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Purpose: The pursuit of a dietary source to increase urine pH and citrate in stone formers has been ongoing for >30 years. Early evidence showed that orange juice (OJ) contains alkali and citrate, but high sugar and ascorbic acid content limited the use of OJ as a viable daily source of alkali. Recently, novel low-calorie OJs have emerged and could potentially be a better option. Methods: Beverages with high concentrations of alkali citrate and malate were identified using ion chromatography. Two low-calorie OJ beverages, in addition to crystal light lemonade beverage (CLLB), were chosen. Healthy volunteers (5 men, 5 women) drank 1 L of OJ or CLLB with 1 L water daily for 7 days, and then completed a 24-hour urinalysis. A washout week was instituted between trial weeks. The study design is a prospective randomized crossover control trial. A paired analysis using comparison of means was used to evaluate low-calorie OJ and CLLB. Volunteers had no prior history of kidney stones and maintained a journal with beverage compliance, side effect (SE), and dietary consumption data. Results: Tropicana 50 (TRP50), Kroger low-calorie OJ (KLCO), and CLLB were found to have a total alkali content of 56.60, 47.9, and 17.3 mEq/L, respectively, based on ion chromatography. Consumption of all three beverages raised urinary citrate (116.6 [-118 to 373, 177.9 [-3 to 359], 155.6 [-4 to 237] ▵mg/day 95% confidence interval) and urinary pH (0.25 [0.08-0.53], 0.74 [0.41-1.07 p  < 0.05], 0.25 [0.25-0.64]), respectively, compared with water phase. Based on journal entries by volunteers, TRP50 had the most SEs (90% participants) felt to be a result of the artificial sweetener (Stevia ® ). Conclusion: Low-calorie OJs, and to a lesser extent CLLB, have alkali and citrate based on ion chromatography. Daily consumption by healthy volunteers of KLCO can raise urinary pH.

Purpose: To determine the effects of regular and diet lemonade upon urinary parameters affecting kidney stone formation., Methods: In this prospective blinded crossover study, 12 healthy participants consumed either 2 L of regular or diet lemonade daily while on a standardized low sodium moderate calcium diet. Twenty four-hour urine collections were obtained at baseline on the controlled diet only and on days 4 and 5 of each treatment phase. There was a 1-week washout period between regular and diet lemonade treatments. Primary outcomes were urine citrate, pH, and volume determined by 24-hour urine collections. Secondary outcomes included the supersaturation of calcium oxalate, calcium phosphate, and uric acid., Results: Urine volume was significantly higher with both regular and diet lemonade consumption compared with baseline values. Urinary citrate significantly increased from baseline with diet lemonade only. Urine pH was unchanged with both beverages. The supersaturation of calcium oxalate significantly decreased with diet lemonade only, whereas supersaturation of calcium phosphate decreased with both beverages. Daily consumption of 2 L of regular and diet lemonade resulted in an intake of 168.4 and 170.2 mEq of citrate but a total alkali intake of 12.2 and 16.0 mEq, respectively. Compared with diet lemonade, regular lemonade provided subjects with 805 additional calories and 225 g of sugar per day., Conclusions: Diet lemonade may provide a low-calorie sugar-free cost-effective option for decreasing the risk of recurrent calcium nephrolithiasis through a significant increase in urine volume, increase in urinary citrate, and reduction in supersaturation of calcium oxalate and calcium phosphate.

Introduction: Cystinuria is an inherited disorder characterized by the impaired reabsorption of the dibasic amino acid, cystine, in the proximal tubule of the nephron. Cystine stones most frequently occur in the first through third decades of life with a decreased incidence in old age. We hypothesize that patients presenting with first stone event at an older age would have more favorable 24-hour urine parameters compared with those patients who present early., Patients: This is a retrospective review of 94 patients with cystinuria and cystine stones who underwent a 24-hour urine test. The patients were divided into four groups based on age at first clinical stone event: group 1, children aged <18 years (n = 37); group 2, 18 to 32 years (n = 19); group 3, 33 to 50 years (n = 19); and group 4, >50 years (n = 19). All 24-hour urines were collected prior to any medical therapy. Statistical analysis was performed using analysis of variance and Tukey pairwise comparison test., Results: There was a significant increase in cystine supersaturation (SS) for the young adults (group 2) as opposed to older adults (groups 3 and 4) though cystine excretion was not different among the groups. A higher urine volume appeared to account for the lower cystine SS in the older adults. The children were found to have cystine SS similar to the young adults., Discussion: In this retrospective study, a correlation was found between the age of onset of cystine stones and 24-hour urine parameters. Cystinuric patients presenting with first stone event at an older age have lower cystine SS and increased urinary volume compared with patients presenting earlier, likely conferring a protective effect.

Purpose: To determine the effect of cystine-binding thiol drugs (CBTD) on urinary cystine capacity in patients with cystinuria., Patients and Methods: Seven cystinuric patients performed two sets of urine collections while on and off CBTD while controlling for all other variables: diet and fluid and alkali intake. They monitored and recorded their diet for 3 days and performed urine collections on days 2 and 3. They then stopped the CBTD for 7 days. On days 8, 9, and 10, they replicated their diets of days 1 through 3 and performed two more urine collections on days 9 and 10. Two patients took D-penicillamine, four took tiopronin, and one took tiopronin and captopril. The cystine capacity was determined, and the values obtained when the patient was on and off the CBTD were compared to determine whether CBTDs affect urinary cystine capacity. To measure the cystine capacity, we used a solid-phase assay in which cystine crystals are added to the urine and incubated for 48 hours. The crystals are spun down and resolubilized in high-pH buffer, and the amount of cystine in the crystals is calculated. The solid phase will take up cystine from urine (negative cystine capacity) that is supersaturated and give up cystine to an undersaturated urine (positive cystine capacity)., Results: All seven patients had significant improvement in urinary cystine capacity on CBTDs. The mean cystine capacity off CBTD was -130.6 +/- 280.8, while the value during CBTD use was 43.1 +/- 131.2 (P < 0.05). On CBTDs, two patients still had negative values, but both had important improvements. The mean urinary volumes were similar on and off CBTD, indicating adequate and similar fluid intake. Urine pH values and urinary excretion of sodium and urea also were comparable, indicating consistency of citrate intake and diet., Conclusions: Our results demonstrate that CBTDs lower the urinary supersaturation of cystine, as shown by a less-negative or more-positive cystine capacity. Cystine capacity can be measured directly, even in the presence of CBTDs. The value of this measurement lies in the potential to monitor the response to the drug, prescribe the minimum effective dose, and potentially decrease the adverse effects often associated with CBTDs.