An uncommon cause of acute kidney injury in young children: Cystinuria

Nalçacıoğlu, Hülya; Özden, Ender; Genç, Gürkan; Yakupoğlu, Yarkın Kamil; Sarıkaya, Şaban; Özkaya, Ozan

doi:10.1016/j.jpurol.2012.08.006

Cited by 10 publications

(8 citation statements)

References 23 publications

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“…In cystine stones, regular follow-up and treatment are important due to the risk of high recurrence rates. On the other hand, it has also the risk of progressive renal failure (1)(2)(3)(4)(5)(6)(7)(8)(13)(14)(15)18,19).…”

Section: Discussionmentioning

confidence: 99%

“…Thus, medical treatment should aim to reduce the cystine level and to increase the solubility of cystine in the urine. High levels of fluid intake, dietary sodium restriction, alkalinization of the urine with potassium citrate to maintain a urine pH>6.5 and the usage of thiol drugs are the main treatments (5,(14)(15)(16)(17)(18)(19)(20).…”

Section: Discussionmentioning

confidence: 99%

“…However, the effectiveness of these treatments remain controversial, and surgery is frequently required to remove urinary stones (9)(10)(11)(12). In addition, it is also accompanied by the risk of progressive renal impairment (13)(14)(15).…”

Section: Introductionmentioning

confidence: 99%

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Sistin Taşı Olan Çocuk Hastaların Değerlendirilmesi: Tek Merkez Deneyimi

Çayci¹,

Acar²,

Tiryaki³

et al. 2020

Turkish Journal of Pediatric Disease

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Objective: Cystinuria is a rare genetic disorder. Many patients suffer from significant recurrent urolithiasis, repeated surgical interventions, and the risk of progressive renal impairment. In the current study, the outcomes of patients with cystine stones were investigated. Material and Methods:A total of Twenty-six cystinuria patients with cystine stones, aged between 3 months and 18 years, in our Pediatric Nephrology Department, were retrospectively analyzed. Results:The mean age of patients at diagnosis was 45.2±45.5 months and 88,5% were male. Sixteen (62%) children had recurrent urinary tract infections. Only 10 (38%) patients showed additional metabolic abnormalities. The urinary pH had significantly increased with treatment and the number of stone recurrence was lower in the patients with urinary pHs ≥ 6.5. There was a significant positive correlation between the last-visit serum creatinine level and the number of surgical interventions. There was no significant correlation between the last-visit eGFR and the number of surgical interventions. On the other hand, eGFR values decreases as the total number of surgical interventions increases. No stone events were observed at the end of the follow-up period in 10 patients (38%) and the stone events per patientyear were 0.36 for all patients. Four patients with low eGFRs at the beginning of the study get normal with treatment after the follow-up period. Conclusion:Cystinuria has significant morbidity if not controlled properly. Despite all treatments, it should be kept in mind that renal impairment may develop in cystine stones with cystinuria and surgical treatment should be planned by considering minimally invasive options.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Sistin Taşı Olan Çocuk Hastaların Değerlendirilmesi: Tek Merkez Deneyimi

Çayci¹,

Acar²,

Tiryaki³

et al. 2020

Turkish Journal of Pediatric Disease

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“…Bilateral stones may also increase the risk for acute kidney injury, particularly in children. Indeed, young children with acute renal failure due to ureteral stones should be tested for cystinuria (Nalcacioglu et al, 2013). Compared with calcium oxalate stone formers, patients with cystinuria are more likely to have abnormal serum creatinine levels and are at higher risk for nephrectomy (Assimos, Leslie, Ng, Streem, & Hart, 2002).…”

Section: Cystinuriamentioning

confidence: 99%

Early Recognition and Management of Rare Kidney Stone Disorders

Goldstein¹,

Goldfarb²

2018

UNJ

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“…The urinary epidermal growth factor (EGF)/ monocyte chemotactic peptide-1 (MCP-1) ratio measured 4 weeks after relief of obstruction correlated with long term outcome, suggesting pathways that favor long term fibrosis are activated quite early. In case series up to 15–23% of patients that present with AKI and obstruction may require short-term dialysis [11, 14], although early relief of obstruction and prompt correction of electrolyte and acid-base imbalances can minimize this risk [15]. …”

Section: Surgical Treatment Of Nephrolithiasis and Long-term Renal Oumentioning

confidence: 99%

Acute and chronic kidney injury in nephrolithiasis

Tang

Lieske

2014

Current Opinion in Nephrology and Hypertension

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Purpose of review Nephrolithiasis is a common systemic disease associated with both acute kidney injury (AKI) and chronic kidney disease (CKD). The purpose of this review is to discuss recent publications on nephrolithiasis-associated kidney damage, with an emphasis on AKI. Recent findings Nephrolithiasis is not a common cause of adult AKI (1–2% of cases), although it may be a more important factor in young children (up to 30%). The primary mechanism of nephrolithiasis-associated AKI is obstructive nephropathy, and factors at presentation with obstructive uropathy predict the likelihood of long-term renal recovery. Crystalline nephropathy is another potential pathway in certain circumstances that is often associated with a worse outcome. Recent studies have elucidated additional pathways whereby calcium oxalate crystals can cause acute injury, implicating innate immunity and intracellular inflammasome pathways. Several large cohort studies have demonstrated an independent association of nephrolithiasis with CKD and ESRD, although the effect size is modest. Urologic comorbidities, urinary infection, and shared underlying risk factors (e.g., diabetes, hypertension) all impact nephrolithiasis-associated CKD risk. Summary Obstructive nephropathy and crystalline nephropathy both contribute to nephrolithiasis-associated AKI, although the latter appears to have a worse prognosis. Nephrolithiasis is an independent albeit small risk factor for CKD. Further study is needed to clarify the incidence and mechanisms of nephrolithiasis-associated AKI, and the relationship between nephrolithiasis-associated AKI and CKD.

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An uncommon cause of acute kidney injury in young children: Cystinuria

Cited by 10 publications

References 23 publications

Sistin Taşı Olan Çocuk Hastaların Değerlendirilmesi: Tek Merkez Deneyimi

Sistin Taşı Olan Çocuk Hastaların Değerlendirilmesi: Tek Merkez Deneyimi

Early Recognition and Management of Rare Kidney Stone Disorders

Acute and chronic kidney injury in nephrolithiasis

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