The treatment of cystinosis with cysteamine and phosphocysteamine in the United Kingdom and Eire

Pediatr Nephrol. 1995 Dec;9(6):685-9. doi: 10.1007/BF00868711.

Abstract

Fifty-nine patients with cystinosis were treated with cysteamine or phosphocysteamine in the United Kingdom up to May 1990. Treatment was started at a median age of 3.2 years (range 0.6-24.8 years) and continued for a median duration of 3.0 years (range 0.01-1.2 years). At the end of the study, 46 (78%) patients remained on treatment. One patient developed end-stage renal failure and 6 died. Efficacy was assessed in the 44 pre-transplant patients. The United Kingdom pre-transplant patients had significantly lower plasma creatinine concentrations at 6 and 8 years than a historical group of patients who did not receive cysteamine (P < 0.0001 and P < 0.0003, respectively). There was no significant difference between pretreatment and final post-treatment height standard deviation scores, suggesting maintenance of growth rate. The leucocyte cystine concentration was less than the accepted upper limit of the treatment range (1 nmol 1/2 cystine/mg protein) in only 21% of determinations. There was no significant difference between the mean pre-treatment and final values of leucocyte cystine concentration. The mean final doses of cysteamine (33 mg/kg per day) and phosphocysteamine (37 mg/kg per day base equivalent) were less than the mean dose (51 mg/kg per day) used in a United States multicentre trial. We conclude that cysteamine treatment was beneficial, but further improvements might be achieved by an improvement in monitoring of therapy.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Cystaphos / therapeutic use*
  • Cysteamine / therapeutic use*
  • Cystine / blood
  • Cystinosis / drug therapy*
  • Cystinosis / mortality
  • Cystinosis / physiopathology
  • Female
  • Growth / drug effects
  • Humans
  • Infant
  • Ireland
  • Kidney Function Tests
  • Leukocytes / metabolism
  • Male
  • Retrospective Studies
  • United Kingdom

Substances

  • Cystine
  • Cysteamine
  • Cystaphos