Multidisciplinary management of Hunter syndrome.
Pediatrics
; 124(6): e1228-39, 2009 Dec.
Article
em En
| MEDLINE
| ID: mdl-19901005
ABSTRACT
Hunter syndrome is a rare, X-linked disorder caused by a deficiency of the lysosomal enzyme iduronate-2-sulfatase. In the absence of sufficient enzyme activity, glycosaminoglycans accumulate in the lysosomes of many tissues and organs and contribute to the multisystem, progressive pathologies seen in Hunter syndrome. The nervous, cardiovascular, respiratory, and musculoskeletal systems can be involved in individuals with Hunter syndrome. Although the management of some clinical problems associated with the disease may seem routine, the management is typically complex and requires the physician to be aware of the special issues surrounding the patient with Hunter syndrome, and a multidisciplinary approach should be taken. Subspecialties such as otorhinolaryngology, neurosurgery, orthopedics, cardiology, anesthesiology, pulmonology, and neurodevelopment will all have a role in management, as will specialty areas such as physiotherapy, audiology, and others. The important management topics are discussed in this review, and the use of enzyme-replacement therapy with recombinant human iduronate-2-sulfatase as a specific treatment for Hunter syndrome is presented.
Texto completo:
1
Coleções:
01-internacional
Temas:
Geral
Base de dados:
MEDLINE
Assunto principal:
Equipe de Assistência ao Paciente
/
Mucopolissacaridose II
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Transplante de Células-Tronco Hematopoéticas
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Comportamento Cooperativo
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Comunicação Interdisciplinar
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Terapia de Reposição de Enzimas
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Iduronato Sulfatase
Tipo de estudo:
Clinical_trials
Limite:
Adolescent
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Adult
/
Child
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Child, preschool
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Humans
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Infant
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Male
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Newborn
Idioma:
En
Revista:
Pediatrics
Ano de publicação:
2009
Tipo de documento:
Article
País de afiliação:
Estados Unidos