Treatment of Cystinuria

Dent, C. E.; Friedman, M.; Green, H.; Watson, L. C. A.

doi:10.1136/bmj.1.5432.403

Cited by 81 publications

(22 citation statements)

References 10 publications

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“…53 Moreover, an extremely low protein diet (20 g per day) was found to reduce urinary cystine excretion by 34%. 39,54 However, this level of protein restriction is not advisable, and might be harmful to patients. 55,56 In our clinic, a dietbased guideline is used to help patients achieve a restricted animal protein intake.…”

Section: Hydrationmentioning

confidence: 98%

“…38 However, this level of fluid intake is not sustainable for most patients and a target of exceeding 3 l per day is more realistic. 39,40 Specific regimens might also be recom mended, often involving waking at night to drink a speci fied volume of fluid. 38,39,41 Traditionally, night was thought to be when most patients were at highest risk of cystine supersaturation.…”

Section: Hydrationmentioning

confidence: 99%

“…39,40 Specific regimens might also be recom mended, often involving waking at night to drink a speci fied volume of fluid. 38,39,41 Traditionally, night was thought to be when most patients were at highest risk of cystine supersaturation. 42 However, studies have not shown a diurnal difference in the number of discrete super saturation episodes.…”

Section: Hydrationmentioning

confidence: 99%

“…This diet includes maintaining a healthy weight, limiting intake of salt and animal protein, and increasing vegeta ble protein (as a substitute for animal protein), fruit and vegetables. 6,33,39,40,[46][47][48][49][50] Historically, the ability of patients to adhere to specific dietary regimens and whether such interventions are too difficult to monitor has been questioned. 40 However, in our multidisciplinary practice, with regular dietetic input and support, patients have made positive changes to their diets.…”

Section: Hydrationmentioning

confidence: 99%

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Cystinuria—a urologist's perspective

et al. 2014

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Cystinuria is a genetic disease that leads to frequent formation of stones. In patients with recurrent stone formation, particularly patients <30 years old or those who have siblings with stone disease, urologists should maintain a high index of suspicion of the diagnosis of cystinuria. Patients with cystinuria require frequent follow-up and a multidisciplinary approach to diagnosis, prevention and management. Patients have reported success in preventing stone episodes by maintaining dietary changes using a tailored review from a specialist dietician. For patients who do not respond to conservative lifestyle measures, medical therapy to alkalinize urine and thiol-binding drugs can help. A pre-emptive approach to the surgical management of cystine stones is recommended by treating smaller stones with minimally invasive techniques before they enlarge to a size that makes management difficult. However, a multimodal approach can be required for larger complex stones. Current cystinuria research is focused on methods of monitoring disease activity, novel drug therapies and genotype-phenotype studies. The future of research is collaboration at a national and international level, facilitated by groups such as the Rare Kidney Stone Consortium and the UK Registry of Rare Kidney Diseases.

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Section: Hydrationmentioning

confidence: 98%

Section: Hydrationmentioning

confidence: 99%

Section: Hydrationmentioning

confidence: 99%

Section: Hydrationmentioning

confidence: 99%

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Cystinuria—a urologist's perspective

et al. 2014

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“…The more detailed radiographic appearances during dissolution of cystine stones in one particular patient (Case 2 of Dent et al, 1965) have led us to consider the possible deleterious effect of mineral contaminants. We therefore report crystallographic analysis of urinary cystine calculi and discuss the results and their possible significance.…”

mentioning

confidence: 99%

Cystine stone dissolution

Shaw¹,

Sutor²

1972

BJR

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The appearances of a cystine kidney stone undergoing dissolution therapy suggested the presence of calcium salts. Contaminants in cystine urinary stones have been investigated by X-ray crystallography.Of 29 stones, four contained sufficient insoluble material to present problems in therapy. The role of inhibitors of calcium oxalate crystal growth in the urine is discussed in relation to these findings.

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Measurement of urinary cystine and cysteinyl‐penicillamine in patients with cystinuria

Sampson

Stewart

Hammond

1986

Biomedical Chromatography

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A high-performance liquid chromatographic method with electrochemical detection (LC/EC) was developed to measure cystine and cysteinyl-penicillamine disulfide in the urine of patients screened or treated for cystinuria. Urine was acidified, centrifuged to remove urinary protein, diluted and injected. The disulfides were separated on a reversed-phase column, reduced at the upstream electrode of a dual electrochemical detector with gold-mercury amalgam (Au/Hg) electrodes and the resultant thiols measured at the downstream electrode. The sample preparation is simple, the analysis rapid, specimens can be easily batched and the specificity of the method is better than those of two other separative procedures with which it was compared. The coefficient of variation for cystine in cystinuric urine is 6.7%, 5.5% and 3.2% for levels of 0.09, 0.52 and 1.02 mmol/l respectively, and for cysteinyl-penicillamine disulfide 2.6% and 7.5% for levels of 0.45 and 0.98 mmol/l respectively. Urine for analysis of these disulfides should not be collected within 24 hours of administration of the radiopaque agent diatrizoate but no other interference to the assay has been noted. This method is suitable as a screen for cystinuria in patients with renal tract calculi, for ongoing monitoring of cystinuric patients and to check patient compliance with d-penicillamine therapy.

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Treatment of Cystinuria

Cited by 81 publications

References 10 publications

Cystinuria—a urologist's perspective

Cystinuria—a urologist's perspective

Cystine stone dissolution

Measurement of urinary cystine and cysteinyl‐penicillamine in patients with cystinuria

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