Rare Diseases Symptoms Automatic Extraction

Extracorporal shock wave lithotripsy in the management of stones in children with oxalosis--still the first choice?

[primary hyperoxaluria type 1]

Primary hyperoxaluria (PH) is a recognised cause of nephrolithiasis. The aim of this study was to evaluate the success of extracorporal shock wave lithotripsy (ESWL) in treating nephrolithiasis in children with PH.This was a retrospective review of patient characteristics, treatments and outcomes of 36 children with oxalate stones due to PH.A total of 52 stones were formed in 28 patients, of which 23 stones were treated with ESWL. Of these 23 stones, ten improved and 13 did not; nine were located in the upper pole, nine in the lower pole and four and one in the pelvic and ureteric areas, respectively. All pelvic and ureteric stones improved, while 66.7 % of upper pole stones and 89.9 % of lower pole stones did not; 20 % of PH type 1 stones improved compared to 47 % of PH type 2 stones. The mean pre- and post-eGFR in stone-improvers was 98.82 and 104.7 ml/min/1.73 m(2), respectively; in the non-improvers, these values were 100.75 and 95.68 ml/min/1.73 m(2), respectively. Mean pre-ESWL stone size in the improved and non-improved groups was 7.3 mm and 8.5 mm respectively.Based on our results, ESWL is not the ideal method of stone therapy for patients with PH. ESWL was more effective in treating pelvic and ureteric stones, with upper pole stone response being better than lower pole response. PH2 patients were more than twice as likely to respond to ESWL treatment. Stone size and prior preventive treatment did not affect outcome. eGFR was not affected by ESWL.

Diseases presenting "ph" symptom

  • congenital diaphragmatic hernia
  • primary hyperoxaluria type 1

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