Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C1832526 (PCC)
 5,967 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hungary's health service (10 million pop.) needs about 2000 kg of albumin, 10 million units of Factor VIII 1-1,5 million units of Factor IX, and 3 million micrograms of anti D IgG a year. The plasma for these products is collected from volunteer blood donors and by plasmapheresis. 90% of the albumin is produced by the Cohn method. To study the efficiency of a new chromatographic plasma fractionation method (3), we installed an integrated system for the production of factor VIII, PCC, albumin and intravenous IgG. About 10% of the total albumin is produced using this system. The paper discusses the results of 3 years of experimentation with the chromatographic system. The problems of the method are also analysed in detail. PCC is now in clinical use, the albumin is under clinical trial and the clinical trial of the i.v. IgG will start soon.
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PMID:Plasma fractionation in blood transfusion service. 311 12

Acquired inhibitors of coagulation factor interaction in nonhemophilic children are usually nonspecific, transient, and unassociated with clinical bleeding. They occur with some frequency and are the most common cause for a prolonged APTT found by routine testing. In children, some association with viral infections and treatment with penicillin has been noted, but their interrelationship with the development of antibodies remains unclear. The exact nature of these antibodies, usually directed against coagulant factor phospholipid, is not clear and multiple antibodies both specific as well as nonspecific may occur. No therapy is generally required. Rarely has the acute development of antibodies directed against specific coagulation factors occurred. The laboratory evaluation of the type of inhibitor is, therefore, most important as specific inhibitors may be associated with life-threatening bleeding situations. Their therapy should probably include attempts at eradication of the inhibitor by immunosuppressive agents or other newer modalities. Unfortunately, little information is available regarding the nature and outcome of specific inhibitors in children. Acquired inhibitors in hemophilic patients occur in about 6 to 10% of patients. Newer approaches to their therapy include activated PCC which have generally improved the outlook for such patients. Treatment regimens involve a knowledge of inhibitor response and the concomitant use of plasmapheresis, high-dose or continuous i.v. Factor VIII, and porcine Factor VIII, followed by activated PCC. The role of immunosuppressive agents and other newer modalities appears promising, however, prospective controlled studies are necessary to evaluate their role in the overall management of such patients.
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PMID:Inhibitors of coagulation in children. 330 77

During a 4-year national cooperative study of factor VIII inhibitors in patients with classic hemophilia, new inhibitors were identified in 31 of 1,306 patients without this finding on entry. The age of patients upon detection of an inhibitor ranged from 2-62 years with a median age of 16 years. The incidence of new inhibitors was 8 per 1000 patient-years of observation. In 29 patients baseline VIII:C was less than or equal to 0.03 units/ml; the other two patients had levels of 0.06 and 0.07 units/ml. Factor VIII:CAg was measured in entry samples of plasma from 27 subjects and generally corresponded to levels of VIII:C; the levels of VIII:CAg ranged from 0.01-0.11 units/ml in 9 cases and were less than 0.01 units/ml in the remaining 18. In no instance did an inhibitor develop without preceding exposure to infused VIII:C, appearing within 8-250 VIII:C exposure-days in all patients and within 75 exposure-days in 10 of 11 patients with maximum inhibitor values greater than 15 Bethesda units/ml. Development of an inhibitor could not be correlated with any of the following variables: bleeding tendency, intercurrent illness, drugs, and selected clinical laboratory tests including blood counts, liver enzymes, and immunoglobulins. On the basis of maximum activity and persistence of inhibitors, a spectrum of patterns could be identified. (1) In Group IA with 9 patients, inhibitors with maximum values greater than 15 Bethesda units/ml persisted throughout the remaining study period. (2) In Group IB with 2 patients with mild classic hemophilia, inhibitors with maximum values greater than 15 Bethesda units/ml disappeared despite varying continued exposure to VIII:C and PCC. (3) In Group IIA with 10 patients, inhibitors with maximum values less than or equal to 15 Bethesda units/ml persisted throughout the remaining study period. (4) In Group IIB with 3 patients, maximum values less than or equal to 15 Bethesda units/ml disappeared within 18 months despite varying continued exposure to VIII:C and PCC. (5) In Group III with 7 patients, an inhibitor with a value of less than 5 Bethesda units/ml was conclusively documented on a sing study period. (4) In Group IIB with 3 patients, maximum values less than or equal to 15 Bethesda units/ml disappeared within 18 months despite varying continued exposure to VIII:C and PCC. (5) In Group III with 7 patients, an inhibitor with a value of less than 5 Bethesda units/ml was conclusively documented on a sing study period.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Clinical patterns of hemophilic patients who develop inhibitors. 643 36