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Titolo:
Treatment of cystinuria
Autore:
Joly, D; Rieu, P; Mejean, A; Gagnadoux, MF; Daudon, M; Jungers, P;
Indirizzi:
Hop Necker Enfants Malad, Dept Nephrol, F-75015 Paris, France Hop Necker Enfants Malad Paris France F-75015 rol, F-75015 Paris, France
Titolo Testata:
PEDIATRIC NEPHROLOGY
fascicolo: 9, volume: 13, anno: 1999,
pagine: 945 - 950
SICI:
0931-041X(199911)13:9<945:TOC>2.0.ZU;2-I
Fonte:
ISI
Lingua:
ENG
Soggetto:
BRUSH-BORDER VESICLES; TIOPRONIN TREATMENT; URINARY CYSTINE; ALPHA-MERCAPTOPROPIONYLGLYCINE; GENETIC-HETEROGENEITY; SODIUM-INTAKE; SLC3A1 GENE; CAPTOPRIL; CALCULI; EXCRETION;
Keywords:
cystine urolithiasis; cystinuria; D-penicillamine; tiopronin;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
53
Recensione:
Indirizzi per estratti:
Indirizzo: Jungers, P Hop Necker Enfants Malad, Dept Nephrol, 149 Rue Sevres, F-75015Paris, France Hop Necker Enfants Malad 149 Rue Sevres Paris France F-75015 e
Citazione:
D. Joly et al., "Treatment of cystinuria", PED NEPHROL, 13(9), 1999, pp. 945-950

Abstract

Cystine urolithiasis is the only clinical expression of cystinuria, an autosomal recessive genetic defect of the transepithelial transport of cystineand other dibasic amino acids in the kidney. Stones form due to the increased excretion of cystine, which is poorly soluble at normal urine pH. Cystine stones are often resistent to extracorporeal shock wave lithotripsy, so that percutaneous surgery or ureteroscopy are the preferred techniques of stone extraction. Medical preventative treatment is based on high diuresis (greater than or equal to 1.5 l/m(2) per day) well distributed throughout the day and night, and urine alkalinization up to pH 7.5 by means of sodium bicarbonate and/or potassium citrate. When these basal measures are ineffective at preventing stone recurrence or dissolving pre-existing stones, sulfhydryl agents such as D-penicillamine or tiopronin, which form highly soluble mixed disulfides with cystine moieties, are to be added to urine dilutionand alkalinization, especially when cystine excretion is in excess of 750 mg/day (3 mmol/day). Frequent clinical and ultrasound follow-up is needed to encourage patient compliance and assess efficacy and tolerance of treatment.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 24/11/20 alle ore 13:47:24