Negligible urinary cysteamine loss in cystinosis patients with Fanconi syndrome

Clinical Nephrology

Volumn 57, Issue 5, 2002, Pages 349-351

  Levtchenko, E N ^a   de Graaf Hess, A ^a   Blom, H J ^a   Monnens, L A H ^a  

^a RADBOUD UNIVERSITY MEDICAL CENTER   (Netherlands)

Author keywords

Cysteamine excretion; Fanconi syndrome; Nephropathic cystinosis; Urine

Indexed keywords

CYSTINE; MERCAPTAMINE;

ADOLESCENT; AMINO ACID TRANSPORT; ARTICLE; CHILD; CLINICAL ARTICLE; CLINICAL FEATURE; CONTROLLED STUDY; CYSTINOSIS; DISEASE COURSE; DRUG URINE LEVEL; FANCONI RENOTUBULAR SYNDROME; HUMAN; INBORN ERROR OF METABOLISM; KIDNEY DISEASE; KIDNEY FAILURE; LYSOSOME; URINE;

ADOLESCENT; CHILD; CYSTEAMINE; CYSTINOSIS; FANCONI SYNDROME; HUMANS;

EID: 0035989758     PISSN: 03010430     EISSN: None     Source Type: Journal    
DOI: 10.5414/CNP57349     Document Type: Article

References (9)

1. New method for determining cystine in leukocytes and fibroblasts
2. Homocysteine and other thiols in plasma and urine: Automated determination and sample stability
3. Cystinosis coming of age
4. Cysteamine: From in vitro efficacy to effectiveness in patients
5. Cysteamine depletes cystinotic leukocyte granular fractions of cystine by the mechanism of disulphide interchange
6. A simple, rapid assay for cysteamine and other thiols
7. Plasma cysteamine concentrations in children treated for cystinosis
8. Recent advances in the treatment of cystinosis
9. A comparison of the effectiveness of cysteamine and phosphocysteamine in elevating plasma cysteamine concentrations and decreasing leukocyte-free cystine in nephropathic cystinosis