Article PubMed Google Scholar. World J Pediatr 12 2 — Pediatr Int. PubMed Google Scholar. Kambham N Postinfectious glomerulonephritis. Adv Anat Pathol 19 5 — Pediatr Nephrol 24 5 — Zaffanello M, Cataldi L, Franchini M, Fanos V Evidence-based treatment limitations prevent any therapeutic recommendation for acute poststreptococcal glomerulonephritis in children.
Kidney Int 84 6 — Clin J Am Soc Nephrol 10 10 — Semin Thromb Hemost 40 4 — Semin Nephrol 31 4 — Pediatr Nephrol 25 2 — Nat Rev Nephrol 5 5 — J Am Soc Nephrol 19 10 — Arch Dis Child 77 4 — Pediatr Nephrol 31 11 — Kidney Int 82 4 — Nephrol Dial Transplant 31 5 — Download references. Farah A. Falix, Michiel J. Oosterveld, Jaap W. You can also search for this author in PubMed Google Scholar. Correspondence to Farah A.
This refers to the article that can be found at doi: Reprints and Permissions. Falix, F. Diagnostic dilemmas in a girl with acute glomerulonephritis: Answers. Pediatr Nephrol 33, 65—69 Download citation. Received : 31 January Revised : 04 February Accepted : 07 February Published : 09 March Issue Date : January Anyone you share the following link with will be able to read this content:. Sorry, a shareable link is not currently available for this article. The rationale for antiplatelet therapy is that platelet consumption is increased in MPGN and that platelets may play a role in glomerular injury.
However, these same authors retracted this conclusion in a subsequent study [ 34 ]. Anti-inflammatory agents, such as corticosteroids, may have useful effects in treating MPGN, but their non-specific nature and adverse effects mean that a high price is paid for any beneficial effect on the renal lesion. More selective forms of anti-inflammatory therapy are becoming available [ 36 ], and these are aimed at individual pro-inflammatory mediators, such as tumor necrosis factor which can be neutralized with specific antibodies or interleukin IL -1 the effect of which can be abrogated using soluble IL-1 receptor antagonist.
Therapies which inhibit complement activation by both the classical and alternative pathways, such as eculizumab an anti-C5 antibody designed to decrease C5a-mediated glomerular damage , should also be considered. Another attractive strategy which merits study is to reduce C3NeF using rituximab a chimeric IgG1 monoclonal antibody that specifically targets the CD20 surface antigen expressed on B lymphocytes.
The improved understanding of the pathogenesis of MPGN is likely to result in the identification of future agents that will modify the intense cellular proliferation and immune deposition seen in this glomerular disease. In summary, the therapeutic options in idiopathic MPGN depend on the level of proteinuria and kidney failure, as shown in Fig.
Management of patients with hepatitis C-associated cryoglobulinemic MPGN is also dictated by the severity of proteinuria and renal dysfunction [ 37 ], necessitating two distinct approaches, as shown in Fig. In all patients, the empiric use of angiotensin-converting enzyme inhibitors or angiotensin II receptor antagonists, as drugs of first choice to treat hypertension and decrease proteinuria, is a common practice that may retard the progression of renal disease.
Treatment of recurrent MPGN in renal transplant is uncertain, but plasma exchange may be useful in patients whose renal function deteriorates rapidly [ 13 ], whereas rituximab seems to be an effective treatment in the presence of cryoglobulinemia [ 2 , 37 ].
Lastly, improvement in renal outcomes for patients with MPGN largely relies on the evaluation of more selective agents in future studies. Treatment options of idiopathic MPGN depending on the level of proteinuria and renal dysfunction. Which of the following statements regarding the recurrence of MPGN in kidney transplantation is true? There is a good evidence that the combination of aspirin and dipyridamole slows the progression of the disease.
Cyclosporine is generally recommended in patients with rapidly progressive renal failure. National Center for Biotechnology Information , U. Pediatric Nephrology Berlin, Germany. Pediatr Nephrol. Published online Nov Bassam Alchi and David Jayne. Author information Article notes Copyright and License information Disclaimer.
Corresponding author. This article has been cited by other articles in PMC. Abstract Membranoproliferative glomerulonephritis is an uncommon kidney disorder characterized by mesangial cell proliferation and structural changes in glomerular capillary walls. Keywords: Cryoglobulinemia, Dense deposit disease, Hypocomplementemia mesangiocapillary, Nephritic factor.
Introduction The term membranoproliferative glomerulonephritis MPGN is often employed to denote a general pattern of glomerular injury seen in a variety of disease processes that share a common pathogenetic mechanism, rather than to describe a single disease entity [ 1 ]. Table 1 Classification of membranoproliferative glomerulonephritis. Open in a separate window. Clinical manifestations Membranoproliferative glomerulonephritis primarily affects children and young adults, with no sex predilection.
Histopathology Type I MPGN The glomeruli often show a global increase in mesangial cellularity and matrix with diffuse endocapillary proliferation. Treatment The efficacy of the various therapeutic regimens tried in MPGN is difficult to assess because of the small patient numbers and short-term nature of published controlled trials; the larger trials carried out to date have been uncontrolled [ 24 ].
Corticosteroids Both retrospective and prospective studies have demonstrated the beneficial effect of alternate-day steroid therapy on renal survival in pediatric patients with MPGN. Cyclophosphamide In one study [ 27 ], 19 pediatric and adult patients with MPGN were treated with an intensive and prolonged regimen of pulse methylprednisolone plus oral prednisone and cyclophosphamide.
Mycophenolate mofetil Mycophenolate mofetil MMF is an anti-proliferative agent that is being increasingly used for treating patients with various forms of immune-mediated renal disease. Cyclosporine The efficacy of cyclosporine was tested in a recent trial involving 18 patients with refractory MPGN who also received small doses of prednisolone 0. Plasma exchange Plasma exchange has not been studied in a controlled manner. Antiplatelet therapy The rationale for antiplatelet therapy is that platelet consumption is increased in MPGN and that platelets may play a role in glomerular injury.
Possible future therapeutic strategies Anti-inflammatory agents, such as corticosteroids, may have useful effects in treating MPGN, but their non-specific nature and adverse effects mean that a high price is paid for any beneficial effect on the renal lesion.
Questions: Answers appear following the question list Which of the following statements regarding the prevalence of MPGN is true? Duplication of the glomerular basement membrane Crescent formation Effacement of the podocyte foot process Positive staining for C4 along the glomerular capillaries Which of the following statements regarding the recurrence of MPGN in kidney transplantation is true?
The rarity of MPGN makes the randomized controlled study design easy to implement There is a good evidence that the combination of aspirin and dipyridamole slows the progression of the disease Steroid therapy has been proven effective for children but not for adults Cyclosporine is generally recommended in patients with rapidly progressive renal failure.
References 1. Kher V, Gulati S. Mesangiocapillary glomerulonephritis. In: Davidson AM, editor. Oxford textbook of clinical nephrology. Oxford: Oxford University Press; Cryoglobulinemia and renal disease. Curr Opin Nephrol Hypertens. Orth SR, Ritz E. The nephrotic syndrome. N Engl J Med. Primary nephrotic syndrome during childhood in Turkey. Pediatr Int. The predominance of membranoproliferative glomerulonephritis in childhood nephrotic syndrome in Ibadan, Nigeria.
West Afr J Med. Renal transplantation, chronic dialysis, and chronic renal insufficiency in children and adolescents. The annual report of the North American pediatric renal transplant cooperative study. Post-MRSA infection glomerulonephritis with marked Staphylococcus aureus cell envelope antigen deposition in glomeruli. J Nephrol. Walker PD. Dense deposit disease: new insights. Pathogenic mechanisms in membranoproliferative glomerulonephritis.
Nakopoulou L. Membranoproliferative glomerulonephritis. Nephrol Dial Transplant. Complement in glomerular injury. Semin Immunopathol. Williams DG. C3 nephritic factor and mesangiocapillary glomerulonephritis. Membranoproliferative glomerulonephritis type II dense deposit disease : an update. J Am Soc Nephrol.
The anticollagen antibodies cross-react with GBM, fixing complement and triggering a cell-mediated inflammatory response in the kidneys and usually the lungs.
The term Goodpasture syndrome Goodpasture Syndrome Goodpasture syndrome, a subtype of pulmonary-renal syndrome, is an autoimmune syndrome consisting of alveolar hemorrhage and glomerulonephritis caused by circulating anti-glomerular basement Glomerulonephritis without alveolar hemorrhage in the presence of anti-GBM antibodies is called anti-GBM glomerulonephritis.
Immunofluorescent staining of renal biopsy tissue demonstrates linear IgG deposits. Immune complex RPGN complicates numerous infectious and connective tissue disorders and also occurs with other primary glomerulopathies. Immunofluorescent staining demonstrates nonspecific granular immune deposits. Pathogenesis is usually unknown.
Pauci-immune RPGN is distinguished by the absence of immune complex or complement deposition on immunofluorescent staining. Immune complexes but no obvious cause such as infection, connective tissue disorder, or glomerular disorder. Manifestations are usually insidious, with weakness, fatigue, fever, nausea, vomiting, anorexia, arthralgia, and abdominal pain. Some patients present similarly to those with postinfectious glomerulonephritis Postinfectious Glomerulonephritis PIGN Postinfectious glomerulonephritis occurs after infection, usually with a nephritogenic strain of group A beta-hemolytic streptococcus.
Diagnosis is suggested by history and urinalysis and confirmed Hypertension is uncommon and rarely severe. Patients with anti-GBM antibody disease may have pulmonary hemorrhage, which can manifest with hemoptysis or be detectable only by finding diffuse alveolar infiltrates on chest x-ray pulmonary-renal syndrome Pulmonary-Renal Syndrome Pulmonary-renal syndrome is diffuse alveolar hemorrhage plus glomerulonephritis, often occurring simultaneously.
Cause is almost always an autoimmune disorder. Diagnosis is by serologic tests There are numerous causes, but autoimmune disorders are most common. Most patients present with dyspnea, cough, hemoptysis Diagnosis is suggested by acute kidney injury Acute Kidney Injury AKI Acute kidney injury is a rapid decrease in renal function over days to weeks, causing an accumulation of nitrogenous products in the blood azotemia with or without reduction in amount of urine Testing includes serum creatinine, urinalysis, complete blood count CBC , serologic tests, and renal biopsy.
Diagnosis is usually by serologic tests and renal biopsy. Urinalysis shows hematuria is always present, and RBC casts are usually present. Complement measurement may be useful in suspected immune complex RPGN because hypocomplementemia is common. Early renal biopsy is essential. The glomerular tuft usually appears hypocellular and collapses. Necrosis within the tuft or involving the crescent may occur and may be the most prominent abnormality.
In such patients, histologic evidence of vasculitis should be sought. Antiglomerular basement membrane antibody disease is characterized by smooth, linear staining of glomerular basement membranes with antibody to IgG. In anti-GBM antibody disease, linear or ribbon-like deposition of IgG along the GBM is most prominent and is often accompanied by linear and sometimes granular deposition of C3. In pauci-immune RPGN, immune staining and deposits are not detected.
However, fibrin occurs within the crescents, regardless of the fluorescence pattern. In idiopathic RPGN, some patients have immune complexes and others have absence of immune staining and deposits. Prognosis improves with early treatment.
Systemic lupus erythematosus Systemic Lupus Erythematosus SLE Systemic lupus erythematosus is a chronic, multisystem, inflammatory disorder of autoimmune etiology, occurring predominantly in young women. Common manifestations may include arthralgias and
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