Diagnosis and management of neonatal purpura fulminans

Seminars in Fetal and Neonatal Medicine

Volumn 16, Issue 6, 2011, Pages 318-322

  Price, V E ^a   Ledingham, D L ^a   Krumpel A ^b   Chan, A K ^c  

^a DALHOUSIE UNIVERSITY   (Canada)

^b UNIVERSITY HOSPITAL MÜNSTER   (Germany)

^c MCMASTER UNIVERSITY   (Canada)

Author keywords

Neonate; Protein C deficiency; Protein S deficiency; Purpura fulminans

Indexed keywords

DROTRECOGIN; FRESH FROZEN PLASMA; HEPARIN; LOW MOLECULAR WEIGHT HEPARIN; PHOSPHOLIPID ANTIBODY; PROTEIN C; PROTEIN S; WARFARIN;

BLEEDING DISORDER; CLINICAL FEATURE; DISSEMINATED INTRAVASCULAR CLOTTING; FETUS; FULMINATING PURPURA; HUMAN; LIVER TRANSPLANTATION; NEWBORN; NEWBORN DISEASE; PARTIAL BLINDNESS; PROTEIN C DEFICIENCY; PROTEIN S DEFICIENCY; RETINA DETACHMENT; REVIEW; SEPSIS; SKIN NECROSIS; SUBSTITUTION THERAPY; THROMBOCYTE DYSFUNCTION; VITREOUS HEMORRHAGE;

DISEASE MANAGEMENT; HUMANS; INFANT, NEWBORN; PROTEIN C DEFICIENCY; PROTEIN S DEFICIENCY; PURPURA FULMINANS;

EID: 80053602997     PISSN: 1744165X     EISSN: 18780946     Source Type: Journal    
DOI: 10.1016/j.siny.2011.07.009     Document Type: Review

References (54)

1. Van Der Horst R.L. Purpura fulminans in a newborn baby. Arch Dis Child 1962, 37:436-441.
2. Sills R.H., Marlar R.A., Montgomery R.R., Deshpande G.N., Humbert J.R. Severe homozygous protein C deficiency. J Pediatr 1984, 105:409-413.
3. Branson H.E., Katz J., Marble R., Griffin J.H. Inherited protein C deficiency and coumarin-responsive chronic relapsing purpura fulminans in a newborn infant. Lancet 1983, 2:1165-1168.
4. Mahasandana C., Suvatte V., Marlar R.A., Manco-Johnson M.J., Jacobson L.J., Hathaway W.E. Neonatal purpura fulminans associated with homozygous protein S deficiency. Lancet 1990, 335:61-62.
5. Mahasandana C., Suvatte V., Chuansumrit A., et al. Homozygous protein s deficiency in an infant with purpura fulminans. J Pediatr 1990, 117:750-753.
6. Reitsma P.H., Bernardi F., Doig R.G., et al. Protein C deficiency: a database of mutations, 1995 update. On behalf of the Subcommittee on Plasma Coagulation Inhibitors of the Scientific and Standardization Committee of the ISTH. Thromb Haemost 1995, 73:876-889.
7. Dogan Y., Aygun D., Yilmaz Y., et al. Severe protein S deficiency associated with heterozygous factor V Leiden mutation in a child with purpura fulminans. Pediatr Hematol Oncol 2003, 20:1-5.
8. Petaja J., Manco-Johnson M.J. Protein C pathway in infants and children. Semin Thromb Hemost 2003, 29:349-362.
9. Esmon C.T. The protein C pathway. Chest 2003, 124:26S-32S.
10. Tuddenham E.G., Takase T., Thomas A.E., et al. Homozygous protein C deficiency with delayed onset of symptoms at 7 to 10 months. Thromb Res 1989, 53:475-484.
11. Marlar R.A., Montgomery R.R., Broekmans A.W. Diagnosis and treatment of homozygous protein C deficiency. Report of the working party on homozygous protein C deficiency of the Subcommittee on Protein C and Protein S, International Committee on Thrombosis and Haemostasis. J Pediatr 1989, 114:528-534.
12. Manco-Johnson M.J., Abshire T.C., Jacobson L.J., Marlar R.A. Severe neonatal protein C deficiency: prevalence and thrombotic risk. J Pediatr 1991, 119:793-798.
13. Hattenbach L.O., Beeg T., Kreuz W., Zubcov A. Ophthalmic manifestation of congenital protein C deficiency. J AAPOS 1999, 3:188-190.
14. Seligsohn U., Berger A., Abend M., et al. Homozygous protein C deficiency manifested by massive venous thrombosis in the newborn. N Engl J Med 1984, 310:559-562.
15. Kirkinen P., Salonvaara M., Nikolajev K., Vanninen R., Heinonen K. Antepartum findings in fetal protein C deficiency. Prenat Diagn 2000, 20:746-749.
16. Mintz-Hittner H.A., Miyashiro M.J., Knight-Nanan D.M., O'Malley R.E., Marlar R.A. Vitreoretinal findings similar to retinopathy of prematurity in infants with compound heterozygous protein S deficiency. Ophthalmology 1999, 106:1525-1530.
17. Monagle P., Chalmers E., Chan A., et al. Antithrombotic therapy in neonates and children: American College of Chest Physicians evidence-based clinical practice guidelines (8th edition). Chest 2008, 133:887S-968S.
18. ten Kate M.K., van der Meer J. Protein S deficiency: a clinical perspective. Haemophilia 2008, 14:1222-1228.
19. Goldenberg N.A., Manco-Johnson M.J. Protein C deficiency. Haemophilia 2008, 14:1214-1221.
20. Zenciroglu A., Karagol B.S., Ipek M.S., Okumus N., Yarali N., Aydin M. Neonatal purpura fulminans secondary to group B streptococcal infection. Pediatr Hematol Oncol 2010, 27:620-625.
21. Lynn N.J., Pauly T.H., Desai N.S. Purpura fulminans in three cases of early-onset neonatal group B streptococcal meningitis. J Perinatol 1991, 11:144-146.
22. Issacman S.H., Heroman W.M., Lightsey A.L. Purpura fulminans following late-onset group B beta-hemolytic streptococcal sepsis. Am J Dis Child 1984, 138:915-916.
23. Zenciroglu A., Ipek M.S., Aydin M., Kara A., Okumus N., Kilic M. Purpura fulminans in a newborn infant with galactosemia. Eur J Pediatr 2010, 169:903-906.
24. MacDonald P.D., Walker I.D., Galea P., Alroomi L.G. Acquired transient protein C deficiency in neonatal cardiac failure. Arch Dis Child 1990, 65:158.
25. Estelles A., Garcia-Plaza I., Dasi A., et al. Severe inherited "homozygous" protein C deficiency in a newborn infant. Thromb Haemost 1984, 52:53-56.
26. Goodwin A.J., Rosendaal F.R., Kottke-Marchant K., Bovill E.G. A review of the technical, diagnostic, and epidemiologic considerations for protein S assays. Arch Pathol Lab Med 2002, 126:1349-1366.
27. Khor B., Van Cott E.M. Laboratory tests for protein C deficiency. Am J Hematol 2010, 85:440-442.
28. Moalic P., Gruel Y., Body G., Foloppe P., Delahousse B., Leroy J. Levels and plasma distribution of free and c4b-bp-bound protein S in human fetuses and full-term newborns. Thromb Res 1988, 49:471-480.
29. Schwarz H.P., Muntean W., Watzke H., Richter B., Griffin J.H. Low total protein S antigen but high protein S activity due to decreased c4b-binding protein in neonates. Blood 1988, 71:562-565.
30. Monagle P., Barnes C., Ignjatovic V., et al. Developmental haemostasis. Impact for clinical haemostasis laboratories. Thromb Haemost 2006, 95:362-372.
31. Andrew M., Paes B., Milner R., et al. Development of the human coagulation system in the healthy premature infant. Blood 1988, 72:1651-1657.
32. Andrew M., Paes B., Milner R., et al. Development of the human coagulation system in the full-term infant. Blood 1987, 70:165-172.
33. Salonvaara M., Kuismanen K., Mononen T., Riikonen P. Diagnosis and treatment of a newborn with homozygous protein C deficiency. Acta Paediatr 2004, 93:137-139.
34. Mulder R., Ten Kate M.K., Kluin-Nelemans H.C., Mulder A.B. Low cut-off values increase diagnostic performance of protein S assays. Thromb Haemost 2010, 104:618-625.
35. Millar D.S., Allgrove J., Rodeck C., Kakkar V.V., Cooper D.N. A homozygous deletion/insertion mutation in the protein C (proc) gene causing neonatal purpura fulminans: prenatal diagnosis in an at-risk pregnancy. Blood Coagul Fibrinolysis 1994, 5:647-649.
36. D'Ursi P., Marino F., Caprera A., Milanesi L., Faioni E.M., Rovida E. PROCMD: A database and 3D web resource for protein C mutants. BMC Bioinformatics 2007, 8(Suppl. 1):S11.
37. Gandrille S., Borgel D., Sala N., et al. Protein S deficiency: a database of mutations - summary of the first update. Thromb Haemost 2000, 84:918.
38. Barnes C., Newall F., Higgins S., Carden S., Monagle P. Perinatal management of patients at high risk of homozygous protein C deficiency. Thromb Haemost 2002, 88:370-371.
39. Reverdiau-Moalic P., Delahousse B., Body G., Bardos P., Leroy J., Gruel Y. Evolution of blood coagulation activators and inhibitors in the healthy human fetus. Blood 1996, 88:900-906.
40. Mibashan R.S., Millar D.S., Rodeck C.H., Nicolaides K.H., Berger A., Seligsohn U. Prenatal diagnosis of hereditary protein C deficiency. N Engl J Med 1985, 313:1607.
41. Hellstern P. Solvent/detergent-treated plasma: composition, efficacy, and safety. Curr Opin Hematol 2004, 11:346-350.
42. Hellstern P., Haubelt H. Manufacture and composition of fresh frozen plasma and virus-inactivated therapeutic plasma preparations: correlation between composition and therapeutic efficacy. Thromb Res 2002, 107(Suppl. 1):S3-S8.
43. Lawrie A.S., Green L., Canciani M.T., et al. The effect of prion reduction in solvent/detergent-treated plasma on haemostatic variables. Vox Sang 2010, 99:232-238.
44. Veldman A., Fischer D., Wong F.Y., et al. Human protein C concentrate in the treatment of purpura fulminans: a retrospective analysis of safety and outcome in 94 pediatric patients. Crit Care 2010, 14. R156.
45. Fischer D., Schloesser R.L., Nold-Petry C.A., Nold M.F., Veldman A. Protein C concentrate in preterm neonates with sepsis. Acta Paediatr 2009, 98:1526-1529.
46. Nadel S., Goldstein B., Williams M.D., et al. Drotrecogin alfa (activated) in children with severe sepsis: a multicentre phase III randomised controlled trial. Lancet 2007, 369:836-843.
47. Manco-Johnson M.J., Knapp-Clevenger R. Activated protein C concentrate reverses purpura fulminans in severe genetic protein C deficiency. J Pediatr Hematol Oncol 2004, 26:25-27.
48. Sanz-Rodriguez C., Gil-Fernandez J.J., Zapater P., et al. Long-term management of homozygous protein C deficiency: replacement therapy with subcutaneous purified protein C concentrate. Thromb Haemost 1999, 81:887-890.
49. Lee M.J., Kim K.M., Kim J.S., Kim Y.J., Lee Y.J., Ghim T.T. Long-term survival of a child with homozygous protein C deficiency successfully treated with living donor liver transplantation. Pediatr Transplant 2009, 13:251-254.
50. de Kort E.H., Vrancken S.L., van Heijst A.F., Binkhorst M., Cuppen M.P., Brons P.P. Long-term subcutaneous protein C replacement in neonatal severe protein C deficiency. Pediatrics 2011, 127:e1338-e1342.
51. Mathias M., Khair K., Burgess C., Liesner R. Subcutaneous administration of protein C concentrate. Pediatr Hematol Oncol 2004, 21:551-556.
52. Monagle P., Andrew M., Halton J., et al. Homozygous protein C deficiency: description of a new mutation and successful treatment with low molecular weight heparin. Thromb Haemost 1998, 79:756-761.
53. Newall F., Bauman M. Point-of-care antithrombotic monitoring in children. Thromb Res 2006, 118:113-121.
54. Muller F.M., Ehrenthal W., Hafner G., Schranz D. Purpura fulminans in severe congenital protein C deficiency: monitoring of treatment with protein C concentrate. Eur J Pediatr 1996, 155:20-25.