Abstract:Selective IgA deficiency associated with glomerulonephritis is rare and no previous reports in childhood have been made of the association of IgA deficiency and membranous glomerulonephritis (MGN). We report a 5-year-old boy with selective IgA deficiency and MGN. He presented with nephrotic syndrome. Percutaneous renal needle biopsy showed diffuse global thickening on light microscopy and heavy IgG and moderate C3 deposits were found on immunofluorescence. Electron microscopy detected extensive global subepith… Show more
“…In children, secondary causes of MN have been associated with conditions such as systemic lupus erythematosus (SLE), hepatitis B or C infection, secondary and congenital syphilis, malaria, and Ebstein Barr Virus (EBV) infection [1, 6, 7]. Other rare underlying causes are C4 deficiency, selective IgA deficiency, or antitubular basement membrane antibodies [8–10]. Fig.…”
The approach to the pediatric patient with membranous nephropathy (MN) can be challenging to the practitioner. The clinical presentation of the child with this histologic entity usually involves some degree of proteinuria ranging from persistent, subnephrotic-ranged proteinuria to overt nephrotic syndrome. Patients often have accompanying microscopic hematuria and may have azotemia or mild hypertension. Children presenting with nephrotic syndrome are often steroid resistant; as such, their biopsy for steroid-resistant nephrotic syndrome results in the diagnosis of MN. The practitioner treating MN in the pediatric patient must weigh the risks of immunosuppressive therapy against the benefits. In general, the child with subnephrotic proteinuria and normal renal function can likely be treated conservatively with angiotensin blockade (angiotensin-converting enzyme inhibitors or angiotensin receptor blockers) without the need for immunosuppressive therapy. Those with nephrotic syndrome are usually treated with steroids initially and often followed by alkylating agents (cyclophosphamide or chlorambucil). Calcineurin inhibitors may also be useful, but the relapse rate after their discontinuation remains high. The absence of controlled studies in children with MN makes treatment recommendations difficult, but until they are available, using the patient’s clinical presentation and risk of disease progression appears to be the most prudent approach.
“…In children, secondary causes of MN have been associated with conditions such as systemic lupus erythematosus (SLE), hepatitis B or C infection, secondary and congenital syphilis, malaria, and Ebstein Barr Virus (EBV) infection [1, 6, 7]. Other rare underlying causes are C4 deficiency, selective IgA deficiency, or antitubular basement membrane antibodies [8–10]. Fig.…”
The approach to the pediatric patient with membranous nephropathy (MN) can be challenging to the practitioner. The clinical presentation of the child with this histologic entity usually involves some degree of proteinuria ranging from persistent, subnephrotic-ranged proteinuria to overt nephrotic syndrome. Patients often have accompanying microscopic hematuria and may have azotemia or mild hypertension. Children presenting with nephrotic syndrome are often steroid resistant; as such, their biopsy for steroid-resistant nephrotic syndrome results in the diagnosis of MN. The practitioner treating MN in the pediatric patient must weigh the risks of immunosuppressive therapy against the benefits. In general, the child with subnephrotic proteinuria and normal renal function can likely be treated conservatively with angiotensin blockade (angiotensin-converting enzyme inhibitors or angiotensin receptor blockers) without the need for immunosuppressive therapy. Those with nephrotic syndrome are usually treated with steroids initially and often followed by alkylating agents (cyclophosphamide or chlorambucil). Calcineurin inhibitors may also be useful, but the relapse rate after their discontinuation remains high. The absence of controlled studies in children with MN makes treatment recommendations difficult, but until they are available, using the patient’s clinical presentation and risk of disease progression appears to be the most prudent approach.
“…The authors underlined the importance to detect IgAD in patients with renal diseases because they frequently require blood transfusion and may experience anaphylactic reactions. Kawasaki et al presented a 5-year-old boy with a primary nephrotic syndrome and IgAD (IgA less than 5 mg/dL) [14]. Oral prednisolone (2 mg kg/day) was started, and the proteinuria became negative at after 14 days of treatment.…”
Background: Imunoglobulin A (IgA) deficiency (IgAD) is the most common form of primary immunodeficiency in Western countries. There have been several reports on IgAD complicated by glomerulonephritis in adults, but only very few cases of IgAD with nephropathy have been reported in children. We present two cases of IgAD with relapsing nephrotic syndrome in pediatric age. Case presentation: A 4-year-old boy and a 2-year-old boy presented with bilateral periorbital oedema and weight gain. The results of laboratory tests revealed IgAD (IgA < 7 mg/dL), normal creatinine, hypoprotidaemia, hypoalbuminaemia, and nephrotic proteinuria. A diagnosis of IgAD and idiopathic nephrotic syndrome was made, and steroid treatment (prednisone 60 mg/mq/day) was started. During steroid tapering, the children experienced several relapses and to obtain a positive outcome they required therapy with human monoclonal anti-CD20 antibodies (rituximab in the first child, ofatumumab in the second one). Conclusions: Our cases highlight that IgAD can be observed in nephrotic syndrome and nephropathy in children with IgAD appears to be complicated and difficult to treat with corticosteroids alone. Further research is needed to better describe the clinical manifestations and pathological pictures among subjects with IgAD and nephrotic syndrome to understand whether IgAD has a prognostic value in children with nephrotic syndrome and to let clinical physicians define a more personalized and appropriate approach for the management of these patients.
“…Sirs, I read with interest the article in this journal entitled "IgA deficiency and membranous glomerulonephritis presenting as nephrotic syndrome" by Kawasaki et al [1]. The authors suggested that an increased level of circulating immune complexes was involved in the pathogenesis of membranous glomerulonephritis (MGN) in patients with IgA deficiency [1].…”
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confidence: 97%
“…The authors suggested that an increased level of circulating immune complexes was involved in the pathogenesis of membranous glomerulonephritis (MGN) in patients with IgA deficiency [1]. However, the renal biopsy of the patient described by Kawasaki et al demonstrated diffuse and global thickening of the glomerular capillary walls in the absence of any mesangial changes, mesangial deposition of immunoglobulin or complements or mesangial electron-dense deposits, findings that are compatible with idiopathic MGN [2].…”
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confidence: 98%
“…Selective IgA deficiency-associated glomerulonephritis is rare, and only 19 patients have been reported [1,3]. Renal biopsy demonstrated mesangial proliferation in the majority of patients with IgA deficiency and glomerulonephritis, thereby suggesting a major role of circulating immune complexes on the development of glomerulonephritis in patients with IgA deficiency [3].…”
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