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Carrasco A Jr et al, 2015: Surgical management of stone disease in patients with primary hyperoxaluria.

Carrasco A Jr, Granberg CF, Gettman MT, Milliner DS, Krambeck AE.
Department of Urology, Mayo Clinic, Rochester, MN.
Department of Nephrology and Hypertension, Mayo Clinic, Rochester, MN.

Abstract

OBJECTIVE: To present our experience with surgical management of nephrolithiasis in patients with primary hyperoxaluria (PH).
METHODS: A retrospective chart review from 1994 to 2012 was performed to identify patients with diagnosis of PH.
RESULTS: A total of 14 patients with PH were identified with a median follow-up of 18.6 years (range, 0.9-51 years). Median ages at initial symptom and subsequent diagnosis were 6.7 years (range, 1.1-35.5 years) and 0.42 years (range, 0-33.25 years), respectively. Patients underwent a total of 54 procedures at our institution, including ureteroscopy (27 [50%]), percutaneous nephrolithotomy (15 [28%]), shock wave lithotripsy (8 [15%]), and combined procedures (4 [7%]). Overall nonintraparenchymal stone-free rate after the first, second, and third procedures were 59%, 76%, and 78%, respectively. On average, 1.6 procedures (range, 1-4) were required to rid patients of symptomatic stones, which subsequently afforded them a mean of 3.62 years (range, 0.25-21.5 years) without the need for additional intervention. There were 6 Clavien grade ≥III complications in 4 patients, including immediate postoperative end-stage renal disease in 3 patients.
CONCLUSION: Despite optimal medical and surgical management, patients experience recurrent acute stone events requiring multiple urologic interventions. Significant complications such as end-stage renal disease can occur secondary to surgical intervention.

Urology. 2015 Mar;85(3):522-6. doi:10.1016/j.urology.2014.11.018. 

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Comments 1

Peter Alken on Monday, 01 June 2015 07:34

The authors also offer a brief look at all 149 PH patients they have seen at Mayo clinic: a median time from presentation to diagnosis of 2,7 years illustrates the urological deficits in establishing the proper diagnosis of PH in a reasonable time.
In those 14 patients completely treated at their institution the stone free status was especially defined: “Given that nephrocalcinosis, in the form of urothelial and parenchymal calcifications commonly found in PH kidneys, makes it difficult to determine if patients are
radiographically stone free, we defined successful treatment as complete clearance of nonintraparenchymal stones (ie, freefloating intraluminal stones) per targeted renal unit.”
Despite these limitations the stone free rate achieved is disappointing.
As to the ESWL treatment of PH patients the authors state: “Although it is difficult to extrapolate the observed side effects of shock-wave energy in animal studies, one cannot ignore the risk of parenchymal damage, particularly in small kidneys. Additionally, there are reports of patient with PH developing progressive renal failure after SWL. Thus, based on our experience with SWL, we rarely perform SWL in patients with PH.” But 6 major complications (Clavien grade≥ III) did not happen after SWL but in 4 patients after PNL. “Three patients developed ESRD within 30 days of the procedure.”
The most important information is in the conclusion:” Finally, it must be recognized that subtle changes in GFR in patients with PH can result in oxalosis crisis and has the potential for the development of ESRD. Therefore, any acute stone event should be treated
expeditiously to prevent oxalosis crisis.”

The authors also offer a brief look at all 149 PH patients they have seen at Mayo clinic: a median time from presentation to diagnosis of 2,7 years illustrates the urological deficits in establishing the proper diagnosis of PH in a reasonable time. In those 14 patients completely treated at their institution the stone free status was especially defined: “Given that nephrocalcinosis, in the form of urothelial and parenchymal calcifications commonly found in PH kidneys, makes it difficult to determine if patients are radiographically stone free, we defined successful treatment as complete clearance of nonintraparenchymal stones (ie, freefloating intraluminal stones) per targeted renal unit.” Despite these limitations the stone free rate achieved is disappointing. As to the ESWL treatment of PH patients the authors state: “Although it is difficult to extrapolate the observed side effects of shock-wave energy in animal studies, one cannot ignore the risk of parenchymal damage, particularly in small kidneys. Additionally, there are reports of patient with PH developing progressive renal failure after SWL. Thus, based on our experience with SWL, we rarely perform SWL in patients with PH.” But 6 major complications (Clavien grade≥ III) did not happen after SWL but in 4 patients after PNL. “Three patients developed ESRD within 30 days of the procedure.” The most important information is in the conclusion:” Finally, it must be recognized that subtle changes in GFR in patients with PH can result in oxalosis crisis and has the potential for the development of ESRD. Therefore, any acute stone event should be treated expeditiously to prevent oxalosis crisis.”
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