This study aimed to define glomerular filtration rate (GFR) decline, hypertension, and proteinuria in a well-defined autosomal recessive polycystic kidney disease (ARPKD) cohort and compared with two congenital kidney disease control groups (hypoplastic/dysplastic disorders and obstructive uropathies). The overall rates of GFR decline did not differ significantly in subjects with ARPKD vs controls. There were no significant differences in rates of hypertension or left ventricular hypertrophy, but subjects with ARPKD had a greater percent on ³3 blood pressure medications, more angiotensin-converting enzyme inhibitor use, and less proteinuria. The authors conclude that the relatively slow rate of GFR decline in subjects with ARPKD and absence of significant proteinuria suggest that these standard clinical measures may have limited utility in assessing therapeutic interventions, and highlight the need for other ARPKD kidney disease progression biomarkers.

The aim of this study was to identify possible urinary risk factors for hematuria in children. The authors retrospectively evaluated clinical onset, family history, and metabolic risk factors of 60 children with idiopathic hematuria but without renal stones or other pathologic conditions that could explain the hematuria. A family history of stone disease was found in 63% of the children. At least one urinary metabolic abnormality was present in 49 patients, while 11 patients had no metabolic abnormality. The most common urinary risk factor was idiopathic hypercalciuria (single or associated) 43.5% of patients, followed by hypocitraturia (single or associated) in 31.7%. The authors also found hyperoxaluria and, less frequently, hypomagnesuria and hyperuricosuria. The authors concluded that asymptomatic idiopathic hematuria in pediatric patients may often be linked to different urinary biochemical abnormalities, similar to what is observed in pediatric kidney stone-formers.