Long-term health outcomes of adults with McCune-Albright syndrome.
Clin Endocrinol (Oxf)
; 87(5): 627-634, 2017 Nov.
Article
en En
| MEDLINE
| ID: mdl-28699175
ABSTRACT
CONTEXT McCune-Albright syndrome (MAS) is associated with numerous health problems. Comprehensive long-term health problems of adults with MAS are less well defined in the literature. OBJECTIVE:
Our objective is to report comprehensive health outcomes of adults with MAS (>18 years).DESIGN:
Retrospective case note review of 16 adults with MAS managed by one clinician. Results expressed as median (range).RESULTS:
The study included 16 adults (seven males) with MAS. Median current age is 29 years (20, 46). Twelve of 16 had craniofacial fibrous dysplasia with five of 12 (42%) with progressive facial asymmetry. Growth hormone excess was observed in six of 16 (38%) and T3-toxicosis in five of 16 (31.3%). Six of the seven men (86%) had abnormalities on testicular ultrasound with one man exhibiting marked atrophy of germ and sertoli cells with reduction in spermatogenesis. Six of the 16 (38%) had cardiorespiratory complications including high output cardiac failure (n,3), hypertension (n,2) and one man with congestive cardiac failure and restrictive lung disease. Six of eight (66%) who had screening endoscopy for upper gastrointestinal polyps show increasing numbers of polyps, with benign histology to date. One woman with a previous history of early puberty presented with early aggressive breast carcinoma, which was positive for GNAS. Two patients had GNAS-positive muscle myomas. Platelet dysfunction with bleeding tendency responsive to platelet transfusion during surgery was seen in four.CONCLUSION:
A range of complex health problems is encountered in adults with MAS. These have important implications for transition of patients with MAS and adult care. Long-term cancer risk is currently unknown but requires careful follow-up.Palabras clave
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Cráneo
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Huesos Faciales
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Displasia Fibrosa Poliostótica
Tipo de estudio:
Observational_studies
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Risk_factors_studies
Límite:
Adult
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Female
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Humans
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Male
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Middle aged
Idioma:
En
Año:
2017
Tipo del documento:
Article