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Lipoprotein glomerulopathy(LPG)

MedGen UID:
382034
•Concept ID:
C2673196
•
Disease or Syndrome
Synonym: LPG
SNOMED CT: Lipoprotein glomerulopathy (446923008)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
•Concept ID:
C0443147
•
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
Autosomal dominant inheritance (Orphanet)
 
Gene (location): APOE (19q13.32)
 
Monarch Initiative: MONDO:0012725
OMIM®: 611771
Orphanet: ORPHA329481

Definition

Lipoprotein glomerulopathy is an uncommon kidney disease characterized by proteinuria, progressive kidney failure, and distinctive lipoprotein thrombi in glomerular capillaries (Saito et al., 2006). It mainly affects people of Japanese and Chinese origin; in these populations, it is associated with mutations in the gene that encodes apolipoprotein E (APOE; 107741). The disorder had rarely been described in Caucasians. [from OMIM]

Clinical features

From HPO
Proteinuria
MedGen UID:
10976
•Concept ID:
C0033687
•
Finding
Increased levels of protein in the urine.
See: Feature record | Search on this feature
Glomerulopathy
MedGen UID:
451033
•Concept ID:
C0268731
•
Disease or Syndrome
Inflammatory or noninflammatory diseases affecting the glomeruli of the nephron.
See: Feature record | Search on this feature
Renal insufficiency
MedGen UID:
332529
•Concept ID:
C1565489
•
Disease or Syndrome
A reduction in the level of performance of the kidneys in areas of function comprising the concentration of urine, removal of wastes, the maintenance of electrolyte balance, homeostasis of blood pressure, and calcium metabolism.
See: Feature record | Search on this feature
Mesangial hypercellularity
MedGen UID:
924090
•Concept ID:
C4281741
•
Cell or Molecular Dysfunction
Increased numbers of mesangial cells per glomerulus, defined as more than 3 nuclei fully surrounded by matrix in one or more mesangial areas, not including perihilar region, on a standard 3-micron-thick tissue section, best evaluated on periodic acid-Schiff (PAS) stain.
See: Feature record | Search on this feature

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLipoprotein glomerulopathy
Follow this link to review classifications for Lipoprotein glomerulopathy in Orphanet.

Professional guidelines

PubMed

Hereditary features, treatment, and prognosis of the lipoprotein glomerulopathy in patients with the APOE Kyoto mutation.
Hu Z, Huang S, Wu Y, Liu Y, Liu X, Su D, Tao Y, Fu P, Zhang X, Peng Z, Zhang S, Yang Y
Kidney Int 2014 Feb;85(2):416-24. Epub 2013 Sep 11 doi: 10.1038/ki.2013.335. PMID: 24025644

Recent clinical studies

Etiology

APOE gene variants in primary dyslipidemia.
Khalil YA, Rabès JP, Boileau C, Varret M
Atherosclerosis 2021 Jul;328:11-22. Epub 2021 May 23 doi: 10.1016/j.atherosclerosis.2021.05.007. PMID: 34058468
Apolipoprotein E in lipoprotein metabolism, health and cardiovascular disease.
Marais AD
Pathology 2019 Feb;51(2):165-176. Epub 2018 Dec 28 doi: 10.1016/j.pathol.2018.11.002. PMID: 30598326
Update on the molecular biology of dyslipidemias.
Ramasamy I
Clin Chim Acta 2016 Feb 15;454:143-85. Epub 2015 Nov 4 doi: 10.1016/j.cca.2015.10.033. PMID: 26546829
Topics in lipoprotein glomerulopathy: an overview.
Saito T, Matsunaga A, Ito K, Nakashima H
Clin Exp Nephrol 2014 Apr;18(2):214-7. Epub 2013 Oct 23 doi: 10.1007/s10157-013-0887-4. PMID: 24149835
Lipoprotein glomerulopathy.
Tsimihodimos V, Elisaf M
Curr Opin Lipidol 2011 Aug;22(4):262-9. doi: 10.1097/MOL.0b013e328345ebb0. PMID: 21464714

Diagnosis

Clinicopathological characteristics and gene mutations in 11 patients with lipoprotein glomerulopathy.
Qin Y, Sun XJ, Hu YF, Jing M, Yu XJ, Zhao MH, Tan Y
Ren Fail 2024 Dec;46(1):2332491. Epub 2024 Mar 26 doi: 10.1080/0886022X.2024.2332491. PMID: 38584145Free PMC Article
APOE gene variants in primary dyslipidemia.
Khalil YA, Rabès JP, Boileau C, Varret M
Atherosclerosis 2021 Jul;328:11-22. Epub 2021 May 23 doi: 10.1016/j.atherosclerosis.2021.05.007. PMID: 34058468
Dysbetalipoproteinaemia: a mixed hyperlipidaemia of remnant lipoproteins due to mutations in apolipoprotein E.
Marais AD, Solomon GA, Blom DJ
Crit Rev Clin Lab Sci 2014 Feb;51(1):46-62. Epub 2014 Jan 9 doi: 10.3109/10408363.2013.870526. PMID: 24405372
Impact of lipoprotein glomerulopathy on the relationship between lipids and renal diseases.
Saito T, Matsunaga A, Oikawa S
Am J Kidney Dis 2006 Feb;47(2):199-211. doi: 10.1053/j.ajkd.2005.10.017. PMID: 16431249
Newer glomerular diseases.
Abt AB, Cohen AH
Semin Nephrol 1996 Nov;16(6):501-10. PMID: 9125794

Therapy

Pathogenesis, histopathologic findings and treatment modalities of lipoprotein glomerulopathy: A review.
Cambruzzi E, Pêgas KL
J Bras Nefrol 2019 Jul-Sep;41(3):393-399. Epub 2018 Nov 8 doi: 10.1590/2175-8239-jbn-2018-0148. PMID: 30421781Free PMC Article
Lipoprotein glomerulopathy in China.
Chen Y
Clin Exp Nephrol 2014 Apr;18(2):218-9. Epub 2013 Oct 29 doi: 10.1007/s10157-013-0873-x. PMID: 24165683
Topics in lipoprotein glomerulopathy: an overview.
Saito T, Matsunaga A, Ito K, Nakashima H
Clin Exp Nephrol 2014 Apr;18(2):214-7. Epub 2013 Oct 23 doi: 10.1007/s10157-013-0887-4. PMID: 24149835
Hereditary features, treatment, and prognosis of the lipoprotein glomerulopathy in patients with the APOE Kyoto mutation.
Hu Z, Huang S, Wu Y, Liu Y, Liu X, Su D, Tao Y, Fu P, Zhang X, Peng Z, Zhang S, Yang Y
Kidney Int 2014 Feb;85(2):416-24. Epub 2013 Sep 11 doi: 10.1038/ki.2013.335. PMID: 24025644
Abnormal lipid metabolism and renal disorders.
Saito T
Tohoku J Exp Med 1997 Mar;181(3):321-37. doi: 10.1620/tjem.181.321. PMID: 9163848

Prognosis

Clinicopathological characteristics and gene mutations in 11 patients with lipoprotein glomerulopathy.
Qin Y, Sun XJ, Hu YF, Jing M, Yu XJ, Zhao MH, Tan Y
Ren Fail 2024 Dec;46(1):2332491. Epub 2024 Mar 26 doi: 10.1080/0886022X.2024.2332491. PMID: 38584145Free PMC Article
APOE gene variants in primary dyslipidemia.
Khalil YA, Rabès JP, Boileau C, Varret M
Atherosclerosis 2021 Jul;328:11-22. Epub 2021 May 23 doi: 10.1016/j.atherosclerosis.2021.05.007. PMID: 34058468
Dysbetalipoproteinaemia: a mixed hyperlipidaemia of remnant lipoproteins due to mutations in apolipoprotein E.
Marais AD, Solomon GA, Blom DJ
Crit Rev Clin Lab Sci 2014 Feb;51(1):46-62. Epub 2014 Jan 9 doi: 10.3109/10408363.2013.870526. PMID: 24405372
Hereditary features, treatment, and prognosis of the lipoprotein glomerulopathy in patients with the APOE Kyoto mutation.
Hu Z, Huang S, Wu Y, Liu Y, Liu X, Su D, Tao Y, Fu P, Zhang X, Peng Z, Zhang S, Yang Y
Kidney Int 2014 Feb;85(2):416-24. Epub 2013 Sep 11 doi: 10.1038/ki.2013.335. PMID: 24025644
Newer glomerular diseases.
Abt AB, Cohen AH
Semin Nephrol 1996 Nov;16(6):501-10. PMID: 9125794

Clinical prediction guides

Clinicopathological characteristics and gene mutations in 11 patients with lipoprotein glomerulopathy.
Qin Y, Sun XJ, Hu YF, Jing M, Yu XJ, Zhao MH, Tan Y
Ren Fail 2024 Dec;46(1):2332491. Epub 2024 Mar 26 doi: 10.1080/0886022X.2024.2332491. PMID: 38584145Free PMC Article
The first case of lipoprotein glomerulopathy complicated with collagen type III glomerulopathy and literature review.
Liu H, Luo C, Li Z, Zhang C, Xiong J
J Nephrol 2023 Apr;36(3):663-667. Epub 2022 Nov 12 doi: 10.1007/s40620-022-01491-x. PMID: 36370330Free PMC Article
Apolipoprotein E-related glomerular disorders.
Saito T, Matsunaga A, Fukunaga M, Nagahama K, Hara S, Muso E
Kidney Int 2020 Feb;97(2):279-288. Epub 2019 Nov 22 doi: 10.1016/j.kint.2019.10.031. PMID: 31874799
Dysbetalipoproteinaemia: a mixed hyperlipidaemia of remnant lipoproteins due to mutations in apolipoprotein E.
Marais AD, Solomon GA, Blom DJ
Crit Rev Clin Lab Sci 2014 Feb;51(1):46-62. Epub 2014 Jan 9 doi: 10.3109/10408363.2013.870526. PMID: 24405372
Lipoprotein glomerulopathy.
Tsimihodimos V, Elisaf M
Curr Opin Lipidol 2011 Aug;22(4):262-9. doi: 10.1097/MOL.0b013e328345ebb0. PMID: 21464714

Supplemental Content

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    • ClinVar
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      Clinical tests in GTR
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      Related records in OMIM
    • OMIM(Genes)
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