JOURNAL ARTICLE
RESEARCH SUPPORT, NON-U.S. GOV'T
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Immunohistochemical and serological characterization of membranous nephropathy in children and adolescents.

BACKGROUND: Membranous nephropathy (MN) is a common cause of nephrotic syndrome in adults, but is less frequent in children. Antibodies against four antigens leading to MN have been described in children: phospholipase A2 receptor 1 (PLA2 R1), thrombospondin type-1 domain-containing 7A (THSD7A), neutral endopeptidase (NEP), and cationic bovine serum albumin (BSA).

METHODS: Twelve children with MN were included in this study. Sera of all patients were analyzed for antibodies against PLA2 R1, THSD7A, NEP, and BSA. All sera were also analyzed using Western blot with human glomerular extracts (HGE) under non reducing conditions. In 5 cases renal biopsies were analyzed for PLA2 R1, THSD7A, NEP, BSA, and all IgG subclasses.

RESULTS: Six patients were PLA2 R1-antibody-positive, whereas THSD7A, NEP, and BSA antibodies were not found in any of our 12 patients. All sera were analyzed by Western blot using human glomerular extracts; however, no further potential antigens were found. Five kidney biopsies from 2 PLA2 R1-antibody-positive and 3 PLA2 R1-antibody-negative patients were available for additional analyses, confirming the diagnosis of PLA2 R1-associated MN in 2 cases, whereas none of the biopsies revealed enhanced staining for THSD7A, NEP or BSA. IgG2 and IgG4 stainings were positive in both patients with PLA2 R1-associated MN and negative in the other biopsies. During follow-up (median 24 months), 4 children with PLA2 R1-associated MN went into remission, preceded by decline of PLA2 R1 antibodies. Five of the 6 PLA2 R1-antibody-negative children went into remission.

CONCLUSIONS: In children with MN, PLA2 R1-associated MN appears to be common, whereas MN associated with THSD7A, NEP or BSA was not encountered. PLA2 R1 antibody levels are closely associated with disease activity, whereas PLA2 R1-antibody-negative patients often have a good prognosis. However, the pathophysiology of MN in a considerable number of children remains unclear.

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