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Biomedical subjects

J Pizzuto

Publications and source records attributed to J Pizzuto.

At least 37 records · Page 2Linked to original sources

Hemostatic defects in the Bernard-Soulier syndrome. Presentation of one case and literature review.

Bernard-Soulier's syndrome (BSS) is a familial hemorrhagic disease that is not very common, but its hemostatic defects have not been explained satisfactorily. In this paper the authors comments on the lack of Ib and Is glycoproteins in the platelet membrane, which is the basic characteristic of platelets with BSS. These proteins contain large amounts of sialic acid and have been identified as the absent membrane marker in said patients. The role played by these alterations in the BSS platelets leads us to suppose that there are no other structural and functional defects in such platelets, other than those related to the absence of membrane marker mentioned. From the above, a discussion follows on the similarity of BSS with von Willebrand's disease and it is concluded that the BSS is due to a scarce molecular concentration of Ib and Is glycoproteins in the platelet membrane and not to a functional defect of such molecules. The case described in this paper is the first one to be published in this country and was studied using the most useful and recommendable tests known at present, to this end.

Adult↗

Acquired von Willebrand's syndrome during autoimmune disorder.

A case with evidence of acquired von Willebrand's syndrome associated with systemic lupus erythmatosus and Sjögren's syndrome is described. The patient, who had no family history of bleeding, presented a haemorrhagic diathesis of recent origin, the bleeding time was prolonged, procoagulant Factor-VIII and von Willebrand factor levels were low and platelet aggregation was decreased with different concentrations of Ristocetin. No improvement was seen after the tranfusion of cryoprecipitates, and there was no increase in procoagulant Factor-VIII. Clinical improvement resulted after treatment with corticosteroids, and later, the laboratory abnormalities characteristic of von Willebrand's disease became normal. The level of procoagulant factor-VIII reached the very high level of 810%.

Adult↗

Adriamycin and splenectomy in the treatment of histiocytic medullary reticulosis.

Histiocytic medullary reticulosis (HMR) is a rare, malignant disorder with unsatisfactory response to various chemotherapeutic agents that have been used up to the present time. The purpose of this communication is to describe the first patient with this disease in whom an immediate, complete remission was obtained using only adriamycin. Four months after institution of this treatment, a staging laparotomy and splenectomy revealed residual disease in the spleen only. Adriamycin was continued as the sole agent, to the full calculated dose, followed by maintenance therapy consisting of cyclophosphamide. The patient continues to be in complete remission 35 months after diagnosis was established.

Adolescent↗

Use of androgens in acquired aplastic anaemia. Relation of response to aetiology and severity.

124 adult patients with acquired aplastic anaemia (A.A.) were studied. 54 presented as severe A.A. (mean survival of the whole group, 2.7 months and in those who died, 2.5 months; mortality 88.8%) while 70 were mild (mean survival of the whole group, 27.2 months and in those who died , 22.7 months; mortality, 28.5%). Sixty-four did not live long enough to have adequate therapeutic trials or developed severe hepatic failure which made it impossible to continue treatment. The effectiveness of 78 therapeutic periods of treatment in the 60 evaluable cases was analyzed as to aetiology and severity. A response was obtained in 31 (40%), of which 24 were with oxymethalone; 17 showed improvement after 6 months of treatment. No conclusions could be drawn as to the effectiveness of methalone, cyclophosphamide and prednisone because of the limited number of treatments. In severe A.A. only one of 13 treatments was effective, whereas 30 of the 65 used in mild A.A. gave a response. Oxymethalone was used in 23 out of 41 (56%). The aetiology, sex and age appeared to have no influence on the response of the 60 patients analyzed. The only factor which appears useful for prognosis as to the effectiveness of treatment is the severity of the case.

Adolescent↗

Pregnancy in patients with autoimmune thrombocytopenic purpura.

The obstetric and hematologic problems in 21 pregnancies of 18 patients with ATP are analyzed: one maternal and one fetal death occurred. Urinary estriol and oxytocinase were measured. Eighteen infants were born by vaginal delivery and three by cesarean section. None of the 20 liveborn infants died, but they had NIP due to the passage of maternal antibodies to the fetus. These results are compared to those reported in previous publications. The obstetric management of these patients should be individualized and carefully planned; it should not be based on the platelet count.

Female↗