{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T18:13:10Z","timestamp":1789582390134,"version":"build-2803163510"},"reference-count":36,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2006,7,11]],"date-time":"2006-07-11T00:00:00Z","timestamp":1152576000000},"content-version":"vor","delay-in-days":5061,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[1992,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>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 \u00b1 15 units\/dl. PC is present in all PCC analyzed; however, a ten\u2010fold 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\u2010deficient children, the PC levels obtained were \u226630 units\/dl post\u2010 infusion and undetectable after 12\u201018 hr. With infusions of PCC, plasma levels of PC obtained were 100\u2010145 units\/dl and &lt;10 units\/dl after 48 hr. The percent recovery and half\u2010lives 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\u2010rich material. Based on this information, a specific replacement protocol has been developed using a PC\u2010rich concentrate. However, several problems may arise with the \u201eless pure\u201d\ufe01 PC\u2010rich concentrates: catheter\u2010tip thrombosis, related large vessel thrombosis and blood\u2010transmitted diseases. With a specific PC concentrate, replacement therapy is a viable alternative for the long\u2010term management\/treatment of homozygous PC deficiency. \u00a9 1992 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajh.2830410106","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T12:58:10Z","timestamp":1172062690000},"page":"24-31","source":"Crossref","is-referenced-by-count":19,"title":["Protein C survival during replacement therapy in homozygous protein C deficiency"],"prefix":"10.1002","volume":"41","author":[{"given":"Richard A.","family":"Marlar","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert R.","family":"Montgomery","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Renee M.","family":"Madden","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard 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