1/5. Are the strokes in moyamoya syndrome associated with down syndrome due to protein c deficiency?Moyamoya syndrome has occasionally been seen in association with down syndrome. We report a child with moyamoya syndrome and down syndrome who was admitted with repeated episodes of strokes; his magnetic resonance imaging and magnetic resonance angiography findings confirmed the presence of occlusive cerebrovascular disease with basal collateral vessels. His protein C levels were significantly decreased during the stroke. Complete clinical recovery was seen during follow-up. This raises the possibility of a link between protein c deficiency and down syndrome with moyamoya syndrome.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/5. Protein C survival during replacement therapy in homozygous protein c deficiency.Homozygous protein C (PC) deficiency is a rare genetic defect that usually results in fatal thrombotic complications (purpura fulminans and DIC), but it can be successfully managed with oral anticoagulants or PC replacement. The successful use of PC replacement for two individuals is described. The activity and antigen levels of PC in fresh frozen plasma (FFP) and prothrombin complex concentrate (PCC) are also reported. The concentration of PC in FFP is 87 /- 15 units/dl. PC is present in all PCC analyzed; however, a ten-fold difference between the various brands and/or lots is noted. The PC activity and antigen correlates well with no significant levels of APC. Upon infusion of FFP into two homozygous PC-deficient children, the PC levels obtained were less than or equal to 30 units/dl post-infusion and undetectable after 12-18 hr. With infusions of PCC, plasma levels of PC obtained were 100-145 units/dl and less than 10 units/dl after 48 hr. The percent recovery and half-lives of PC from FFP and PCC were 49.8% and 7.8 hr, and 84% and 7.4 hr, respectively. One infant was treated every 48 hr for 2 years without significant purpura fulminans or DIC complications. The levels of the other PC system components did not change during the infusion of the PC-rich material. Based on this information, a specific replacement protocol has been developed using a PC-rich concentrate. However, several problems may arise with the "less pure" PC-rich concentrates: catheter-tip thrombosis, related large vessel thrombosis and blood-transmitted diseases. With a specific PC concentrate, replacement therapy is a viable alternative for the long-term management/treatment of homozygous PC deficiency.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/5. An adult case of moyamoya syndrome that developed dural sinus thrombosis associated with protein c deficiency: case report and literature review.We describe a 54-year-old woman exhibiting MMS who developed delayed dural sinus thrombosis associated with PCD. Angiographic findings of the patient were so unusual that bilateral internal carotid arteries were occluded between their origin and the carotid fork with extensive development of collateral circulation via the external carotid arteries and the posterior cerebral arteries instead of moyamoya vessels at the base of the brain. Seven years after bilateral cerebral revascularization surgery, intracerebral hemorrhage occurred caused by dural sinus thrombosis. In the treatment for the patient with MMS associated with PCD, risk of sinus thrombosis should be taken into account.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/5. cardiopulmonary bypass for a coronary artery bypass graft patient with heterozygous protein c deficiency and protein s deficiency.cardiopulmonary bypass (CPB) poses great risks for hypercoagulable patients and requires management techniques to ensure an optimal outcome free from thrombotic events. This case report reviews perfusion management techniques that may contribute to a safer CPB experience for a patient deficient in both protein C and protein S. A patient with heterozygous protein c deficiency is at increased risk of thrombosis, especially in the venous circulation. Since it is an essential cofactor for activated protein C, deficiency of free protein S is also linked to a hypercoagulable condition. A 52-year-old male presented to our institution with a past medical history of hypercoagulable state, multiple deep vein thromboses, pulmonary embolisms, and stroke. He was scheduled for two-vessel coronary artery bypass graft surgery to be followed by right carotid endarterectomy (RCEA) before discharge. The anesthesia and perfusion teams worked closely together to ensure that fresh frozen plasma (FFP) was given intraoperatively at appropriate times. heparin dose response and protamine dosage was determined with hemostasis management system (HMS) analysis. The closed CPB circuit and cannulae were Carmeda bonded. Rapid autologous priming, along with the use of a hemoconcentrator, kept the hematocrit above 21 during CPB. Zero-balance ultrafiltration and leukocyte depletion were initiated during rewarming to aid in attenuation of the inflammatory response. To conserve coagulation factors, all pump blood was ultrafiltrated post-CPB and returned to the patient. Laboratory samples drawn on postoperative day (POD) one measured normal protein C activity with subnormal protein S activity. On POD six, the patient underwent RCEA and he was discharged on POD eight without complications.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/5. Functional deficiency of protein C associated with mesenteric venous thrombosis and splenic infarction.A 16-year-old girl had abdominal pain, hematemesis, and fever. Radiographic evaluation showed her to have an infarcted spleen and jejunum. Pathologic description revealed thrombi involving the vessels of both organs. A hypercoagulation evaluation of the patient and her family resulted in the diagnosis of hereditary protein C activity deficiency. She is unusual both in the diagnosis of protein C activity deficiency and in her appearance with mesenteric and splenic venous thrombosis. This appearance has not been previously reported in the literature and should be considered in the differential diagnosis of any thrombotic event, even in the face of a negative family history.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |