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Titolo:
Scleroderma in South Australia: epidemiological observations of possible pathogenic significance
Autore:
Roberts-Thomson, PJ; Jones, M; Hakendorf, P; Dharmapatni, AASSK; Walker, JG; Macfarlane, JG; Smith, MD; Ahern, MJ;
Indirizzi:
Flinders Univ S Australia, Flinders Med Ctr, Clin Epidemiol Unit, Adelaide, SA 5001, Australia Flinders Univ S Australia Adelaide SA Australia 5001 , SA 5001, Australia Flinders Univ S Australia, Dept Anat & Histol, Adelaide, SA 5001, Australia Flinders Univ S Australia Adelaide SA Australia 5001 , SA 5001, Australia Univ Cambridge, Sch Med, Cambridge, England Univ Cambridge Cambridge England Cambridge, Sch Med, Cambridge, England Univ Adelaide, Dept Immunul Allergy & Arthrit, Adelaide, SA, Australia Univ Adelaide Adelaide SA Australia y & Arthrit, Adelaide, SA, Australia
Titolo Testata:
INTERNAL MEDICINE JOURNAL
fascicolo: 4, volume: 31, anno: 2001,
pagine: 220 - 229
SICI:
1444-0903(200105/06)31:4<220:SISAEO>2.0.ZU;2-R
Fonte:
ISI
Lingua:
ENG
Soggetto:
SYSTEMIC-SCLEROSIS SCLERODERMA; PREVALENCE;
Keywords:
epidemiology; prevalence; scleroderma; stochastic illness; systemic sclerosis;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
17
Recensione:
Indirizzi per estratti:
Indirizzo: Roberts-Thomson, PJ Flinders Med Ctr, Dept Immunol Allergy & Arthrit, Bedford Pk, SA 5042, Australia Flinders Med Ctr Bedford Pk SA Australia 5042 stralia
Citazione:
P.J. Roberts-Thomson et al., "Scleroderma in South Australia: epidemiological observations of possible pathogenic significance", INTERN M J, 31(4), 2001, pp. 220-229

Abstract

Background: Scleroderma is an autoimmune disorder of unknown cause. Previous epidemiological studies have suggested some regional clustering and associations with occupations involving exposure to silica dusts and hydrocarbons. Aims: To determine: (i) prevalence and incidence of scleroderma in South Australia (SA), a state with a stable population living in well-defined urban, rural and industrial regions, (ii) hospital separation rates, (iii) cumulative survival rates, (iv) gender and disease subclassification, (v) geographical residency and occupations, (vi) familial associations and (vii) ageat onset versus age-specific incidence rate. Methods: Creation of the South Australian Scleroderma Register (SASR) to identify demographics and clinical and serological features of all scleroderma patients resident in SA. Results: Scleroderma occurs in South Australia with a point prevalence of 23 per 10(5) and an incidence of approximately 1/15th of this. However, this prevalence is high compared with other regional world studies. Scleroderma is predominantly a female disease, with most patients having the limited form of scleroderma characterized by the presence of the anticentromere antibody and a mean survival of 27.6 years. In contrast, diffuse scleroderma is a less benign disease occurring at an early age of onset and has a mean survival of 9.6 years. Scleroderma occurs throughout SA without regional localization. Weak associations are seen in males, but not females, with occupations involving possible environmental exposure to silica or hydrocarbons. Scleroderma rarely occurs in families. Conclusion: No strong genetic or environmental influences were found to account for the relatively common occurrence of scleroderma in SA. The age atonset versus age-specific incidence curve suggests that scleroderma can beconsidered as a stochastic illness involving a number of random events occurring in a predisposed population. These random events may involve mutations of pivotal somatic genes.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 23/01/21 alle ore 08:54:09