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DOI: 10.1055/s-0041-106902
Gerinnungsstörungen im Kindesalter
Publication History
Publication Date:
11 January 2016 (online)
![](https://www.thieme-connect.de/media/intensiv-u2d/201601/lookinside/thumbnails/250_10-1055-s-0041-106902-1.jpg)
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Eine schwere angeborene Blutungsneigung manifestiert sich in der Regel im ersten Lebensjahr. Zu den typischen Symptomen zählen u. a. intrakranielle Blutung, Hämatome nach Bagatellverletzungen, flächenhafte Petechien, Purpura, Ekchymosen oder auch Gelenkblutungen.
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Vor der Labordiagnostik sind eine körperliche Untersuchung und insbesondere das Erheben der Eigen- und Familienanamnese wichtig.
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Laborergebnisse muss man bei Kindern anhand altersentsprechender Referenzwerte interpretieren, da sich diese teils erheblich von den Werten für Erwachsene unterscheiden.
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Eine spezifische Therapie einer Gerinnungsstörung im Kindesalter ist erst nach Abschluss der umfassenden klinischen und laborchemischen Diagnostik möglich.
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Diagnostik und Therapie einer angeborenen Blutgerinnungsstörung erfordern eine fachübergreifende Zusammenarbeit zwischen Neonatologe, Pädiater, Hämostaseologe und Labormediziner.
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Literatur
- 1 Reverdiau-Moalic P, Delahousse B, Bardos P et al. Evolution of blood coagulation activators and inhibitors in the healthy human fetus. Blood 1996; 88: 900-906
- 2 Andrew M, Payes B, Milner R et al. Development of the human coagulation system in the healthy premature infant. Blood 1988; 72: 1651-1657
- 3 Andrew M, Payes B, Johnston M. Development of the hemostatic system in the neonate and young infant. Am J Pediatr Hematol Oncol 1990; 12: 95-104
- 4 Attard C, van der Straaten T, Karlaftis V et al. Developmental hemostasis: age-specific differences in the levels of hemostatic proteins. J Thromb Haemost 2013; 11: 1850-1854
- 5 Witt I, Müller H, Künzer W. Evidence for the existence of fetal fibrinogen. Thromb Diath Haemorrh 1969; 22: 101-109
- 6 Cvirn G, Gallistl S, Muntean W. Effects of antithrombin and protein C on thrombin generation in newborn and adult plasma. Thromb Res 1999; 93: 183-190
- 7 Andrew M, Schmidt B, Mitchell L et al. Thrombin generation in newborn plasma is critically dependent on the concentration of prothrombin. Thromb Haemost 1990; 63: 27-30
- 8 Summaria L. Comparison of human normal, full-term, fetal and adult plasminogen by physical and chemical analyses. Haemostasis 1989; 19: 266-273
- 9 Saxonhouse MA, Sola MC. Platelet function in term and preterm neonates. Clin Perinatol 2004; 31: 15-18
- 10 Levy-Shraga Y, Maayan-Metzger A, Lubetsky A et al. Platelet function of newborns as tested by the Cone and Plate(let) analyser correlates with gestational age. Acts Haematol 2006; 115: 152-156
- 11 Del Vecchio A, Latini G, Henry E et al. Template bleeding time of 240 neonates born at 24 to 41 weeks gestation. J Perinatol 2008; 28: 427-423
- 12 Rajasekhar D, Barnard MR, Bednarek F. Platelet hyporeactivity in very low birth weight neonates. Thromb Haemost 1997; 77: 1002-1007
- 13 Israels SJ, Odaibo FS, Robertson C et al. Deficient Thromboxane synthesis and response in platelets from premature infants. Pediatr Res 1997; 41: 218-223
- 14 Yoffe G, Buchanan GR. Intracranial hemorrhage in newborn and young infants with hemophilia. J Pediatr 1988; 113: 333-336
- 15 Abbondanzo SL, Gootenberg JE, Lofts R et al. Intracranial hemorrhage in congenital deficiency of FXIII. Am J Pediatr Hematol Oncol 1988; 10: 65-68
- 16 Struwe FE. Intracranial hemorrhage and occlusive hydrocephalus in hereditary bleeding disorders. Dev Med Child Neurol 1970; 22: 165
- 17 Kenet G, Chan AK, Soucie JM et al. Bleeding disorders in neonates. Haemophilia 2010; 16: 168-174
- 18 Bidlingmaier C, Grote V, Budde U et al. Prospective evaluation of a pediatric bleeding questionnaire and the ISTH bleeding assessment tool in children and parents in routine clinical practice. J Thromb Haemost 2012; 10: 1335-1341
- 19 Doymaz S, Zinger M, Sweberg T. Risk factors associated with intracranial hemorrhage in neonates with persistent pulmonary hypertension on ECMO. J Intensive care 2015; DOI: 10.1186/s40560-015-0071-x.
- 20 Retter A, Barrett NA. The management of abnormal haemostasis in the ICU. Anesthesia 2015; 70: 121-127
- 21 Whittaker B, Christiaans SC, Altice JL et al. Early coagulopathy is an independent predictor of mortality in children after severe trauma. Shock 2013; 39: 421-426
- 22 Bulutco FS, Özbek U, Polat B et al. Which may be effective to reduce blood loss after cardiac operations in cyanotic children: tranexamic acid, aprotinin or a combination?. Ped Anesthesia 2005; 15: 41-46
- 23 Monagle P, Barnes C, Ignjatovic V et al. Developmental haemostasis. Impact for clinical haemostasis laboratories. Thromb Haemost 2006; 95: 362-372
- 24 Ehrenforth S, Junker R, Koch HG et al. Multicentre evaluation of combined prothrombotic defects associated with thrombophilia in childhood. Childhood Thrombophilia Study Group. Eur J Pediatr 1999; 158 (Suppl. 03) S97-104
- 25 Nowak-Göttl U, Fröhlich B, Thedieck S et al. Association of the protein Z ATG haplotype with symptomatic nonvascular stroke or thromboembolism in white children: a family-based cohort study. Blood 2009; 113: 2336-2341
- 26 Beheiri A, Langer C, Düring C et al. Role of elevated alpha2-macroglobulin revisited: results of a case-control study in children with symptomatic thromboembolism. J Thromb Haemost 2007; 5: 1179-1184
- 27 Kreuz W, Stoll M, Junker R et al. Familial elevated factor VIII in children with symptomatic venous thrombosis and post-thrombotic syndrome: results of a multicenter study. Arterioscler Thromb Vasc Biol 2006; 26: 1901-1906
- 28 Burghaus B, Langer C, Thedieck S et al. Elevated alpha1-antitrypsin is a risk factor for arterial ischemic stroke in childhood. Acta Haematol 2006; 115: 186-191
- 29 Duering C, Kosch A, Langer C et al. Total tissue factor pathway inhibitor is an independent risk factor for symptomatic paediatric venous thromboembolism and stroke. Thromb Haemost 2004; 92: 707-712
- 30 Sainio S, Jarvenpaa AL, Renlund M et al. Throbocytopenia in term infants: a population-based study. Obstet Gynecol 2000; 95: 441-446
- 31 Holzhauer S, Zieger B. Diagnosis and management of neonatal thrombocytopenia. Sem Fet Neonal Med 2011; 16: 305-310
- 32 Peterson JA, Farland JG, Curtis BR et al. Neonatal alloimmune thrombocytopenia: pathogenesis, diagnosis and management. Br J Haematol 2013; 16: 3-14
- 33 Kamphuis MM, Paridaans NP, Porcelijn L et al. Incidence and consequences of neonatal alloimmune thrombocytopenia: a systematic review. Pediatrics 2014; 133: 715-721
- 34 Roberts JA, Murray NA. Neonatal thrombocytopenia. Cur Hematol Rep 2006; 5: 55-63
- 35 Ferrer-Marin F, Lju ZJ, Gutti R et al. Neonatal thronbocytopenia and megakaryocytopoiesis. Semin Hematol 2010; 47: 281-288
- 36 Castle V, Andrew M, Kelton J et al. Frequency and mechanism of neonatal thrombocytopenia. J Pediatr 1986; 108: 749-755
- 37 Sola-Visner M, Saxonhouse MA, Brown RE. Neonatal thrombocytopenia: what we do and dont’t know. Early Hum Dev 2008; 84: 499-506
- 38 Katz JA, Moake JL, McPherson PD et al. Relationship between human development and disappearance of unusually large von Willebrand factor multime rs from plasma. Blood 1989; 73: 1851-1858
- 39 Schlegel N, Bardet V, Kenet G et al. Diagnostic and therapeutic considerations on inherited platelet disorders in neonates and children. Klin Padiatr 2010; 222: 209-214
- 40 Halimeh S, Angelis G, Sander A et al. Multiplate whole blood impedance point of care aggregometry: preliminary reference values in healthy infants, children and adolescents. Klin Padiatr 2010; 222: 158-163
- 41 Sandrock-Lang K, Oldenburg J, Wiegering V et al. Characterisation of patients with Glanzmann thrombasthenia and identification of 17 novel mutations. Thromb Haemost 2015; 113: 782-791
- 42 Khair K, Liessner R. Bruising and bleeding in infants and children – a practical approach. Br J Haematol 2006; 133: 221-231
- 43 Brady KM, Easley RB, Tobias JD. Recombinant activated factor VII (rFVIIa) treatment in infants with hemorrhage. Ped Anest 2006; 16: 1042-1046
- 44 Brettlet D, Levine P. Clinical manifestations and therapy of inherited coagulation factor deficiencies. In: Colman RW, Hirsh J, Marder VJ, et al., eds. Hemostasis and Thrombosis: Basic Principles and clinical practice. Philadelphia: J.B Lippincott; 1999: 169-183
- 45 Minford A, Behnisch W, Brons P et al. Subcutaneous protein C concentrate in the management of severe protein C deficiency – experience from 12 centres. Br J Haematol 2014; 164: 414-421
- 46 Kitchen S, Olson JD, Preston FE. Quality in Laboratory Hemostasis and Thrombosis. Hoboken: Wiley-Blackwell; 2009
- 47 Manner D, Kurnik K, Junker R et al. thromboermbolische Ereignisse im Kindes- und Jugendalter. Pädiatrie up2date 2010; 1: 1-16