The challenge of cystine and struvite stone formers: clinical, metabolic and surgical assessment

Yamaçake, Kleiton; Marchini, Giovanni Scala; Reis, Sabrina T.; Danilovic, Alexandre; Vicentini, Fábio Carvalho; Torricelli, Fábio César Miranda; Srougi, Miguel; Mazzucchi, Eduardo

doi:10.1590/s1677-5538.ibju.2015.0741

Cited by 7 publications

(16 citation statements)

References 22 publications

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“…10, 11, 17-20, 23-34 20 studies reported outcomes from surgical interventions in cystinuria patients. 10, 11, 17-20, 24-26, 29, 30, 32-40 Of the 24 studies, 15 reported combined intervention methods with alkalinizing compounds, pharmacological drugs and/or surgical procedures. 10, 11, 17-20, 24-27, 29, 30, 32-34 No studies directly compared pharmacological intervention to surgical intervention.…”

Section: Resultsmentioning

confidence: 99%

“…19 studies reported mean patient age, which ranged from 7.4 to 49 years. 10, 17-20, 23-28, 31, 32, 34-38, 40 Of the 24 studies, 5 reported median patient age, which ranged from 6 to 18.5 years. 11, 29, 30, 33, 39…”

Section: Resultsmentioning

confidence: 99%

“…Either single or combined surgical procedures were partially effective in removing stones. 10, 11, 17-20, 24-26, 29, 30, 32-40 Ahmed et al . reported cystine stone patients needed repeated surgical procedures to remove stones including ESWL which accounted for 61%, URS 21%, PCNL 12% and OP 6% of surgical procedures.…”

Section: Resultsmentioning

confidence: 99%

“…25 Stone free rate after first PCNL was 0% in cystine stone group and 40% in struvite stone group, and final stone free rate after a secondary procedure was increased to 70% in cystine stone group and 80% in struvite stone group. 40 RIRS had a 100% stone free rate and it was considered a safe intervention in pediatric cystine stone management. 20…”

Section: Resultsmentioning

confidence: 99%

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Intervention strategies for cystinuria: A systematic review

Bhatt

Deshpande

Jones‐Freeman

et al. 2020

Preprint

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Purpose: This systematic review evaluates the current intervention strategies for cystinuria and assesses their quality and efficacy in order to determine the need to identify new and improved strategies for treatment. Materials and Methods: A literature search for interventions in cystinuria was conducted on key electronic databases for studies published between 1996 and 2019. Quality was assessed using Methodological Index for Non-Randomized Studies (MINORS). Studies meeting the inclusion criteria were assessed for study design, patient characteristics and outcomes of interventions, including urinary cystine levels, stone-free rate and stone recurrence rate. A qualitative and critical analysis was performed. Results: Common treatment strategies for cystinuria include hydration and diuresis, alkali therapy and thiol-based therapeutics. Conservative therapies such as adequate hydration and urinary alkalization effectively increased urinary pH, decreased urinary cystine levels and the formation of cystine stones. Second line agents reported included thiols such as Tiopronin, D-penicillamine and captopril. Non-invasive surgical procedures were found to reduce operative trauma and preserved renal function. Combined treatment approaches with hydration and thiols after surgical procedures were associated with less stones and reduced stone recurrence rates. Patient compliance to interventions was often poor and contributed to recurrent cystine stones. Conclusions: Despite existing pharmacological intervention strategies, cystinuria patients frequently require surgical procedures. A more detailed understanding of the mechanisms of pathogenesis of cystinuria as well as an evaluation of patients on an individual basis may be beneficial in reducing the severity of cystinuria, by reducing stone recurrence and associated renal complications.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Intervention strategies for cystinuria: A systematic review

Bhatt

Deshpande

Jones‐Freeman

et al. 2020

Preprint

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“…Despite constituting only 1% of adult and 6–10% of paediatric urinary tract calculi [ 1 – 3 ], patients with cystinuria are often the most challenging stone formers to manage [ 4 , 5 ]. With initial stone presentation often within the first two decades of life and recurrence rates of 60% or greater [ 6 ], cystinurics require lifelong treatment and intensive follow-up.…”

Section: Introductionmentioning

confidence: 99%

Journey of a cystinuric patient with a long-term follow-up from a medical stone clinic: necessity to be SaFER (stone and fragments entirely removed)

2018

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There is a lack of studies looking at the longitudinal follow-up of patients with cystine stones. We wanted to assess the journey of cystinuric patients through our specialist metabolic stone clinic to improve the understanding of episodes, interventions and current outcomes in this patient cohort. After ethical approval, all patients who attended our metabolic stone clinic from 1994 to 2014 with at least one cystine stone episode were included in our study. Data were retrospectively analysed for patient demographics, stone episodes or intervention, clinical parameters and patient compliance. Over a period of 21 years, 16 patients with a median age of 15.5 years underwent a mean follow-up of 8.6 years (1-21 years). The mean number of surgical interventions was 3.1 (1-8/patient), but patients who were stone free after their first treatment had lower recurrences (p = 0.91) and lower number of interventions during their follow-up (2.7/patient, compared to those who were not stone free at 4/patient). During their follow-up period, patients with < 3 interventions had a significantly better renal function than those with ≥ 3 surgical interventions (p = 0.04). Additionally, linear regression analysis showed that eGFR was demonstrated to decline with increasing numbers of stone episodes (r 2 = 0.169). It was also noted that patients who began early medical management remained stone free during follow-up compared to those who had medical management after ≥ 2 stone episodes, of whom all had a recurrent episode. Our long-term longitudinal study of cystine stone formers highlights that patients who are stone free and receive early metabolic stone screening and medical management after their initial presentation have the lowest recurrence rates and tend to preserve their renal function. Hence, prompt referral for metabolic assessment, and the stone and fragments entirely removed (SaFER) principles are key to preventing stone episodes and improving long-term function.

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