Spectrum of Inherited Bleeding Disorders and the Clinical Severity of Hemophilia a at Kenyatta National Hospital

Abstract

IBDs are a heterogeneous group of disorders that occur globally though their prevalence varies among the different population groups, with the highest prevalence seen in developed countries. Limited information is available regarding these defects in developing countries with much of the scientific data coming from developed countries. Over the years there has been marked improvement in the diagnostic procedures for IBDs thus improving the clinicians’ ability to give accurate diagnosis and thus improved treatment and care. Describing the current epidemiological and clinical spectrum of Inherited Bleeding disorders in one of the largest Teaching and Referral Hospital in Kenya would offer valuable preliminary data and provide critical insights in understanding these defects in developing countries. Broad Objective To Describe the Clinical Spectrum of Inherited Bleeding Disorders in patients attending the Kenyatta National Hospital. Specific Objectives To Determine the Socio-Demographic features in patients with Inherited Bleeding Disorders attending the Kenyatta National Hospital. To Determine the Types of Inherited bleeding disorders in patients attending the Kenyatta National Hospital. To Determine the Clinical Presentations of Inherited bleeding disorders in patients attending the Kenyatta National Hospital. Methodology This was a retrospective descriptive study, describing the spectrum of IBD at the Kenyatta National Hospital. Patients records were extracted from the medical records department and included all patients suspected or diagnosed with IBD over a 7year period (2010 to 2017). Results Of the 140 medical records of patients, majority had Hemophilia 115 (82%) with HA having 106 (75.7%) cases and HB 9 (6.4%) cases with undiagnosed group 22 (15.8%) and one rare case of Congenital Afibrinogenemia was identified. Their ages ranged from 2 -56 and most were males 136 (97%). The patients locality was wide spread representing 21 out of 47 counties in Kenya. xvi The most frequent bleeding manifestation experienced was Hemarthrosis 88 (62.9%) and cutaneous bleeds 61 (43.6%). Conclusion HA accounted for the commonest IBD at the referral hospital with majority of patients ranging between the age of 11-20 years with a nationwide distribution. Recommendation: Targeted population-based studies (Haemophilia gene carriers, gynaecological bleeds; Menorrhagia, PPH) to comprehensively define the IBD spectrum