Ponad 7000 publikacji medycznych!
Statystyki za 2021 rok:
odsłony: 8 805 378
Artykuły w Czytelni Medycznej o SARS-CoV-2/Covid-19

Poniżej zamieściliśmy fragment artykułu. Informacja nt. dostępu do pełnej treści artykułu tutaj
© Borgis - New Medicine 4/2012, s. 99-101
*Anna Gorzelnik1, Monika Jabłońska-Jesionowska1, Lidia Zawadzka-Głos1, Anna Klukowska2
Perioperative management of children with von Willebrand disease and tonsillar hypertrophy in ENT Department of Children Hospital in Warsaw Medical University
1Department of Pediatric Otolaryngology, Medical University of Warsaw, Poland
Head of Department: Lidia Zawadzka-Głos, MD, PhD
2Department of Pediatrics, Hematology and Oncology, Medical University of Warsaw, Poland
Head of Department: prof. Michał Matysiak, MD, PhD
Summary
Introduction. The tonsillar hypertrophy are the most common children’s disease. The treatment of choice is surgery. Regardless of surgery procedure perioperative bleeding is still present. The problem with homeostasis occurs in 0.5-2 percent of cases due to bleeding disorders. The most common inherited coagulopaties is von Willebrand disease (vWD). vWD commonly presents with epistaxis, excessive bleeding following soft tissue trauma, ecchymosis. Three type of vWD have been described. The treatment of hemorrhage in patients with vWD is i.v. infusion of Desmopressin (Minirin), concentrate containing factor VIII/vWF or antifibrinolytic drugs.
Material and methods. From January 2008 to December 2011 in the Department of Pediatric Otholaryngology of Medical University of Warsaw we treated by surgery 16 children with von Willebrand disease because of the tonsillar hypertrophy.
Results. 8 children with vWD and tonsillar hypertrophy underwent adenoidectomy and 8 adenoidotonsillotomy. Von Willebrand disease has been recognized because of clinical symptoms and blood tests in preoperative time. We treated by surgery 14 children with type 1 and 2 children with type 2 of vWD. 14 children has received Desmopressin (Minirin) intravenously and 2 children has received factor VIII-vWF concentrate (HaemateP).
8 children has received etamsylat (Cyclonamina), all 16 children has received as antifibrinolytic therapy tranexamicum acidum (Exacyl).
Conclusions. Adenoidectomy with/or without tonsillotomy in children with von Willebrand disease seems to be safe procedure by using adequate treatment of haemostasis.
INTRODUCTION
The tonsillar hypertrophy are the most common children’s disease. The treatment of choice is surgery by adenoidectomy with/or without tonsillotomy. Regardless of surgery procedure perioperative bleeding is still present, but normally is stopped spontaneously. In 0.5-2 percent of cases we have a problem with homeostasis and some special treatment using haemostatic drugs has to be used. In 0.04 percent of patients the compatibility blood has to be transfused. The cause of severe hemorrhage is perioperative trauma of soft tissues in naso- or oropharynx, not complete excision of tonsils and haemorrhagic or vascular diathesis.
The most common inherited bleeding disorder is von Willebrand disease (vWD). 1% of general population has insufficiency or dysfunction of von Willebrand factor (vWF). Most of those deficiencies are clinically asymptomatic. Only in 1 of 10 cases of vWD the abnormal bleeding is observed. vWD is inherited in an autosomal -dominant often then autosomal-recessive pattern. vWF is encoded by a gen on chromosome 12 and synthesized in vascular endothelial cells and megacariocytes. The vWF has two haemostatic functions: preventing degradation of factor VIII and mediates platelet adhesion to damaged endothelium.
vWD commonly presents with epistaxis, excessive bleeding following soft tissue trauma, ecchymosis. Three type of vWD have been described. The most common is type 1 (75% of all) in them the bleeding is very mild, in type 2 bleeding is more excessive and in type 3 most severe due to absent of vWF. In type 1 the coagulopathy is characterized by partial quantitative vWF deficiency and in type 2 by qualitative defect in vWF. The treatment of hemorrhage in patients with vWD is i.v. infusion of Desmopressin (Minirin), concentrate containing factor VIII/vWF or antifibrinolytic drugs. The knowledge of the type of vWD is very important in preoperative care to plan surgery procedures or substitute treatment.
AIM
The aim of this study was to asses perioperative management of adenoidectomy or adenotonsillotomy in children with von Willebrand disease.
MATERIAL AND METHODS
From January 2008 to December 2011 in the Department of Pediatric Otholaryngology of Medical University of Warsaw we treated by surgery 16 children with von Willebrand disease. All of them has tonsillar hypertrophy. It was 5 girls and 11 boys in age from 3 to 12 year. We analyzed preoperative laboratory tests, surgery technique, perioperative treatment of haemostasis and postoperative complications.
RESULTS
All children with tonsillar hypertrophy and von Willebrand disease has been treated by surgery using La Force adenotome or Becman’s knife and tonsillotome. 8 children underwent adenoidectomy and 8 adenoidotonsillotomy. At the same time in 3 children lower level of XII factor has been diagnosed and in 1 child spherocytosis. Von Willebrand disease has been recognized because of clinical symptoms and blood tests in preoperative time. The activated partial thromboplastin time (aPTT) was from 30.2 to 61,8 seconds and was extended in 9 children. Activity of ristocetin cofactor of vWF (vWF:RCo) was from 36.12 to 54.2 and was decreased in all children, the level of antigen vWF (vWF:Ag) was from 38,4-52,8 and was decreased in 15 children. The activity of factor VIII was from 47,3 to 92.8 and was decreased in 1 child. The plates level was normal in all children, as well as the INR factor (0.98 to 1,23). 10 children has blood group “0”, 4 children “A” and 2 children “B”.

Powyżej zamieściliśmy fragment artykułu, do którego możesz uzyskać pełny dostęp.
Mam kod dostępu
  • Aby uzyskać płatny dostęp do pełnej treści powyższego artykułu albo wszystkich artykułów (w zależności od wybranej opcji), należy wprowadzić kod.
  • Wprowadzając kod, akceptują Państwo treść Regulaminu oraz potwierdzają zapoznanie się z nim.
  • Aby kupić kod proszę skorzystać z jednej z poniższych opcji.

Opcja #1

19

Wybieram
  • dostęp do tego artykułu
  • dostęp na 7 dni

uzyskany kod musi być wprowadzony na stronie artykułu, do którego został wykupiony

Opcja #2

49

Wybieram
  • dostęp do tego i pozostałych ponad 7000 artykułów
  • dostęp na 30 dni
  • najpopularniejsza opcja

Opcja #3

119

Wybieram
  • dostęp do tego i pozostałych ponad 7000 artykułów
  • dostęp na 90 dni
  • oszczędzasz 28 zł
Piśmiennictwo
1. Massimo F: Surgical prophylaxis in von Willebrand’s disease: a difficult balance to manager. Blood Transfus 2008; 6: 33-38. 2. Sadler JE, Mannucci PM, Berntorp E et al.: Impast, diagnosis and treatment of von Willebrand disease. Thromb Haemost 2000, 84: 160-174. 3. Chmielik M, Zając B: Operacje migdałków podniebiennych i migdałka gardłowego u dzieci – metody operacyjne i wskazania do zabiegów. Magazyn Otolaryngologiczny 2003; Supl. 4. 4. Klukowska A: Skazy krwotoczne u dzieci, Pediatria Ogólna; 19(3). 5. Klukowska A, Łaguna P, Skrzypczyk-Smalisz K, Koziołek R: Choroba von Willebranda u dzieci, Nowa Pediatria 2008, 3. 6. Janczarski M, Ruszkowska I, Cetnarowicz H, Łopaciuk S: Stosowanie dożylnie dezamino-d-arginino-wazopresyny (DDAVP) u chorych na hemofilię i chorobę von Willebranda. Acta Haematol Pol 1990: 21; 16-21. 7. Zdziarska J, Chojnowski K, Klukowska A et al.: Postępowanie w chorobie von Willebranda. Med Prakt 2008; 12: 1-24. 8. Lewandowski K, Barańska M: Aktualne możliwości diagnozowania choroby von Willebranda. Pol Arch Med Wewn 2004; 112: 1141-1149. 9. Feredici AB, Baundo F, Caracciolo C et al.: Clinical efficacy of highly purified doubly virus-inactivated factor VIII/von Willebrand factor concventrate in the treatment of Willebrand disease: a retrospective clinical study. Haemophilia 2002; 8: 761-767. 10. Bogusz M, Lewandowski K: Patofizjologia i leczenie choroby von Willebranda – aktualne poglądy. Adv Clin Exp Med 2004; 13: 677-687. 11. Kuczkowski J, Wlazłowski M, Narożny W et al.: Postępowanie okołooperacyjne w chorobie von Willebranda u dzieci. Otolaryngologia 2009; 8(4): 173-176. 12. Allen GC, Armfield DR, Bontempo FA et al.: Adenotonsillectomy in children with von Willebrand disease. Arch Otolaryngol Head Neck Surg 1999; 125: 547-551. 13. Laffan M, Brown SA, Collins PW et al.: The diagnosis of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctor’s Organization. Haemophilia 2004; 10: 199-217. 14. Pasi KJ, Collins PW, Keeling DM et al.: Management of von Willebrand disease; a guideline for the UK Haemophilia Centre Doctor’s Organization. Haemophilia 2004; 10: 218-231. 15. Ginsburg D: The molecular biology of von Willebrand disease. Haemophilia 1999; 5 (Suppl. 2): 19-27. 16. Ingerslev J: Von Willebrand factor, factor VIII and the factor VIII/von Willebrand factor complex. Laegerforeningens Forlag, Copenhagen 1990; 5-17. 17. Zwack, GC, Derkay CS: The utility of preoperative hemostatic assessment in adenotonsillectomy. Int J Pediatr Otorhinolaryngol 1997; 39: 67-76. 18. Lillicrap D: Von Willebrand disease – phenotype versus genotype: deficiency versus disease. Thrombosis Research 2007, 120: S11-S16.
otrzymano: 2012-10-08
zaakceptowano do druku: 2012-10-31

Adres do korespondencji:
*Anna Gorzelnik
Department of Pediatric Otolaryngology Medical University of Warsaw
24 Marszałkowska St., 00-576 Warsaw
tel./fax: +48 (22) 628-05-84
e-mail: laryngologia@litewska.edu.pl

New Medicine 4/2012
Strona internetowa czasopisma New Medicine