Top

European Journal of Pediatrics

Published in:

Open Access 01-01-2012 | Review

Clinical practice

The bleeding child. Part I: primary hemostatic disorders

Authors: C. Heleen van Ommen, Marjolein Peters

Published in: European Journal of Pediatrics | Issue 1/2012

Abstract

Mucocutaneous bleeding is common in childhood and may be the result of primary hemostatic disorders such as vascular abnormalities, von Willebrand disease, thrombocytopenia, and platelet dysfunction. A detailed bleeding history and physical examination are essential to distinguish between normal and abnormal bleeding and to decide whether it is necessary to perform further laboratory evaluation. Initial laboratory tests include complete blood count, peripheral blood smear, mean platelet volume, von Willebrand factor (VWF) antigen assay, VWF ristocetin cofactor activity, and factor VIII activity. Once thrombocytopenia and von Willebrand disease have been excluded, platelet function should be tested by platelet aggregation. Additional specific diagnostic tests, such as platelet secretion tests and flow cytometry for the detection of platelet surface glycoprotein expression, are needed to confirm the raised hypothesis.

Andrew M, Paes B, Bowker J, Vegh P (1990) Evaluation of an automated bleeding time device in the newborn. Am J Hematol 35:275–277PubMedCrossRef

Arad ID, Alpan G, Sznajderman SD, Eldor A (1986) The mean platelet volume (MPV) in the neonatal period. Am J Perinatol 3:1–3PubMedCrossRef

Balduini CL, Cattaneo M, Fabris F, Gresele P, Iolascon A, Pulcinelli FM, Savoia A (2003) Inherited thrombocytopenias: a proposed diagnostic algorithm from the Italian Gruppo di Studio delle Piastrine. Haematologica 88:582–592PubMed

Boccardo P, Remuzzi G, Galbusera M (2004) Platelet dysfunction in renal failure. Semin Thromb Hemost 30:579–589PubMedCrossRef

Boudewijns M, Raes M, Peeters V, Mewis A, Cartuyvels R, Magerman K, Rummens JL (2003) Evaluation of platelet function on cord blood in 80 healthy term neonates using the Platelet Function Analyser (PFA-100); shorter in vitro bleeding times in neonates than adults. Eur J Pediatr 162:212–213PubMed

Bowman M, Hopman WM, Rapson D, Lillicrap D, Silva M, James P (2010) A prospective evaluation of the prevalence of symptomatic von Willebrand disease (VWD) in a pediatric primary care population. Pediatr Blood Cancer 55:171–173PubMed

Bowman M, Riddel J, Rand ML, Tosetto A, Silva M, James PD (2009) Evaluation of the diagnostic utility for von Willebrand disease of a pediatric bleeding questionnaire. J Thromb Haemost 7:1418–1421PubMedCrossRef

Cariappa R, Wilhite TR, Parvin CA, Luchtman-Jones L (2003) Comparison of PFA-100 and bleeding time testing in pediatric patients with suspected hemorrhagic problems. J Pediatr Hematol Oncol 25:474–479PubMedCrossRef

Forestier F, Daffos F, Galacteros F, Bardakjian J, Rainaut M, Beuzard Y (1986) Hematological values of 163 normal fetuses between 18 and 30 weeks of gestation. Pediatr Res 20:342–346PubMedCrossRef

10.

Ghevaert C, Campbell K, Walton J, Smith GA, Allen D, Williamson LM, Ouwehand WH, Ranasinghe E (2007) Management and outcome of 200 cases of fetomaternal alloimmune thrombocytopenia. Transfusion 47:901–910PubMedCrossRef

11.

Harrison P, Mumford A (2009) Screening tests of platelet function: update on their appropriate uses for diagnostic testing. Semin Thromb Hemost 35:150–157PubMedCrossRef

12.

Israels SJ (2009) Diagnostic evaluation of platelet function disorders in neonates and children: an update. Semin Thromb Hemost 35:181–188PubMedCrossRef

13.

Israels SJ, Kahr WH, Blanchette VS, Luban NL, Rivard GE, Rand ML (2011) Platelet disorders in children: a diagnostic approach. Pediatr Blood Cancer 56:975–983PubMedCrossRef

14.

Kaleelrahman M, Minford A, Parapia LA (2004) Use of recombinant factor VIIa in inherited platelet disorders. Br J Haematol 125:95–96PubMedCrossRef

15.

Katz JA, Moake JL, McPherson PD, Weinstein MJ, Moise KJ, Carpenter RJ, Sala DJ (1989) Relationship between human development and disappearance of unusually large von Willebrand factor multimers from plasma. Blood 73:1851–1858PubMed

16.

Kaufmann JE, Vischer UM (2003) Cellular mechanisms of the hemostatic effects of desmopressin (DDAVP). J Thromb Haemost 1:682–689PubMedCrossRef

17.

Kitchen S, Jennings I, Woods TA, Kitchen DP, Walker ID, Preston FE (2006) Laboratory tests for measurement of von Willebrand factor show poor agreement among different centers: results from the United Kingdom National External Quality Assessment Scheme for Blood Coagulation. Semin Thromb Hemost 32:492–498PubMedCrossRef

18.

Knofler R, Olivieri M, Weickardt S, Eberl W, Streif W (2007) First results of the THROMKID study: a quality project for the registration of children and adolescents with hereditary platelet function defects in Germany, Austria, and Switzerland. Hamostaseologie 27:48–53PubMed

19.

Knofler R, Weissbach G, Kuhlisch E (1998) Platelet function tests in childhood measuring aggregation and release reaction in whole blood. Semin Thromb Hemost 24:513–521PubMedCrossRef

20.

Koenig S, Gerstner T, Keller A, Teich M, Longin E, Dempfle CE (2008) High incidence of vaproate-induced coagulation disorders in children receiving valproic acid: a prospective study. Blood Coagul Fibrinolysis 19:375–382PubMedCrossRef

21.

Kriz N, Rinder CS, Rinder HM (2009) Physiology of hemostasis: with relevance to current and future laboratory testing. Clin Lab Med 29:159–174PubMedCrossRef

22.

Lippi G, Manzato F, Franchini M, Brocco G, Florenziani G, Guidi G (2001) Establishment of reference values for the PFA-100 platelet function analyzer in pediatrics. Clin Exp Med 1:69–70PubMedCrossRef

23.

Litalien C, Jacqz-Aigrain E (2001) Risks and benefits of nonsteroidal anti-inflammatory drugs in children: a comparison with paracetamol. Paediatr Drugs 3:817–858PubMedCrossRef

24.

Malfait F, De PA (2009) Bleeding in the heritable connective tissue disorders: mechanisms, diagnosis and treatment. Blood Rev 23:191–197PubMedCrossRef

25.

Marcus PD, Nire KG, Grooms L, Klima J, O'Brien SH (2011) The power of a standardized bleeding score in diagnosing paediatric type 1 von Willebrand's disease and platelet function defects. Haemophilia 17:223–227PubMedCrossRef

26.

Patzke J, Schneppenheim R (2010) Laboratory diagnosis of von Willebrand disease. Hamostaseologie 30:203–206PubMed

27.

Provan D, Stasi R, Newland AC, Blanchette VS, Bolton-Maggs P, Bussel JB, Chong BH, Cines DB, Gernsheimer TB, Godeau B, Grainger J, Greer I, Hunt BJ, Imbach PA, Lyons G, McMillan R, Rodeghiero F, Sanz MA, Tarantino M, Watson S, Young J, Kuter DJ (2010) International consensus report on the investigation and management of primary immune thrombocytopenia. Blood 115:168–186PubMedCrossRef

28.

Roberts I, Murray NA (2008) Neonatal thrombocytopenia. Semin Fetal Neonatal Med 13:256–264PubMedCrossRef

29.

Roberts I, Stanworth S, Murray NA (2008) Thrombocytopenia in the neonate. Blood Rev 22:173–186PubMedCrossRef

30.

Rodeghiero F, Castaman G, Tosetto A, Batlle J, Baudo F, Cappelletti A, Casana P, De Bosch N, Eikenboom JC, Federici AB, Lethagen S, Linari S, Srivastava A (2005) The discriminant power of bleeding history for the diagnosis of type 1 von Willebrand disease: an international, multicenter study. J Thromb Haemost 3:2619–2626PubMedCrossRef

31.

Roschitz B, Thaller S, Koestenberger M, Wirnsberger A, Leschnik B, Fritsch P, Muntean W (2007) PFA-100 closure times in preoperative screening in 500 pediatric patients. Thromb Haemost 98:243–247PubMed

32.

Sugar NF, Taylor JA, Feldman KW (1999) Bruises in infants and toddlers: those who don’t cruise rarely bruise. Puget Sound Pediatric Research Network. Arch Pediatr Adolesc Med 153:399–403PubMed

33.

Tauer JT, Gneuss A, Lohse JE, Jurgens T, Knofler R (2011) Evaluation of desmopressin effect on primary haemostasis in pediatric patients with aspirin-like defect as hereditary thrombocytopathy. Klin Padiatr 223:169–172PubMedCrossRef

34.

Tosetto A, Castaman G, Rodeghiero F (2008) Bleeding scores in inherited bleeding disorders: clinical or research tools? Haemophilia 14:415–422PubMedCrossRef

Title: Clinical practice
The bleeding child. Part I: primary hemostatic disorders
Authors: C. Heleen van Ommen
Marjolein Peters
Publication date: 01-01-2012
Publisher: Springer-Verlag
Published in: European Journal of Pediatrics / Issue 1/2012
Print ISSN: 0340-6199
Electronic ISSN: 1432-1076
DOI: https://doi.org/10.1007/s00431-011-1532-4

At a glance: The STEP trials

Springer Medicine

Clinical practice

The bleeding child. Part I: primary hemostatic disorders

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

Please log in to get access to this content

Other articles of this Issue 1/2012

Discordant expression of a new WT1 gene mutation in a family with monozygotic twins presenting with congenital nephrotic syndrome

Unilateral gynecomastia in a prepubertal boy

Serum procalcitonin is not an early marker of pulmonary exacerbation in children with cystic fibrosis

Magnetic resonance imaging of the brain in adenylosuccinate lyase deficiency: a report of seven cases and a review of the literature

Effect of exclusive breastfeeding on the development of children’s cognitive function in the Krakow prospective birth cohort study

Reply to the correspondence letter by Se Jin Park et al. What dose of intravenous immunoglobulin should be administered in Kawasaki disease with suspected systemic capillary leak syndrome?