1/7. [Perioperative management of a patient with purpura fulminans syndrome due to protein c deficiency]PURPOSE: Protein C is a vitamin k-dependent anticoagulant and homozygous protein C deficiency is a rare fatal thrombotic disease. This report describes the perioperative management of homozygous protein c deficiency in a patient who underwent a total of three surgical procedures under general anesthesia and the successful use of activated protein C concentrate. CLINICAL FEATURES: A female baby, who developed disseminated intravascular coagulation and purpura fulminans shortly after birth, was diagnosed as purpura fulminans syndrome due to homozygous protein c deficiency. At one month of age, she suffered bilateral retinal detachment and glaucoma due to retinal hemorrhage. After marked improvement of her condition after administration of activated protein C concentrate, she underwent a left iridectomy and implantation of a Broviak catheter under general anesthesia. Her intraoperative course was uncomplicated but, on postoperative day four, she presented another episode of massive cutaneous necrosis and gangrene. Activated protein C concentrate was administered again, with good results. She underwent replacement of a Broviak catheter at four months of age, and right iridectomy for glaucoma at eight months. Both were uneventful. CONCLUSION: The perioperative management of homozygous protein c deficiency and purpura fulminans requires appropriate measures for thromboembolic prophylaxis. Sufficient iv fluid administration is necessary. attention should be paid to decrease the risk of tissue compression such as that associated with positioning, blood pressure cuff, and endotracheal intubation, which may cause necrosis over pressure points. Replacement therapy with activated protein C concentrate appears safe and effective. The anesthetic management is reviewed and discussed.- - - - - - - - - - ranking = 1keywords = catheter (Clic here for more details about this article) |
2/7. Renal allograft loss due to proximal extension of ileofemoral deep venous thrombosis.Renal allograft recipients with thrombophilic (hypercoagulable) states are at higher risk for early allograft loss. Presumably, the combination of endothelial injury at surgery and thrombophilia predisposes to arterial or venous thrombosis. Of 270 consecutive renal transplants at our center one allograft failed secondary to renovascular thrombosis. At exploration the iliac and renal veins were thrombosed. thrombectomy and re-implantation were attempted, but unsuccessful. Also noted at surgery was extensive clot in the femoral vein that could not be removed by embolectomy catheters. Post-operatively, a Doppler ultrasound confirmed the presence of extensive deep venous thrombosis (DVT) in the femoral and popliteal veins. The adherent nature of this clot, the extent of clot found less than 12 h after renal transplantation and the absence of leg edema suggested that the DVT existed prior to surgery. This case demonstrates that a pre-existing, asymptomatic DVT can precipitate allograft thrombosis and highlights the importance of diagnosing thrombophilia in patients undergoing renal transplantation. Current practices in our unit have evolved to include screening for thrombophilia in all patients with a suggestive history. As thrombophilic states are increasingly appreciated in the end-stage renal disease population, effective management of these patients while on hemodialysis and at the time of renal transplantation presents an ongoing challenge.- - - - - - - - - - ranking = 0.5keywords = catheter (Clic here for more details about this article) |
3/7. 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 = 0.5keywords = catheter (Clic here for more details about this article) |
4/7. Subcutaneous administration of protein C concentrate.Children born with severe homozygous protein c deficiency do not survive beyond the neonatal period unless they receive protein C replacement. Protein C concentrate (Baxter Biosciences, Vienna) is usually given intravenously via a central venous catheter The authors describe 2 children in whom protein C concentrate was successfully administered by subcutaneous infusion after they had had recurrent central line infections. Alternate daily doses of 250-350 IU/kg resulted in trough protein C levels of > 25 IU/dL. In the follow-up period of 1-2 years neither child has had a thrombotic episode or purpuric skin lesions, and infusions are managed in the home by their parents.- - - - - - - - - - ranking = 0.5keywords = catheter (Clic here for more details about this article) |
5/7. Pulmonary thromboendarterectomy for chronic pulmonary thromboembolism in protein C deficiency.Pulmonary thromboendarterectomy was performed on a patient with chronic pulmonary thromboembolism showing thrombophilia. The patient was a 56-year-old female with the above condition complicated by congenital protein c deficiency. She was admitted to our hospital with severe dyspnea accompanied by right ventricular failure. A pulmonary arteriogram showed occlusion and stenosis from lobar to segmental arteries. cardiac catheterization showed marked pulmonary hypertension. A lung perfusion scintigram revealed multiple defects in the right and left lungs. After the insertion of an inferior vena cava filter, she was operated on. Following a median sternotomy, thromboendarterectomy of the bilateral pulmonary arteries was performed using deep hypothermia and intermittent circulatory arrest. Circulatory arrest was employed in three periods totaling up to 36 minutes. After surgery, she had improvements in pulmonary hypertension and pulmonary vascular resistance. She maintained improved lung functions, and remained in the new york heart association functional class I for more than two years and eight months after surgery.- - - - - - - - - - ranking = 0.5keywords = catheter (Clic here for more details about this article) |
6/7. Homozygous protein c deficiency: early treatment with warfarin.We present a case of homozygous protein c deficiency with neonatal purpura fulminans and disseminated intravascular coagulopathy (DIC) starting shortly after birth. In addition, the infant had vitreal eye hemorrhages and intraparenchymal brain infarction, apparently as intrauterine events. Within 15 hours of institution of fresh frozen plasma (FFP) infusions the DIC resolved and the progression of purpura fulminans reversed. warfarin (0.4 mg/kg/day) was started on the fifth day of life, followed by gradual tapering of the FFP infusions. There were no recurrences of purpura, areas of skin necrosis healed without the need for skin grafting, and the areas of brain infarction resolved without apparent sequelae. The eye and brain lesions may be intrauterine events and appear to be a regular feature of this syndrome. family studies are essential to establish the diagnosis, although there may be no family history of thromboembolic events, as in this case. Homozygous protein c deficiency is a rare disorder, but one in which early recognition and intervention may be lifesaving. Ours is the youngest patient yet reported to be treated with warfarin anticoagulation. We were thus able to avoid the complications of long-term plasma therapy as well as the potential thrombotic complications of central venous catheter placement.- - - - - - - - - - ranking = 0.5keywords = catheter (Clic here for more details about this article) |
7/7. Combined factor vii and protein c deficiency found in a patient with peripheral pulmonary artery stenosis accompanied by progressive pulmonary hypertension and hemoptysis.A congenital deficiency of factor vii and protein C was found in a 21-year-old female suffering from recurrent and progressive attacks of dyspnea and hemoptysis over the last four years. She has been followed in our Department since the age of 17 under a diagnosis of peripheral pulmonary artery stenosis and pulmonary hypertension as confirmed by cardiac catheterization and angiography. Prolonged prothrombin time repeatedly examined during this time period prompted us to perform detailed coagulation studies. We found that factor vii and protein C were both half normal in activity as well as in antigen. Three other members of her immediate family were also found to be affected with this combined deficiency. Since the genes encoding factor vii and protein C are located in different chromosomes, the 13th and the second chromosomes, respectively, expression of the combined hereditary deficiency is a random and very rare association on the basis of frequencies of 1:50,000 for factor vii and 1:16,000 for protein C deficiencies.- - - - - - - - - - ranking = 0.5keywords = catheter (Clic here for more details about this article) |