Renal function in glycogen storage disease type I, natural course, and renopreservative effects of ACE inhibition.

Background And Objectives: Renal failure is a major complication in glycogen storage disease type I (GSD I). We studied the natural course of renal function in GSD I patients. We studied differences between patients in optimal and nonoptimal metabolic control and possible renoprotective effects of angiotensin converting enzyme inhibition.

Altered activity of plasma hemopexin in patients with minimal change disease in relapse.

Since an active isoform of plasma hemopexin (Hx) has been proposed to be a potential effector molecule in minimal change disease (MCD), we tested plasma and urine samples from subjects with MCD in relapse (n = 18) or in remission (n = 23) (after treatment with prednisolone) for presence or activity of Hx. For comparison, plasma or urine from proteinuric subjects with focal and segmental glomerulosclerosis (FSGS, n = 11), membranoproliferative glomerulonephritis (MPGN, n = 9), IgA nephropathy (n = 5) or healthy control donors (n = 10), were incorporated into the study. Electrophoresis and Western blotting methods were used for evaluation of the Hx status, whereas protease activity of Hx was tested upon kidney tissue in vitro according to standard methods.