Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.4.21.5 (thrombin)
 33,306 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An 18-month-old boy was admitted to our hospital with sudden onset of paraplegia, analgesia of the lower limbs, dysuria and constipation. His gestational and birth histories were unremarkable. Past history revealed he had lymphangioma in his left inguinal region, and had been treated in another hospital. Neurological examination revealed flaccid paraplegia, analgesia below Th12 dermatome and dysuria. MRI revealed an intramedullary high intensity lesion surrounded by round low intensity areas located from TH11 to L2 vertebral levels, suggesting the existence of vascular tumor or spinal AVM. Spinal angiogram revealed arteriovenous fistula with large intramedullary aneurysmal vascular dilatation from T12 to L2 vertebral level. The feeder was the Adamkiewicz artery which branched from the left Th12 intercostal artery. First, artificial embolization with thrombin gelfoam was performed successfully. However, follow-up MRI showed an image of flow void in the aneurysm again, indicating recanalization of the AVF. Therefore, an operation was undertaken on October 24th, 1988. The patient was placed in prone position and osteoplastic laminotomy from Th10-L2 was performed. The thrombus and wall of the aneurysm were mostly removed through the lumbosacral midline myelotomy for decompression. Then, the feeder and drainers were ligated. Postoperative course was uneventful. 2.5 years after the operation, he still had flaccid paralysis at the ankle joints bilaterally, analgesia below L4 dermatome, neurogenic bladder and constipation.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[An infant case of spinal arteriovenous malformation with a large venous aneurysm]. 157 80

We present an 18-year-old patient with Degos' disease who manifested the sudden onset of paraplegia. Neurological examination revealed a rt-hemianopsia, paraparesis (with a sensory level at Th12), and a neurogenic bladder. Brain MRI showed multiple cerebral infarctions accompanied by small hemorrhagic areas and GdDTPA enhancement of the dura. A cerebral angiogram displayed stenosis, ectasia, and aneurysms involving the peripheral branch of arteries. Stenoses also were observed in the celiac artery and small arteries in the 1t-kidney. Skin biopsy exhibited hyperkeratosis, atrophy of the epidermis, and necrobiosis of the collagen layer. Laboratory examinations disclosed persistent elevations of the thrombin-antithrombin III complex (TAT), plasmin-alpha 2 plasmin inhibitor complex (PIC), and cytotoxic T cell subset (CD8+CD11-), illustrating the coagulative, fibrinolytic, and immunological implications of this disease.
 ...
PMID:Degos' disease: radiological and immunological aspects. 894 89

Deep vein thrombosis (DVT) is a frequent event in patients with spinal cord injury, even with prophylactic anticoagulant therapy. Lower limb paralysis is a known major risk factor for venous thrombosis, supposedly due to the venostasis in relation with total immobility. The main goal of this study was to evaluate the endothelial response to anoxia to determine whether recovery of fibrinolytic potential occurs in patients subjected to forced bedrest because of a spinal cord injury and whether this recovery is related to the incidence and/or evolution of DVT. We evaluated vascular endothelium reactivity in the lower limbs no longer submitted to the hydrostatic pressure of the erected position in 15 patients with paraplegia or tetraplegia and in 10 normal volunteers after venous occlusion produced by the application of 10 cm Hg pressure to the lower limb for 15 min comparatively to the upper limb used as reference. Among the 15 patients, 10 whose spinal cord injury had occurred 1 to 6 months earlier were still receiving prophylactic anticoagulant therapy, whereas the five other patients were not receiving prophylactic anticoagulants because the injury dated back 6 months or more. After venostasis, tissue plasminogen activator (tPA) increased significantly in both patients and controls in the upper limb (tPA levels twofold and threefold respectively in controls and patients) but showed no significant changes in the lower limb; prolonged immobility did not allow recovery in the lower limbs of a level of fibrinolytic responsiveness identical to that in the upper limbs. The plasminogen activator inhibitor (PAI1) remained unchanged after anoxia, although wide interindividual variations were seen. Natural coagulation inhibitors and circulating blood stigmates of hypercoagulability were measured. None of the patients had abnormally low levels of coagulation inhibitors (ie, antithrombin III, protein C and protein S levels were normal). Seventy-five per cent of patients (prophylactically anticoagulated or not) had very high levels of fibrin degradation products (D. Dimer levels sevenfold to eightfold those of the controls), but all patients had normal levels of thrombin-antithrombin complexes and prothrombin fragments 1 + 2. The permanence of the thrombotic process characterized by an increase in D. Dimer levels without recovery of fibrinolytic potential suggests a proposal for the patients an indefinite antithrombotic treatment at curative doses.
 ...
PMID:Endothelial fibrinolytic reactivity and the risk of deep venous thrombosis after spinal cord injury. 907 65

We report a 57-year-old woman with optic-spinal form of active multiple sclerosis, who developed a large lobar type hemorrhage of the brain. She initially suffered from left visual loss, and three month later, she was hospitalized with paraplegia and total sensory loss up to the fourth thoracic level accompanied by sphincteric disturbance. Diagnosis of clinically probable multiple sclerosis was based on the relapsing-remitting clinical course and laboratory findings. Five months after admission, she developed sudden consciousness loss. Brain CT scan showed a massive hemorrhage in the right frontal to parietal lobe. The patient had no risk factors for cerebral hemorrhage including hypertension. Histopathological study of brain tissues obtained at surgical evacuation of hematoma did not reveal any malignancy, and congo-red staining of this specimen was negative. We analyzed coagulation, fibrinolytic, and endothelial parameters during the follow-up period. von Willebrand factor (vWF) as a marker for endothelial damages was elevated persistently. Moreover, thrombin-antithrombin III complex (TAT) as a marker for activation of coagulation was also elevated constantly throughout the clinical course. The findings suggest that fragility of the vascular walls and permeability changes associated with immunological mechanisms might have resulted in the cerebral hemorrhage. Although there are few reports of cerebral hemorrhage in patients with multiple sclerosis, it has been reported that vascular wall damage is an important aspect of the pathology of multiple sclerosis and acute cerebral vascular damage may sometimes occur in multiple sclerosis. We propose that coagulation studies including the endothelial marker such as vWF would provide a useful information regarding the risk of cerebrovascular complication in multiple sclerosis.
 ...
PMID:[A case of optic-spinal form of multiple sclerosis with lobar type large cerebral hemorrhage]. 939 61

Protein Z is a vitamin-K-dependent plasma glycoprotein synthesized by the liver, showing great structural similarity to other vitamin-K-dependent coagulation factors such as factors II, IX, X and protein C and S. Protein Z seems to assist haemostasis by binding thrombin and promoting its association with phospholipid surfaces, and it downregulates coagulation by forming a complex with the plasma protein-Z-dependent protease inhibitor, which inhibits activated factor Xa. Studies in patients with a bleeding tendency of unknown origin during and after surgery displayed diminished protein-Z-concentrations in about 50 % of the patients with recurrent bleeding. We report about a 19 year old patient, who suffers from a posttraumatic paraplegia subTh 8 since childhood. In 1998 a correction operation in order to reduce scoliosis with restrictive ventilatory defects had to be stopped before successful spondylodesis because of massive bleeding. After a second intraoperative bleeding incident and exclusion of other more frequent thrombocytic or plasmatic causes of hypocoagulability protein-Z-deficiency finally was diagnosed. Under substitution of protein-Z using PPSB (Beriplex P/N) a repeatedly postponed implantation of a sphincter-externus (Brindley-) stimulator could be performed without bleeding complications in 2001, and this was also true for two other urological interventions in 2002. This report about repeated life-threatening intraoperative bleeding in a patient with protein-Z-deficiency, which could be successfully counteracted using selected plasma concentrates with guaranteed protein-Z-amounts, underlines the importance of protein-Z-assessment in some rare cases of bleeding tendency of "unknown origin" and documents the preventive plasma Protein-Z-levels achieved with the substitution of PPSB.
 ...
PMID:[Protein-z-deficiency as a rare case of perioperative bleeding]. 1297 41

Penetrating gunshot wounds (GSWs) to the abdominal aorta are frequently lethal. Alternative management options for treatment of traumatic pseudoaneurysms of the abdominal aorta are illustrated by three patient case histories. Patient A sustained two GSWs to the abdomen (midepigastrium, right subcostal region). He was hypotensive in the field. Emergent laparotomy was undertaken with suture ligature of a celiac injury and distal pancreatectomy/splenectomy for a pancreatic injury. Postoperative abdominal CT for an intraabdominal infection with leukocytosis revealed a 4 cm traumatic pseudoaneurysm of the abdominal aorta that extended from the suprarenal aorta to the level of the renal arteries. Six weeks later, he underwent an open repair. Patient B sustained multiple GSWs to his right arm and right upper quadrant. He was hemodynamically stable. He underwent abdominal exploration for a grade 3 liver laceration. Postoperative abdominal CT revealed a supraceliac abdominal aortic pseudoaneurysm. An aortogram demonstrated a 1.5 cm defect in the aortic wall above the celiac trunk communicating with the inferior vena cava (IVC). He underwent endovascular repair with covered aortic stent graft. Patient C sustained multiple thoracoabdominal GSWs. He was hemodynamically stable. Emergent laparotomy revealed multiple left colonic perforations, two duodenal lacerations, and an unsalvageable left kidney laceration. Postoperatively, he developed a duodenal-cutaneous fistula with multiple intraabdominal abscesses. Serial CT scans revealed an enlarging infrarenal aortic pseudoaneurysm. He underwent angiographic coil embolization and intraarterial injection of thrombin into the pseudoaneurysm sac. The average time from injury to surgical treatment was 46 days (range 29-67). Postoperatively, none of the patients developed paraplegia. Advances in endovascular techniques have provided options to deal with traumatic pseudoaneurysms of the abdominal aorta. In a hemodynamically stable patient with a traumatic pseudoaneurysm, careful selection of a specific intervention can be tailored to the clinical scenario electively.
 ...
PMID:Treatment options for traumatic pseudoaneurysms of the paravisceral abdominal aorta. 1601 May 2

The patient was a 36-year-old man who had been aware of a mild bleeding tendency since childhood, but did not show any history of severe bleeding. After lumbar epidural block was performed for pain caused by lumbar disc herniation, the patient developed paraplegia due to an acute epidural hematoma, and rectovesical disorder. He was admitted to our hospital, and wide fenestration and hematoma evacuation were performed. Because of persistent bleeding, reoperation was performed to achieve hemostasis. Although factor VIII antigen was 138%, its activity was decreased to 18% of normal. A diagnosis of cross-reacting material positive mild hemophilia A was made. Postoperative injection of a factor VIII preparation resulted in complete hemostasis. The activated partial thromboplastin time was within normal range. A thrombin generation test showed reduced endogenous thrombin potential, peak thrombin levels, and prolonged time-to-peak levels. The thrombin generation test, which allows comprehensive assessment of the coagulation profile, was useful for diagnosis and treatment of this case.
 ...
PMID:[Mild hemophilia A diagnosed at the onset of acute epidural hematoma after lumbar epidural block]. 2140 28