To describe the in-hospital epidemiology of haemophilia A and B in the US we analysed the National Inpatient Sample (NIS), a stratified probability sample of 20% of all hospital discharges in the US for the year 2007. We applied sampling weights to represent all hospital discharges for haemophilia A and B identified using ICD-9 codes 286.0 and 286.1, respectively. Haemophilia (A or B) was one of all the listed diagnoses in 9737 discharges and principal diagnosis in 1684 discharges. The most common associated diagnoses in discharges with Haemophilia in adults and children were hypertension (28.1 ± 1.6%) and central line infections (15.2 ± 1.8%) respectively. No Hepatitis C or HIV was reported in children. Among 212 deaths, associated diagnoses included sepsis (37.9%), heart failure (30.2%), respiratory failure (28.3%), pneumonia (24.5%), HIV (14.2%), hepatic coma (5.2%) and intracranial haemorrhage (2.3%). All fifteen reported paediatric deaths occurred on day zero of life, the commonest associated diagnoses being Intraventricular haemorrhage and newborn haemorrhage-NOS (33% each). Median age of in-hospital mortality for diagnosis of Haemophilia was 68.3 years as compared to 72.3 years for all males for all hospitalizations in NIS combined. Mean hospital charges for haemophilia of $76823 ± 5530 were significantly higher than those from all causes of hospitalization of $26,120 ± 562. In-hospital mortality is rare in children with haemophilia beyond the neonatal period and age of mortality in adults is approaching that of the general male population. Hospitalization in children is most often due to central line infections and hospitalization and death in adults is primarily due to age-related illnesses.
[Show abstract][Hide abstract] ABSTRACT: With advances in care, increasing numbers of people with hemophilia achieve near-normal life expectancies and present with typical age-related cardiovascular conditions. Evidence-based guidelines for medical or surgical management of cardiovascular conditions in individuals with hemophilia are limited. Published recommendations exist for the management of some common cardiovascular conditions (e.g., ischemic heart disease, atrial fibrillation), but identifying optimal strategies for anticoagulant or antithrombotic therapy constitutes the primary challenge of managing non-operative cardiovascular disease in people with hemophilia. In general, as long as factor concentrates or other hemostatic therapies maintain adequate hemostasis, the recommended medical and surgical management of cardiovascular disease in people with hemophilia parallels that in individuals without hemophilia. The presence of factor inhibitors complicates hemophilia management. Published outcomes of cardiovascular disease treatment in people with hemophilia are similar to those in the general population. Specific knowledge about factor replacement, factor inhibitors, and disease-specific treatment distinguishes the cardiovascular care of people with hemophilia from similar care of individuals without this rare bleeding disorder. Furthermore, a multidisciplinary approach incorporating a hematologist with an onsite coagulation laboratory, ideally associated with a hemophilia treatment center, is integral to the management of cardiovascular disease in people with hemophilia.This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License, where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially. http://creativecommons.org/licenses/by-nc-nd/3.0. American Society of Plastic Surgeons.
Cardiology in Review 11/2014; 23(2). DOI:10.1097/CRD.0000000000000045 · 2.41 Impact Factor
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