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Acute Nephritic Syndrome with Diffuse Membranoproliferative Glomerulonephritis
Acute Nephritic Syndrome with Diffuse Mesangiocapillary Glomerulonephritis

Presentation

C3GN usually presents with a membranoproliferative pattern of injury. Crescents may or may not be present. However, we have noted a severe necrotizing and crescentic glomerulonephritis in a small subset of C3GN patients. [mayoclinic.pure.elsevier.com]

Presenting features comprise any of proteinuria, hematuria, hypertension and renal failure. [ncbi.nlm.nih.gov]

How changes in complement regulation leads to very diverse presentations might be related to the underlying defect. [renalfellow.blogspot.com]

Workup

Given the complexity of disease pathogenesis in C3G, a patient-tailored approach including a comprehensive workup of complement abnormalities is necessary to evaluate the best treatment options. [ncbi.nlm.nih.gov]

This is especially important in the workup of glomerulonephritis of uncertain etiology. Sometimes, the pattern of C3/C4 levels can give a clue as to the specific diagnosis. [renalfellow.blogspot.com]

Autoimmune workup was performed to determine the etiology of this patient’s disease. [cureus.com]

Histopathologic changes include swelling of the glomerular tufts and infiltration with polymorphonucleocytes (see Workup: Histologic Findings ). Immunofluorescence reveals deposition of immunoglobulins and complement. [emedicine.medscape.com]

In all cases, routine workup including light, immunofluorescence and electron microscopy was performed. Clinical information was obtained from the charts. The Institutional Review Board at the Mayo Clinic approved this study. [ndt.oxfordjournals.org]

Treatment

The effect was sustained at more than 2 years with only two courses of treatment and an excellent tolerance. Rituximab could be proposed as a treatment of C3GP associated with antibodies interfering with complement alternative pathway. [ncbi.nlm.nih.gov]

[…] clinical trials to further investigate CCX168 in the treatment of C3G. [globenewswire.com]

Case diagnosis/treatment: We present the clinical features, kidney biopsy findings, complement evaluation, treatment, and follow-up of four C3GN patients. [mayoclinic.pure.elsevier.com]

[…] step with targeted treatment. [karger.com]

Prognosis

Prognosis is often worse in males with CFHR5 nephropathy. Prognosis of C3G Patients with C3G have a variable clinical course, though the prognosis is generally poor and there are no effective treatments. [atypicalhus.co.uk]

Likewise, the data available on prognosis and response to different assayed treatments is very scarce. 7. [revistanefrologia.com]

Its poor prognosis and onset in young populations makes the detailed study of new therapeutic alternatives for this disease essential. Recently eculizumab, an anti-C5 antibody, has demonstrated effectiveness in the treatment of these patients. [ncbi.nlm.nih.gov]

Prognosis - C3 Glomerulopathy Renal prognosis is poor, with a 30% risk of end stage renal disease at 2-years. The risk of recurrence in the transplanted kidney is over 70%, with more than a 50% chance of graft loss. [checkorphan.org]

The prognosis depends on the underlying disease and the overall health of the patient. The occurrence of cardiopulmonary or neurologic complications worsens the prognosis. [emedicine.medscape.com]

Etiology

CONCLUSIONS: As this multimodal therapeutic approach was effective in all four treated patients with suspected autoimmune etiology of C3G, it offers a treatment option for severely affected patients with this rare disease until more specific regimens [ncbi.nlm.nih.gov]

This is especially important in the workup of glomerulonephritis of uncertain etiology. Sometimes, the pattern of C3/C4 levels can give a clue as to the specific diagnosis. [renalfellow.blogspot.com]

The idiopathic etiology that could be accepted in immunoglobulin positive group corresponded to 49.3% of out casuistic, we do not know if electron microscopy could help to find the etiology in this cases. [bjn.org.br]

They are organised into groups, and further divided into clinical, etiological or histopathological sub-types. [orpha.net]

Conclusion Our study strengthens the evidence that paraproteinemia is an underlying etiology of C3GP. [omicsonline.org]

Epidemiology

Keywords: complement system proteins; epidemiology; glomerulonephritis, membranoproliferative; microscopy, fluorescence. RESUMO: INTRODUÇÃO: Uma nova classificação para glomerulonefrite membranoproliferativa foi proposta na literatura. [bjn.org.br]

Changing epidemiology of acute post-streptococcal glomerulonephritis in Northeast Florida: a comparative study. Pediatr Nephrol. 2008 Jul. 23(7):1101-6. [Medline]. Longcope WT, O'Brien DP, McGuire J, Hansen OC, Denny ER. [emedicine.medscape.com]

The epidemiology, clinical presentation, outcomes (patient and graft survival) and anti-HLA alloantibodies were evaluated. [scielo.mec.pt]

Pathophysiology

We discuss the pathophysiology of C3 glomerulopathy, with evidence for alternative pathway dysregulation obtained from affected individuals and complement factor H (Cfh)-deficient animal models. [ncbi.nlm.nih.gov]

Definition  Glomerular disorders in which dysregulation of the complement system is either  The key pathophysiological factor  The major mediator of glomerular damage 7.  Proposed re-classification  Ig-mediated disease -classical complement pathway [slideshare.net]

Prevention

By investigating its effects in vitro using multiple assays of complement activity, we show that Cp40 prevents complement-mediated lysis of sheep erythrocytes in sera from C3G patients, prevents complement dysregulation in the presence of patient-derived [ncbi.nlm.nih.gov]

However, vaccination may not be sufficient to prevent infection. [nice.org.uk]

When kidney failure ensues, dialysis and kidney transplantation become available options for treatment Thus far, no preventive methods or guidelines have been described for this disease. [dovemed.com]

[…] thereby preventing propagation of the TCC. 8. [slideplayer.com]

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