ANKMJ
Ankyra Medical Journal

Ankyra Medical Journal (AnkMJ), formerly known as the Journal of Translational and Practical Medicine, regularly publishes international quality issues in the field of Medicine in the light of current information.

eISSN: 3023-655X
Creative Commons CC BY-NC-ND 4.0 International License.
EndNote Style

Online Manuscript System

Index
Original Article
Intracranial hemorrhage in children with hemophilia
Abstract
Aims: Hemophilias are inherited bleeding disorders, in which the patients generally present with clinical complaints of hemarthrosis. Intracranial hemorrhage (ICH) is one of the severe bleeding types with the highest mortality and morbidity throughout childhood, as well as in patients with a diagnosis of hemophilia. Herein, a single-center experience of intracranial hemorrhage in children with hemophilia is presented.
Methods: The files and hospital records of the patients with the diagnosis of hemophilia who were followed up by the Pediatric Hematology and Oncology Department of Erciyes University between the years 1993-2022 were evaluated retrospectively.
Results: A total of 81 patients with hemophilia were evaluated. Among them, 9 patients developed ICH. All patients had severe disease. The mean age of incidence ICH was 2,6 months (4 days- 8,7 months). All the ICH episodes were observed within the first year of life. Four patients were diagnosed with the ICH episode initially. None of the patients had primary prophylaxis. The majority of them were admitted with neurological signs and symptoms. There was no ICH-related mortality in our study and all of the patients are being followed up in our department.
Conclusion: Intracranial hemorrhage remains important in patients with congenital bleeding disorders, especially in hemophilia; with difficulties in diagnosis, management, and treatment.

Keywords : Children, hemophilia, intracranial hemorrhage

Download Article


1. Sarangi SN, Acharya SS. Disorders of Coagulation. In:Lanzkowsky P, editor. Manual of pediatric hematology andoncology. 7th ed. San Diego, CA: Elsevier Academic Press;2022. pp 287-339.
2. Paola JD, Montgomery RR, Gill JC, Flood V. Hemophilia andvon Willebrand Disease. In: Nathan and Oskı’s Hematology andOncology of Infancy and Chıldhood. 8th ed. Philadelphia, CA:Elsevier Academic Press; 2015. pp 1028-1054.
3. Hemophilia Diagnosis and Treatment Guidelines. Version 1.2,September 2021. CA: Turkish Society of Hematology
4. Gök V, Ünal E. Comprehensive approach to hemophilia. JHealth Sci Med 2022; 5: 1199-206.
5. Kasper CK, Lin JC. Prevalence of sporadic and familialhaemophilia. Haemophilia 2007; 13: 90-2.
6. Atik T, Işık E, Onay H, et al. Factor 8 gene mutation spectrumof 270 patients with hemophilia a: identification of 36 novelmutations. Turk J Haematol 2020; 37: 145-53.
7. Richards M, Lavigne Lissalde G, Combescure C, et al. Neonatalbleeding in haemophilia: a European cohort study. Br JHaematol 2012; 156: 374-82.
8. Kulkarni R, Presley RJ, Lusher JM, et al. Complications ofhaemophilia in babies (first two years of life): a report fromthe Centers for Disease Control and Prevention Universal DataCollection System. Haemophilia 2017; 23: 207-14.
9. Zwagemaker AF, Gouw SC, Jansen JS, et al. Incidence andmortality rates of intracranial hemorrhage in hemophilia: asystematic review and meta-analysis. Blood 2021; 138: 2853-73.
10. Patiroglu T, Ozdemir MA, Unal E, et al. Intracranial hemorrhagein children with congenital factor deficiencies. Childs Nerv Syst2011; 27: 1963-6.
11. Ljung RC. Intracranial haemorrhage in haemophilia A and B.Br J Haematol 2007; 140: 378-84.
12. Bladen M, Main E, Khair K, Hubert N, Koutoumanou E, LiesnerR. The incidence, risk and functional outcomes of intracranialhaemorrhage in children with inherited bleeding disorders atone haemophilia center. Haemophilia 2016; 22: 556-63.
13. Witmer C, Presley R, Kulkarni R, Soucie JM, Manno CS,Raffini L. Associations between intracranial haemorrhage andprescribed prophylaxis in a large cohort of haemophilia patientsin the United States. Br J Haematol 2011; 152: 211-6.
14. Zanon E, Pasca S, Demartis F, et al. Intracranial Haemorrhagein Haemophilia Patients Is Still an Open Issue: The Final Resultsof the Italian EMO.REC Registry. J Clin Med 2022; 11: 1969.
15. Fink S, Kunzmann S, Andres O, Eyrich M, Wiegering V.Haemophilia in extreme prematüre infants: increased risk forintracranial haemorrhage? J Matern Fetal Neonatal Med 2014;27: 621-4.
16. Davies J, Kadir RA. Mode of delivery and cranial bleeding innewborns with haemophilia: a systematic review and meta-analysis of the literature. Haemophilia 2016; 22: 32-8.
17. Andersson NG, Chalmers EA, Kenet G, et al. Mode of deliveryin hemophilia: vaginal delivery and cesarean section carrysimilar risks for intracranial hemorrhages and other majorbleeds. Haematologica 2019; 104: 2100-6.
18. Moreira A, Das H. Acute life-threatening hemorrhage inneonates with severe hemophilia a: a report of 3 cases. J InvestigMed High Impact Case Rep 2018; 6: 2324709618800349.
19. Bayoumi MAA, Khider WM, Elmalik EE. Congenitalhaemophilia A presenting with subgaleal and intracranialhaemorrhage following instrumental delivery. BMJ Case Rep2022; 15: e248030.
20. Samur BM, Samur TG, Çiflikli FE, et al. Evaluation of primarycare physicians' approaches to hemophilia and bleedingdisorders: a questionnaire survey. Blood Coagul Fibrinolysis2022; 33: 381-8.
21. Tabibian S, Motlagh H, Naderi M, Dorgalaleh A. Intracranialhemorrhage in congenital bleeding disorders. Blood CoagulFibrinolysis 2018; 29: 1-11.
22. Zanon E, Iorio A, Rocino A, et al. Intracranial haemorrhage inthe Italian population of haemophilia patients with and withoutinhibitors. Haemophilia 2012; 18: 39-45.
23. Ljung RC. Intracranial haemorrhage in haemophilia A and B.Br J Haematol 2008; 140: 378-84.
24. Nagel K, Pai MK, Paes BA, Chan AK. Diagnosis and treatmentof intracranial hemorrhage in children with hemophilia. BloodCoagul Fibrinolysis 2013; 24: 23-7.
25. Kracht MJ, Stein A, Knoll C, et al. A proposed algorithm forevaluation and management of pediatric hemophilia patientswho present to the emergency department with head trauma.Pediatr Blood Cancer 2022; 69: e29811.
26. Aras M, Oral S. Management of intracranial hemorrhage inhemophilia A patients. Childs Nerv Syst 2020; 36: 2041-6.
27. Andersson NG, Wu R, Carcao M, et al. Long-term follow-up of neonatal intracranial haemorrhage in children with severehaemophilia. Br J Haematol 2020; 190: e101-e104.
28. Rohaert C, Labarque V. Spontaneous intracranial haemorrhageas initial presentation of haemophilia in infants and children: Acase report and systematic literature review. Haemophilia 2021;27: e398-e401.
Article available in :
Volume 1, Issue 3, 2022
Page : 85-88
https://doi.org/10.51271/JTPM-0024
Article Information
Received : 16 Kas 2022
Accepted : 13 Ara 2022
Published : 26 Ara 2022
Corresponding Author :

Şefika Akyol: Division of Pediatric Hematology and Oncology, Department of Pediatrics, Faculty of Medicine, Erciyes University, Kayseri, Turkiye

[email protected]
Citation : Akyol Ş, Koçak Gül D, Yılmaz E, Karaman ZF, Özcan A, Küçük A, Gök V, Aydın F, Per H, Karakükcü M, Patıroğlu T, Özdemir MA, Ünal E. Intracranial hemorrhage in children with hemophilia. J Transl Pract Med. 2022;1(3): 85-88.

Read: 354
Cited: 2
Download : 243
_Footer