摘要:

We describe a male infant with congenital deficiency of coagulation Factor XIII who presented in the immediate postnatal period with umbilical stump bleeding and suffered a severe intracranial hemorrhage at 2 months of age. Factor XIII, also known as “fibrin-stabilizing factor,” is a transpeptidase that produces strong covalent bonds between soluble fibrin monomers formed during coagulation. Presumptive diagnosis of Factor XIII deficiency was made with a clot solubility screening test, and confirmation was accomplished by demonstrating the absence of cross-linked fibrin chains by electrophoresis. This patient had received replacement therapy for 2 years, initially with intravenous fresh frozen plasma, and recently with Fibrogammin (Hoechst-Roussel Pharmaceuticals), a European Factor XIII concentrate soon to be available, in the United States. Factor XIII deficiency is associated with a high incidence of life-threatening complications, notably intracranial hemorrhage. In light of the long half-life of this factor and the relatively low risk associated with new Factor XIII concentrates, such as Fibrogammin, prophylactic life-long replacement therapy should be considered for patients with severe Factor XIII deficiency.

ISSN:0192-8562    

出版商:OVID    

年代:1988

数据来源: OVID

摘要:

We report two group B Rho(D)-positive bone marrow transplant recipients who had sudden onset of massive intravascular hemolysis following the use of intravenous immunoglobulin (i.v. Ig). The first case developed chills and hypotension followed by hemoglobinuria with the first infusion of i.v. Ig. More severe symptoms occurred when infusion was reattempted. The second case developed hemoglobinuria without other symptomatology. In both patients, direct and indirect antiglobulin tests became positive following the use of i.v. Ig. Sera and eluates demonstrated IgG anti-B. Samples of i.v. Ig received by both patients contained both anti-B and anti-A and were incompatible with patients red blood cells. These serologic findings indicate acute hemolysis secondary to the presence of IgG anti-B in i.v. Ig preparations.

ISSN:0192-8562    

出版商:OVID    

年代:1988

数据来源: OVID

摘要:

To assess the prognostic value of the cellular DNA content measured by flow cytometry in acute lymphoblastic leukemia (ALL), we studied the pretreatment distribution of the DNA content in marrow blasts from 74 children with this disease. They were divided into standard-and high-risk groups according to their white blood cell counts and age at the time of diagnosis and were followed for up to 44 months. Abnormal DNA stemlines were detected in 26 children, and all of them had a hyperdiploid DNA content (DNA index of >1.0). The duration of remission was significantly longer in those with hyperdiploid DNA stemlines than in those with diploid DNA stemlines in both risk groups. In the standard-risk group, the DNA content and common ALL antigen were significant prognostic factors, especially the former. In the high-risk group, the DNA content also showed an independent significant correlation with the clinical outcome.

ISSN:0192-8562    

出版商:OVID    

年代:1988

数据来源: OVID

ISSN:0192-8562    

出版商:OVID    

年代:1988

数据来源: OVID

摘要:

In older children and adults the use of thrombolytic drugs appears to be a promising medical approach to thromboembolic disease. However, experience in newborns is limited, with both failure and success having been reported. In this article, the fibrinolytic mechanism is delineated. The similarities and differences in this mechanism between newborns and adults are indicated. It is clear that our understanding of the newborn's fibrinolytic system is limited. Thrombolytic therapy is reviewed. It is recommended that future clinical studies be designed to include three essential features: (a) a uniform clinical staging, (b) a dose-response relationship with a plasminogen activator based on in vivo and in vitro studies, and (c) detailed studies of the newborn's fibrinolytic mechanism before and during thrombolytic therapy.

ISSN:0192-8562    

出版商:OVID    

年代:1988

数据来源: OVID

ISSN:0192-8562    

出版商:OVID    

年代:1988

数据来源: OVID