Langerhans' cell histiocytosis: experience from a single center.
Indian Pediatr
;
2008 Aug; 45(8): 685-8
Artículo
en Inglés
| IMSEAR
| ID: sea-8493
ABSTRACT
Clinical profile, disease-distribution and outcome of Langerhans cell histiocytosis (LCH) is presented in this retrospective analysis. There were 69 children with LCH from January 1986 to December 2004. Diagnosis was presumptive in the majority. The age ranged from 2 months to 12 years. Multisystem disease was documented in 48 (69.6%) children. Evidence of hepatic dysfunction was detected in 25 (36.2%). An elevated serum alkaline phosphatase was a prominent observation in patients with hepatic involvement. Children with localized disease received oral steroids, while cases with disseminated/multi-system disease were treated with prednisolone and, vinblastine or etoposide. 20 (54%) children with disseminated disease and organ dysfunction died. A favorable outcome was documented in all but one case with localized disease. Portal hypertension developed in 3 cases, all of whom had a fatal outcome. Twelve (17.4%) patients had diabetes insipidus. Disseminated disease with organ dysfunction was observed to be a predictor of fatal outcome.
Texto completo:
Disponible
Índice:
IMSEAR (Asia Sudoriental)
Asunto principal:
Humanos
/
Áreas de Influencia de Salud
/
Preescolar
/
Histiocitosis de Células de Langerhans
/
Estudios Retrospectivos
/
Diabetes Insípida
/
Atención Ambulatoria
/
India
/
Lactante
/
Hígado
Tipo de estudio:
Estudio observacional
/
Estudio pronóstico
País/Región como asunto:
Asia
Idioma:
Inglés
Revista:
Indian Pediatr
Año:
2008
Tipo del documento:
Artículo
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