منابع مشابه
[Protein C deficiency].
Severe protein C deficiency (i.e. protein C activity <1 IU dL(-1)) is a rare autosomal recessive disorder that usually presents in the neonatal period with purpura fulminans (PF) and severe disseminated intravascular coagulation (DIC), often with concomitant venous thromboembolism (VTE). Recurrent thrombotic episodes (PF, DIC, or VTE) are common. Homozygotes and compound heterozygotes often pos...
متن کاملAcquired free protein S deficiency in children with steroid resistant nephrosis.
Plasma and urine concentrations of protein S were measured in five children with steroid-resistant nephrotic syndrome. It was found that plasma free protein S was reduced in three out of the five patients studied. Thus, acquired free protein S deficiency does occur in children with nephrotic syndrome and is one of many factors which may place them at risk for a thromboembolic event.
متن کاملProtein C deficiency in liver disease.
Protein C is a vitamin K-dependent zymogen of a serine protease that is found in blood plasma. The active form, activated protein C, can inhibit blood coagulation and stimulate fibrinolysis. Protein C is synthesized in the liver as a single chain protein. Its synthesis requires several post-translational modifications including carboxylation of glutamic acid residues, hydroxylation of aspartic ...
متن کاملAcquired apolipoprotein B deficiency with chronic hepatitis C virus infection.
Chronic hepatitis C virus (HCV) infection is often associated with fatty liver. Apolipoprotein B (ApoB) deficiency is one of the known causes of fatty liver and acquired ApoB deficiency has recently been reported with HCV infection. We report two patients (47-year-old lady and 48-year-old man) who had asymptomatic transaminase elevation, fatty liver, anti-HCV positive with high viral load (geno...
متن کاملAcquired C 1 Inhibitor ( Cl - INH ) Deficiency Type
The response of two patients with autoantibody-mediated Clinhibitor (Cl-INH) deficiency to replacement therapy with Cl-INH was studied over a period of 3 d. In patient 1 an acute attack of angioedema was successfully managed by infusion of 1,000 U ofC1-INH concentrate. C1-INH function returned to normal levels within 30 min., while CH50 and C4 peaked after 6-7 h and Cl hemolytic activity reache...
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 1999
ISSN: 0031-3998,1530-0447
DOI: 10.1203/00006450-199906000-00281