Cationic antigens in poststreptococcal glomerulonephritis

Antigen charge is an important factor in the pathogenesis of experimental immune complex glomerulonephritis. Its potential role in man was investigated in post-streptococcal glomerulonephritis, a disease where the causative agent is known. Cationic, extracellular streptococcal antigens were detected in 8 of 18 renal biopsies from patients with acute poststreptococcal glomerulonephritis (APSGN). The antigen was found mainly in earlier biopsies in which both IgG and IgM were present. Patients' sera taken at the time of biopsy contained antibody to cationic, streptococcal antigens. Cationic moieties are known to have affinity for the glomerular basement membrane and it is possible that the type of antigen described here initiates APSGN via in situ immune complex formation.     

Post-streptococcal glomerulonephritis and uveitis—a case report

We hereby present the first case report of a child with concomitant post-streptococcal glomerulonephritis (PSGN) and uveitis. Pediatricians should be familiar with this entity and recognize signs and symptoms of uveitis in children with PSGN.     

Acute post-streptococcal glomerulonephritis in a renal allograft

We report a 12-year-old male with acute post-streptococcal glomerulonephritis (APSGN) occurring 1 year after a cadaveric renal transplant. Although recurrent and de novo renal transplant glomerulonephritides have been well described in large series of adult and pediatric renal transplant recipients, post-infectious glomerulonephritis has been rarely reported, and APSGN has never been reported in either adult or pediatric transplant series. We speculate on the reasons for the lack of occurrence of APSGN in renal transplant recipients.     

Crescentic post-streptococcal glomerulonephritis with nephrotic syndrome in the adult: is aggressive therapy warranted?

The prognosis for adults with acute post-streptococcal glomerulonephritis (PSGN) who present with crescentic glomerulonephritis and nephrotic proteinuria is not known. We report a patient with rapidly progressive glomerulonephritis and nephrotic-range proteinuria following acute pharyngitis, in whom serologic and kidney biopsy findings led to a diagnosis of PSGN. The patient was treated with corticosteroids and anti-hypertensive medications resulting in improvement in renal function and decrease in proteinuria. These results suggest that aggressive treatment of crescentic PSGN with nephrotic syndrome can result in a favorable outcome.     

Acute Post-Bacterial Glomerulonephritis in Renal Transplant Patients: Description of Three Cases and Review of the Literature

Only a few cases of acute post-infectious glomerulonephritis have been described in renal transplant patients. We report here three cases of acute post-bacterial glomerulonephritis in renal transplants. In contrast to the classic cases of post-streptococcal glomerulonephritis the type of infection was heterogeneous: respectively, Escherichia coli bacteremia, a skin abscess, and cholangitis. The clinical presentation was characterized by a deterioration of graft function in two of our three patients. Acute renal dysfunction recovered in both patients, but in the long term the outcome was severe; two of the three patients lost their graft function. It is difficult to ascertain whether progression was due to chronic allograft nephropathy, to glomerulonephritis, or both. It may be concluded that acute post-infectious glomerulonephritis is a possible, although rare, complication in renal transplant recipients. It has an unusual presentation and may have a poor outcome in the long term. The role of therapy, if any, is still undefined.     

Histopathological atlas of renal diseases: Acute post-streptococcal glomerulonephritis

Acute post-streptococcal glomerulonephritis, also known as "proliferative-exudative nephritis" is morphologically characterized by intense endocapillary proliferation and massive accumulation of inflammatory cells.     

Histologically confirmed superimposition of post-streptococcal acute glomerulonephritis during IgA nephropathy

We describe a 39-year-old Japanese man with post-streptococcal acute glomerulonephritis (PSAGN) super-imposed on long-term immunoglobulin A nephropathy (IgA-N). The histological findings of the first renal biopsy, done at 21 years of age, revealed mild mesangial proliferative glomerulonephritis with mesangial IgA deposition. Nineteen years later, acute nephritic syndrome with hypocomplementemia and an increasing anti-streptolysin O (ASO) titer developed 2 weeks after the onset of an upper respiratory infection. A second renal biopsy revealed severe segmental endocapillary proliferative and exudative glomerulonephritis, with fibrocellular crescents in about 40% of the glomeruli. Immunofluorescence showed that more C3 than IgA was deposited in the mesangium and that the IgA deposits had decreased. Electron microscopy revealed hump electron-dense deposits on the epithelial side of the glomerular basement membrane. These features were consistent with PSAGN superimposed on IgA-N. After 2 weeks of observation, blood pressure, C3 level, and ASO titer had returned to normal, although the persisting nephritic syndrome necessitated steroid therapy. Six months after the onset of the acute nephritic syndrome, the patient remained asymptomatic, except for microhematuria.     

Meningococcal disease associated with an acute post-streptococcal complement deficiency

Chronic deficiencies in the complement pathway proteins are associated with an increased risk of meningococcal disease. Such deficiencies are caused by primary congenital immunodeficiency of a complement protein, properdin or mannose binding lectin, or are secondary to consumption of complement by systemic lupus erythematosus (SLE) or membranoproliferative glomerulonephritis (MPGN). Whatever the cause, the complement deficiency is always chronic. Here we report a case of meningococcal disease (MCD) in a child with a transient complement deficiency (CD), caused by post-streptococcal glomerulonephritis (PSGN).     

Irreversible renal failure in acute post-streptococcal glomerulonephritis.

Irreversible renal failure in sporadic acute post-streptococcal glomerulonephritis rarely occurs. Most patients have extracapillary proliferation. Complete recovery from the epidemic form is the rule. It is possible that the two conditions are different clinicopathological entities.     

Acute glomerulonephritis with bacteriuria: a probable etiologic relationship

Twenty-one cases of acute glomerulonephritis in children with no previous history of renal disease were studied. Urinary infection with a rising titre of serum agglutinins against the organisms isolated from urine was found in 5 cases. No evidence of previous streptococcal infection was found in these cases. In the meantime all 8 cases with post-streptococcal glomerulonephritis remained without bacteriuria. In one case acute glomerulonephritis followed virus hepatitis, and in the remaining 7 cases the cause of glomerulonephritis was unknown. It is suggested that in predisposed patients the bacteria present in urinary infections might act as antigens starting immunologic reactions in the glomeruli, leading to glomerulonephritis. The final proof of this theory awaits immunofluorescence identification of these antigens in the glomeruli.     

Acute post-streptococcal glomerulonephritis in an 8-month-old girl

We describe a middle class Sicilian girl who at 8 months of age suffered from acute post-streptococcal glomerulonephritis documented by clinical history, a positive type 12 -haemolytic streptococcus throat culture, a raised anti-sterptolysin O titre and a low C3 rising to normal after 8 weeks.     

Acute post-streptococcal glomerulonephritis in a 14-month-old boy: why is this uncommon?

Acute post-streptococcal glomerulonephritis (APSGN) is rare in children under 2 years of age. This is related in part to the disease patterns of group A streptococcus (GAS) and in part to impaired immunogenicity in infants. We report the case of a 14-month-old child with APSGN following GAS pharyngitis. This case illustrates that APSGN needs to be considered in the evaluation of both gross and microscopic hematuria in this age group. We review the literature of both GAS and APSGN and discuss the pathogenesis and epidemiologic reasons for this association.     

Persistent anti-DNA antibodies and DNA-anti-DNA complexes in post-streptococcal glomerulonephritis

Two patients with the typical clinical, serological and pathological features of acute post-streptococcal glomerulonephritis were found to have elevated serum concentrations of DNA-anti-DNA complexes, and, in one case, of anti-DNA antibody, both single and double stranded. The DNA-anti-DNA complexes were found to persist for 15 and 21 months respectively following the initial illness, despite rapid resolution of the clinical features and severe renal dysfunction. It is suggested that the DNA-anti-DNA complexes may have a pathogenetic role in post-streptococcal nephritis, or alternatively act as a marker of those patients who will develop nephritis after a streptococcal infection.     

A rare case of juvenile acute non-proliferative glomerulonephritis with a 6-year follow-up experience

Juvenile acute non-proliferative glomerulonephritis (JANG) is a relatively new clinico-pathological entity established in 2000. A PubMed search revealed only two published reports encompassing 14 cases of JANG. It is distinguished from acute post-streptococcal glomerulonephritis by the presence of normal serum complement and the absence of diffuse proliferation of mesangial cells in the glomeruli. We report a case of a 4-year-old child that presented with a clinical scenario and renal biopsy findings consistent with JANG. She responded well to treatment with high-dose methylprednisolone for 3 days, followed by gradual taper with oral prednisone. She was monitored closely for a period of 6 years and continues to do well with normal renal parameters and without any medications.     

Outcome of severe acute post-streptococcal glomerulonephritis in New Zealand children

Post-streptococcal glomerulonephritis (PSGN) is the commonest cause of severe acute glomerulonephritis in New Zealand children, with the majority (85%) of the patients being of either Pacific Island or Maori ethnicity. We have performed a retrospective study on 27 pediatric patients with acute PSGN. Of these patients, those with crescentic glomerulonephritis (n = 11) had a greater tendency (72.7%) for needing acute dialysis and were left with persistent urinary sediment abnormalities after a mean follow-up of 3.2 years (95% confidence interval 2.1–4.3). The efficacy of immunosuppression in the group with crescentic disease was uncertain. The severity of renal histopathological abnormalities as judged by the total biopsy score did not correlate with either presentation or eventual outcome. Severe childhood acute post-streptococcal glomerulonephritis, although uncommon, results in significant long-term renal morbidity, particularly among Maori and Pacific Island children.     

Cerebral vasculitis in acute post-streptococcal glomerulonephritis

Neurological complications in acute post-streptococcal glomerulonephritis (APSGN) have been traditionally attributed to hypertensive encephalopathy. A 9-year-old girl with biopsy-documented APSGN developed seizures at a time she was normotensive and biochemically well balanced. Computed tomography of her brain was consistent with vasculitis, a finding which was also clinically supported by apparent vasculitic involvement of other organ systems. All clinical, laboratory and radiological abnormalities resolved with recovery from the APSGN. We suggest that on rare occasions neurological complications in APSGN may result from involvement of the central nervous system in multiorgan transient vasculitis.     

Streptococcal pyrogenic exotoxin B antibodies in a mouse model of glomerulonephritis

Streptococcal pyrogenic exotoxin B is an extracellular cysteine protease. Only nephritis-associated strains of group A streptococci secrete this protease and this may be involved in the pathogenesis of post-streptococcal glomerulonephritis. Mice were actively immunized with a recombinant protease inactive exotoxin B mutant or passively immunized with exotoxin B antibody. Characteristics of glomerulonephritis were measured using histology, immunoglobulin deposition, complement activation, cell infiltration, and proteinuria. None of the mice given bovine serum albumin or exotoxin A as controls showed any marked changes. Immunoglobulin deposition, complement activation, and leukocyte infiltration occurred only in the glomeruli of exotoxin B-hyperimmunized mice. One particular anti-exotoxin B monoclonal antibody, 10G, was cross-reactive with kidney endothelial cells and it caused kidney injury and proteinuria when infused into mice. This cross-reactivity may be involved in the pathogenesis of glomerulonephritis following group A streptococcal infection.     

Rapid development of diffuse crescents in post-streptococcal glomerulonephritis

A 14-year-old boy presented with 3 days of hematuria and oliguria following impetigo. Renal biopsy showed diffuse endocapillary proliferative glomerulonephritis with subendothelial electron dense deposits but no crescents. Due to recrudescence of clinical abnormalities after initial improvement, the patient was re-biopsied 10 days later. This biopsy showed circumferential crescents in 83% of the glomeruli. Treatment with intensive plasma exchange, prednisolone, cyclophosphamide and dipyridamole was accompanied by prompt improvement in renal function. The need to carefully monitor the course in patients with acute post-streptococcal glomerulonephritis is emphasized.     

End-stage renal disease and acute glomerulonephritis in Goajiro Indians

BACKGROUND: Goajiro Indians are a semi-nomad tribe that live on the Goajiro peninsula, in the northwestern part of Venezuela. We investigated the incidence of end-stage renal disease (ESRD) and post-streptococcal glomerulonephritis (PSGN) among Goajiros and to determine if it was increased and whether congenital endowment of low number of nephrons (as indicated by low birth weight) was a contributing factor in their predisposition to chronic renal failure (CRF). METHODS: The incidence of ESRD and the attack rate of poststreptococcal glomerulonephritis (PSGN) among Goajiros during the period December 1991 through December 1998 were evaluated from the records of the University Hospital, in Maracaibo, which is the referral center for Goajiro Indians. Demographic characteristics and birth weight were obtained from the records of the Regional Public Health Service. Subclinical reduction in renal functioning mass was investigated in 11 healthy Goajiros with a standardized tubular stress test that determines the increment in tubular secretion of creatinine (TSCr) resulting from the intravenous administration of a bolus of creatinine. RESULTS: The incidence of ESRD among Goajiros was 220 patients per million inhabitants per year, 1.7 times higher than the incidence for the country. The attack rate of post-streptococcal glomerulonephritis is nearly double among Goajiro Indians (2.9 +/- 1.3 cases/100,000 inhabitants/year) than in the general population in the neighboring Maracaibo city (1.5 +/- 0.3, P < 0.02). Low weight birth was common among Goajiros; as many as 23% of newborns weigh less than 1000 g. The stimulated TSCr in healthy Goajiros was 30% lower than in controls (P < 0.001). CONCLUSIONS: Goajiro Indians have a high incidence of ESRD. A high attack rate of PSGN and low nephron endowment in combination may be responsible, at least in part, for the increased risk of ESRD in this population.     

Serum complement levels before and after the onset of acute post-streptococcal glomerulonephritis. A case report

Low serum C3, properdin, and C5 levels found in the acute stage of acute post-streptococcal glomerulonephritis (APSGN) indicate the presence of aggressive complement activation. We followed serum complement component levels in a child hospitalized with erysipelas who developed APSGN on the 2nd hospital day. Her initial serum sample, obtained prior to the clinical onset of nephritis, had a low properdin level and normal C3 and C5 levels despite the presence of C3 splitting activity. Two days later she developed gross hematuria and subsequent sera contained low C3, properdin, and C5 levels, as is usual in APSGN. These observations suggest that complement activation, predominantly through the alternative pathway, precedes the clinical onset of APSGN.