Abstract | PURPOSE: MATERIALS AND METHODS: We reviewed the charts of all 11 children with cystinuria treated at our clinic using a gradual dose escalation of penicillamine. Mean +/- SD patient age at diagnosis was 5.8 +/- 4.3 years (range 1.2 to 12). We tracked urinary cystine concentration before and after initiation of treatment, penicillamine side effects and incidence of new stones during maintenance therapy. RESULTS: During the gradual escalation of penicillamine to the target dose none of the 11 patients experienced toxicity and all had improved urinary cystine concentration (mean +/- SD percent reduction 54% +/- 25%, range 5% to 81%). We followed the patients for a total of 1,203 months (mean +/- SD 109 +/- 73 months, range 41 to 221), periodically assessing urinary cystine concentration, urine protein content, complete blood count, blood urea nitrogen, creatinine and liver function. During this time only 2 patients experienced significant side effects and no patient had stones or stone crises while compliant with treatment. CONCLUSIONS: In our cohort penicillamine was well tolerated after gradual initiation and it reduced urinary cystine concentration. Long-term compliance with the medication appeared to protect patients from acute stone crises.
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Authors | Ralph J DeBerardinis, Curtis R Coughlin 2nd, Paige Kaplan |
Journal | The Journal of urology
(J Urol)
Vol. 180
Issue 6
Pg. 2620-3
(Dec 2008)
ISSN: 1527-3792 [Electronic] United States |
PMID | 18951580
(Publication Type: Journal Article)
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Chemical References |
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Topics |
- Adolescent
- Child
- Child, Preschool
- Cystinuria
(complications, drug therapy)
- Female
- Humans
- Infant
- Male
- Penicillamine
(therapeutic use)
- Retrospective Studies
- United States
- Urinary Calculi
(etiology, prevention & control)
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