Cerebral Infarction ; etiology ; Enzyme Activation ; Humans ; Infant ; Male ; Protein C Deficiency ; complications ; Protein S Deficiency ; complications

Anemia, Hypochromic ; etiology ; Humans ; Iron ; metabolism ; Protein Deficiency ; complications ; congenital ; Transferrin ; deficiency

Portal vein thrombosis (PVT) is an uncommon cause of presinusoidal portal hypertension. Among various hepatoportal disorders, noncirrhotic portal hypertension conditions such as idiopathic portal hypertension (IPH) are considered to have a close relation with PVT. PVT is known to have several predisposing conditions, including infection, malignancies, and coagulation disorders. There is growing interest and recognition that deficiencies in proteins C and S are associated with a hypercoagulable state. These deficiencies are regarded as key factors of systemic hypercoagulability and recurrent venous thromboembolism. We report the case of a 19-year-old male diagnosed as IPH with PVT and combined deficiencies in proteins C and S.
Humans ; Hypertension, Portal/complications/*diagnosis/pathology ; Male ; *Portal Vein ; Protein C Deficiency/*complications ; Protein S Deficiency/*complications ; Tomography, X-Ray Computed ; Venous Thrombosis/complications/*diagnosis/pathology ; Young Adult

The prevalences of deficiencies in antithrombin III (AT III), protein C (PC), protein S (PS) and in the activated protein C (APC) resistance in the thrombotic population of the Trakya region, Turkey were investigated. 37 patients with venous thrombosis (VT) and 17 patients with arterial thrombosis (ArT) were included in this study. The mean ages of the patients with VT and ArT were 46 years (range 20-70) and 38 years (range 32-40), respectively. The activity of AT III was measured by commercially available immuno-turbidimetric assay. The activities of PC and PS were determined by coagulometric assay. The APC resistance was measured using a modified APTT-based clotting assay. Among the VT patients, there were 2 cases (5.4%) with AT III, 5 (13.51%) with PC deficiency, 5 (13.51%) with PS deficiency and 2 (5.4%) with APC resistance. In the ArT patient group, there was 1 patient (5.88%) with AT III, 3 (17.64%) with PC deficiency, 1 (5.88%) with PS deficiency and no APC resistant patients, while there was one (2.08%) with PC deficiency and one (2.08%) with APC resistance in the control group (49 persons, mean age 41 years). The relative risk of thrombosis (odds ratio) was 1.7 in the deficiency of PC and 5.6 in the deficiency of PS. The data presented suggests that the prevalences of AT III, PC and PS deficiencies causing thrombophilia in the Trakya region of Turkey are higher than in other reported studies while the APC resistance is lower than in others. Further studies including more patients would be required to clarify these discrepancies.
Activated Protein C Resistance/complications ; Adult ; Antithrombin III Deficiency/complications ; Human ; Middle Age ; Prevalence ; Protein C Deficiency/complications ; Protein S Deficiency/complications ; Risk Factors ; Thrombophilia/epidemiology* ; Thrombosis/etiology ; Turkey/epidemiology

Protein S is found in two forms in plasma; as free and functionally active protein S, and complexed to C4b-binding protein. Patients with nephrotic syndrome are at risk for arterial and venous thrombosis at various localizations, and acquired protein S deficiency due to the selective urinary loss of the free form may be a risk factor for the development of thromboembolic complications. We report a case of cerebral arterial thrombosis associated with decreased level of free protein S antigen (44%) in a 39-year-old female patient with nephrotic syndrome.
Adult ; Case Report ; Female ; Human ; Intracranial Embolism and Thrombosis/*etiology ; Nephrotic Syndrome/*complications ; Protein S Deficiency/*complications

The pathological changes of the gastric rumen produced by rocking stress in 38 young male albino rats fed on low protein diet for a short period and in 20 young male albino rats fed on low protein diet only as the control rats, were investigated macroscopically and histologically. The ulcerative lesions of the gastric rumen were observed in 17 out of 38 experimental rats accompanied with hyperplasia and cornification of the ruminal epithelial layer and more or less edematous changes of the tunica propria near the ulcer site. The authors, finding of the ruminal ulcer in albino rats produced by rocking stress associated with feeding on a low protein diet for a short period were compared with the results of others. The authors discussed other opinions concerning of the gastric ruminal ulcer of the albino rats produced in various ways.
Animal ; Male ; Protein Deficiency/complications* ; Rats ; Rumen* ; Stomach Ulcer/etiology* ; Stomach Ulcer/pathology ; Stress*

OBJECTIVETo investigate the mechanism of anticoagulation protein defect in the pathogenesis of unexplained recurrent miscarriage.

METHODSFifty-seven patients with a history of unexplained abortion were enrolled as the investigation group for tests of protein C, protein S, antithrombin III (AT-III), as well as activated protein C resistance (APC-R). The control group consisted of fifty healthy women with a history of normal pregnancy and delivery. Blood samples were obtained for, measuring serum activity of protein C, protein S, AT-III, and APC-R. Patients with positive APC-R were tested for factor V (FV) Leiden gene mutation by PCR-RFLP method.

RESULTSOf the 57 patients, 12 (21.1%), 1 (1.8%), and 5 (8.8%) cases were found with protein S, protein C, and AT-III deficiency respectively, and 13 (22.8%) cases with positive results of APC-R. Of the control group, no protein C or AT-III deficiency was ever found, whereas 2 (4.0%) volunteers were presented with protein S deficiency and 3 (6.0%) with positive results of APC-R. No FV Leiden gene mutation was identified in all the patients with positive APC-R results. Late spontaneous abortion cases had higher incidence of anticoagulation protein defect than the early cases.

CONCLUSIONAnticoagulation protein defect may play a role in the pathogenesis of fetal loss, especially for those occurring in late stage of pregnancy.

Abortion, Habitual ; blood ; etiology ; Activated Protein C Resistance ; blood ; complications ; genetics ; Adult ; Antithrombin III ; metabolism ; Antithrombin III Deficiency ; blood ; complications ; Factor V ; genetics ; Female ; Humans ; Point Mutation ; Protein C ; metabolism ; Protein C Deficiency ; blood ; complications ; Protein S ; metabolism ; Protein S Deficiency ; blood ; complications

Acute Disease ; Adult ; Female ; Humans ; Mesenteric Ischemia ; etiology ; surgery ; Pregnancy ; Pregnancy Complications ; surgery ; Protein S Deficiency ; complications ; Remission Induction

The purpose of this study was to evaluate the early outcome of endovascular management in patients with iliofemoral deep venous thrombosis (DVT) due to iliac vein compression syndrome (IVCS) and protein C and/or S deficiency. Between September 2000 and January 2003, catheter-directed thrombolysis was performed in 11 patients with a diagnosis of acute iliofemoral DVT: 7 with protein C and/or S deficiency and 4 without protein C and/or S deficiency. After thrombolysis, the diagnosis of IVCS was confirmed in 6 patients: 4 with protein C and/or S deficiency and 2 without protein C and/or S deficiency. Further intervention consisted of angioplasty and stent placement was performed. Four patients with IVCS and protein C and/or S deficiency were included in this study. The immediate technical and clinical success rates were 100% in all 4 patients. There were no complications or clinically detectable pulmonary emboli. This initial experience suggests that endovascular management of iliofemoral DVT due to IVCS in patients with protein C and/or S deficiency is safe and effective.
Adult ; Aged ; Female ; Humans ; Iliac Vein ; Male ; Middle Aged ; Plasminogen Activators/administration & dosage ; Protein C Deficiency/*complications ; Protein S Deficiency/*complications ; Research Support, Non-U.S. Gov't ; *Thrombolytic Therapy ; Treatment Outcome ; Urinary Plasminogen Activator/administration & dosage ; Venous Thrombosis/*complications/*drug therapy

Biomarkers ; Cerebral Hemorrhage ; etiology ; prevention & control ; Humans ; Infant, Newborn ; Protein Precursors ; Prothrombin ; Vitamin K Deficiency ; complications ; diagnosis ; Vitamin K Deficiency Bleeding ; etiology ; pathology ; prevention & control