ESPN 50th Annual Meeting

ESPN 2017


 
Clinical and immunopathological study of glomerulopathies with dominant mesangial immunoglobulin M expression in Belarusian children
NATALLIA TUR 1 VICTORIA SAVOSH 2 TATJANA LETKOUSKAYA 2 INA KAZYRA 2 EUGENIJ CHERSTVOY 2 ALIAKSANDR SUKALO 2

1- 2ND CHILDRENS HOSPITAL MINSK
2- BELARUS STATE MEDICAL UNIVERSITY
 
Introduction:

IgM nephropathy (IgMN) is a primary glomerulopathy characterised by diffuse mesangial immunoglobulin M (IgM) deposits. IgMN has been proposed as variant of glomerulopathy between minimal change disease and focal segmental glomerulosclerosis. It usually presents with steroid resistant or dependent nephrotic syndrome. 

Material and methods:

As material for research have served 8 kidneys biopsies patients with diffuse mesangial IgM positive on immunohistochemistry, whether there was single or dominant positivity, executed on the basis of the Republic Center of Pediatric Nephrology and Renal Replacement Therapy in Minsk/Belarus between January 2013 and March 2017. All the cases included were classified as primary glomerulopathy.

Results:

The age of patients ranged from 3 year to 16 years; there were 4 boys and 4 girls. The initial manifestation was nephrotic syndrome in 5 of 8 children, of whom 2 were steroid resistant, three were steroid dependent. Three of nephrotic patients also presented high blood pressure level and haematuria. One child had nephrotic proteinuria with microhaematuria, two children were haematuric with non-nephrotic proteinuria.

The most frequent glomerular morphological finding was diffuse mesangial hypercellularity: in 6 of 8 patients. In two cases, focal and segmental glomerulosclerosis was found. Tubulointerstitial changes (atrophy of the tubular epithelium, interstitial fibrosis or inflammation) were found in 5 of 8 cases. All biopsies had diffuse mesangial dominant positivity for IgM and in five cases there were also focal and segmental deposits of the immunoglobulin A, G and/or C3, C1q complement fractions. 

Conclusions:

The clinical manifestations and morphological findings in pediatric patients with IgMN is highly variable. The long-term prognosis of these patients and their response to steroid therapy are still unknown. Therefore, this question demands the further studying.