Journal of Veterinary Internal Medicine · 2001 · 42 citations · 32 references
MedicineUrologyVeterinary PharmacologyBladder ManagementCanine Internal MedicineUrolithiasisCystine StonesStone Recurrence PreventionCumulative Urinary ExcretionCystine Stone EvaluationCystine Stone FormationCystine Stone RecurrenceRecurrence Rate AnalysisRenal Adverse EffectsTiopronin TherapyUrinary Cystine Excretion
The purpose of this study was to summarize 14 years of clinical experience with medical treatment of 88 cystinuric dogs. Of special interest was evaluation of recurrence rate of cystine uroliths and adverse effects during long-term tiopronin treatment. Twenty-six different breeds were recognized, and the most common breeds were Dachshunds, Tibetan Spaniels, and Basset Hounds. In 76 of 88 treated dogs (86%), re-formation of cystine uroliths was prevented. Recurrence rate of cystine uroliths changed from 7 months before to 18 months during tiopronin treatment. On 28 occasions, bladder stones were found, and in about 60% of the dogs, the uroliths dissolved. Quantitative measurement of the urinary excretion of cystine showed a significantly (P < .03) higher excretion of cystine in dogs with recurrent urolith formation than in dogs with only 1 urolith episode. Another finding was a significant (P = .02) decrease in urinary cystine excretion in older (>5 years) than in younger (<5 years) dogs. Adverse effects were found in 11 dogs, and the most severe signs were aggressiveness and myopathy. All signs disappeared when tiopronin treatment was stopped. In conclusion, this study emphasizes the importance of an individual strategy for lifelong treatment of cystinuria. In addition to increasing water intake, chemical modification of the cysteine molecule into a more soluble form by means of tiopronin is useful. In dogs with re-formed cystine uroliths, dissolution may be induced by increasing the tiopronin dosage to 40 mg/kg body weight per day. In dogs with a low urolith recurrence rate and low urinary cystine excretion, the tiopronin dosage may be decreased or treatment discontinued.
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Non-type I cystinuria caused by mutations in SLC7A9, encoding a subunit (bo,+AT) of rBAT
Lídia Feliubadaló, Jesús Purroy, F. Rousaud et al. · Nature Genetics · 1999 · 335 citations
Effect of Penicillamine on Cystinuria
J.C. Crawhall, E. F. Scowen, R. W. E. Watts · BMJ · 1963 · 256 citations · Full text
Management of Cystine Nephrolithiasis with Alpha-Mercaptopropionylglycine
Charles Y.C. Pak, Cindy J. Fuller, Khashayar Sakhaee et al. · The Journal of Urology · 1986 · 220 citations
Medicine, Nephrology, Pediatrics +11