<abstract xmlns="http://www.w3.org/1999/xhtml"><sec><h3>Introduction</h3><p>Haemophilia is a rare hereditary haemorrhagic disease caused by coagulation factor VIII (haemophilia A) or IX (haemophilia B) deficiency. Very few data exist on this disease in Congo. This survey aims to describe the epidemiological and clinical aspects of the children affected.</p></sec><sec><h3>Materials and methods</h3><p>A descriptive cross-sectional study was carried out in the haematology department of the Brazzaville University Hospital over a period of two years. Children (under 18 years of age) with haemophilia and with a factor VIII or IX level less than or equal to 30% were identified. The parameters analysed included age, diagnostic delay, type and severity of haemophilia, type and frequency of bleeding manifestations, complications and history of transfusion.</p></sec><sec><h3>Results</h3><p>Nineteen patients were identified with an average age at diagnosis of four years. The average time to diagnosis was six years, and the most frequent first known bleeding episode was haemorrhage during circumcision. Family history was found in 14 cases. There were 13 cases of haemophilia A and six cases of haemophilia B. Fourteen cases were severe haemophilia; no mild cases were identified. Haemorrhagic manifestations included haemarthrosis, haematomas and mucocutaneous haemorrhages. The average number of haemorrhagic episodes per year was 12. Haemophilic arthropathy was present at diagnosis in seven cases, with the main location being the knee. The average number of hospitalisations before diagnosis was two. Sixteen patients had been transfused at least once.</p></sec><sec><h3>Conclusion</h3><p>Although circumcision is the most frequent first known haemorrhagic manifestation of haemophilia in Congo, patients are often diagnosed late, sometimes with severe osteoarticular complications. Further measures are needed to help ensure early diagnosis and improve care.</p></sec></abstract>

IntroductionHaemophilia is a rare hereditary haemorrhagic disease caused by coagulation factor VIII (haemophilia A) or IX (haemophilia B) deficiency. Very few data exist on this disease in Congo. This survey aims to describe the epidemiological and clinical aspects of the children affected.Materials and methodsA descriptive cross-sectional study was carried out in the haematology department of the Brazzaville University Hospital over a period of two years. Children (under 18 years of age) with haemophilia and with a factor VIII or IX level less than or equal to 30% were identified. The parameters analysed included age, diagnostic delay, type and severity of haemophilia, type and frequency of bleeding manifestations, complications and history of transfusion.ResultsNineteen patients were identified with an average age at diagnosis of four years. The average time to diagnosis was six years, and the most frequent first known bleeding episode was haemorrhage during circumcision. Family history was found in 14 cases. There were 13 cases of haemophilia A and six cases of haemophilia B. Fourteen cases were severe haemophilia; no mild cases were identified. Haemorrhagic manifestations included haemarthrosis, haematomas and mucocutaneous haemorrhages. The average number of haemorrhagic episodes per year was 12. Haemophilic arthropathy was present at diagnosis in seven cases, with the main location being the knee. The average number of hospitalisations before diagnosis was two. Sixteen patients had been transfused at least once.ConclusionAlthough circumcision is the most frequent first known haemorrhagic manifestation of haemophilia in Congo, patients are often diagnosed late, sometimes with severe osteoarticular complications. Further measures are needed to help ensure early diagnosis and improve care.

Clinical profile of children with haemophilia at the University Hospital of Brazzaville

Faculty of Health Sciences, Marien Ngouabi University of Brazzaville

The Journal of Haemophilia Practice

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

IntroductionHaemophilia is a rare hereditary haemorrhagic disease caused by coagulation factor VIII (haemophilia A) or IX (haemophilia B) deficiency. Very few...

	VARIABLE	NUMBER	FREQUENCY (%)
Gender	MaleFemale	190	100%0
Age group at diagnosis	< 1 year1–5 years>5 years	3313	15.8%15.8%68.4%
Type of haemophilia	AB	136	68.4%31.6%
Severity of haemophilia	MildModerateSevere	0514	0%26.3%73.7%
First known abnormal bleeding	Haemorrhage after circumcisionHaematomaOral haemorrhage	1261	63.2%31.6%5.2%
Circumstance of diagnosis	HaemorrhageSystematic screening	172	89.5%10.5%
Previous history of blood transfusion	YesNo	163	89.5%10.5%
Arthropathy at diagnosis	YesNo	712	36.8%63.2%

Clinical profile of children with haemophilia at the University Hospital of Brazzaville

Article Category: Clinical Research

Published Online: Jun 17, 2020

Page range: 53 - 58

DOI: https://doi.org/10.17225/jhp00159

Keywords
Children, haemophilia, circumcision, haemorrhage, arthropathy, Congo

© 2020 FO Galiba Atipo Tsiba et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Figure 1

Figure 2

Parameters related to children diagnosed with haemophilia in the Haematology Department of Brazzaville University Hospital from June 2017 to May 2019

Clinical profile of children with haemophilia at the University Hospital of Brazzaville

Article Category: Clinical Research

Published Online: Jun 17, 2020

Page range: 53 - 58

DOI: https://doi.org/10.17225/jhp00159

KeywordsChildren, haemophilia, circumcision, haemorrhage, arthropathy, Congo

© 2020 FO Galiba Atipo Tsiba et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Figure 1

Figure 2

Parameters related to children diagnosed with haemophilia in the Haematology Department of Brazzaville University Hospital from June 2017 to May 2019

Keywords
Children, haemophilia, circumcision, haemorrhage, arthropathy, Congo