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]

• Turkish

  In conclusion, although there were some differences between the Spanish [ 8 ] and the Turkish study, the Turkish study does not confirm the putative efficacy of mycophenolate-based immunosuppressive treatment for C3 glomerulopathy. [karger.com]

  A novel CFHR5 mutation associated with C3 glomerulonephritis in a Turkish girl Besbas, Nesrin; Gulhan, Bora; Gucer, Safak; Korkmaz, Emine; Ozaltin, Fatih 2013-12-05 00:00:00 C3 glomerulopathy defines a subgroup of membranoproliferative glomerulonephritis [deepdyve.com]

  Familial studies have shed some important information such as the deletion of CFH codon noted in sisters from a consanguineous Turkish family with C3GN ( 30, 31 ). The result was a mutant CFH with defective binding to C3b ( 32 ). [frontiersin.org]

  A novel CFHR5 mutation associated with C3 glomerulonephritis in a Turkish girl. [ncbi.nlm.nih.gov]

• Aspiration

  The bone marrow aspiration showed atypical plasma cells >90% of marrow cells with some normal hematopoietic elements. X-rays revealed a patchy distribution of skeletal lesions. Cryoglobulinemia was negative. [indianjnephrol.org]

• Periodontitis

  The company plans to move its novel drug candidates to the clinic, starting with applications in C3G, paroxysmal nocturnal hemoglobinuria (PNH), ABO-incompatible kidney transplantation and periodontal disease, and potentially including other complement-mediated [fiercepharma.com]

• Retinal Lesion

  These retinal lesions can be associated with choroidal neovascularization and central serous chorioretinopathy (CSCR). [ncbi.nlm.nih.gov]

• Suggestibility

  CONCLUSIONS: We provide new insights into the pathogenesis of Ig-MPGN/C3G that underscore the complex nature of these diseases and suggest that the current C3G classification may miss many cases associated with abnormalities of the complement alternative [ncbi.nlm.nih.gov]

• Urinary Urgency

  Three months before presentation, she had an attack of gross hematuria with urinary urgency and dysuria, for which she started looking for medical attention at the local hospital. [journals.lww.com]

• Neonatal Onset

  Cryopyrin-associated periodic syndromes (CAPS) are autoinflammatory diseases comprising three clinically overlapping disorders: familial cold urticarial syndrome (FCAS), Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease [ncbi.nlm.nih.gov]

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]

• Hyperglobulinemia

  She was found to have gross hematuria (red blood cell count 80 × 10 6 /mL, pleomorphic type), elevated serum creatinine (2.9 mg/dL), anemia (hemoglobin 63 g/L), and hyperglobulinemia (41.0 g/L), and was treated with traditional Chinese medicine. [journals.lww.com]

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 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]

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]

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]

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]

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]