Research Article
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Year 2022, Volume: 5 Issue: 1, 22 - 25, 17.01.2022
https://doi.org/10.32322/jhsm.984860

Abstract

References

  • Sandoval C, Dong S, Visintainer P, Ozkaynak MF, Jayabose S. Clinical and laboratory features of 178 children with recurrent epistaxis. J Pediatr Hematol Oncol 2002; 47-9.
  • Recht M, Chitlur M, Lam D. Epistaxis as a common presenting symptom of Glanzmann’s thrombasthenia, a rare qualitative platelet disorder: illustrative case examples. Case Rep Emerg Med 2017; 2017: 8796425.
  • Natalia Rydz , Paula D James. The evolution and value of bleeding assessment tools. J Thromb Haemost 2012; 10: 2223-9.
  • Sarah H O'Brien. Bleeding scores: are they really useful? Hematology Am Soc Hematol Educ Program 2012; 2012: 152-6.
  • Bowman M, Riddel J, Rand ML, Tosetto A, Silva M, James PD. Evaluation of the diagnostic utility for von Willebrand disease of a pediatric bleeding questionnaire. J Thromb Haemost 2009; 7: 1418–21.
  • Bui J, Martyres D, James PD, et al. Validation of the school age self-administered pediatric bleeding questionnaire (Self-PBQ) in children aged 8-12 years. Pediatr Blood Cancer 2019; 66:e27709
  • Tosetto A, Rodeghiero F, Castaman G, et al. A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 VWD). J Thromb Haemost 2006; 4: 766-73.
  • Rojnuckarin P, Akkawat B, Intragumtornchai T. Von Willebrand factor (VWF) antigen levels and function in healthy Thais. Southeast Asian J Trop Med Public Health 2005; 36: 1292-7.
  • Macfarlane DE, Stibbe J, Kirby EP, Zucker MB, Grant RA, McPherson J. Letter: a method for assaying von Willebrand factor (ristocetin cofactor). Thromb Diath Haemorrh 1975; 34: 306-8.
  • Kitchen S, Gray E, Mertens K. Monitoring of modified factor VIII and IX products. Haemophilia 2014; 20: 36-42.
  • Nichols WL, Hultin MB, James AH, et al. Von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel Report (USA). Haemophilia 2008; 14: 171-232.
  • Leebeek F, Eikenboom J. Von Willebrand’s disease. N Engl J Med 2016; 375: 2067-80.
  • James PD, Connell NT, Ameer B, et al. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease. Blood Adv 2021; 5: 280-300.
  • Tosetto A, Rodeghiero F, Castaman G, et al. A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 VWD). J Thromb Haemost 2006; 4: 766-73.
  • Tosetto A, Castaman G, Rodeghiero F. Bleeding scores in inherited bleeding disorders: clinical or research tools? Haemophilia 2008; 14: 415-22.
  • Biss TT, Blanchette VS, Bowman M, et al. Quantification of bleeding symptoms in children with von Willebrand disease or a platelet function disorder: use of a standardized pediatric bleeding score. Blood 2007; 110: 636a.
  • Belen B, Kocak U, Isik M, et al. Evaluation of pediatric bleeding questionnaire in Turkish children with Von Willebrand disease and platelet function disorders. Clin Appl Thromb Hemost 2015; 21: 565-9.
  • Malec LM, Moore CG. Validation study of the composite score to identify Von Willebrand disease in children. J Pediatr Hematol Oncol 2016; 38: 139-42.

The predictive value of bleeding score on the diagnosis of Von Willebrand disease in children applied to the hematologic clinic with epistaxis

Year 2022, Volume: 5 Issue: 1, 22 - 25, 17.01.2022
https://doi.org/10.32322/jhsm.984860

Abstract

Aim: Epistaxis may be a symptom of an inherited bleeding disease.. We aimed to analyze an approved pediatric bleeding score (PBS) as a screening test for von Willebrand Disease (VWD) in children with epistaxis
Material and Method: We retrospectively reviewed the medical records of pediatric patients, who applied to the Pediatric Hematology Department with the complaint of epistaxis between January 2018 and December 2019.
Results: One hundred and sixty eight patients enrolled in this study There were 65(38.7%) girls and 103(61.3%) boys, with a mean age of 114±49 months (range 8 months to 18 years).The PBS of 34 patients was greater than/ or equal to 2. Factor 8, von Willebrand factor antigen, and von Willebrand Ristocetin cofactor levels were significantly lower in patients with PBS≥2 compared to those in patients with PBS<2 (%73±43 vs % 91±29, p=0.03; 87±44 vs 106±29 IU/dl, p=0.03; 72±39 vs 98±30 IU/dl, p=0.001, respectively). While 15 (44%) of 34 patients with PBS ≥ 2 diagnosed VWD, but in the group with PBS<2, VWD was diagnosed for only 4 children (0.02%) (4/134). The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of PBS for diagnosis of VWD was 79.0%, 87.2%, 44%, and 97% respectively.
Conclusion: PBS could be integrated into the evaluation of children suspected of having a bleeding disorder such as VWD in pediatrician’s offices. Our cut off value 2 appears to be significant in exclusion of VWD, since its high negative predictive value.

References

  • Sandoval C, Dong S, Visintainer P, Ozkaynak MF, Jayabose S. Clinical and laboratory features of 178 children with recurrent epistaxis. J Pediatr Hematol Oncol 2002; 47-9.
  • Recht M, Chitlur M, Lam D. Epistaxis as a common presenting symptom of Glanzmann’s thrombasthenia, a rare qualitative platelet disorder: illustrative case examples. Case Rep Emerg Med 2017; 2017: 8796425.
  • Natalia Rydz , Paula D James. The evolution and value of bleeding assessment tools. J Thromb Haemost 2012; 10: 2223-9.
  • Sarah H O'Brien. Bleeding scores: are they really useful? Hematology Am Soc Hematol Educ Program 2012; 2012: 152-6.
  • Bowman M, Riddel J, Rand ML, Tosetto A, Silva M, James PD. Evaluation of the diagnostic utility for von Willebrand disease of a pediatric bleeding questionnaire. J Thromb Haemost 2009; 7: 1418–21.
  • Bui J, Martyres D, James PD, et al. Validation of the school age self-administered pediatric bleeding questionnaire (Self-PBQ) in children aged 8-12 years. Pediatr Blood Cancer 2019; 66:e27709
  • Tosetto A, Rodeghiero F, Castaman G, et al. A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 VWD). J Thromb Haemost 2006; 4: 766-73.
  • Rojnuckarin P, Akkawat B, Intragumtornchai T. Von Willebrand factor (VWF) antigen levels and function in healthy Thais. Southeast Asian J Trop Med Public Health 2005; 36: 1292-7.
  • Macfarlane DE, Stibbe J, Kirby EP, Zucker MB, Grant RA, McPherson J. Letter: a method for assaying von Willebrand factor (ristocetin cofactor). Thromb Diath Haemorrh 1975; 34: 306-8.
  • Kitchen S, Gray E, Mertens K. Monitoring of modified factor VIII and IX products. Haemophilia 2014; 20: 36-42.
  • Nichols WL, Hultin MB, James AH, et al. Von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel Report (USA). Haemophilia 2008; 14: 171-232.
  • Leebeek F, Eikenboom J. Von Willebrand’s disease. N Engl J Med 2016; 375: 2067-80.
  • James PD, Connell NT, Ameer B, et al. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease. Blood Adv 2021; 5: 280-300.
  • Tosetto A, Rodeghiero F, Castaman G, et al. A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 VWD). J Thromb Haemost 2006; 4: 766-73.
  • Tosetto A, Castaman G, Rodeghiero F. Bleeding scores in inherited bleeding disorders: clinical or research tools? Haemophilia 2008; 14: 415-22.
  • Biss TT, Blanchette VS, Bowman M, et al. Quantification of bleeding symptoms in children with von Willebrand disease or a platelet function disorder: use of a standardized pediatric bleeding score. Blood 2007; 110: 636a.
  • Belen B, Kocak U, Isik M, et al. Evaluation of pediatric bleeding questionnaire in Turkish children with Von Willebrand disease and platelet function disorders. Clin Appl Thromb Hemost 2015; 21: 565-9.
  • Malec LM, Moore CG. Validation study of the composite score to identify Von Willebrand disease in children. J Pediatr Hematol Oncol 2016; 38: 139-42.
There are 18 citations in total.

Details

Primary Language English
Subjects Health Care Administration
Journal Section Original Article
Authors

Ayşegül Alpcan 0000-0001-9447-4263

Serkan Tursun 0000-0003-3354-6360

Yaşar Kandur 0000-0002-8361-5558

Mustafa Çağlar Yörgüç 0000-0001-5616-5603

Meryem Albayrak 0000-0003-2711-5150

Publication Date January 17, 2022
Published in Issue Year 2022 Volume: 5 Issue: 1

Cite

AMA Alpcan A, Tursun S, Kandur Y, Yörgüç MÇ, Albayrak M. The predictive value of bleeding score on the diagnosis of Von Willebrand disease in children applied to the hematologic clinic with epistaxis. J Health Sci Med / JHSM. January 2022;5(1):22-25. doi:10.32322/jhsm.984860

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