Plasma levels of adrenomedullin in patients with adrenoleukodystrophy/adrenomyeloneuropathy
| Autor: | Graziamaria Ubertini, Marco Cappa, S. Benedetti, Carla Bizzarri, A. Di Biase, Emilio D'Erasmo, Claudio Letizia, Subioli S |
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| Rok vydání: | 2004 |
| Předmět: |
Adult
Male medicine.medical_specialty endocrine system diseases Adolescent Endocrinology Diabetes and Metabolism Pheochromocytoma Adrenomedullin Endocrinology Addison Disease Internal medicine medicine addison's disease adrenoleukodystrophy adrenomedullin adrenomyeloneuropathy central nervous system demyelination Humans In patient Adrenoleukodystrophy Child Gene business.industry Plasma levels Middle Aged medicine.disease Addison's disease Case-Control Studies Pediatrics Perinatology and Child Health Hypotensive peptide business Peptides hormones hormone substitutes and hormone antagonists Adrenal Insufficiency |
| Zdroj: | Hormone research. 63(2) |
| ISSN: | 0301-0163 |
| Popis: | Background/Aims: Adrenomedullin (AM) is a recently purified hypotensive peptide and its encoding gene has been sequenced from a human pheochromocytoma. High levels of AM have been shown in Addison’s disease (AD). X-linked adrenoleukodystrophy/adrenomyeloneuropathy (ALD/AMN) is a peculiar adrenal insufficiency due to an accumulation of very-long chain fatty acid in adrenal cells and it is very often associated with a devastating demyelination of the central nervous system. Methods: We studied the AM plasma levels of 22 patients with ALD/AMN (18 with hypoadrenalism, ALDa, and 4 with normal adrenal function, ALDb) and compared them with 18 males with classical AD and 16 normal male subjects. All patients with hyposurrenalism were studied before treatment with hydrocortisone. Results: Both patients with ALD/AMN and AD showed increased levels of AM and all of them showed a significant difference from the control group (p < 0.0001). The plasma renin activity was higher in all patient groups than in the control group (p Conclusions: Our data indicate that plasma AM levels in ALDa, ALDb and AD are higher than controls. These results were previously described in untreated AD. While classical AD patients show complete adrenal insufficiency (both mineralocorticoid and glucocorticoid defects), ALD/AMN patients show a less compromised glomerular function, indicating that AM is not completely correlated with mineralocorticoid insufficiency, and that the exact mechanism responsible for the increased AM levels in ALD/AMN is still unknown. |
| Databáze: | OpenAIRE |
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