Biomedical subjects
Publications and source records attributed to B Cuesta.
A 44 year old woman with multiple myeloma and cutaneous involvement is presented. She had a painless tumor over the malar region. Microscopic study showed a cutaneous infiltration of plasma cells. Survival from diagnosis lasted 5 months. Over this period, multiple and progressive cutaneous nodules appeared, having the same structural characteristics.
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The first autochthonous case of kala-azar in the province of Navarra is reported. The epidemiology, diagnostic procedures and the criterion for cure are related. The patient showed a very good response to treatment with pentavalent organic antimonials.
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The possible existence of acquired dysfibrinogenemia was investigated in blood samples from 30 patients with liver cirrhosis, 15 newborns and 30 healthy control subjects. Alterations of thrombin time were found in newborns and in 14 cirrhotic patients; glucide fraction levels were measured in these subjects and an increase in sialic acid content was observed. Its functional role was studied by comparing thrombin time and electrophoretic mobility of purified and desialylated forms of fibrinogen. We observed a thrombin time normalization, which was initially prolonged upon the removal of the sialic acid. The anodic electrophoretic mobility underwent changes due to the removal of sialic acid.
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A combination of antiplatelet agents, corticosteroids, plasmapheresis and fresh plasma achieved complete remission in a 35-yr old man diagnosed of thrombotic thrombocytopenic purpura who presented with hemolytic anemia, renal and neurologic damage. No symptoms 9 months after finishing treatment have been observed. Platelet and lactate dehydrogenase levels were the most useful analytical parameters in the follow-up.
Inherited qualitative abnormalities of fibrinogen have been documented in 144 families. These dysfibrinogenemias have been inherited as autosomal dominant traits and usually are clinically silent, but in some cases are associated with bleeding, thrombosis, or defective wound healing. Dysfibrinogenemias may be associated with defects in any of the three basic steps in the thrombin-fibrinogen reaction, i.e., cleavage of the fibrinopeptides by thrombin, polymerization, and fibrin cross-linking. Biochemical studies of several abnormal fibrinogens have demonstrated that the functional defects are the result of single amino acid substitutions. Most of the reported cases may be distinguished by functional criteria and by the physicochemical behavior and biochemical nature of the abnormal protein.
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