title: 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.
authors.affiliation: Universidade Federal de Ciencias da Saude de Porto Alegre.
authors.forename: Karla Lais
authors.initials: KL
authors.lastname: Pêgas
authors.affiliation: Santa Casa de Porto Alegre.
authors.forename: Roberta
authors.initials: R
authors.lastname: Rohde
authors.affiliation: Universidade Federal de Ciencias da Saude de Porto Alegre.
authors.forename: Clotilde Druck
authors.initials: CD
authors.lastname: Garcia
authors.affiliation: Universidade Federal de Ciencias da Saude de Porto Alegre.
authors.forename: Viviane de Barros
authors.initials: Vde B
authors.lastname: Bittencourt
authors.affiliation: Universidade Federal de Ciencias da Saude de Porto Alegre.
authors.forename: Elizete
authors.initials: E
authors.lastname: Keitel
authors.affiliation: Universidade Federal de Ciencias da Saude de Porto Alegre.
authors.forename: José Antonio Tesser
authors.initials: JA
authors.lastname: Poloni
authors.affiliation: Instituto de Cardiologia/Fundacao Universitaria de Cardiologia.
authors.forename: Eduardo
authors.initials: E
authors.lastname: Cambruzzi
mesh_terms: D001938:Brazil; D002648:Child; D006801:Humans; D007674:Kidney Diseases / Q000150:complications / Q000175:diagnosis*; D008297:Male; D009404:Nephrotic Syndrome / Q000209:etiology; D035583:Rare Diseases