[Extractions in hemophilic patients. Control of hemostasis with DDAVP].
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Biomedical subjects
Publications and source records attributed to L Rota.
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Sixteen cases are reported of dilatation of supra-aortic vessels; in 14/16 patients the vessel involved was either the subclavian artery or the brachiocephalich trunk. Special attention is paid to the choice of patients--the ideal one presenting with a single uncalcified lesion, with stenosis more than 50% of diameter; the symptoms have recently appeared, with a significant difference (more than 20 mmHg) in the pressure of the two arms. The technical aspects of the angioplastic procedure are discussed, especially in order to preserve the intracranial circulation and to limit possible complications. The presence of reversed blood flow in the vertebral artery is extremely important to preserve intracranial circulation from possible embolism; normal flow obtained at the end of the procedure is therefore an useful proof of successful dilatation. The advantages are stressed of dilatation over the surgical techniques used in the past. Finally, the importance is emphasized of a correct follow-up and doppler control of supra-aortic circulation.
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30 patients with a previous myocardial infarction who had undergone diagnostic conventional left ventriculography and coronary arteriography had left ventriculography also performed by digital subtraction angiography after intravenous injection of contrast medium; the mean interval between the two investigations was 2.3 days. The conventional and the digital subtracted left ventricular images were computer-processed; global ejection fraction (obtained by the volumes calculated with the Dodge method) and segmental wall motion (from a quantitative analysis performed according to the Stanford method) were compared. We found a close correlation for the ejection fraction obtained by the two techniques (r = 0.89, p less than 0.01); absolute values proved to be slightly higher in the digital subtraction angiography. Segmental wall motion was significantly correlated too (p less than 0.05 for all the segments of the ventricular silhouette but for the one corresponding to the base of the heart). Digital intravenous angiography provides an accurate and less invasive method to evaluate left ventricular function.
Bronchial arteries embolization is a routine treatment of hemophtysis. In patients affected by cystic fibrosis hemophtysis is often very serious, dangerous for their life. The extent of pulmonary lesions, the frequent bilaterality and respiratory dysfunction are contraindications to operation. The authors report their experience on three patients affected by cystic fibrosis with hemophtysis, in which bronchial embolization has been the only therapeutic choice. It performed a good result with immediate stopping of hemophtysis. In all three cases results were not serious relapses, but only rare slight hemophtysis.
Thirty patients with myocardial infarction were studied using Digital Subtraction Ventriculography with Intravenous injection (DSVIV) between 3 and 6 days from myocardial infarction and were checked after 30 and 90 days. The variation of ejection fraction in the controls was not important. The wall motion provided precise indications regarding the infarctual regions. Digital subtraction ventriculography seems to be a very interesting tool in the study of the left ventricle in the evolution of myocardial infarction, thanks to a low invasivity, providing data on ventricular function.
Intravenous digital angiography was performed in 92 patients with pulmonary diseases. The examination yielded satisfactory results in 82/92 cases (89%). The most promising fields of application of pulmonary digital angiography are: vascular malformations (3 cases: 3%), pulmonary emphysema (24 cases: 26%) and pulmonary embolism (38 cases: 41%). In tumoral masses (14 cases: 15%) digital angiography showed dislocation of the surrounding pulmonary vessels but failed to demonstrate the intrinsic vascularity of the mass, deriving from the bronchial arteries. In post-lobectomy controls (10 cases: 11%) the examination gave a good demonstration of the absence of vascular supply at the site of the previous surgery. Intravenous digital angiography shows many technical limitations, namely a reduced resolution power and a small field of view; in addition, it requires patient immobility, which results sometimes impossible in critically ill patients. In spite of these inconvenients, however, intravenous digital angiography appears to be an important diagnostic step for the evaluation of pulmonary embolism and vascular malformations: in these conditions, digital angiography can be the procedure of choice.
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These studies were designed with the purpose of providing clinico-pharmacological information relevant to the use of DDAVP in the management of mild haemophilia and von Willebrand's disease (VWD). In healthy subjects, intravenous DDAVP produced its maximal response at a dose of 0.3 micrograms/kg. The extent of the increase in factor VIII coagulant activity (VIII:C) and factor VIII related antigen (VIIIR:Ag) induced by this dose was not significantly different from that observed with the same dose in haemophiliacs and VWD patients. In these, the bleeding time was not shortened. DDAVP given intranasally was followed by a two-fold increase of VIII:C. This route of administration might be adopted to provide an emergency aid in bleeding patients and to yield higher VIII:C levels in blood donors. In healthy subjects, the half-disappearance time of autologous VIII:C after increase induced by i.v. DDAVP is similar to that observed in patients with VWD treated in the same conditions, whereas the response appears to be more prolonged in haemophiliacs. This study shows that the consistency of the VIII:C response tends to decrease when repeated doses are given to healthy subjects. Repeatedly-treated haemophiliacs and VWD patients showed varied patterns, ranging from no change of the response to its early abolishment.
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The authors report on their experience of two cases of splenic artery aneurysm in patients with cirrhosis and portal hypertension. It is underlined the determinant, causal role of the hemodynamic changes occurring in the circulation of the liver, spleen and portal vein in patients with or without portal hypertension. Above changes are thought to be responsible of the formation of splenic artery aneurysm in line with the current literature.
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Chronic liver disease is not often reported in patients with haemophilia. Although a high incidence of abnormal liver function tests has been reported, the clinical significance of these findings and their relation to chronic liver disease cannot be established without a liver biopsy. The results of this procedure, carried out in 11 patients with severe haemophilia A and B, in whom SGOT had been persistently raised for three years, are reported. Five patients had chronic active hepatitis, four had chronic persistent hepatitis, one had cirrhosis, and one alcoholic hepatitis. No haemorrhagic complication followed the biopsy procedure, which was carried out in patients given prophylactic clotting factor concentrates. These results suggest that duration of abnormal liver function tests is likely to represent liver disease in haemophiliacs, and that biopsy should be considered to establish the diagnosis and plan a suitable therapeutic programme.
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