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Membranous nephropathy in children: clinical presentation and therapeutic approach

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Abstract

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.

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Acknowledgments

The authors thank Xu Zeng, Department of Laboratory Medicine and Pathology, Wayne State University, Detroit, MI, USA, for his assistance with the renal pathology slides.

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Authors and Affiliations

  1. The Carman and Ann Adams Department of Pediatrics, Division of Nephrology, Children’s Hospital of Michigan, Wayne State University School of Medicine, Detroit, MI, USA

    Shina Menon & Rudolph P. Valentini

  2. The Carman and Ann Adams Department of Pediatrics, Division of Nephrology, Children’s Hospital of Michigan, Wayne State University School of Medicine, 3901 Beaubien Blvd, Detroit, MI, 48201-2196, USA

    Rudolph P. Valentini

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  1. Shina Menon
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  2. Rudolph P. Valentini
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Correspondence to Rudolph P. Valentini.

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Answers

1. b

2. a

3. e

4. a

5. e

Questions

Questions

(Answers appear following the reference list)

  1. 1.

    Which of the following would be the LEAST expected clinical presentation of a child diagnosed with idiopathic MN?

  1. a.

    Asymptomatic proteinuria

  2. b.

    Azotemia

  3. c.

    Hypertension

  4. d.

    Microscopic hematuria

  5. e.

    Nephrotic syndrome

  1. 2.

    All of the following would be an expected association of secondary MN EXCEPT?

  1. a.

    Celiac disease

  2. b.

    Hepatitis B infection

  3. c.

    Nonsteroidal anti-inflammatory drugs

  4. d.

    Sarcoidosis

  5. e.

    Systemic lupus erythematosus

  1. 3.

    A key histologic feature distinguishing MN associated with systemic lupus erythematosus from that of idiopathic MN is

  1. a.

    C3 staining on immunofluorescence

  2. b.

    Granular staining for IgG

  3. c.

    Subepithelial electron-dense deposits

  4. d.

    Thickened glomerular capillary walls

  5. e.

    Tubuloreticular inclusions

  1. 4

    Which of the following treatment strategies best characterizes the approach to a child with MN?

  1. a.

    Alkylating agents with corticosteroids should be considered for children with MN and nephrotic syndrome and steroid-resistant disease

  2. b.

    ACEi should be reserved for patients with refractory, nephrotic-range proteinuria

  3. c.

    Calcineurin inhibitors appear to be effective in MN, with a low relapse rate upon discontinuation of medication

  4. d.

    Oral corticosteroids should be used in all patients irrespective of clinical presentation

  5. e.

    Rituximab appears to be a suitable second-line therapy for children with steroid-resistant MN based on published reports

  1. 5.

    Which of the following factors are associated with a favorable prognosis in the pediatric patient with MN?

  1. a.

    Age <14 years

  2. b.

    Azotemia

  3. c.

    Male gender

  4. d.

    Tubulointerstitial fibrosis

  5. e.

    Subnephrotic proteinuria

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Menon, S., Valentini, R.P. Membranous nephropathy in children: clinical presentation and therapeutic approach. Pediatr Nephrol 25, 1419–1428 (2010). https://doi.org/10.1007/s00467-009-1324-5

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