Lipoprotein glomerulopathy: a case report of a rare disease in a brazilian child
Open accessAbstract
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.
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LPG is more often reported in eastern Asian countries, but cases have also been observed in Europe and America.
“It is more often reported in eastern Asian countries, however cases have also been observed in Europe and America [2–4].”
From Lipoprotein glomerulopathy associated with the Osaka/Kurashiki APOE variant: two cases identified in Latin America (2021) · cites this paper as [2]
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Lipoprotein Glomerulopathy: Glomerular Lipoprotein Thrombi in a Patient With Hyperlipoproteinemia
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Lipoprotein Glomerulopathy, First Case Report from Canada
Ting, J. A., McRae, S. A., Schwartz, D., Barbour, S. J., & Riazy, M. (2022) · International Journal of Nephrology and Renovascular Disease · doi:10.2147/ijnrd.s364890 · Cited by 7 · see citing works
da Silveira-Neto, J. N., de Oliveira Ahn, G. J., de Menezes Neves, P. D. M., Baptista, V. A. F., de Almeida Araújo, S., Wanderley, D. C., Watanabe, A., Watanabe, E. H., Murai, N. M., Bertollo, E. M. G., et al. (2021) · Diagnostic Pathology · doi:10.1186/s13000-021-01119-x · Cited by 6 · see citing works
- Authors
- Pêgas, K. L., Rohde, R., Garcia, C. D., Bittencourt, V. D. B., Keitel, E., Poloni, J. A. T., & Cambruzzi, E.
- Affiliations
- Santa Casa Hospital (BR)
- Universidade Federal de Ciências da Saúde de Porto Alegre (BR)
- Fundação Universitária de Cardiologia (BR)
- Venue
- Jornal Brasileiro de Nefrologia
- Publisher
- FapUNIFESP (SciELO)
- Published
- 2014
- Volume
- 36
- Issue
- 1
- Pages
- 93-95
- ISSN
- 0101-2800 (ISSN-L)
- OpenAlex
- W2081285898
- Cited by
- 8
- References
- 2
- Citations by year
- 2024: 2 · 2022: 2 · 2021: 1 · 2019: 1 · 2018: 1 · 2016: 1
- FWCI
- 0.26
- Type
- journal article
- Language
- en
- Open access
- diamond
- doi.org
- Licence
- cc-by-nc
- Locations
- Brazilian Journal of Nephrology (publishedVersion, cc-by-nc)
- PubMed (publishedVersion)
- Americanae (AECID Library) (publishedVersion, other-oa)
- LA Referencia (Red Federada de Repositorios Institucionales de Publicaciones Científicas) (publishedVersion, other-oa)
- Americanae (AECID Library) (submittedVersion, other-oa)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Lume (Universidade Federal do Rio Grande do Sul) (submittedVersion, other-oa)
- LA Referencia (Red Federada de Repositorios Institucionales de Publicaciones Científicas) (submittedVersion, cc-by-nc-sa)
- Topics
- Health Sciences › Medicine › Nephrology › Renal Diseases and Glomerulopathies
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Lipid metabolism and disorders
- Health Sciences › Medicine › Nephrology › Chronic Kidney Disease and Diabetes
- Keywords
- lipoprotein glomerulopathy, apolipoprotein E, nephrotic syndrome, steroid resistance, proteinuria, lipid-lowering therapy, pediatric kidney disease, renal failure
- MeSH terms
- Brazil, Child, Humans, Kidney Diseases, Male, Nephrotic Syndrome, Rare Diseases
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