-++ or ++-++, was associated with a milder anaemia. Two copies of these alleles were necessary to produce a significant clinical effect. Increased expression of the G gamma-globin gene and higher production of haemoglobin F, which could reduce the overall globin chain imbalance, were also associated with homozygosity for the Xmn I cleavage site and thus with less severe anaemia. However, this effect was not seen in Xmn I site heterozygotes. Whether the effects of the Xmn I polymorphism, HbF concentration and G gamma/A gamma ratio act separately or through common mechanisms in reducing anaemia remains to be ascertained.

Severity differences in β‐thalassaemia/haemoglobin E syndromes: implication of genetic factors

In Thailand, a and B thalassemia, hemoglobin (Hb) E, and Hb Constant Spring (Con Sp) are The frequencies are 20%-30% for a thalassemia, 3%-9% for thalassemia, up to 52% for Hb E and at least 4% for Hb Con Sp. The abnormal genes in different combinations lead to over 60 thalassemic syndromes. TABLE 1 shows the numbers of patients with major thalassemic diseases examined in our unit, exclusive of the Hb Bart's hydrops fetalis. This is in the department of medicine; in the department of pediatrics of this hospital they have seen more or less the same numbers of thalassemic patients. Clinical features, although of extreme interest, will not be described here; however, certain other salient points will be discussed.

Thalassemia in thailand

Examination for circulating platelet aggregates according to Wu and Hoak revealed increased circulating platelet aggregates in 71% of splenectomized and 35% of nonsplenectomized patients with beta (0)-thalassaemia/Hb E disease. This may be causally related to the newly observed high incidences of pulmonary artery thrombosis and hypoxaemia in splenectomized thalassaemic patients. It is recommended that anti-platelet aggregation drugs such as aspirin and/or dipyridamole are given to thalassaemic patients after splenectomy.

Winichagoon P

Papers

Hydroxyurea increases hemoglobin F levels and improves the effectiveness of erythropoiesis in beta-thalassemia/hemoglobin E disease.

Severity differences in β‐thalassaemia/haemoglobin E syndromes: implication of genetic factors

Thalassemia in thailand

Increased circulating platelet aggregates in thalassaemia.

Variable severity of Southeast Asian beta 0-thalassemia/Hb E disease.