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Titolo:
Schonlein-Henoch purpura in children and adults - Diagnosis, pathophysiology and management
Autore:
Rostoker, G;
Indirizzi:
Ctr Hosp Prive Claude Galien, Serv Nephrol & Dialyse, F-91480 Quincy Sous Senart, France Ctr Hosp Prive Claude Galien Quincy Sous Senart France F-91480 t, France
Titolo Testata:
BIODRUGS
fascicolo: 2, volume: 15, anno: 2001,
pagine: 99 - 138
SICI:
1173-8804(2001)15:2<99:SPICAA>2.0.ZU;2-P
Fonte:
ISI
Lingua:
ENG
Soggetto:
IMMUNOGLOBULIN-A NEPHROPATHY; VON-WILLEBRAND-FACTOR; IGA-IMMUNE-COMPLEXES; CUTANEOUS LEUKOCYTOCLASTIC VASCULITIS; SYSTEMIC LUPUS-ERYTHEMATOSUS; FAMILIAL MEDITERRANEAN FEVER; EARLY PREDNISONE TREATMENT; PARVOVIRUS B19 INFECTION; CONVERTING ENZYME GENE; NECROSIS-FACTOR-ALPHA;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
328
Recensione:
Indirizzi per estratti:
Indirizzo: Rostoker, G Ctr Hosp Prive Claude Galien, Serv Nephrol & Dialyse, 20 RouteBoussy St Antoine, F-91480 Quincy Sous Senart, France Ctr Hosp Prive Claude Galien 20 Route Boussy St Antoine Quincy Sous Senart France F-91480
Citazione:
G. Rostoker, "Schonlein-Henoch purpura in children and adults - Diagnosis, pathophysiology and management", BIODRUGS, 15(2), 2001, pp. 99-138

Abstract

Schonlein-Henoch syndrome is a form of systemic small-vessel vasculitis, characterised by vascular and/or mesangial immunoglobulin Al deposits. The main clinical manifestations are vascular purpura, predominating on the lower limbs, and articular, gastrointestinal and renal symptoms. Pulmonary, cardiac, genital and neurological symptoms have also be observed. The syndromeusually affects children, whereas it is rare in adults. The frequency of renal involvement varies between individual studies (from20 to 100%). Renal manifestations are usually mild and transient, althoughchronic nephropathies may occur. Overall, an estimated 2% of children withSchonlein-Henoch purpura progress to renal failure and up to 20% of children with nephritis treated in specialised centres require haemodialysis. Therenal prognosis appears to be worse in adults. Aetiological investigationsare required, as a triggering factor is found in approximately half the patients (e.g. viral, bacterial and parasitic infections, drugs, toxins, systemic diseases and cancer). Dapsone has beneficial effects on cutaneous, gastrointestinal and articular manifestations in adults, especially those with chronic forms. Corticosteroids may be useful for refractory abdominal pain. Methylprednisolone pulsetherapy, immunosuppressive drugs (e.g. cyclophosphamide and azathioprine),plasma exchange and polyclonal immunoglobulin therapy are beneficial in very rare life-threatening forms of the disease and in rare instances where renal function is compromised.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 28/09/20 alle ore 23:08:14