Adrenomyeloneuropathie: Eine häufige Ursache des Morbus Addison*

Autor:	K. Ehlenz, C. Baerwald, Armin Steinmetz, H. Kaffarnik, C. Pilz, J. R. Schafer, P. von Wichert, D. H. Hunneman
Rok vydání:	2008
Předmět:	endocrine system Pediatrics medicine.medical_specialty endocrine system diseases business.industry Spastic paraparesis Mean age General Medicine Disease medicine.disease University hospital Normal limit Addison's disease medicine Adrenoleukodystrophy business
Zdroj:	DMW - Deutsche Medizinische Wochenschrift. 119:327-331
ISSN:	1439-4413 0012-0472
DOI:	10.1055/s-2008-1058698
Popis:	Adrenomyeloneuropathy (AMN) is a "milder form" of adrenoleukodystrophy with a X-linked inheritance. Abnormal catabolism of the very long-chain fatty acids (VLCFA) results in Addison's disease and spastic paraparesis. The VLCFA concentration was measured in 23 of 26 patients with Addison's disease (mean age 48.5 [20-75] years) being treated at the University Hospital Marburg during May, 1991. The concentration was elevated in four of the 12 men with the disease, while it was within normal limits in the 11 women. Only two patients had paraparesis-like neurological deficits. This finding suggests that AMN is not as rare as has been supposed. It is recommended that the concentration of VLCFA be measured in all patients with Addison's disease, because an increase could have important consequences.
Databáze:	OpenAIRE
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