Clinical and functional evaluation of the joint status of hemophiliac adults at a Brazilian blood center

Ferreira, Adriana; Bustamante-Teixeira, Maria Teresa; Leite, Isabel Cristina Gonçalves; Corrêa, Camila Soares Lima; Rodrigues, Daniela de Oliveira Werneck; Cruz, Danielle Teles da

doi:10.5581/1516-8484.20130010

Cited by 23 publications

(23 citation statements)

References 18 publications

(19 reference statements)

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“…Our data suggest that haemophilic joint damage starts at an early age and progresses until the age of 12-16 years, by which time aggregative damage approaches maximum but some further progression in arthropathy is possible. Several studies have reported a positive relationship between age and HJHS: a Lithuanian study (n = 20) showed higher HJHS in older children (mean score = 24.5) compared with younger (mean score = 11.6) [22]; a group from London showed a positive association of HJHS with age [23], and a cohort of 39 individuals with haemophilia from Brazil showed a significant positive correlation of Gilbert score with age [24]. Despite the fact that in each of these studies, the cohorts were either on prophylaxis or on-demand treatment, the joint health scores increased with age, however, the magnitude of increase was not as great as observed in our cohort.…”

Section: Discussionmentioning

confidence: 98%

Joint health scores in a haemophilia A cohort from Pakistan with minimal or no access to factor VIII concentrate: correlation with thrombin generation and underlying mutation

et al. 2013

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Haemophilia A is associated with recurrent joint bleeding which leads to synovitis and debilitating arthropathy. Coagulation factor VIII level is an important determinant of bleed number and development of arthropathy . The aim of this study was to compare the haemophilia joint health score (HJHS) and Gilbert score with severity, age, thrombin generation (TG) and underlying mutation in a haemophilia A cohort which had minimal access to haemostatic replacement therapy. Ninety-two haemophilia A individuals were recruited from Pakistan. Age, age at first bleed, target joints, haemophilic arthropathy joints, HJHS and Gilbert score were recorded. A strong correlation was found between HJHS and Gilbert score (r = 0.98), both were significantly higher in severe (n = 59) compared with non-severe (n = 29) individuals before the age of 12 years (P ≤ 0.01) but not thereafter. When individuals were divided according to developmental age (<12 years, 12-16 years and >16 years), both HJHS and Gilbert score were significantly lower in the youngest group (P ≤ 0.001), there was no difference between 12-16 years and >16 years. In severe individuals there was no correlation between in vitro TG and joint score, whereas in non-severe individuals there was a weak negative correlation. In the severe group, no significant difference was observed for either joint score according to the underlying mutation type (inversion, missense, nonsense, frameshift). In this cohort of haemophilia A individuals with minimal access to haemostatic treatment, haemophilic arthropathy correlated with severity and age; among severe individuals, joint health scores did not relate to either the underlying mutation or in vitro TG.

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Section: Discussionmentioning

confidence: 98%

Joint health scores in a haemophilia A cohort from Pakistan with minimal or no access to factor VIII concentrate: correlation with thrombin generation and underlying mutation

et al. 2013

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“…Increasing pain, muscle weakness and atrophy along with an increased risk of falling are key features of haemophilic arthropathy . Persons with mild haemophilia were excluded because they have no to very little haemophilic arthropathy . Various variables, known as fall risk factors in community‐dwelling older persons, were questioned with a standardized test battery in adult PWH.…”

Section: Discussionmentioning

confidence: 99%

Falling and fall risk factors in adults with haemophilia: an exploratory study

et al. 2014

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Falls are a particular risk in persons with haemophilia (PWH) because of damaged joints, high risk of bleeding, possible impact on the musculoskeletal system and functioning and costs associated with treatment for these fall-related injuries. In addition, fall risk increases with age and PWH are increasingly entering the over 65 age group. The aim of this study was to determine the occurrence of falls during the past year and to explore which fall risk factors are present in community-dwelling PWH. Dutch speaking community-dwelling adults were included from the age of 40 years with severe or moderate haemophilia A or B, independent in their mobility and registered at the University Hospitals Leuven. They were asked to come to the haemophilia centre; otherwise a telephone survey was conducted. Demographic and social variables, medical variables, fall evaluation and clinical variables were queried. From the 89 PWH, 74 (83.1%) participated in the study. Twenty-four (32.4%) fell in the past year, and 10 of them (41.7%) more than once with an average of four falls. Living conditions, physical activity, avoidance of winter sports due to fear of falling, orthopaedic status, urinary incontinence and mobility impairments are potential fall risk factors in adult PWH. This exploratory study indicates that PWH are attentive to falling since they are at higher risk for falls and because of the serious consequences it might have. Screening and fall prevention should be stimulated in the daily practice of haemophilia care.

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“…The Brazilian Ministry of Health implemented a primary prophylaxis program in Brazil only at the end of 2011 because of the high cost of the coagulation factors. This program has benefited children aged 3 years or younger with severe haemophilia (or moderate haemophilia with coagulation factor VIII or IX levels below 2%) . Therefore, most adult patients with severe or moderate haemophilia A or B started the replacement of clotting factors late and developed severe sequelae due to repetitive bleeding, increased frequency of intra‐articular bleeding, with more risk of developing target joints (a joint in which recurrent bleeding has occurred four or more times in the past 6 months) and haemophilic arthropathy.…”

Section: Introductionmentioning

confidence: 99%

Translation, validation and reliability of the functional capacity questionnaire Haemophilia Activities List for haemophilia patients in Brazil

et al. 2019

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Introduction To date, none of the available assessment instruments to evaluate functional abilities for individuals with haemophilia has been translated and validated in Brazil. Aim To translate, and test the construct validity, internal consistency and the reliability of the Haemophilia Activities List (HAL) for the Brazilian population with severe and moderate haemophilia (type A or B) and to investigate differences in the self‐perception of functional abilities in patients adults with haemophilia classified according to the presence of joint bleeding and the performance of orthopaedic surgeries. Methods The translation and transcultural adaptation following the steps: (a) translation; (b) synthesis (consensual version); (c) back‐translation; (d) review by the committee of experts; (e) pretest in the target population; and (f) final version of the instrument. Correlations between HAL scores and the scores of the Health Assessment Questionnaire (HAQ) and Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) were determined for construct validation. Reliability was tested using the test and retest method, and internal consistency was calculated by Cronbach's α. Results The HAL‐BR was applied to 52 individuals with haemophilia (34.51 ± 12.87 years). The process of translation and cross‐cultural adaptation produced similar versions between the translations A moderate correlation was observed between HAL and the HAQ (r = −0.55) and WOMAC scores (r = −0.58). The reliability was ICC = 0.972, CI (0.917‐0.997) ICC = 0.876, CI (0.631‐0.978) for inter‐ and intra‐examiners. No difference was found in the total HAL score between the groups with and without bleeding. The group subjected to surgery compared to the group of not subjected to surgery presented in the HAL domains worse function in ‘lying down/sitting/kneeling/standing’, ‘self‐care’ and ‘complex lower extremities activities’ as well as in total HAL score. Conclusion The Brazilian version of the HAL was proven to be a valid and reliable evaluation tool for adults with haemophilia in Brazil.

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Clinical and functional evaluation of the joint status of hemophiliac adults at a Brazilian blood center

Cited by 23 publications

References 18 publications

Joint health scores in a haemophilia A cohort from Pakistan with minimal or no access to factor VIII concentrate: correlation with thrombin generation and underlying mutation

Joint health scores in a haemophilia A cohort from Pakistan with minimal or no access to factor VIII concentrate: correlation with thrombin generation and underlying mutation

Falling and fall risk factors in adults with haemophilia: an exploratory study

Translation, validation and reliability of the functional capacity questionnaire Haemophilia Activities List for haemophilia patients in Brazil

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