Quantitation of bleeding symptoms in a national registry of patients with inherited platelet disorders.
Blood Cells Mol Dis
; 67: 59-62, 2017 09.
Article
em En
| MEDLINE
| ID: mdl-27998672
ABSTRACT
BACKGROUND:
Inherited platelet deficiency and/or dysfunction may be more common in the general population than has previously been appreciated. In 2013 the Israeli Inherited Platelet Disorder (IPD) Registry was established.METHODS:
Clinical and laboratory data were collected to pre-specified registration forms. The study protocol was approved by the local hospital ethics committees.RESULTS:
To date we have included in the registry 89 patients (male 52%) from 79 families. Most patients (74%) have a not-yet specified inherited thrombocytopenia (n=39) or non-specific platelet function disorder (n=27). Full clinical data were available for 81 (91%) patients. The median (range) age at presentation and time of follow-up were 1.8years (1day-17.8years) and 4.7 (0-26) years, respectively. The Pediatric Bleeding Questionnaire was available for 78patients; abnormal bleeding score (≥2) was recorded in 47 (52.8%, 95% CI 42%-63.5%) patients and was less frequent in patients followed for isolated thrombocytopenia. Abnormal score was associated with a longer time of follow-up, OR 1.19 (95% CI 1.04-1.36).CONCLUSION:
Long term follow-up of patients with IPDs is important as bleeding risks may increase with time. We expect that clinical and laboratory information of patients/families with IPDs gathered in a systemic format will allow for better diagnosis and treatment of these patients.Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Transtornos Plaquetários
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Plaquetas
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Hemorragia
Tipo de estudo:
Diagnostic_studies
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Guideline
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Observational_studies
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Prognostic_studies
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Risk_factors_studies
Limite:
Adolescent
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Adult
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Child
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Child, preschool
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Female
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Humans
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Infant
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Male
Idioma:
En
Revista:
Blood Cells Mol Dis
Assunto da revista:
HEMATOLOGIA
Ano de publicação:
2017
Tipo de documento:
Article