PubMed · 6832492
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J Aicardi. 1983. Neonatal spinal-cord dysfunction.. https://doi.org/10.1111/j.1469-8749.1983.tb13728.x
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Recent studies investigating thrombotic thrombocytopenic purpura (TTP) have implicated abnormal plasma von Willebrand factor (vWF)-cleaving metalloprotease activity in this disorder. It has been proposed that a metalloprotease cleaves unusually large (UL) multimers of vWF, which enter the circulation from the endothelium. Abnormal metalloprotease activity could result in ULvWF, which could participate in TTP. However, the diagnostic specificity of abnormalities in the plasma metalloprotease activity has not been established. A prospective study of vWF protease activity was performed using samples from 20 healthy controls, 20 patients with acute TTP, 20 patients with immune idiopathic thrombocytopenic purpura (ITP), 10 patients with disseminated intravascular thrombocytopenia (DIC), 10 patients with systemic lupus erythematosus (SLE,) and 5 thrombocytopenic patients with leukemia. Studies were performed blinded to the diagnosis. Samples from hospitalized patients with normal platelet counts were also tested. The vWF digests and multimer analysis were done using previously described methods. Six laboratory personnel independently scored each of the multimer gels. Reduced protease activity was observed in 9 of 20 patients with TTP. Reduced activity was also observed in 6 of 20 patients with ITP, 6 of 10 patients with DIC, 5 of 10 patients with SLE, 1 of 5 patients with leukemia, 2 of 20 healthy controls, and 3 of 25 hospitalized patients. This study indicates that abnormalities of vWF protease activity are not restricted to patients with the diagnosis of TTP.
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PURPOSE: The purpose of our study was to clarify the frequency of these causes. PATIENTS AND METHODS: Retrospective study using reports of newborns in the neonatal unit in Sousse (Tunisia) from 1991 to 1996, hospitalized for hemorrhagic syndrome defined by bleeding, exteriorized or not, whatever its importance, severity, causes and the associated clinical and biological disorders. Isolated meningeal hemorrhages, limited cutaneo-mucous hemorrhages (conjunctival hemorrhages, bruises), and genital crises of the newborn, were excluded. RESULTS: One hundred and fifty-five hemorrhagic syndromes were observed from 7,128 newborn infants (2.17% of hospitalization). Sex ratio was 1.42. Prematurity rate was 35.7%. The Apgar score was < 7 at one minute in 40.7% of cases. Disorders associated with hemorrhagic syndromes were observed in 118 newborn infants (76.1%) with a predominance of neonatal infections (35.6%). The etiology of neonatal hemorrhages was specified in 93% of cases: newborn hemorrhagic disease (27.7%), disseminated intravascular coagulation (27.1%), isolated thrombocytopenia (9%), digestive lesions (13.5%), and obstetrical trauma (2.6%). CONCLUSION: The frequency of the newborns hemorrhagic syndromes underlines the need for its systematic prevention by vitamin K in the antenatal period to the mother and after birth to the newborn.