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Cited by 5Renal Diseases and GlomerulopathiesLipid metabolism and disordersChronic Kidney Disease and Diabetes

Case Report: A Pediatric Case of Lipoprotein Glomerulopathy in China and Literature Review

Open access
Song, Yue, Yang, Changqiang, Liu, Lan, Wang, Hua·2021·Frontiers in Pediatrics
DOI 10.3389/fped.2021.684814PMC PMC8429817
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Abstract

Background: Lipoprotein glomerulopathy is a rare kidney disease characterized by lipoprotein thrombi in the glomerulus. Here, we report a case of lipoprotein glomerulopathy in a Chinese pediatric patient. Furthermore, we summarized the clinical features and genetic characteristics of lipoprotein glomerulopathy in China. Case Presentation: An 11-year-old Chinese girl presented with nephrotic syndrome with anemia (98 g/L). After excluding secondary causes, primary nephrotic syndrome was considered. Treatment with prednisone (60 mg/day) did not improve her condition. Renal biopsy showed marked dilation of the capillary lumen with lipoprotein thrombi and positive oil red O staining. Genetic testing revealed the genetic variant c.127C > T (p.R43C), known as the Kyoto mutation of the APOE gene. These findings are consistent with the diagnosis of lipoprotein glomerulopathy. Prednisone was gradually tapered and captopril was initiated. A 2-year follow-up revealed elevated urine protein and serum creatinine levels. We also reviewed 17 pediatric and 156 adult cases of lipoprotein glomerulopathy reported in China from the year of creation to 2021. The most common clinical features were edema, hematuria, hypertriglyceridemia, and increased serum apoE levels. Extra-renal manifestations included anemia, splenomegaly, and cardiac lipoprotein deposition. Conclusion: APOE Kyoto is the most common mutation in patients with lipoprotein glomerulopathy. In China, homozygosity for E3 (E3/3) is the most common isoform.

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  • APOE gene mutation leads to elevated serum apolipoprotein E (ApoE) levels and glomerular capillary occlusion by massive lipoprotein thrombi composed of ApoB and ApoE, causing glomerular damage and renal impairment.

    “APOE gene mutation leads to elevated serum apolipoprotein E (ApoE) levels, and glomerular capillary lumens become occluded by massive lipoprotein thrombi composed of ApoB and ApoE, thereby resulting in glomerular damage and renal impairment.1, 2, 3”

    From The First Case of Lipoprotein Glomerulopathy in a Patient With Male Infertility (2026) · cites this paper as [3]

    The Introduction describes massive lipoprotein thrombi in dilated glomerular capillaries and renal disease; the Discussion reports increased serum apoE in patients with lipoprotein glomerulopathy. But the paper does not establish that an APOE mutation invariably causes the full sequence claimed: its Discussion says mutations are not necessary for the disease and its mechanisms remain unclear. The passages also do not identify the thrombi as composed of ApoB and ApoE. (LLM, conf=0.87)

    Experimental — accuracy not yet measured

  • Lipoprotein glomerulopathy (LPG) is a rare hereditary glomerular disease characterized by lipoprotein thrombi deposition in glomerular capillaries and caused by pathogenic variants in the APOE gene.

    “Lipoprotein glomerulopathy (LPG) is a rare hereditary glomerular disease with lipoprotein thrombi deposition in glomerular capillaries, which is caused by pathogenic variants in APOE gene.”

    From The First Case of Lipoprotein Glomerulopathy in a Patient With Male Infertility (2026) · cites this paper as [3]

    The Introduction describes LPG as a rare hereditary renal disorder and identifies lipoprotein thrombi in glomerular capillaries as its key histopathologic feature. The Discussion links APOE mutations to LPG but also reports cases without mutations and explicitly says an APOE mutation is not necessary for LPG to develop. Thus, the claim overstates APOE variants as a universal cause. (LLM, conf=0.88)

    Experimental — accuracy not yet measured

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Research works printed but not linked: 23

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Cited by

Sorted by citations

Authors
Song, Y., Yang, C., Liu, L., & Wang, H.
Affiliations
Sichuan University (CN)
Corresponding author
Wang, Hua
ORCID
Venue
Frontiers in Pediatrics
Publisher
Frontiers Media SA
Published
2021-08-27
Volume
9
Pages
684814-684814
ISSN
2296-2360 (ISSN-L)
DOI
10.3389/fped.2021.684814
PMC
PMC8429817
OpenAlex
W3197976314
Cited by
5
References
6
Citations by year
2026: 2 · 2024: 1 · 2022: 2
FWCI
0.51
Type
journal article
Language
en
Open access
Licence
Locations
  • Frontiers in Pediatrics (publishedVersion, cc-by)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
  • DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
Funders
National Natural Science Foundation of China
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, APOE gene, idiopathic nephrotic syndrome, pediatric kidney disease, renal biopsy, prednisone, captopril, hypertriglyceridemia, anemia, splenomegaly, APOE isoforms, China

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