Kidney biopsy findings in children with sickle cell disease: a Midwest Pediatric Nephrology Consortium study.

BACKGROUND: Renal damage is a progressive complication of sickle cell disease (SCD). Microalbuminuria is common in children with SCD, while a smaller number of children have more severe renal manifestations necessitating kidney biopsy. There is limited information on renal biopsy findings in children with SCD and subsequent management and outcome. METHODS: This is a multicenter retrospective analysis of renal biopsy findings and clinical outcomes in children and adolescents with SCD. We included children and adolescents (age ≤ 20 years) with SCD who had a kidney biopsy performed at a pediatric nephrology unit. The clinical indication for biopsy, biopsy findings, subsequent treatments, and outcomes were analyzed. RESULTS: Thirty-six SCD patients (ages 4-19 years) were identified from 14 centers with a median follow-up of 2.6 years (0.4-10.4 years). The indications for biopsy were proteinuria (92%) and elevated creatinine (30%). All biopsies had abnormal findings, including mesangial hypercellularity (75%), focal segmental glomerulosclerosis (30%), membranoproliferative glomerulonephritis (16%), and thrombotic microangiopathy (2%). There was increased use of hydroxyurea, angiotensin-converting-enzyme inhibitors, and angiotensin receptor blockers following renal biopsy. At last follow-up, 3 patients were deceased, 2 developed insulin-dependent diabetes mellitus, 6 initiated chronic hemodialysis, 1 received a bone marrow transplant, and 1 received a kidney transplant. CONCLUSIONS: Renal biopsies, while not commonly performed in children with SCD, were universally abnormal. Outcomes were poor in this cohort of patients despite a variety of post-biopsy interventions. Effective early intervention to prevent chronic kidney disease (CKD) is needed to reduce morbidity and mortality in children with SCD.

Extracorporeal shockwave lithotripsy for upper tract urolithiasis.

PURPOSE OF REVIEW: For the last three decades, extracorporeal shockwave lithotripsy (SWL) has been the mainstay of management of urolithiasis; recognized widely by patients and physicians for its noninvasive approach and good outcomes. Recent challenges by endoscopic approaches have driven ongoing research to refine indications, define outcomes and explore innovations. RECENT FINDINGS: Utilization of SWL remains high, despite increasing utilization of endoscopic approaches. Patient selection is critical--outcomes with percutaneous nephrolithotomy and ureteroscopy after failed SWL are not as good as those obtained in patients who have not had prior SWL. A structured training in ultrasound localization and proper patient positioning can have dramatic impacts on stone-free results. Stone size, location, Hounsfield unit stone attenuation and stone volume remain important predictors of outcomes. Renal cysts may negatively impact outcomes with SWL. SUMMARY: These recent studies highlight important considerations for patient selection, SWL technique and follow-up for patients undergoing SWL. New technologies hold promise but require further study.