Long term morbidity and health related quality of life after multi-system Langerhans cell histiocytosis.
Eur J Cancer
; 42(15): 2563-9, 2006 Oct.
Article
em En
| MEDLINE
| ID: mdl-16959486
ABSTRACT
BACKGROUND:
Langerhans' cell histiocytosis, a clonal multisystem disorder, can affect children or adults resulting in long term sequelae. However, the overall morbidity for survivors has not been formally determined. PATIENTS ANDMETHODS:
We performed a cross-sectional study of 40 unselected long term survivors of childhood multisystem Langerhans cell histiocytosis, involving clinical examination, health-related quality of life assessment, brain imaging, neuropsychometry, endocrine assessment, respiratory function tests and audiometry. A specific 'morbidity score' was devised to measure outcome.RESULTS:
Seventy-five percent of patients had detectable long term sequelae, hypothalamic-pituitary dysfunction (50%), cognitive dysfunction (20%) and cerebellar involvement (17.5%) being the most common. Half had moderate to severe morbidity, and the worst-affected patients were unable to lead an independent adult life. Health-related quality of life, which correlated well with the morbidity score (p<0.001), was adversely affected in >50% of patients.CONCLUSION:
Organ damage from multisystem Langerhans cell histiocytosis causes long term morbidity extending into adult life. Carefully planned, multidisciplinary follow up is essential to ensure early recognition of problems with appropriate interventions to reduce the impact on patients' 'quality of life'.
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Base de dados:
MEDLINE
Assunto principal:
Qualidade de Vida
/
Histiocitose de Células de Langerhans
/
Sobreviventes
Tipo de estudo:
Observational_studies
/
Prevalence_studies
/
Prognostic_studies
/
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
Ano de publicação:
2006
Tipo de documento:
Article