Emergency department visits in children with hemophilia

Division of Hematology/Oncology, Carman Ann Adams Children's Hospital of Michigan, Wayne State University, Detroit, Michigan. .
Pediatric Blood & Cancer (Impact Factor: 2.39). 07/2013; 60(7). DOI: 10.1002/pbc.24401
Source: PubMed


The pediatric emergency department (ED) management of bleeding and other complications of hemophilia constitutes an increasingly important component of hemophilia therapy. This retrospective study examined the overall ED use by children with hemophilia in a single center, with a particular aim to investigate visits related to injury or bleeding, and those related to blood stream infection in patients with a central venous catheter (CVC).

Electronic medical records of patients with hemophilia presenting to Children's Hospital of Michigan ED were reviewed. Different categories of ED visits over a 5-year period (January 2006-December 2010) were examined.

There were 536 ED visits from 84 male patients (median age 4 years, range 0-21) with hemophilia over the 5-year period. The reasons for ED visits were: injury or bleeding (61.2%); suspected CVC-related infection (11.8%); causes unrelated to hemophilia (19.2%); and routine clotting factor infusion (7.8%). Eighteen visits from six patients were secondary to injury or bleeding in a patient not yet diagnosed with hemophilia. An intracranial hemorrhage was detected in five visits. Overall, 5.4% of all visits represented distinct episodes of bloodstream infection.

The pediatric ED is an indispensable component of the overall hemophilia care, because: (1) patients with potentially lethal problems such as ICH or CVC-related infection may present to the ED for their initial management; (2) previously undiagnosed patients with hemophilia may also present to the ED for their first bleeding episodes, initiating the diagnostic investigations; (3) the ED provides after-hours treatment service for many episodes of injury or bleeding, and also for clotting factor infusion.

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    ABSTRACT: Hemophilia A is a rare inherited bleeding disorder resulting from factor VIII deficiency and is a group of diseases characterized by intra-articular and intramuscular bleeding. In this study, we aimed to retrospectively evaluate the treatment outcomes, demographic and clinical characteristics of our patients who were treated and followed up for last 17 years in our pediatric hematology unit with a diagnosis of Hemophilia A. The medical records of 83 patients who were diagnosed with Hemophilia A and followed up between 1997 and 2014 in our hospital's pediatric hematology clinic were reviewed retrospectively. The demographic data, prophylaxis state, development of inhibitors and clinical characteristics of the patients were evaluated. When the complaints at presentation were examined, it was found that 27 (32%) patients had hemarthrosis, 24 (29%) patients had ecchymosis and hematoma, 13 (16%) patients had prolonged bleeding after trauma or cut, 10 (12%) patients had gingival, mouth or nose bleeding, 4 (5%) patients had prolonged bleeding after circumcision, 4 (5%) patients had gastrointestinal bleeding, 1 (1%) patient had hematuria. Fifty (60%) patients were considered severe hemophilia A, 20 (24%) patients were considered moderate hemophilia A and 13 (16%) patients were considered mild hemophilia A according to factor activity. Among severe hemophilia A patients, primary prophylaxis was being administered in 2 (2%) patients and secondary prophylaxis was being administered in 40 (48%) patients. Inhibitor positivity was found in 8 (10%) of these patients. It is found that hemophilic artropathy developed in 17 patients and 8 of these 17 patients had undergone radioisotope synovectomy. Treatment of severe bleeding in hemophilia A patients should be performed in hospital and the presence of inhibitor must be investigated in cases of uncontrolled bleeding where adequate doses of factor concentrates have been administered for treatment. In order to decrease the development of inhibitor, prophlaxis should be suggested to patients rather than repetetive treatment when bleeding occurs. The radioactive synovectomy should not be overlooked in countries like ours in which factors can not be used adequately.
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