Lipoprotein glomerulopathy: a case report of a rare disease in a Brazilian child.

Abstract

Lipoprotein glomerulopathy (LPG) is a rare autosomal recessive glomerulopathy associated with the deposition of lipoprotein thrombi in the capillary lumina due to apoE gene mutations. Abnormal plasma lipoprotein profile and marked increase in serum apoliprotein E (apoE) are characteristic clinical data. The compromised patients can present nephrotic syndrome, hematuria, and progressive renal failure. Herein, the authors present the first described case of LPG in a Brazilian male patient, 11 years, who presented with a steroid-resistant nephrotic syndrome. Renal function was normal. Kidney biopsy showed markedly enlarged glomerulus, with dilated capillary loops and weak eosinophilic lipoprotein thrombi in the capillary lumina. Interstitium, tubules, arteries, and veins showed normal histologic aspect. Genotypic study for the apoE gene showed the presence of the alleles E3 and E4. The diagnosis of LPG was then performed. The patient received lipid-lowering treatment. After 2 years of follow-up, renal function is gradually decreasing, with persisting heavy proteinuria, despite a marked decrease in serum cholesterol and triglycerides levels.

Cite this paper

@article{Pgas2014LipoproteinGA, title={Lipoprotein glomerulopathy: a case report of a rare disease in a Brazilian child.}, author={Karla La{\'i}s P{\^e}gas and Roberta Rohde and Clotilde Druck Garcia and Viviane de Barros Bittencourt and Elizete Keitel and Jos{\'e} Ant{\^o}nio Tesser Poloni and Eduardo Cambruzzi}, journal={Jornal brasileiro de nefrologia : ʹorgão oficial de Sociedades Brasileira e Latino-Americana de Nefrologia}, year={2014}, volume={36 1}, pages={93-5} }