Abstract Number: PB0581
Meeting: ISTH 2021 Congress
Background: Intracranial haemorrhage (ICH) is one of the potential life threatening complications in person with haemophilia (PAH) that can result in long‐term morbidity. There are very few reports in Africa and Egypt especially in children.
Aims: Our study will assess the prevalence, risk factors and outcome of ICH in PAH.
Methods: This is a retrospective study on all PAH from 2006 to 2021 .We collected data on all children who had presented with ICH to Cairo University Paediatric Hospital. ICH was confirmed by clinical and computed tomographic imaging data. Clinical data were extracted at baseline and during the follow-up.
Results: 18/ 250 (6.8%) PWH (15 HA, 12 severe whether by factor activity and/or phenotypically, 1 moderate, 2 mild; 3HB, 1 mild, 2 severe) with a mean age of 9. 4 years who developed cerebral bleeds at a mean age of 3 years were enrolled. 58.8% were a product of consanguineous marriage. ICH was the initial presenting symptom in 33.3%. 6 children had inhibitors (3 high titre) that nearly all developed after the onset of the cerebral bleed. Symptoms of ICH included pallor in 44.4%, vomiting in 55.6%, seizures and disturbed consciousness level in 77.8% and squin t in 11%. Our studied cohort developed 23 episodes of cerebral bleeds mainly post-traumatic (70%), including 15 subdural, 7 intracerebral/intraventricular, and 1 brainstem. Mortality and morbidity is usually associated with late presentation to hospital 82% were managed conservatively with replacement therapy for 1 to 3 weeks. 22.2% died of severe brain insult, 15.4% developed a cognitive impairment with hydrocephalic changes and visual impairment in one, 1 squint. Eight children are currently on biweekly secondary prophylaxis.
Conclusions: ICH is not a rare complication in our paediatric Egyptian cohort with an unfavourable outcome. Young age, lack of primary prophylaxis and trauma are the risk factors in our cohort.
To cite this abstract in AMA style:Abdelwahab M. Intracranial Haemorrhage (ICH) in Egyptian Children with Haemophilia A and B: A 15 Year Retrospective Single Center Study [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 1). https://abstracts.isth.org/abstract/intracranial-haemorrhage-ich-in-egyptian-children-with-haemophilia-a-and-b-a-15-year-retrospective-single-center-study/. Accessed September 16, 2021.
« Back to ISTH 2021 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/intracranial-haemorrhage-ich-in-egyptian-children-with-haemophilia-a-and-b-a-15-year-retrospective-single-center-study/