Childhood-onset IgA nephropathy: is long-term recovery possible?

Background: IgA nephropathy (IgAN) is the most common glomerulonephritis worldwide. While studies have primarily focused on identifying risk factors for disease progression, very few data exist on the likelihood of achieving complete recovery from the disease.

Methods: We conducted a single-center retrospective study on all consecutive patients with biopsy-proven IgAN diagnosed between 1986 and 2018 in our pediatric center.

Persistently active interferon-γ pathway and expansion of T-bet B cells in a subset of patients with childhood-onset systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is an autoimmune disease causing significant morbidity and mortality, despite important improvements in its management in the last decades. The objective of this work is to investigate the role of IFN-γ in the pathogenesis of childhood-onset systemic lupus erythematosus (cSLE), evaluating the crosstalk between IFN-α and IFN-γ and the expression of T-bet, a transcription factor induced by IFN-γ, in B cells of patients with cSLE. Expression levels of both IFN-α and IFN-γ-induced genes were upregulated in patients with cSLE.

Treatment of idiopathic nephrotic syndrome at onset: a comparison between 8- and 12-week regimens in everyday clinical practice.

Background: Optimal steroid treatment at onset of idiopathic nephrotic syndrome is still debated. The aim of this study was to analyze the clinical outcome at 24 months of follow-up in patients admitted to our unit for the first episode of steroid-sensitive nephrotic syndrome comparing two different steroid regimens.

Methods: We collected data on patients treated from 1992 to 2007 with prednisone according to the International Study on Kidney Diseases in Children 8-week regimen and since 2008 according to the Arbeitsgemeinschaft fur Padiatrische Nephrologie 12-week regimen.

IgM on the surface of T cells: a novel biomarker of pediatric-onset systemic lupus erythematosus.

Background: Children with systemic lupus erythematosus (SLE) frequently have kidney involvement. Lupus nephritis sometimes presents alone, without systemic SLE features, representing the so-called full-house nephropathy (FHN). Distinguishing patients with SLE or FHN has therapeutic and prognostic implications.

A case of haemolytic uraemic syndrome (HUS) revealed an outbreak of Shiga toxin-2-producing O26:H11 infection in a nursery, with long-lasting shedders and person-to-person transmission, Italy 2015.

Shiga toxin-producing (STEC) represents a major issue for public health because of the severity of the associated illnesses, including haemolytic uraemic syndrome (HUS). In 2015, investigation of a case of HUS revealed an outbreak of Shiga toxin-2-producing O26 : H11 infection in a nursery in Italy. The investigation showed that the infection was transmitted to cases' contacts via person to person.

Outcome of childhood-onset full-house nephropathy.

Background: Patients with full-house nephropathy (FHN) present renal lesions that are indistinguishable from those of lupus nephritis (LN) but lack the systemic features necessary to meet diagnostic criteria for systemic lupus erithematosus (SLE). Some have been reported to develop a delayed SLE with time. The clinical outcome of children having FHN without SLE has never been reported.

Lupus nephritis in children and adolescents: results of the Italian Collaborative Study.

Background: Lupus nephritis (LN) strongly affects the outcome in children with systemic lupus erythematosus (SLE). Many patients, however, have renal disease at onset, but lack a sufficient number of criteria to be diagnosed as SLE and develop delayed symptoms over time (d-SLE). Data on the clinical course, long-term outcome and predictors of disease progression in children with LN are scant.

Usefulness of skin immunofluorescence for distinguishing SLE from SLE-like renal lesions: a pilot study.

Lupus nephritis (LN) may represent a diagnostic problem, particularly in pediatric patients that present with typical histological lesions but do not fulfill the American Rheumatism Association (ARA) criteria for the diagnosis of systemic lupus erythematosus (SLE). Based on the well-described deposition of immunoglobulins (Ig) and complement at the dermoepithelial junction in SLE, we hypothesized that skin biopsies may help in the diagnosis of LN. To test this hypothesis, we carried out a pilot study, performing a skin biopsy in 22 patients with LN and 13 patients with lupus-like lesions, regardless of the time elapsed from onset of renal disease.

Risk factors for cyclosporin A nephrotoxicity in children with steroid-dependant nephrotic syndrome.

Background And Objectives: Cyclosporin A (CsA) is a well-established treatment for steroid-dependent nephrotic syndrome (SDNS) that may, however, cause chronic ischemic renal lesions. The objective of the study was to assess the prevalence of CsA nephrotoxicity (CsAN) in protocol biopsies of children with SDNS.

Design, Settings, Participants, & Measurements: From 1990 through 2008, we performed 71 renal biopsies in 53 patients with SDNS.

Genetic risk factors in typical haemolytic uraemic syndrome.

Background: Haemolytic uraemic syndrome (HUS) is a disorder characterized by thrombotic microangiopathy, which is caused in 'typical forms' by gastrointestinal infections with Escherichia Coli species that produce verotoxins. Several studies have identified negative prognostic factors of the disease, among which prolonged oliguria, neurological involvement and increased leukocytosis have been more consistently reported. We have hypothesized that the genetic background may also predispose to the development of typical forms of HUS and may influence the clinical course of the disease.

Blood pressure in the long-term follow-up of children with hemolytic uremic syndrome.

The hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure (ARF) in young children. Most patients recover from the acute phase of the illness but they may develop arterial hypertension(AH) after many years, even in the absence of signs of renal impairment during short-term follow-up. In this study, we performed casual blood pressure (BP) measurement, 24-h blood pressure monitoring (ABPM), and a Bruce walking treadmill study (ET) in 24 children (aged 5-15 years, 13 males, 11 females) with a history of HUS and normal renal function during follow-up (median 5.

Risk factors for poor renal prognosis in children with hemolytic uremic syndrome.

Many factors have been proposed as predictors of poor renal prognosis in children with hemolytic uremic syndrome (HUS), but their role is still controversial. Our aim was to detect the most reliable early predictors of poor renal prognosis to promptly identify children at major risk of bad outcome who could eventually benefit from early specific treatments, such as plasmapheresis. Prognostic factors identifiable at onset of HUS were evaluated by survival analysis and a proportional hazard model.

Shiga toxin-producing Escherichia coli infections associated with hemolytic uremic syndrome, Italy, 1988-2000.

The mean annual incidence of hemolytic uremic syndrome in persons View Article and Find Full Text PDF

Role of non-polio enterovirus infection in pediatric hemolytic uremic syndrome.

Verocytotoxin-producing Escherichia coli(VTEC) infections cause most cases of hemolytic uremic syndrome (HUS); 10-30% of patients, however, are negative for VTEC infection. The etiology of HUS in VTEC-negative cases remains poorly understood. Before the association between VTEC infection and HUS was recognized, sporadic cases of HUS with enterovirus infection were reported in the literature.

Delayed onset of systemic lupus erythematosus in patients with "full-house" nephropathy.

Three patients are described who presented with a glomerulopathy suggestive of lupus nephritis in the absence of other clinical and biological evidence of systemic lupus erythematosus (SLE). Renal biopsies showed a "full-house" immunofluorescence pattern and two patients also had cytoplasmic tubuloreticular inclusions by electron microscopy. All these patients developed antinuclear and anti-double-stranded DNA antibodies 3, 5, and 10 years after their original presentation.

Retrospective study of plasma exchange in patients with idiopathic rapidly progressive glomerulonephritis and vasculitis.

A retrospective study of 48 patients was conducted to evaluate the efficacy of plasma exchange in children with idiopathic rapidly progressive glomerulonephritis (IRPGN), and renal or non-renal vasculitis. All patients were followed up at a single centre over a 15 year period. Treatment consisted of corticosteroids and/or cytotoxic agents.

Pheno-genotyping of verotoxin 2 (VT2)-producing Escherichia coli causing haemorrhagic colitis and haemolytic uraemic syndrome by direct analysis of patients' stools.

The subtype of verotoxin 2 (VT2) found in 22 VT2-positive stool samples from severely diseased Italian and German children with haemorrhagic colitis or haemolytic uraemic syndrome, or both, and that produced by the corresponding VT-producing Escherichia coli (VTEC) strains isolated from the stools were studied by cytotoxicity seroneutralisation assays and by polymerase chain reaction (PCR) amplification of the VT2 B-subunit gene, followed by restriction fragment length polymorphism (RFLP) analysis. The free faecal toxin was serotyped as the classical VT2 in 21 stool samples, and as the VT2 variant VT2c in one. For all but one of the VTEC isolates, the toxin phenotype was consistent with the type of VT produced in vivo and found in the corresponding stool samples.

Macromolecular IgA and abnormal IgA reactivity in sera from children with IgA nephropathy. Italian Collaborative Paediatric IgA Nephropathy Study.

This multicenter study investigated the characteristics of circulating IgA molecules in 77 children: 42 had primary IgA nephropathy (IgAN), 20 were non-IgA glomerulonephritides (CGN) and 15 had urological problems (U). Fifteen assays were employed including the detection of macromolecular IgA [IgA immune complexes (IgAIC) by the conglutinin (K) assay, heavy molecular weight IgA in 2.5% polyethylene glycol (PEG), mixed IgA/IgGIC (Jacalin assay), IgA-Fibronectin (IgA-F) aggregates]IgA antibodies to alimentary antigens (gliadin, glycgli, glutein, ovalbumin, bovine serum albumin) and IgA binding to mesangial antigens (fibronectin, laminin, type IV collagen) or polycations (poly-L-lysine).

Haemolytic-uraemic syndrome in childhood: surveillance and case-control studies in Italy. Italian HUS Study Group.

Seventy-six cases of haemolytic-uraemic syndrome (HUS) were collected over a 4-year period during a surveillance and case-control study. The annual incidence of 0.2 per 100,000 children aged 0-14 years is lower than in other countries; 34% had no prodromal diarrhoea.

A community outbreak of haemolytic-uraemic syndrome in children occurring in a large area of northern Italy over a period of several months.

From March to October 1993, 15 cases of haemolytic-uraemic syndrome (HUS) in children were detected in a large area of northern Italy, where only 8 cases had occurred in the previous 5 years. Analysis of stool and serum specimens obtained from 14 cases showed evidence of Verotoxin-producing Escherichia coli (VTEC) infection in 13. Serum antibodies to the E.

Acute tubulointerstitial nephritis occurring with 1-year lapse in identical twins.

We report monozygotic female twins who developed acute tubulointerstitial nephritis, with identical histological features and similar clinical symptoms, 1 year apart. Both patients presented with acute renal failure; only one developed bilateral uveitis after the onset of the nephritis.

[IgA serology in idiopathic IgA deposit nephropathy in children].

It is generally thought that in primary IgA nephropathy (IgAN) an altered immune response favours high levels of serum IgA directed against environmental or mesangial antigens (Ag) leading to circulating IgA containing immune complexes (IgAIC). The aim of this multicenter collaborative study was to evaluate some of the IgA immunologic abnormalities in children with IgAN. We investigated 42 children with biopsy-proved IgAN, 21 children with non-IgA glomerulonephritides (CD) and 15 with previous urologic disorders without any evidence of immunologic disease (U).