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. ...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.展开更多
文摘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.
