Study of bleeding disorders (hemophilia) in children in Beni-Suef university hospital

Document Type : Original Article

Authors

1 Depatrement of Pediatrics, Faculty of Medicine, Beni-Suef University, Egypt

2 Depatrement of Clinical and Chemical Pathology, Faculty of Medicine, Beni-Suef University, Egypt.

Abstract

The goal of this study is to study the clinical spectrum of hemophiliac children (age <18 years) in Beni-Suef university hospital. A cross-sectional study conducted in the pediatric hematology clinic of Beni-Suef university hospital, over a period of 12 months. Out of a total of 112 cases enrolled, 91% were diagnosed as hemophilia A. While (9%) were diagnosed as hemophilia B. Positive family history was found in (46%) of cases. The mean age of onset of symptoms and diagnosis was 1.7 ± 1.4 and 3.8 ± 3.8 years, respectively. ), the most common bleeding symptom was bruises (86.5), the most serious was intracranial hemorrhage (9.6%). While haemarthrosis was in (7.7%).Conclusion: Occurrence of posttraumatic bleeds and gum bleeds in an otherwise normal child should warn the clinician for evaluation of hemophilia. Hemophilias are distributed worldwide and have heterogeneous presentation depending upon the disease severity. Knowledge of the spectrum of presentation of hemophilia in the local population helps in early diagnosis and planning of management.

Keywords

Main Subjects

References

  1. Bolton-Maggs PH, Pasi KJ. Haemophilias A and B. Lancet. 2003; 361:1801–9.
  2. Biggs R, Douglas AS, Macfarlane RG, Dacie JV, Pitney WR, Merskey C. Christmas disease: A condition previously mistaken for haemophilia. Br Med J. 1952; 2:1378–82.
  3. Mannucci PM, Tuddenham EG. The hemophilias - From royal genes to gene therapy. N Engl J Med. 2001; 344:1773–9.
  4. White GC, 2nd, Rosendaal F, Aledort LM, Lusher JM, Rothschild C, Ingerslev J. Factor VIII and Factor IX Subcommittee. Definitions in hemophilia. Recommendation of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost. 2001; 85:560.
  5. Kar A, Mirkazemi R, Singh P, Potnis-Lele M, Lohade S, Lalwani A, et al. Disability in Indian patients with haemophilia. Haemophilia. 2007; 13:398–404.
  6. Skinner MW, Lillicrap DP, McMillan J, Castro Ozelo M, Pierce GF. What is a cure and how do we get there? Haemophilia. 2004; 10(Suppl 4):115–8.
  7. Mammen J, Nair SC, Srivastava A. External quality assessment scheme for hemostasis in India. Semin Thromb Hemost. 2007; 33:265–72.
  8. Uddin MM, Rahman MJ, Rahman MM, Sultana SA, Shah MS. Clinico-pathological study on haemophilia: An analysis of 50 cases. J Bangladesh Coll Phys Surg. 2006; 24:50–3.
  9. Karim MA, Siddique R, Jamal CY, Islam A. Clinical profile of haemophilia in children in a tertiary care hospital. Bangladesh J Child Health. 2013; 37:90–6.
  10. Kar A, Potnis-Lele M. Descriptive epidemiology of haemophilia in Maharashtra, India. Haemophilia. 2001; 7:561–7.
  11. Ling SC, Chan GC, Shing MM, Yuen HL, Lee AC, Chan CW, et al. Children and Adolescents with haemophilia in Hong Kong: An epidemiological and clinical review. HK J Paediatr. 2006; 11:13–9.
  12. Soucie JM, Evatt B, Jackson D. Occurrence of hemophilia in the United States. The Hemophilia Surveillance System Project Investigators. Am J Hematol. 1998; 59:288–94.
  13. Mohsin S, Saeed T, Hussain S, Mahmood S, Sohail S. Clinical manifestations and complications of haemophilia A in Pakistan. Ann Pak Inst Med Sci. 2010; 6:168–71.
  14. Hazewinkel MH, Hoogerwerf JJ, Hesseling PB, Hartley P, MacLean PE, Peters M, et al. Haemophilia patients aged 0-18 years in the Western Cape. S Afr Med J. 2003; 93:793–6.
  15. Rodgers MG, Greenburg SC. Wintrobe's Clinical Haematology. 10th ed. Baltimore: Wilkins; 1998. Inherited coagulation disorders; pp. 1683–732.
  16. Kim KY, Yang CH, Cho MJ, Lee M. Comprehensive clinical and statistical analysis of hemophilia in Korea. J Korean Med Sci. 1988; 3:107–15.
  17. Zafar T, Ikram N, Amanat S, Zafar A, Hassan K. Clinico hematological spectrum of haemophilia. J Rawalpindi Med Coll. 2006; 10:54–60.
  18. Tonbary YA, Elashry R, Zaki Mel S. Descriptive epidemiology of hemophilia and other coagulation disorders in mansoura, egypt: Retrospective analysis. Mediterr J Hematol Infect Dis. 2010; 2:e2010025.
  19. Qasim Z, Naseem L, Asif N, Hassan K. Haemophilia; pattern of clinical presentation and disease severity. Int J Pathol. 2013; 11:58–63.
  20. Handelsman JE. The knee joint in hemophilia. Orthop Clin North Am. 1979; 10:139–73.
  21. Ljung RC. Intracranial haemorrhage in haemophilia A and B. Br J Haematol. 2008; 140:378–84.
  22. Klinge J, Auberger K, Auerswald G, Brackmann HH, Mauz-Körholz C, Kreuz W. Prevalence and outcome of intracranial haemorrhage in haemophiliacs - A survey of the paediatric group of the German Society of Thrombosis and Haemostasis (GTH) Eur J Pediatr. 1999; 158(Suppl 3):S162–5.
  23. Kulkarni R, Lusher JM. Intracranial and extracranial hemorrhages in newborns with hemophilia: A review of the literature. J Pediatr Hematol Oncol. 1999; 21:289–95.
  24. Kulkarni R, Soucie JM, Lusher J, Presley R, Shapiro A, Gill J, et al. Sites of initial bleeding episodes, mode of delivery and age of diagnosis in babies with haemophilia diagnosed before the age of 2 years: A report from The Centers for Disease Control and Prevention's (CDC) Universal Data Collection (UDC) project. Haemophilia. 2009; 15:1281–90.
  25. Richards M, Fijnvandraat K, Lavigne-Lissalde G. A European study of major neoanatal haemorrhage in haemophilia. J Thromb Haemost. 2009; 7(Suppl 2):S99.
  26. Tarantino MD, Gupta SL, Brusky RM. The incidence and outcome of intracranial haemorrhage in newborns with haemophilia: Analysis of the Nationwide Inpatient Sample database. Haemophilia. 2007; 13:380–2.
  27. Andrew M, Paes B, Milner R, Johnston M, Mitchell L, Tollefsen DM, et al. Development of the human coagulation system in the full-term infant. Blood. 1987; 70:165–72.
  28. Andrew M, Paes B, Milner R, Johnston M, Mitchell L, Tollefsen DM, et al. Development of the human coagulation system in the healthy premature infant. Blood. 1988; 72:1651–7.
  29. Conway JH, Hilgartner MW. Initial presentations of pediatric hemophiliacs. Arch Pediatr Adolesc Med. 1994; 148:589–94.
  30. Jamil I, Bayoumy M, Iran D, Adler B. Pediatric severe haemophilia: Initial presentation, characteristics and complications. Internet J Hematol. 2004; 1:1–5.
  31. Mansour-Ghanaei F, Fallah MS, Shafaghi A, Yousefi-Mashhoor M, Ramezani N, Farzaneh F, et al. Prevalence of hepatitis B and C seromarkers and abnormal liver function tests among hemophiliacs in Guilan (northern province of Iran) Med Sci Monit. 2002; 8:CR797–800.
  32. Sharifi-Mood B, Eshghi P, Sanei-Moghaddam E, Hashemi M. Hepatitis B and C virus infections in patients with hemophilia in Zahedan, southeast Iran. Saudi Med J. 2007; 28:1516–9. Mittal M, Zaman S, Bhatnagar N, Gajjar M. Transfusion transmitted infections in patients with hemophilia: A study from a tertiary care hospital in Western India. Internet J Infect Dis. 2013; 12:1–4.
Egyptian Journal of Medical Research
Volume 3, Issue 4
October 2022
Pages 254-261

Files

Share

How to cite

Statistics