The hyper-IgE syndrome is a multisystem disorder that affects the dentition, the skeleton, connective tissue, and the immune system. It is inherited as a single-locus autosomal dominant trait with variable expressivity.

Reductions in BP with NHD are accompanied by regression of LVH.

Focal and segmental glomerulosclerosis (FSGS) is one of the most common primary glomerular diseases to terminate in ESRD.A complete remission (CR) confers an excellent long-term prognosis, but the quantitative benefits of partial remissions (PR) have not been defined. This study evaluated the rate of renal function decline (slope of creatinine clearance) and renal survival in nephrotic FSGS patients with CR, PR, or no remission. It also examined relapse rate from remission and its impact on outcome. Multivariate analysis included clinical and laboratory data at presentation and over follow-up, BP control, the agents used, and immunosuppressive therapy. The study cohort was 281 nephrotic FSGS patients who had a minimum of 12 mo of observation and were identified from the Toronto Glomerulonephritis Registry. Over a median follow-up of 65 mo, 55 experienced a CR, 117 had a PR, and 109 had no remission. A PR was independently predictive of slope and survival from renal failure by multivariate analysis (adjusted time-dependent hazard ratio, 0.48; 95% confidence interval, 0.24 to 0.96; P ‫؍‬ 0.04). Immunosuppression with high-dose prednisone was associated with a higher rate of PR and CR. Relapse from PR was frequent (56%) and associated with a more rapid rate of renal function decline and worse renal survival compared with relapse-free partial remitters. Only female gender and the nadir of proteinuria during remission were associated with a sustained remission. A PR in proteinuria and its maintenance are important therapeutic targets in FSGS, with implications for both slowing progression rate and improving renal survival.J (FSGS) is one of the most common causes of primary glomerular disease that terminates in renal failure (1,2). In most series, its 10-yr survival is in the 40 to 60% range (3-7).Severity of proteinuria at onset and during follow-up have been associated with a poor outcome in several series (3-8).Although there is strong evidence that nephrotic patients who experience a complete remission (CR) have a very favorable prognosis (9 -11), the long-term outcome in adults with only a reduction in proteinuria has seldom been reported (11,12). Despite the lack of evidence of its value as a valid surrogate for improved renal survival in FSGS, it is often reported as a positive finding in clinical studies (11)(12)(13)(14).This study addresses the long-term implications of a partial remission (PR) in nephrotic FSGS patients. It compares the rate of renal function decline, relapse, renal failure, and treatment effects among patients with a PR, CR, and no remission (NR). Materials and MethodsAll FSGS patients from the Toronto Glomerulonephritis Registry were considered for this study. This database began in 1974 and includes all biopsy-proven cases of glomerulonephritis from the Toronto area. Patient information at onset is compiled using a standard form, and registrars perform a periodic prospective assessment of the patients' clinical status, medications, and laboratory results (15). We reviewed all previous m...