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Titolo:
ADRENOMYELONEUROPATHY, A COMMON-CAUSE OF ADDISONS-DISEASE
Autore:
SCHAFER JR; EHLENZ K; STEINMETZ A; PILZ C; HUNNEMAN DH; BAERWALD C; VONWICHERT P; KAFFARNIK H;
Indirizzi:
UNIV MARBURG,ZENTRUM INNERE MED,ENDOKRINOL & STOFFWECHSEL ABT,BALDINGERSTR D-35033 MARBURG GERMANY UNIV MARBURG,ZENTRUM INNERE MED,MED POLIKLIN D-35033 MARBURG GERMANY UNIV GOTTINGEN,KINDERKLIN D-37075 GOTTINGEN GERMANY
Titolo Testata:
Deutsche Medizinische Wochenschrift
fascicolo: 10, volume: 119, anno: 1994,
pagine: 327 - 331
Fonte:
ISI
Lingua:
GER
Soggetto:
X-LINKED ADRENOLEUKODYSTROPHY; PIGMENT GENE REGION; CHAIN FATTY-ACIDS; THERAPY; MANIFESTATIONS; DISORDERS; DIAGNOSIS; OXIDATION;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
29
Recensione:
Indirizzi per estratti:
Citazione:
J.R. Schafer et al., "ADRENOMYELONEUROPATHY, A COMMON-CAUSE OF ADDISONS-DISEASE", Deutsche Medizinische Wochenschrift, 119(10), 1994, pp. 327-331

Abstract

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 ashas 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.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 25/09/20 alle ore 10:23:30