Abstract
Background: Hemophilia, a constitutional bleeding disease, has always been present in Madagascar considering children who died after bleeding circumcision, as reported in the family history of the diagnosed patients. Hemophilia is serious because of the potentially fatal risk of hemorrhage. The aim of this study was to evaluate the clinical, outcome and therapeutic aspects of inpatients with hemophilia in the Surgical Resuscitation Unit of JRA Hospital in Antananarivo. A descriptive and observational study was led about patients with hemophilia cared in the Surgical Resuscitation Unit from January 2011 to March 2018, studying age, type and severity of hemophilia, reason and duration of hospitalization, treatment instituted and outcome of patients. Results and comments: Thirty-six hemophiliacs (0.2%) were enrolled. The mean age was 9.52 years old; 52.78% were with hemophilia B and 47.22% with hemophilia A, mainly severe. Clinical manifestations were muscle hematomas (25.71%), gum bleeding (14%), epistaxis (14.28%), gastrointestinal bleeding (11.42%), intracranial hemorrhage (11.42%), post circumcision bleeding (11.42%), hematuria, intraperitoneal hematomas and hemarthrosis. Treatment was based on factor concentrate substitution when available. The length of stay ranged from one to thirty days. The evolution was favorable except for two deaths related to delayed management of intracranial hemorrhage. Three patients with hemophilia A developed inhibitors. The results showed that throughout these years of study, a change in management was noted alongside. Conclusion: Hemophilia cases requiring hospitalization were managed in surgical resuscitation unit. The evolution was mainly related to the availability of clotting factor concentrates in coagulation factors, the delay in taking care of the patients and the presence of specialized staff.
Highlights
Blood has always been linked to life; one cannot live without blood
Clinical manifestations were muscle hematomas (25.71%), gum bleeding (14%), epistaxis (14.28%), gastrointestinal bleeding (11.42%), intracranial hemorrhage (11.42%), post circumcision bleeding (11.42%), hematuria, intraperitoneal hematomas and hemarthrosis
Of the 14,658 patients hospitalized in the Surgical Resuscitation Unit during the study period, 36 (0.27%) obviously male patients were with hemophilia
Summary
Blood has always been linked to life; one cannot live without blood. Blood losses are provoked; there are those related to blood abnormalities. Hemophilia is one of them, a constitutional hemorrhagic disease, serious due to risk of fatal hemorrhage, genetically transmitted in a recessive mode linked to the X chromosome This genetic abnormality, resulting in a deficiency of a coagulation factor VIII for hemophilia A or coagulation factor IX for hemophilia B, has not spared Madagascar [1]. Its prevalence is not completely known in Africa for several reasons: rarity of the disease, high cost of its management, insufficient number of specialists in hematology and absence of adequate laboratories for the biological diagnosis of this disease It is, established that hemophilia is a ubiquitous condition with an annual incidence of 1 in 5000 male births and an estimated population prevalence of 1 in 10,000 to 12,000 regardless of race or geographical area [2] [3].
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