{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,31]],"date-time":"2025-10-31T19:21:16Z","timestamp":1761938476358,"version":"build-2065373602"},"reference-count":45,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[1996,1,1]],"date-time":"1996-01-01T00:00:00Z","timestamp":820454400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[1996,1,1]],"date-time":"1996-01-01T00:00:00Z","timestamp":820454400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[1996,1,1]],"date-time":"1996-01-01T00:00:00Z","timestamp":820454400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[1996,1,1]],"date-time":"1996-01-01T00:00:00Z","timestamp":820454400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Eur J Pediatr"],"published-print":{"date-parts":[[1996,1]]},"DOI":"10.1007\/bf02115621","type":"journal-article","created":{"date-parts":[[2005,9,15]],"date-time":"2005-09-15T00:49:30Z","timestamp":1126745370000},"page":"20-25","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":28,"title":["Purpura fulminans in severe congenital protein C deficiency: Monitoring of treatment with protein C concentrate"],"prefix":"10.1007","volume":"155","author":[{"given":"F. -M.","family":"M\u00fcller","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"W.","family":"Ehrenthal","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G.","family":"Hafner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D.","family":"Schranz","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","reference":[{"key":"BF02115621_CR1","doi-asserted-by":"crossref","first-page":"1998","DOI":"10.1182\/blood.V80.8.1998.1998","volume":"80","author":"M Andrew","year":"1992","unstructured":"Andrew M, Vegh P, Johnston M, Bowker J, Ofosu F, Mitchell L (1992) Maturation of the hemostatic system during childhood. Blood 80: 1998\u20132005","journal-title":"Blood"},{"key":"BF02115621_CR2","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/BF01697402","volume":"64","author":"K Auberger","year":"1992","unstructured":"Auberger K (1992) Evaluation of a new protein-C concentrate and comparison of protein-C assays in a child with congenital protein-C deficiency. Ann Hematol 64: 1\u20136","journal-title":"Ann Hematol"},{"issue":"6","key":"BF02115621_CR3","doi-asserted-by":"crossref","first-page":"340","DOI":"10.1056\/NEJM198308113090604","volume":"309","author":"AW Broekmans","year":"1983","unstructured":"Broekmans AW, Veltkamp JJ, Bertina RM (1983) Congenital protein C deficiency and venous thromboembolism. N Engl J Med 309 (6): 340\u2013344","journal-title":"N Engl J Med"},{"key":"BF02115621_CR4","doi-asserted-by":"crossref","first-page":"435","DOI":"10.1016\/S0140-6736(88)91231-7","volume":"I","author":"JF Casella","year":"1988","unstructured":"Casella JF, Lewis JH, Bontempo FA, et al (1988) Successful treatment of homozygous protein C deficiency by hepatic transplantation. Lancet I: 435\u2013438","journal-title":"Lancet"},{"issue":"20","key":"BF02115621_CR5","doi-asserted-by":"crossref","first-page":"1298","DOI":"10.1056\/NEJM198605153142006","volume":"314","author":"LH Clouse","year":"1986","unstructured":"Clouse LH, Comp PC (1986) The regulation of hemostasis: the protein C system. N Engl J Med 314 (20): 1298\u20131304","journal-title":"N Engl J Med"},{"issue":"4","key":"BF02115621_CR6","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1055\/s-2007-1002682","volume":"16","author":"PC Comp","year":"1990","unstructured":"Comp PC, Elrod JP, Karzenski S (1990) Warfarin-induced skin necrosis. Semin Thromb Hemost 16 (4): 293\u2013298","journal-title":"Semin Thromb Hemost"},{"key":"BF02115621_CR7","doi-asserted-by":"crossref","first-page":"1221","DOI":"10.1172\/JCI110368","volume":"68","author":"PC Comp","year":"1981","unstructured":"Comp PC, Esmon CT (1981) Generation of fibrinolytic activity by infusion of activated protein C into dogs. J Clin Invest 68: 1221\u20131228","journal-title":"J Clin Invest"},{"key":"BF02115621_CR8","doi-asserted-by":"crossref","first-page":"1159","DOI":"10.1182\/blood.V82.4.1159.1159","volume":"82","author":"J Conard","year":"1993","unstructured":"Conard J, Bauer K, Gruber A, Griffin JH, Schwarz HP, Horellou MH, Samama MM, Rosenberg RD (1993) Normalization of markers of coagulation activation with a purified protein C concentrate in adults with homozygous protein C deficiency. Blood 82: 1159\u20131164","journal-title":"Blood"},{"key":"BF02115621_CR9","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1097\/00001721-199304010-00013","volume":"4","author":"CE Dempfle","year":"1993","unstructured":"Dempfle CE, Dollman M, Lill H, Puzzovio D, Dessauer A (1993) Binding of a new monoclonal antibody against N-terminal heptapeptide of fibrin polymerization site \u201cA\u201d: effects of fibrinogen and fibrinogen derivatives, and pretreatment of samples with NaSCN. Blood Coagul Fibrinolysis 4: 79\u201386","journal-title":"Blood Coagul Fibrinolysis"},{"key":"BF02115621_CR10","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1055\/s-0038-1649478","volume":"70","author":"V De Stefano","year":"1993","unstructured":"De Stefano V, Mastrangelo S, Schwarz HP, Pola P, Flore R, Bizzi B, Leone G (1993) Replacement therapy with a purified protein C concentrate during initiation of oral anticoagulation in severe protein C congenital deficiency. Thromb Haemost 70: 247\u2013249","journal-title":"Thromb Haemost"},{"key":"BF02115621_CR11","doi-asserted-by":"crossref","first-page":"1565","DOI":"10.1056\/NEJM199111283252207","volume":"325","author":"M Dreyfus","year":"1991","unstructured":"Dreyfus M, Magny JF, Bridey F et al (1991) Treatment of homozygous protein C deficiency and neonatal purpura fulminans with a purified protein C concentrate. N Engl J Med 325: 1565\u20131568","journal-title":"N Engl J Med"},{"key":"BF02115621_CR12","first-page":"577","volume":"30","author":"R Egbring","year":"1989","unstructured":"Egbring R, Seitz R (1989) Angeborene H\u00e4mostasest\u00f6rungen mit Thromboseneigung. Internist 30: 577\u2013586","journal-title":"Internist"},{"key":"BF02115621_CR13","doi-asserted-by":"crossref","first-page":"4743","DOI":"10.1016\/S0021-9258(18)83649-3","volume":"264","author":"CT Esmon","year":"1989","unstructured":"Esmon CT (1989) The roles of protein and thrombomodulin in the regulation of blood coagulation. J Biol Chem 264: 4743\u20134746","journal-title":"J Biol Chem"},{"key":"BF02115621_CR14","doi-asserted-by":"crossref","first-page":"538","DOI":"10.1097\/00007611-199005000-00013","volume":"83","author":"CE Fiore","year":"1990","unstructured":"Fiore CE, Tamburino C, Foti R, Grimaldi D (1990) Reduced axial bone mineral content in patients taking an oral anticoagulant. South Med J 83: 538\u2013542","journal-title":"South Med J"},{"key":"BF02115621_CR15","doi-asserted-by":"crossref","first-page":"634","DOI":"10.1016\/S0140-6736(85)92167-1","volume":"I","author":"I Garcia-Plaza","year":"1985","unstructured":"Garcia-Plaza I, Jimencz-Astorga C, Borrego D, et al (1985) Coumarin prophylaxis for fulminant purpura syndrome due to homozygous protein C deficiency. Lancet I: 634\u2013635","journal-title":"Lancet"},{"key":"BF02115621_CR16","doi-asserted-by":"crossref","first-page":"418","DOI":"10.1542\/peds.91.2.418","volume":"91","author":"WT Gerson","year":"1993","unstructured":"Gerson WT, Dickerman JD, Bovill EG, Golden E (1993) Severe acquired protein C deficiency in purpura fulminans associated with disseminated intravascular coagulation: Treatment with Protein C Concentrate. Pediatrics 91: 418\u2013422","journal-title":"Pediatrics"},{"issue":"4","key":"BF02115621_CR17","first-page":"395","volume":"11","author":"KR Hartmann","year":"1989","unstructured":"Hartmann KR, Manco-Johnson M, Rawlings JS, et al (1989) Homozygous protein C deficiency: early treatment with warfarin. Am J Pediatr Hematol Oncol 11 (4): 395\u2013401","journal-title":"Am J Pediatr Hematol Oncol"},{"issue":"6","key":"BF02115621_CR18","doi-asserted-by":"crossref","first-page":"934","DOI":"10.1016\/S0022-3476(05)80346-4","volume":"121","author":"IN Horowitz","year":"1992","unstructured":"Horowitz IN, Galuis AG, Gomperts ED (1992) Arterial thrombosis and protein S deficiency. J Pediatr 121 (6): 934\u2013937","journal-title":"J Pediatr"},{"key":"BF02115621_CR19","doi-asserted-by":"crossref","first-page":"464","DOI":"10.1055\/s-0038-1647516","volume":"59","author":"AR Hubbard","year":"1988","unstructured":"Hubbard AR (1988) Standardization of protein C in plasma: establishment of an international standard. Thromb Haemost 59: 464\u2013467","journal-title":"Thromb Haemost"},{"issue":"3","key":"BF02115621_CR20","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1097\/00001721-199106000-00001","volume":"2","author":"DW Jones","year":"1991","unstructured":"Jones DW, Mackie IJ, Winter M, et al (1991) Detection of protein C deficiency during oral anticoagulant therapy\u2014use of the protein C: factor VII ratio. Blood Coagul Fibrinolysis 2 (3): 407\u2013411","journal-title":"Blood Coagul Fibrinolysis"},{"key":"BF02115621_CR21","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1007\/BF01709832","volume":"18","author":"F Leclerc","year":"1992","unstructured":"Leclerc F, Hazelzet J, Jude B, et al (1992) Protein C and S deficiency in severe infectious purpura of children: a collaborative study of 40 cases. Intensive Care Med 18: 202\u2013205","journal-title":"Intensive Care Med"},{"key":"BF02115621_CR22","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1016\/0140-6736(90)90201-F","volume":"335","author":"C Mahasandana","year":"1990","unstructured":"Mahasandana C, Suvatte V, Marlar RA, et al (1990) Neonatal purpura fulminans associated with homozygous protein S deficiency. Lancet 335: 61\u201362","journal-title":"Lancet"},{"key":"BF02115621_CR23","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1002\/ajh.2830400116","volume":"40","author":"MJ Manco-Johnson","year":"1992","unstructured":"Manco-Johnson MJ, Nuss R (1992) Protein C concentrate prevents peripartum thrombosis. Am J Hematol 40: 69\u201370","journal-title":"Am J Hematol"},{"key":"BF02115621_CR24","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1016\/S0022-3476(88)80284-1","volume":"113","author":"MJ Manco-Johnson","year":"1988","unstructured":"Manco-Johnson MJ, Marlar RA, Jacobson LJ, et al (1988) Severe protein C deficiency in newborn infants. J Pediatr 113: 359\u2013363","journal-title":"J Pediatr"},{"issue":"3","key":"BF02115621_CR25","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1097\/00001721-199008000-00010","volume":"1","author":"RA Marlar","year":"1990","unstructured":"Marlar RA, Mastovich S (1990) Hereditary protein C deficiency: a review of the genetics, clinical presentation, diagnosis and treatment. Blood Coagul Fibrinolysis 1 (3): 319\u2013330","journal-title":"Blood Coagul Fibrinolysis"},{"issue":"4","key":"BF02115621_CR26","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1055\/s-2007-1002683","volume":"16","author":"RA Marlar","year":"1990","unstructured":"Marlar RA, Neumann A (1990) Neonatal purpura fulminans due to homozygous protein C or protein S deficiencies. Semin Thromb Hemost 16 (4): 299\u2013304","journal-title":"Semin Thromb Hemost"},{"issue":"3","key":"BF02115621_CR27","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1055\/s-0038-1646629","volume":"61","author":"RA Marlar","year":"1989","unstructured":"Marlar RA, Montgomery RR, Broekmans AW (1989) Report on the diagnosis and treatment of homozygous protein C deficiency. Thromb Haemost 61 (3): 529\u2013531","journal-title":"Thromb Haemost"},{"key":"BF02115621_CR28","doi-asserted-by":"crossref","first-page":"528","DOI":"10.1016\/S0022-3476(89)80688-2","volume":"114","author":"RA Marlar","year":"1989","unstructured":"Marlar RA, Montgomery RR, Brockmans AW (1989) Diagnosis and treatment of homozygous protein C deficiency. J Pediatr 114: 528\u2013534","journal-title":"J Pediatr"},{"issue":"2","key":"BF02115621_CR29","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1055\/s-2007-1002665","volume":"16","author":"JP Miletich","year":"1990","unstructured":"Miletich JP (1990) Laboratory diagnosis of protein C deficiency. Semin Thromb Hemost 16 (2): 169\u2013176","journal-title":"Semin Thromb Hemost"},{"issue":"6","key":"BF02115621_CR30","doi-asserted-by":"crossref","first-page":"1704","DOI":"10.1182\/blood.V69.6.1704.1704","volume":"69","author":"J Mimuro","year":"1987","unstructured":"Mimuro J, Sakata Y, Wakabayashi K, et al (1987) Level of protein C determined by combined assays during disseminated intravascular coagulation and oral anticoagulation. Blood 69 (6): 1704\u20131711","journal-title":"Blood"},{"issue":"5","key":"BF02115621_CR31","doi-asserted-by":"crossref","first-page":"843","DOI":"10.1016\/S0022-3476(86)80708-9","volume":"109","author":"M Nardi","year":"1986","unstructured":"Nardi M, Karpatkin M (1986) Prothrombin and protein C in early childhood: Normal adult levels are not achieved unitl the fourth year of life. J Pediatr 109 (5): 843\u2013845","journal-title":"J Pediatr"},{"key":"BF02115621_CR32","doi-asserted-by":"crossref","first-page":"960","DOI":"10.1136\/adc.67.7.960","volume":"67","author":"J O'Sullivan","year":"1992","unstructured":"O'Sullivan J, Chatuverdi R, Bennett MK, Hunter S (1992) Protein S deficiency: early presentation and pulmonary hypertension. Arch Dis Child 67: 960\u2013961","journal-title":"Arch Dis Child"},{"issue":"3","key":"BF02115621_CR33","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1055\/s-0038-1645056","volume":"63","author":"I Pabinger","year":"1990","unstructured":"Pabinger I, Kyrle PA, Speiser W (1990) Diagnosis of protein C deficiency in patients on oral anticoagulant treatment: comparison of three different functional protein C assays. Thromb Haemost 63 (3): 407\u2013412","journal-title":"Thromb Haemost"},{"key":"BF02115621_CR34","doi-asserted-by":"crossref","first-page":"705","DOI":"10.1016\/0049-3848(87)90110-1","volume":"47","author":"I Pabinger-Fasching","year":"1987","unstructured":"Pabinger-Fasching I, Lechner K, Niessner H, et al (1987) Protein C and coagulation factor levels during the initial phase of oral anticoagulant therapy (low dose regimen) in a patient with heterozygous protein C deficiency. Thromb Res 47: 705\u2013708","journal-title":"Thromb Res"},{"key":"BF02115621_CR35","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1055\/s-0038-1646768","volume":"59","author":"H Pelzer","year":"1988","unstructured":"Pelzer H, Schwarz A, Heimburger N (1988) Determination of human thrombin-antithrombin III complex in plasma with an enzyme-linked immunosorbent assay. Thromb Haemost 59: 101\u2013106","journal-title":"Thromb Haemost"},{"key":"BF02115621_CR36","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1055\/s-0038-1647475","volume":"65","author":"H Pelzer","year":"1991","unstructured":"Pelzer H, Schwarz A, St\u00fcber W (1991) Determination of human prothrombin activation fragment 1+2 in plasma with an antibody against a synthetic peptide. Thromb Haemost 65: 153\u2013159","journal-title":"Thromb Haemost"},{"issue":"2","key":"BF02115621_CR37","doi-asserted-by":"crossref","first-page":"165","DOI":"10.3109\/08880019009033387","volume":"7","author":"P Petrini","year":"1990","unstructured":"Petrini P, Segnestam K, Ekelund H, Egberg N (1990) Homozygous protein C deficiency in two siblings. Pediatr Hematol Oncol 7 (2): 165\u2013175","journal-title":"Pediatr Hematol Oncol"},{"issue":"4","key":"BF02115621_CR38","doi-asserted-by":"crossref","first-page":"890","DOI":"10.1182\/blood.V78.4.890.890","volume":"78","author":"PH Reitsma","year":"1991","unstructured":"Reitsma PH, Poort SR, Allaart CF, et al (1991) The spectrum of genetic defects in a panel of 40 dutch families with symptomatic protein C deficiency type, I: heterogeneity and founder effects. Blood 78 (4): 890\u2013894","journal-title":"Blood"},{"issue":"1","key":"BF02115621_CR39","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1111\/j.1365-2257.1989.tb00180.x","volume":"11","author":"CR Sanders","year":"1989","unstructured":"Sanders CR, Majer RV, Green PJ (1989) Protein C deficiency: spontaneous healing of necrotic rash on warfarin (letter). Clin Lab Haematol 11 (1): 74\u201375","journal-title":"Clin Lab Haematol"},{"key":"BF02115621_CR40","doi-asserted-by":"crossref","first-page":"226","DOI":"10.1111\/j.1651-2227.1985.tb10954.x","volume":"74","author":"F Schettini","year":"1985","unstructured":"Schettini F, Mattia D de, Altomare M, et al (1985) Post-natal development of protein C in full-term newborns. Acta Paediatr Scand 74: 226\u2013229","journal-title":"Acta Paediatr Scand"},{"key":"BF02115621_CR41","doi-asserted-by":"crossref","first-page":"753","DOI":"10.1001\/archderm.1993.01680270091012","volume":"129","author":"W Schramm","year":"1993","unstructured":"Schramm W, Spannagl M, Bauer KA, Rosenberg R, Birkner B, Linnau Y, Schwarz HP (1993) Treatment of coumarin-induced skin necrosis with a monoclonal antibody purified protein C concentrate. Arch Dermatol 129: 753\u2013756","journal-title":"Arch Dermatol"},{"issue":"9","key":"BF02115621_CR42","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1056\/NEJM198403013100904","volume":"310","author":"U Seligsohn","year":"1984","unstructured":"Seligsohn U, Berger A, Abend M, et al (1984) Homozygous protein C deficiency manifested by massive venous thrombosis in the newborn. N Engl J Med 310 (9): 559\u2013562","journal-title":"N Engl J Med"},{"key":"BF02115621_CR43","first-page":"51","volume":"12","author":"KE St\u00f6rtzer","year":"1988","unstructured":"St\u00f6rtzer KE, Amiral J, Spannuth E (1988) Neue Methoden zur spezifischen Bestimmung von Fibrinspaltprodukten (D-Dimere). Lab Med 12: 51\u201359","journal-title":"Lab Med"},{"key":"BF02115621_CR44","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1055\/s-2008-1033940","volume":"197","author":"H Wehinger","year":"1985","unstructured":"Wehinger H, Geiger E, Freudenberg V, et al (1985) Schwerer heredit\u00e4rer Protein-C-Mangel bei einem Neugeborenen mit Purpura fulminans\u2014erfolgreiche Behandlung mit Phenprocoumon. Klin P\u00e4diatr 197: 116\u2013120","journal-title":"Klin P\u00e4diatr"},{"key":"BF02115621_CR45","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1055\/s-0038-1655195","volume":"10","author":"I Witt","year":"1990","unstructured":"Witt I, Wehinger H (1990) Das Protein-C-System bei Kindern. H\u00e4mostaseologie 10: 125\u2013129","journal-title":"H\u00e4mostaseologie"}],"container-title":["European Journal of Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/BF02115621.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/BF02115621\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/BF02115621","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/BF02115621.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,7,29]],"date-time":"2022-07-29T00:02:57Z","timestamp":1659052977000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/BF02115621"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1996,1]]},"references-count":45,"journal-issue":{"issue":"1","published-print":{"date-parts":[[1996,1]]}},"alternative-id":["BF02115621"],"URL":"https:\/\/doi.org\/10.1007\/bf02115621","relation":{},"ISSN":["0340-6199","1432-1076"],"issn-type":[{"type":"print","value":"0340-6199"},{"type":"electronic","value":"1432-1076"}],"subject":[],"published":{"date-parts":[[1996,1]]},"assertion":[{"value":"16 May 1994","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"4 April 1995","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"This content has been made available to all.","name":"free","label":"Free to read"}]}}