UNCOMMON CAUSE OF STEROID-RESISTANT NEPHROTIC SYNDROME, CASE REPORT

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https://storage.unitedwebnetwork.com/files/1099/78da97e359ce7680f4871bf9d5c0d19a.pdf

Abstract Title

First Name *

Sofia Victoria

Last Name *

Sanchez Cestona

Co-author 1

Marianela Diorio marianela.diorio17@gmail.com HNRG CABA CABA

Co-author 2

Dario Jesus Martinez Guillot dariomartinezguillot@hotmail.com HNRG CABA CABA

Co-author 3

Melisa Tatiana Medrano tatimedrano85@gmail.com HNRG La PAZ LA PAZ

Co-author 4

Victoria Torregiani torregianivictoria@gmail.com Fundación Hospitalaria CABA CABA

Co-author 5

Maria Jose Romaris dra.romaris.pediatra@gmail.com Cesac 11 CABA CABA

Co-author 6

Alicia Fayad aliciafayad@gmail.com HNRG CABA CABA

Co-author 7

Liern José Miguel jliern@yahoo.com HNRG CABA CABA

Co-author 8

Co-author 9

Co-author 10

Co-author 11

Co-author 12

Co-author 13

Co-author 14

Co-author 15

Introduction

Nephrotic syndrome (NS) is the most common primary glomerulopathy in children. Only 10% are steroid-resistant (SRNS), being more frequent in patients over 12 years of age. The main histopathology in SRNS in children is focal segmental glomerular sclerosis (FSGS), followed by minimal change disease (MCD) and diffuse mesangial sclerosis. Membranous glomerulonephritis (MGN) is detected in less than 1% of cases. In children, 50% of primary membranous GN present anti-PLA2R. Early detection of anti-PLA2R in patients with SRNS has diagnostic value and defines treatment response.

Methods

Presentation of two case reports.

Results

We present two cases of SRNS in previously healthy pediatric patients, who started standard treatment.

The first case is a 17-year-old adolescent, right monorrenal, admitted to the clinic due to nephrotic syndrome and hypertension with proteinuria of 8 g/l. He presented negative viral and parasitic serologies, C3 92 mg/dl, C4 19 mg/dl, negative FAN, anti-DNA, ANCA c and p, and negative PPD. Treatment with meprednisone (MPD) was initiated and continued for six weeks without response. Subsequently, he received cyclosporine (CsA). Kidney biopsy was performed, reporting membranous nephropathy, for which the Ponticelli scheme was initiated. He persisted with 6.3 g/l of proteinuria. PLA2R antibodies were requested, being positive.

The second patient is a 10-year-old patient who began with nephrotic syndrome (6 g/l proteinuria) associated with hematuria in the context of lower limbs pseudoangiomatosis secondary to a viral infection. Viral and parasitic serologies were negative. C3 103 mg/dl, C4 15 mg/dl, FAN, anti-DNA and PPD were negative. After 6 weeks of continuous treatment with MPD, he persisted with massive proteinuria (4.5 g/l), kidney biopsy showed mesangiocapillary glomerulonephritis with a proliferative membranous pattern. Treatment with CsA was started without response after 5 months, persisting with proteinuria (6.4 g/l). PLA2R antibodies were positive (1/40).

Conclusions

PLA2R antibodies should be measured in every pediatric patient with SRNS as part of the initial diagnostic workup.

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