The development of a pediatric emergency transport system.

This article discusses the role of the pediatric emergency transport system as an outreach modality of the pediatric intensive care unit. Most of the discussion focuses on the Denver model.

The association of familial liver disease, subepidermal immunoproteins, and membranoproliferative glomerulonephritis.

Herein we report a new familial form of hepatic disease. Each of the four patients had splenomegaly, hypersplenism, a small liver, biochemical evidence of hepatic excretory dysfunction and hepatocellular damage, kidneys without demonstrable cysts, and normal blood pressue. An evaluation of serum immunoproteins, autoantibodies, histocompatibility antigens, and mixed lymphocyte reactivity further defined the immunologic features of this syndrome. Extrahepatic manifestations included a papulosquamous dermatitis with deposition of immunoglobulins and complement in both normal and abnormal skin, a membranoproliferative glomerulonephritis with subendothelial deposits, arthritis, and pericardial, pleural, and synovial effusions.

The role of complement, immunoglobulin and bacterial antigen in coagulase-negative staphylococcal shunt nephritis.

We describe three patients with arrested hydrocephalus in whom glomerulonephritis developed secondary to Staphylococcus epidermidis bacteremia from an infected ventriculoatrial shunt. Investigation of the immune-mediated renal disease associated with this chronic infection showed that (1) complement depletion during the acute phase of bacteremia and nephritis was predominantly via the classic pathway; (2) rheumatoid factor was associated with bacteremia, fever, proteinuria and low complement levels; (3) early complement components (C1q, C4, C3), immunoglobulin (predominantly immunoglobulin M [IgM], Staph. epidermidis antigen(s) and electron denxe subendothelial deposits were localized within the renal glomerulus; (4) C1q, and IgM derived from patient serums, were the most prominent in vitro immunoreactants to Staph. epidermidis cell walls; and (5) the causative organisms, Staph. epidermidis, shared common antigens with Staph. aureus, and antibody from patient serums cross reacted with extracts from both of these organisms.

Acute eosinophilic interstitial nephritis and renal failure with bone marrow-lymph node granulomas and anterior uveitis. A new syndrome.

We describe two patients with a unique granulomatous syndrome who presented with renal failure secondary to diffuse eosinophilic interstitial nephritis. Both had bilateral anterior uveitis, bone marrow granulomas, hypergammaglobulinemia and an increased sedimentation rate. One patient had lymph node granulomas and an immunoglobulin G (IgG) rheumatoid factor. An extensive investigation for an etiologic agent was unrewarding, and neither patient could be placed into any existing diagnostic category. Over a period of 2 years both patients have experienced improved renal function and dissolution of their bone marrow granulomas.

The secretory immune system and renal disease.

An immunopathological analysis of renal tissue from 105 patients was undertaken: (1) to clarify the relationship of the secretory immune system to renal diseases in which glomerular deposits of immunoglobulin A, (alpha chain), occurred; (2) to determine the lower nephron localization of secretory component and alpha chain in renal disease. This study, which included twenty-four patients with glomerular deposits of alpha chain, failed to reveal glomerular localization of secretory IgA. Secretory component was not found in renal tubular cells in kidneys with normal or minimally abnormal renal histology. In contradistinction to these findings, significant amounts of secretory component were found in tubular epithelial cells and casts in tissue from fifty-one patients with morphological evidence of significant renal damage; this localization had no correlation with glomerular deposits of IgA, IgM or other immunoreactants. Alpha Chain was rarely found in the tubular epithelium or in interstitial round cells; fifteen patients had alpha chain in casts. We conclude that the glomerular localization of immunoglobulin in glomerulonephritis is not derived from the secretory immune system, and the IgA present in glomeruli is not secretory IgA. The finding of secretory component in tubular cells in diseased kidneys without alpha chain may support an hypothesis for an independent role for secretory component in renal disease, apart from its function in the transport and stabilization of secretory IgA.