Contributory metabolic factors in the development of nephrolithiasis in patients with medullary sponge kidney

Yamada, Takashi; Kobayashi, Chizuko; Hayashi, Tetsuo; Yoshida, Ayako; Toma, Hiroshi

doi:10.1053/ajkd.2001.24515

Cited by 31 publications

(28 citation statements)

References 15 publications

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“…Hypercalcuria was present in 21% of 24 stone-forming patients with MSK in our series. Likewise, there are differences in reported prevalence of hyperoxaluria in MSK patients, from 0% of 17 patients [14] to 45% of 22 [13] , compared with our finding of hyperoxaluria in 17% of 24 patients. Local dietary factors, such as a low calcium and high oxalate diet seen in the Japanese population, may account for the data published by Yagisawa et al [13] .…”

Section: Discussioncontrasting

confidence: 96%

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A Review of the Natural Progression of Medullary Sponge Kidney and a Novel Grading System Based on Intravenous Urography Findings

et al. 2007

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Purpose: Medullary sponge kidney (MSK) is a developmental abnormality of the medullary pyramids of the kidney, characterised by cystic dilatations of the collecting ducts. We investigated a cohort of patients with MSK to gain further information about its presentation, clinical course and treatment required. We devised a grading system based upon findings at diagnostic intravenous urography (IVU) and established a relationship between severity of IVU findings and severity of disease. Materials and Methods: The clinical notes and imaging of 29 patients with MSK were analysed. The severity of IVU findings was classified as follows: grade 1 (one calyx, unilateral), grade 2 (one calyx, bilateral), grade 3 (more than one calyx, unilateral) and grade 4 (more than one calyx, bilateral). Results: The age range at diagnosis was 12–69 (mean 39), mean follow-up period was 12.7 years. Increasing grade of IVU findings correlated with more frequent symptomatic stone episodes (grade 1: 0.09 episodes per patient per year; grade 4: 0.34). Higher grade was also related to the number of hospital admissions (grade 1: 0.182 per patient per year; grade 4: 0.282) and the number of procedures required (either surgery or extracorporeal shock wave lithotripsy; grade 1: 0.0 interventions per patient per year; grade 4: 0.24). Conclusions: MSK presents over a wide age range and can cause long periods of intermittent episodes. Patients can be graded using a novel system based on IVU findings, which correlates with severity of disease. Management strategies such as intensity of follow-up can be modified using this grading.

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Section: Discussioncontrasting

confidence: 96%

“…Hypercalcuria is a common finding in stone-forming patients with MSK. Previous studies have reported a wide range of prevalence, from 9% of 22 patients [13] , 34% of 35 patients [5] through to 88% of 17 patients [14] . Hypercalcuria was present in 21% of 24 stone-forming patients with MSK in our series.…”

Section: Discussionmentioning

confidence: 98%

A Review of the Natural Progression of Medullary Sponge Kidney and a Novel Grading System Based on Intravenous Urography Findings

et al. 2007

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“…However, a subset of MSK patients are clinically silent [2] and the diagnosis is incidentally picked up on renal imaging for other indications. The strong association of MSK with nephrolithiasis, especially calcium stones [12], is explained by the tendency of urinary stasis in the ectatic collecting ducts and the association of high quantities of stone promoters like hypercalciuria [13] and reduced levels of inhibitors like citrate and magnesium [14] in the urine. The presence of high urinary pH (due to dRTA) can specifically increase the risk for calcium phosphate precipitation and stone formation [6], and phosphate stones are seen more frequently than oxalate [13].…”

Section: Introductionmentioning

confidence: 99%

CT Urography for the Diagnosis of Medullary Sponge Kidney

Koraishy

Ngo²,

Israel³

2014

Am J Nephrol

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Background: Medullary sponge kidney (MSK) is characterized by malformation of the terminal collecting ducts and is associated with an increased risk of nephrolithiasis, nephrocalcinosis, urinary tract infections, renal acidification defects, and reduced bone density. It has been historically diagnosed with intravenous pyelography (IVP), which is falling out of favor as an imaging modality. CT urography (CTU) performed with multidetector CT (MDCT) has been shown to create images of the renal collecting system with similar detail as IVP; however, its utility in diagnosing MSK has not been defined. Case Report: We present the first 15 patients with recurrent symptomatic nephrolithiasis who were evaluated in our renal stone clinic with CTU. Four patients were diagnosed with MSK after visualization of the characteristic radiologic findings. Discussion: CTU effectively demonstrates the characteristic radiologic findings of MSK including collecting tubule dilatation, medullary nephrocalcinosis, nephrolithiasis, and medullary cysts. Dose reduction protocols can reduce radiation exposure below that associated with conventional IVP. We propose CTU be considered for the diagnosis of MSK.

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“…This stagnant flow in dilated intramedullary collecting duct and particularly in cysts, predisposes to infection. In addition to such anatomic abnormalities, low levels of urinary inhibitors of stones seem to contribute to the pathogenesis of nephrolithiasis in patients with MSK [3]. MSK patients have defects in urinary acidification and concentration mechanism which may, in part, be the result of functional abnormality of the terminal collecting ducts [6].…”

Section: Discussionmentioning

confidence: 99%

“…The frequency of medullary sponge kidney is one case per 5000 general population, and the prevalence may be as much as 1 case per 1000 population in urology clinics. In addition, medullary sponge kidney has been identified in 12-20% of patients who form calcium stones [3]. The first sign of medullary sponge kidney is usually a UTI or a kidney stone.…”

Section: Introductionmentioning

confidence: 99%

Medullary Sponge Kidney with Incomplete Renal Tubular Acidosis: A Poorly Understood Disease

Gupta¹,

Jain²,

Chandra³

et al. 2016

JCR

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Abstract:Medullary sponge kidney (MSK) is usually a benign process which may remain asymptomatic and undetected for life. Incomplete distal renal tubular acidosis (RTA) may be found in as many as 30% to 40% of these patients. Clinical presentation usually occurs after 20 years of age. It may present with renal colic, recurrent urinary tract infection (UTI) and gross hematuria. We are presenting a case of 30 year old female who presented with recurrent urinary tract infection, underwent multiple surgeries for renal stone and due to insufficient follow up developed complications related to forgotten ureteric stent. She finally landed up as poorly functioning symptomatic right kidney and non-functioning left kidney for which nephrectomy was done. This case highlights the importance of earlier diagnosis, proper management and frequent follow up to preserve renal function in a case of MSK.

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Contributory metabolic factors in the development of nephrolithiasis in patients with medullary sponge kidney

Cited by 31 publications

References 15 publications

A Review of the Natural Progression of Medullary Sponge Kidney and a Novel Grading System Based on Intravenous Urography Findings

A Review of the Natural Progression of Medullary Sponge Kidney and a Novel Grading System Based on Intravenous Urography Findings

CT Urography for the Diagnosis of Medullary Sponge Kidney

Medullary Sponge Kidney with Incomplete Renal Tubular Acidosis: A Poorly Understood Disease

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