Bleeding assessment in a large cohort of patients with Osteogenesis Imperfecta.
Orphanet J Rare Dis
; 19(1): 61, 2024 Feb 12.
Article
en En
| MEDLINE
| ID: mdl-38347577
ABSTRACT
BACKGROUND:
Osteogenesis Imperfecta (OI) is characterised by bone fragility. Among several features, easy bruising and multiple case reports on haemorrhagic events have been reported. This paper describes the diverse manifestations of bleeding and bruising in a large cohort of 328 OI patients. The aim of this study is to provide insight in the diverse aspects and therapeutic considerations of bleedings in OI.METHODS:
This descriptive cohort study was conducted at the National Expert Center for adults with OI in the Netherlands. Bleeding was assessed by the validated self-bleeding assessment tool (Self-BAT) The tool was distributed among 328 adults with different clinically confirmed types of OI.RESULTS:
195 of 328 invited patients (completion rate 60%) with OI type 1 (n = 144), OI type 3 (n = 17) and OI type 4 (n = 34), aged between 18 and 82 years, completed the tool. Self-BAT scores were above the normal range in 42% of all patients. For males Self-BAT scores were increased in 37% with a mean score of 3.7, ranged between 0 and 18. For females the Self-BAT scores were increased in 44% with a mean of 5.4 and a range of 0-24. No statistical differences in OI subtypes were found.CONCLUSIONS:
Bleeding tendency appears to be a relevant complication in OI patients as this study confirms the presumption of bleeding tendency. There are specific recommendations to clinicians who treat OI patients to consider an assessment of bleeding tendency and use potential interventions to reduce haemorrhagic complications and improve quality of life.Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Osteogénesis Imperfecta
Tipo de estudio:
Etiology_studies
/
Guideline
/
Incidence_studies
/
Observational_studies
/
Risk_factors_studies
Aspecto:
Patient_preference
Límite:
Adolescent
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Adult
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Aged
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Aged80
/
Female
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Humans
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Male
/
Middle aged
País/Región como asunto:
Europa
Idioma:
En
Revista:
Orphanet J Rare Dis
Asunto de la revista:
MEDICINA
Año:
2024
Tipo del documento:
Article
País de afiliación:
Países Bajos