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Thyroid function in Fabry disease before and after enzyme replacement therapy.
MedLine Citation:
PMID:  21460782     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
METHODS: Study population included 14 FD patients (7 females, 7 males, aged 21-62 years) and 14 sex- and age-matched normal subjects. Thyroid function was evaluated in each patient at baseline and after the beginning of ERT with rh-a-galactosidase-A (1 mg/kg/BW every 2 weeks) for three years.
RESULTS: TSH levels were higher in FD patients than in controls (P<0.05). In FD patients, TSH levels were higher before than after ERT (1.9±0.2 vs 1.2±0.2 mU/L, P<0.01) while fT3 and fT4 levels were normal at baseline and unchanged after ERT. At baseline, TSH levels were >3 mU/L in three patients and normalize after ERT. Anti-Tg and/or anti-TPO titres were positive in 14% of patients and 21% of controls. After ERT, the rate of autoimmunity was unchanged. At the thyroid ultrasonography, a slight hypoechoic pattern was found in 71% of patients at baseline and decreased to 43% after ERT.
CONCLUSION: Primary hypothyroidism in FD patients is reverted after long-term ERT. A screening of thyroid function and periodical re-evaluation during ERT is mandatory in all FD patients.
Authors:
A Faggiano; R Severino; V Ramundo; R Russo; L Vuolo; M Del Prete; F Marciello; G Lombardi; B Cianciaruso; A Colao; A Pisani
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Minerva endocrinologica     Volume:  36     ISSN:  0391-1977     ISO Abbreviation:  Minerva Endocrinol.     Publication Date:  2011 Mar 
Date Detail:
Created Date:  2011-04-04     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8406505     Medline TA:  Minerva Endocrinol     Country:  Italy    
Other Details:
Languages:  eng     Pagination:  1-5     Citation Subset:  IM    
Affiliation:
Department of Molecular and Clinical Endocrinology and Oncology, Federico II University, Naples, Italy - afaggian@unina.it.
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