Document Detail

Urinary free (unconjugated) metadrenalines in different hereditary forms of catecholamine-secreting phaeochromocytoma/paraganglioma.
MedLine Citation:
PMID:  22914444     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Catecholamine-producing neuroendocrine tumours are found in chromaffin cells of the adrenal medulla (phaeochromocytoma) or extra-adrenal paraganglia (paraganglioma), known collectively as PPGLs. In approximately a quarter or more of cases of PPGL, these rare tumours arise as a result of germline mutations of several tumour susceptibility genes. At the Crosshouse laboratory, urine tests include free metadrenalines (fMAs) (also known as free metanephrines) which demonstrate superior sensitivity over that obtained by urinary vanillyl mandelic acid, catecholamines or plasma catecholamines in the diagnosis of PPGL. This retrospective audit was to determine if urinary fMAs offered discrimination among the hereditary forms of PPGL.
METHODS: Retrospective biochemical and genetic data were gathered from 1997 to 2011. The identified urine specimens were those obtained at the time of first diagnosis or recurrence of PPGL. Results of catecholamines and metabolites were standardized as multiples of their respective relevant upper reference limits (URLs).
RESULTS: Results were available for 29 affected patients (15 females and 14 males), median age 26 (range 9-63) years, comprising three mutation groups: succinate dehydrogenase subunit B or D ([SDHB/D] 16 patients), multiple endocrine neoplasia type 2 ([MEN 2] 6 patients) and von Hippel-Lindau disease ([VHL] 7 patients). The parent catecholamines exhibited increased values for noradrenaline (NA) and/or adrenaline (AD) for 25/29 (86.2%) patients. Either or both free normetadrenaline (fNMA) and fMA were elevated in 29/29 (100%) patients.
CONCLUSIONS: The ratio of the multiples of URL for fMA/fNMA displayed a clearer separation of MEN 2 patients from those with SDHB/D or VHL than did the equivalent AD/NA ratio.
D Fraser Davidson; Nicola Bradshaw; Colin G Perry; Robert Lindsay; E Marie Freel
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Publication Detail:
Type:  Journal Article     Date:  2012-08-22
Journal Detail:
Title:  Annals of clinical biochemistry     Volume:  49     ISSN:  1758-1001     ISO Abbreviation:  Ann. Clin. Biochem.     Publication Date:  2012 Sep 
Date Detail:
Created Date:  2012-09-17     Completed Date:  2013-01-29     Revised Date:  2014-02-20    
Medline Journal Info:
Nlm Unique ID:  0324055     Medline TA:  Ann Clin Biochem     Country:  England    
Other Details:
Languages:  eng     Pagination:  486-90     Citation Subset:  IM    
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MeSH Terms
Adrenal Gland Neoplasms / genetics,  urine*
Catecholamines / urine*
Clinical Chemistry Tests
Metanephrine / urine*
Middle Aged
Paraganglioma / genetics,  urine*
Pheochromocytoma / genetics,  urine*
Retrospective Studies
Grant Support
G0802803//Medical Research Council
Reg. No./Substance:
0/Catecholamines; 5001-33-2/Metanephrine

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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