The coagulation system in children: Developmental and pathophysiological considerations

Seminars in Thrombosis and Hemostasis

Volumn 37, Issue 7, 2011, Pages 723-729

  Ignjatovic, Vera ^a   Mertyn, Eliza ^b   Monagle, Paul ^b  

^a MURDOCH CHILDREN'S RESEARCH INSTITUTE   (Australia)

^b UNIVERSITY OF MELBOURNE   (Australia)

Author keywords

children; coagulation; Development; plasma proteins; thromboembolism

Indexed keywords

ACENOCOUMAROL; ALPHA 2 MACROGLOBULIN; ANTITHROMBIN; FIBRINOGEN; HEPARIN; PHENPROCOUMON; PROTEIN C; SERINE PROTEINASE INHIBITOR; WARFARIN;

AGE; ANGIOGENESIS; BLOOD CLOTTING; CHILD DEVELOPMENT; CHILDHOOD DISEASE; CONFERENCE PAPER; HEMOSTASIS; HUMAN; INFANT DISEASE; LIVER GRAFT; LUNG DISEASE; MOLECULAR WEIGHT; NONHUMAN; POSTTRANSLATIONAL MODIFICATIONS; PRIORITY JOURNAL; PROTEIN MODIFICATION; PROTEIN PROCESSING; RISK; RISK FACTOR; THROMBOEMBOLISM; VASCULAR ENDOTHELIUM;

ADULT; AGING; ANTICOAGULANTS; ANTITHROMBIN III; BLOOD COAGULATION; CHILD; CHILD DEVELOPMENT; ENOXAPARIN; FIBRINOGEN; HEMOSTASIS; HUMANS; INFANT, NEWBORN; LIVER TRANSPLANTATION; MOLECULAR WEIGHT; NEOVASCULARIZATION, PHYSIOLOGIC; THROMBOEMBOLISM;

EID: 84255197949     PISSN: 00946176     EISSN: 10989064     Source Type: Journal    
DOI: 10.1055/s-0031-1297162     Document Type: Conference Paper

References (91)

1. Monagle P, Ignjatovic V, Savoia H. Hemostasis in neonates and children: pitfalls and dilemmas. Blood Rev 2010 24 2 63-68
2. Andrew M, Paes B, Johnston M. Development of the hemostatic system in the neonate and young infant. Am J Pediatr Hematol Oncol 1990 12 1 95-104 (Pubitemid 20132100)
3. Andrew M, Paes B, Milner R et al. Development of the human coagulation system in the full-term infant. Blood 1987 70 1 165-172 (Pubitemid 17111257)
4. Andrew M, Vegh P, Johnston M, Bowker J, Ofosu F, Mitchell L. Maturation of the hemostatic system during childhood. Blood 1992 80 8 1998-2005
5. Monagle P, Barnes C, Ignjatovic V et al. Developmental haemostasis. Impact for clinical haemostasis laboratories. Thromb Haemost 2006 95 2 362-372
6. Monagle P, Chan A, de Veber G, Massicotte P. Developmental Hemostasis. Pediatric Thromboembolism and Stroke Handbook. 3rd ed. Hamilton, Ontario BC Decker Inc. 2006 3-30
7. Ignjatovic V, Lai C, Summerhayes R et al. Age-related differences in plasma proteins: how plasma proteins change from neonates to adults. PLoS ONE 2011 6 2 e17213
8. Mari D, Mannucci P M., Coppola R, Bottasso B, Bauer K A., Rosenberg R D. Hypercoagulability in centenarians: the paradox of successful aging. Blood 1995 85 11 3144-3149
9. Bock SC. Antithrombin III and heparin cofactor II. In: Colman RW, Hirsh J, Marder VJ, Clowes AW, George JN, eds. Hemostasis and Thrombosis: Basic Principles and Clinical Practice. 4th ed. Philadelphia, PA Lippincott Williams & Wilkins 2001 321-334
10. Monagle P, Hagstrom J. Developmental haemostasis. In: Polin RA, Fox WW, Abman SH, eds. Fetal and Neonatal Physiology. 3rd ed. St. Louis Elsevier 2003 1435-1447
11. Witt I, Müller H. Phosphorus and hexose content of human foetal fibrinogen. Biochim Biophys Acta 1970 221 2 402-404
12. Hamulyák K, Nieuwenhuizen W, Devilée P P., Hemker H C. Reevaluation of some properties of fibrinogen, purified from cord blood of normal newborns. Thromb Res 1983 32 3 301-310 (Pubitemid 14239986)
13. Witt I, Müller H, Künzer W. Evidence for the existence of foetal fibrinogen. Thromb Diath Haemorrh 1969 22 1 101-109
14. Miller B E., Tosone S R., Guzzetta N A., Miller J L., Brosius K K. Fibrinogen in children undergoing cardiac surgery: is it effective? Anesth Analg 2004 99 5 1341-1346 (Pubitemid 39407278)
15. Martinez J, MacDonald K A., Palascak J E. The role of sialic acid in the dysfibrinogenemia associated with liver disease: distribution of sialic acid on the constituent chains. Blood 1983 61 6 1196-1202 (Pubitemid 13099909)
16. Dang C V., Shin C K., Bell W R., Nagaswami C, Weisel J W. Fibrinogen sialic acid residues are low affinity calcium-binding sites that influence fibrin assembly. J Biol Chem 1989 264 25 15104-15108 (Pubitemid 19237344)
17. Ignjatovic V, Ilhan A, Monagle P. Evidence for age-related differences in human fibrinogen. Blood Coagul Fibrinolysis 2011 22 2 110-117
18. Manco-Johnson M J., Spedale S, Peters M et al. Identification of a unique form of protein C in the ovine fetus: developmentally linked transition to the adult form. Pediatr Res 1995 37 3 365-372
19. Niessen R W., Lamping R J., Peters M, Lamers W H., Sturk A. Fetal and neonatal development of antithrombin III plasma activity and liver messenger RNA levels in sheep. Pediatr Res 1996 39 4 Pt 1 685-691 (Pubitemid 26128157)
20. Lisman T, Platto M, Meijers J C., Haagsma E B., Colledan M, Porte R J. The hemostatic status of pediatric recipients of adult liver grafts suggests that plasma levels of hemostatic proteins are not regulated by the liver. Blood 2011 117 6 2070-2072
21. Mosnier L O., Zlokovic B V., Griffin J H. The cytoprotective protein C pathway. Blood 2007 109 8 3161-3172 (Pubitemid 46572500)
22. Taylor F B. Jr, Kinasewitz G T. The diagnosis and management of disseminated intravascular coagulation. Curr Hematol Rep 2002 1 1 34-40
23. Andrew M, Mitchell L, Vegh P, Ofosu F. Thrombin regulation in children differs from adults in the absence and presence of heparin. Thromb Haemost 1994 72 6 836-842
24. Gibson B E., Chalmers E, Bolton-Maggs P, Henderson D J., Lynn R. Thrombembolism in childhood: a prospective 2 year BPSU study in the United Kingdom. Thromb Haemost 2003 1 Suppl 1 OC422
25. Massicotte P, Leaker M, Marzinotto V et al. Enhanced thrombin regulation during warfarin therapy in children compared to adults. Thromb Haemost 1998 80 4 570-574 (Pubitemid 28479340)
26. Mitchell L G., Andrew M, Hanna K et al, Prophylactic Antithrombin Replacement in Kids with Acute Lymphoblastic Leukemia Treated with Asparaginase Group (PARKAA). A prospective cohort study determining the prevalence of thrombotic events in children with acute lymphoblastic leukemia and a central venous line who are treated with L-asparaginase: results of the Prophylactic Antithrombin Replacement in Kids with Acute Lymphoblastic Leukemia Treated with Asparaginase (PARKAA) Study. Cancer 2003 97 2 508-516 (Pubitemid 36133819)
27. Monagle P, Adams M, Mahoney M et al. Outcome of pediatric thromboembolic disease: a report from the Canadian Childhood Thrombophilia Registry. Pediatr Res 2000 47 6 763-766 (Pubitemid 30365435)
28. Newall F, Wallace T, Crock C et al. Venous thromboembolic disease: a single-centre case series study. J Paediatr Child Health 2006 42 12 803-807 (Pubitemid 44690828)
29. Nowak-Göttl U, Heinecke A, von Kries R, Nürnberger W, Münchow N, Junker R. Thrombotic events revisited in children with acute lymphoblastic leukemia: impact of concomitant Escherichia coli asparaginase/prednisone administration. Thromb Res 2001 103 3 165-172 (Pubitemid 32728101)
30. Nowak-Göttl U, von Kries R, Göbel U. Neonatal symptomatic thromboembolism in Germany: two year survey. Arch Dis Child Fetal Neonatal Ed 1997 76 3 F163-F167
31. Schmidt B, Andrew M. Neonatal thrombosis: report of a prospective Canadian and international registry. Pediatrics 1995 96 5 Pt 1 939-943
32. van Ommen C H., Heijboer H, Büller H R., Hirasing R A., Heijmans H S., Peters M. Venous thromboembolism in childhood: a prospective two-year registry in The Netherlands. J Pediatr 2001 139 5 676-681 (Pubitemid 33097433)
33. White R H. The epidemiology of venous thromboembolism. Circulation 2003 107 23, Suppl 1 I4-I8 (Pubitemid 41289265)
34. Kuhle S, Massicotte P, Chan A et al. Systemic thromboembolism in children. Data from the 1-800-NO-CLOTS Consultation Service. Thromb Haemost 2004 92 4 722-728
35. Raffini L, Huang Y S., Witmer C, Feudtner C. Dramatic increase in venous thromboembolism in children's hospitals in the United States from 2001 to 2007. Pediatrics 2009 124 4 1001-1008
36. Bjarke B, Herin P, Blombck M. Neonatal aortic thrombosis. A possible clinical manifestation of congenital antithrombin 3 deficiency. Acta Paediatr Scand 1974 63 2 297-301
37. De Stefano V, Leone G, Ferrelli R et al. Severe deep vein thrombosis in a 2-year-old child with protein S deficiency. Thromb Haemost 1987 58 4 1089
38. Israels S J., Seshia S S. Childhood stroke associated with protein C or S deficiency. J Pediatr 1987 111 4 562-564 (Pubitemid 17140536)
39. Mannino F L., Trauner D A. Stroke in neonates. J Pediatr 1983 102 4 605-610 (Pubitemid 13114879)
40. Shapiro M E., Rodvien R, Bauer K A., Salzman E W. Acute aortic thrombosis in antithrombin III deficiency. JAMA 1981 245 17 1759-1761 (Pubitemid 11096228)
41. Andrassy K, Ritz E, Bommer J. Hypercoagulability in the nephrotic syndrome. Klin Wochenschr 1980 58 19 1029-1036 (Pubitemid 10011225)
42. Kanfer A, Kleinknecht D, Broyer M, Josso F. Coagulation studies in 45 cases of nephrotic syndrome without uremia. Thromb Diath Haemorrh 1970 24 3 562-571
43. Kauffmann R H., Veltkamp J J., Van Tilburg N H., Van Es L A. Acquired antithrombin III deficiency and thrombosis in the nephrotic syndrome. Am J Med 1978 65 4 607-613 (Pubitemid 9054939)
44. Kuhlmann U, Blttler W, Pouliadis G, Siegenthaler W. [Complications of nephrotic syndrome with special reference to thromboembolic accidents]. Schweiz Med Wochenschr 1979 109 6 200-209 (Pubitemid 9099662)
45. Schrader J, Köstering H, Züchner C, Kaiser H, Kramer P, Scheler F. Antithrombin III-Bestimmungim Schnelltest: Ein Vergleich mit Partigen-Platten undeinem chromogenen Substrat. Lab Med 1981 5 211-218
46. Thaler E, Balzar E, Kopsa H, Pinggera W F. Acquired antithrombin III deficiency in patients with glomerular proteinuria. Haemostasis 1978 7 5 257-272 (Pubitemid 9003241)
47. Hirsh J. Heparin. N Engl J Med 1991 324 22 1565-1574
48. Olson S T., Björk I, Shore J D. Kinetic characterization of heparin-catalyzed and uncatalyzed inhibition of blood coagulation proteinases by antithrombin. Methods Enzymol 1993 222 525-559
49. Carrell R W., Stein P E., Fermi G, Wardell M R. Biological implications of a 3 A structure of dimeric antithrombin. Structure 1994 2 4 257-270
50. Schreuder H A., de Boer B, Dijkema R et al. The intact and cleaved human antithrombin III complex as a model for serpin-proteinase interactions. Nat Struct Biol 1994 1 1 48-54 (Pubitemid 24974779)
51. Levi M. All heparins are equal, but some are more equal than others. J Thromb Haemost 2003 1 5 884-885
52. Schedin-Weiss S, Richard B, Hjelm R, Olson S T. Antiangiogenic forms of antithrombin specifically bind to the anticoagulant heparin sequence. Biochemistry 2008 47 51 13610-13619
53. Chan A K., Berry L R., Paredes N, Parmar N. Isoform composition of antithrombin in a covalent antithrombin-heparin complex. Biochem Biophys Res Commun 2003 309 4 986-991 (Pubitemid 37122627)
54. Picard V, Ersdal-Badju E, Bock S C. Partial glycosylation of antithrombin III asparagine-135 is caused by the serine in the third position of its N-glycosylation consensus sequence and is responsible for production of the beta-antithrombin III isoform with enhanced heparin affinity. Biochemistry 1995 34 26 8433-8440
55. Carlson T H., Atencio A C., Simon T L. Comparison of the behaviour in vivo of two molecular forms of antithrombin III. Biochem J 1985 225 3 557-564 (Pubitemid 15174244)
56. Witmer M R., Hatton M W. Antithrombin III-beta associates more readily than antithrombin III-alpha with uninjured and de-endothelialized aortic wall in vitro and in vivo. Arterioscler Thromb 1991 11 3 530-539
57. Turk B, Brieditis I, Bock S C., Olson S T., Björk I. The oligosaccharide side chain on Asn-135 of alpha-antithrombin, absent in beta-antithrombin, decreases the heparin affinity of the inhibitor by affecting the heparin-induced conformational change. Biochemistry 1997 36 22 6682-6691 (Pubitemid 27242326)
58. Schedin-Weiss S, Richard B, Hjelm R, Olson S T. Antiangiogenic forms of antithrombin specifically bind to the anticoagulant heparin sequence. Biochemistry 2008 47 51 13610-13619
59. Adcock D M., Fink L M., Marlar R A., Cavallo F, Zangari M. The hemostatic system and malignancy. Clin Lymphoma Myeloma 2008 8 4 230-236
60. Akl E A., van Doormaal F F., Barba M et al. Parenteral anticoagulation for prolonging survival in patients with cancer who have no other indication for anticoagulation. Cochrane Database Syst Rev 2007 3 CD006652
61. Wojtukiewicz M Z., Sierko E, Rak J. Contribution of the hemostatic system to angiogenesis in cancer. Semin Thromb Hemost 2004 30 1 5-20 (Pubitemid 38457297)
62. Schmidt B, Gillie P, Mitchell L, Andrew M, Caco C, Roberts R. A placebo-controlled randomized trial of antithrombin therapy in neonatal respiratory distress syndrome. Am J Respir Crit Care Med 1998 158 2 470-476 (Pubitemid 28378175)
63. Ganrot P O. Inhibition of plasmin activity by alpha-2-macroglobulin. Clin Chim Acta 1967 16 2 328-329
64. Sottrup-Jensen L. Alpha-macroglobulins: structure, shape, and mechanism of proteinase complex formation. J Biol Chem 1989 264 20 11539-11542 (Pubitemid 19185316)
65. Blacker D, Wilcox M A., Laird N M. et al. Alpha-2 macroglobulin is genetically associated with Alzheimer disease. Nat Genet 1998 19 4 357-360 (Pubitemid 28357907)
66. Baker A H., Edwards D R., Murphy G. Metalloproteinase inhibitors: biological actions and therapeutic opportunities. J Cell Sci 2002 115 Pt 19 3719-3727 (Pubitemid 35203598)
67. French K, Yerbury J J., Wilson M R. Protease activation of alpha2-macroglobulin modulates a chaperone-like action with broad specificity. Biochemistry 2008 47 4 1176-1185 (Pubitemid 351161874)
68. Andrew M, Paes B, Milner R et al. Development of the human coagulation system in the healthy premature infant. Blood 1988 72 5 1651-1657 (Pubitemid 18268649)
69. Mitchell L, Piovella F, Ofosu F, Andrew M. Alpha-2-macroglobulin may provide protection from thromboembolic events in antithrombin III-deficient children. Blood 1991 78 9 2299-2304
70. Schmidt B, Mitchell L, Ofosu F A., Andrew M. Alpha-2-macroglobulin is an important progressive inhibitor of thrombin in neonatal and infant plasma. Thromb Haemost 1989 62 4 1074-1077 (Pubitemid 20011492)
71. Ignjatovic V, Greenway A, Summerhayes R, Monagle P. Thrombin generation: the functional role of alpha-2-macroglobulin and influence of developmental haemostasis. Br J Haematol 2007 138 3 366-368 (Pubitemid 47035395)
72. Ling X, Delorme M, Berry L et al. alpha 2-Macroglobulin remains as important as antithrombin III for thrombin regulation in cord plasma in the presence of endothelial cell surfaces. Pediatr Res 1995 37 3 373-378
73. Guzzetta N A., Miller B E., Todd K et al. Clinical measures of heparin's effect and thrombin inhibitor levels in pediatric patients with congenital heart disease. Anesth Analg 2006 103 5 1131-1138 (Pubitemid 44659368)
74. Albisetti M, Chan A K., McCrindle B W., Wong D, Monagle P, Andrew M. Impaired fibrinolytic activity is present in children with dyslipidemias. Pediatr Res 2004 55 4 576-580 (Pubitemid 38405842)
75. Ignjatovic V, Furmedge J, Newall F et al. Age-related differences in heparin response. Thromb Res 2006 118 6 741-745 (Pubitemid 44647091)
76. Ignjatovic V, Summerhayes R, Newall F, Monagle P T. The in vitro response to low-molecular-weight heparin is not age-dependent in children. Thromb Haemost 2010 103 4 855-856
77. Newall F, Ignjatovic V, Summerhayes R et al. In vivo age dependency of unfractionated heparin in infants and children. Thromb Res 2009 123 5 710-714
78. Ignjatovic V, Straka E, Summerhayes R, Monagle P. Age-specific differences in binding of heparin to plasma proteins. J Thromb Haemost 2010 8 6 1290-1294
79. Andrew M, Ofosu F, Schmidt B, Brooker L, Hirsh J, Buchanan M R. Heparin clearance and ex vivo recovery in newborn piglets and adult pigs. Thromb Res 1988 52 6 517-527 (Pubitemid 19028224)
80. McDonald M M., Jacobson L J., Hay W W. Jr, Hathaway W E. Heparin clearance in the newborn. Pediatr Res 1981 15 7 1015-1018 (Pubitemid 11087048)
81. Turner Gomes S, Nitschmann E, Benson L, Burrows P, Andrew M. Heparin is cleared faster in children with congenital heart disease than adults. J Am Coll Cardiol 1993 21 2 59a
82. Newall F, Ignjatovic V, Johnston L et al. Age is a determinant factor for measures of concentration and effect in children requiring unfractionated heparin. Thromb Haemost 2010 103 5 1085-1090
83. Ignjatovic V, Najid S, Newall F, Summerhayes R, Monagle P. Dosing and monitoring of enoxaparin (Low molecular weight heparin) therapy in children. Br J Haematol 2010 149 5 734-738
84. Trame M N., Mitchell L, Krümpel A, Male C, Hempel G, Nowak-Göttl U. Population pharmacokinetics of enoxaparin in infants, children and adolescents during secondary thromboembolic prophylaxis: a cohort study. J Thromb Haemost 2010 8 9 1950-1958
85. Andrew M, Marzinotto V, Brooker L A. et al. Oral anticoagulation therapy in pediatric patients: a prospective study. Thromb Haemost 1994 71 3 265-269 (Pubitemid 24083732)
86. Streif W, Andrew M, Marzinotto V et al. Analysis of warfarin therapy in pediatric patients: a prospective cohort study of 319 patients. Blood 1999 94 9 3007-3014 (Pubitemid 29512704)
87. Carpentieri U, Nghiem Q X., Harris L C. Clinical experience with an oral anticoagulant in children. Arch Dis Child 1976 51 6 445-448
88. Doyle J J., Koren G, Cheng M Y., Blanchette V S. Anticoagulation with sodium warfarin in children: effect of a loading regimen. J Pediatr 1988 113 6 1095-1097 (Pubitemid 19014266)
89. Evans D I., Rowlands M, Poller L. Survey of oral anticoagulant treatment in children. J Clin Pathol 1992 45 8 707-708
90. Bonduel M, Sciuccati G, Hepner M et al. Acenocoumarol therapy in pediatric patients. J Thromb Haemost 2003 1 8 1740-1743
91. Piquet P, Losay J, Doubine S. [Acenocoumarol (Sintrom) and fluinidione (Previscan) in pediatrics after cardiac surgical procedures]. Arch Pediatr 2002 9 11 1137-1144