Document Detail

The orthopaedic status of severe haemophiliacs in Spain.
MedLine Citation:
PMID:  10792475     Owner:  NLM     Status:  MEDLINE    
This paper provides an outline of the results obtained in a cross-sectional study conducted primarily with the aim of ascertaining orthopaedic complications in a group of young severe A and B haemophiliacs, the effects which these complications have on the medical resources used with these patients, and the impact of severe haemophilia on their quality of life. Its secondary aim was to link their current orthopaedic status to the type of treatment they had received prior to the study. Eleven Spanish hospitals took part in this study, monitoring 70 severe haemophiliacs (FVIII:C <2%) without inhibitors who had a mean age of 21.6 years and a median age of 22. Retrospective data collected from birth to the conclusion of the study were used and, for certain variables, data from the last 12 months. The type of treatment given had been on-demand treatment, together with prophylaxis of variable time periods, which in 32 cases (45.7%) were prolonged (>6 months). In 40 cases (57.1%) the patients underwent one or more periods of prophylaxis. Thirty-three patients (47.8%) had over 1000 days of administration of factors VIII and IX. The analysis of the total study group reveal an average of 348 bleeding episodes per patient. The findings of this study revealed that 84.3% of these patients suffer from articular complaints on the Gilbert scale, and 85.7% on the Pettersson scale. In addition, pain was reported in 16.1% of the joints, the most frequently affected being the ankle joints. Twenty-six patients (37%) had undergone orthopaedic surgery from the time of birth to the conclusion of the study. The quality of life of the severe haemophiliacs reviewed seems to have been affected. During the last 12 months, there were 216 outpatient haematological visits and 176 orthopaedic-rehabilitation visits, as well as 12 radiological explorations and two hospitalizations. During these 12 months, medical expenditure totalled $55 473 per patient per year, the most important item being factor concentrates VIII/IX ($54 119 per patient per year). The type of treatment given to these patients (on-demand and/or as secondary prophylaxis) was found to be incapable of preventing haemophilic arthropathy or guaranteeing an acceptable quality of life, although both the administration of coagulant factor concentrate to such patients and the financial resources dedicated to their treatment was very high. Consequently, a strategic change is to be considered for the prevention of haemophilic arthropathy, based on the introduction of replacement treatment involving continuous administration of factors VIII and IX in primary prophylaxis regimens from the first years of life.
J A Aznar; M Magallón; F Querol; E Gorina; J M Tusell
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Publication Detail:
Type:  Journal Article; Multicenter Study    
Journal Detail:
Title:  Haemophilia : the official journal of the World Federation of Hemophilia     Volume:  6     ISSN:  1351-8216     ISO Abbreviation:  Haemophilia     Publication Date:  2000 May 
Date Detail:
Created Date:  2000-07-14     Completed Date:  2000-07-14     Revised Date:  2009-10-21    
Medline Journal Info:
Nlm Unique ID:  9442916     Medline TA:  Haemophilia     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  170-6     Citation Subset:  IM    
University Hospital La Fe, Congenital Coagulopathy Unit and Physiotherapy Department of the University of Valencia, Valencia, Spain.
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MeSH Terms
Cohort Studies
Costs and Cost Analysis
Cross-Sectional Studies
Factor IX / administration & dosage
Factor VIII / administration & dosage
Hemarthrosis / etiology
Hemophilia A / complications*,  economics,  therapy
Hemophilia B / complications,  economics,  therapy
Hemorrhage / etiology,  psychology,  therapy
Joint Diseases / etiology,  pathology*,  surgery
Mental Health
Orthopedic Procedures
Pain / etiology,  psychology
Physical Fitness / psychology
Quality of Life
Retrospective Studies
Severity of Illness Index
Socioeconomic Factors
Spain / epidemiology
Statistics, Nonparametric
Reg. No./Substance:
9001-27-8/Factor VIII; 9001-28-9/Factor IX

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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