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Titolo:
LEUKODYSTROPHY INCIDENCE IN GERMANY
Autore:
HEIM P; CLAUSSEN M; HOFFMANN B; CONZELMANN E; GARTNER J; HARZER K; HUNNEMAN DH; KOHLER W; KURLEMANN G; KOHLSCHUTTER A;
Indirizzi:
UNIV HAMBURG,KINDERKLIN,DEPT PEDIAT,MARTINISTR 52 D-20246 HAMBURG GERMANY UNIV HAMBURG,KINDERKLIN,DEPT PEDIAT D-20246 HAMBURG GERMANY UNIV HAMBURG,INST MATH & COMP SCI MED D-20246 HAMBURG GERMANY UNIV WURZBURG,THEODOR BOVERI INST BIOSCI WURZBURG GERMANY UNIV DUSSELDORF,CHILDRENS HOSP D-4000 DUSSELDORF GERMANY UNIV TUBINGEN,INST BRAIN RES D-7400 TUBINGEN GERMANY UNIV GOTTINGEN,CHILDRENS HOSP D-3400 GOTTINGEN GERMANY KRANKENHAUS MOABIT BERLIN GERMANY UNIV MUNSTER,CHILDRENS HOSP D-4400 MUNSTER GERMANY
Titolo Testata:
American journal of medical genetics
fascicolo: 4, volume: 71, anno: 1997,
pagine: 475 - 478
SICI:
0148-7299(1997)71:4<475:LIIG>2.0.ZU;2-G
Fonte:
ISI
Lingua:
ENG
Soggetto:
BONE-MARROW TRANSPLANTATION; METACHROMATIC LEUKODYSTROPHY; ADRENOLEUKODYSTROPHY; ADRENOMYELONEUROPATHY; PATIENT;
Keywords:
EPIDEMIOLOGY; INCIDENCE; LEUKODYSTROPHY; ADRENOLEUKODYSTROPHY; METACHROMATIC LEUKODYSTROPHY; KRABBE DISEASE; CANAVAN DISEASE; ALEXANDER DISEASE; PELIZAEUS-MERZBACHER DISEASE; PEROXISOMAL DISORDERS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
20
Recensione:
Indirizzi per estratti:
Citazione:
P. Heim et al., "LEUKODYSTROPHY INCIDENCE IN GERMANY", American journal of medical genetics, 71(4), 1997, pp. 475-478

Abstract

Through a survey of all departments of pediatrics, neurology and neuropathology in Germany, we calculated the incidence of all major forms of leukodystrophy. Only diagnoses based on specific biochemical tests in association with typical findings and/or neuroradiologically provenwhite matter involvement were accepted, In accordance with these strict criteria, 617 cases of leuko-dystrophy were found (incidence of allforms: app. 2.0/100,000), Minimal incidence was estimated at 0.8/100,000 for adrenoleugodystrophy/adrenomyeloneuropathy (ALD/AMN), 0.6/100,000 for metachromatic leukodystrophy (MLD), and 0.6/100,000 for Krabbedisease. Thus ALD/AMN is apparently underdiagnosed in Germany. A considerable proportion of leukodystrophies could not be classified in spite of adequate diagnostic procedures in experienced centers. (C) 1997 Wiley-Liss, Inc.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 25/09/20 alle ore 09:33:06