Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019045 (hemoglobinopathies)
 2,704 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sixty four cases of retinal and vitreous hemorrhages are reported during a 15 months prospective study in Bamako. Main diseases associated with hemorrhages are high blood pressure (56% of cases), hemoglobinopathies (33%) and diabetes mellitus (23%). In 28% of cases several aetiologies are connected. SC hemoglobin is a frequent aetiology of vitreous hemorrhage (40%). Hemoglobin AS and AC, generally asymptomatic, are also liable to hemorrhages. Terson and Eales syndromes, Werlhof disease, hemophilia and AIDS are most uncommon. In 8% of cases there is not any aetiology.
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PMID:[General causes of retinal and vitreous hemorrhages in Mali]. 181 88

The prevalence of von Willebrand's disease (vWd) in Zulia State, Venezuela, was studied among patients referred because of hemorrhagic symptoms. Ninety six cases out of 684 patients aged six months to fifty years, were diagnosed as vWd by measuring FvW, FVIII and Ristocetin cofactor in plasma, along with determinations of the bleeding time and platelet aggregation with ristocetin. Crossed immune electrophoresis and determination of FvW concentration in platelets were also carried out in several patients. The prevalence of the disease in Zulia State was 42.9/10(6) inhabitants and in the metropolitan area of Maracaibo (capital city of Zulia State) was 73/10(6) inhabitants. Eighty eight per cent of the patients were classified as vWd Type I, 2% as vWd Type IIb and 4.1% as Type III. Five cases were not classified. Three patients with Type I vWd also suffered from Autoimmune Thrombocytopenic Purpura, one had a SC hemoglobinopathy and in one patient the disease coexisted with mitral valve prolapse. The need for a proper attention to the patients with vWd is emphasized, as its prevalence in the region is similar to that found in european countries, where a great effort is applied to assure the adequate therapy and social support.
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PMID:[Epidemiology of von Willebrand's disease in the state of Zulia, Venezuela]. 182 22

Splenectomy is therapeutic for a large host of conditions. It is a consequence of expanding the list of disorders and liberalizing the indications for splenectomy in many diseases. Red blood cells disorders: autoimmune hemolytic anemia, hereditary spherocytosis, hemoglobinopathies and thalassemia are prone to splenectomy after failure of medical therapy. A variety of thrombocytopenic disorders are improved by splenectomy, and the most common indication for splenectomy is ITP (idiopathic thrombocytopenic purpura). Splenectomy is successful in reversing hypersplenism in a spectrum of disease called myeloproliferative disorders. Relief of symptoms from splenomegaly is also achieved, but it does not affect the inexorable course of the disorder. The role of splenectomy in white blood cells disorders (leukemias and lymphomas) is only palliative and facilitates chemotherapy. Splenectomy in patients with hemathologic disorders imparts a risk of fulminant and life threatening infection "overwhelming postsplenectomy sepsis" that can be obviated by appropriate treatment. Although splenectomy for hemathologic disorders is only therapeutic and not curative, the relief of symptoms and for some disorders facilitation of chemotherapy leads to better quality of life and longer survival.
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PMID:Splenectomy for haematological disorders. 2479 4