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Abnormal glomerular endothelial cell morphology

MedGen UID:
1778727
•Concept ID:
C5539471
•
Finding
HPO: HP:0033272

Definition

An abnormal structure of the glomerular endothelial cells, which are highly specialized cells with fenestrae and a charged luminal glycocalyx layer that contribute to the filtration barrier. [from HPO]

Professional guidelines

PubMed

POEMS syndrome: Update on diagnosis, risk-stratification, and management.
Dispenzieri A
Am J Hematol 2023 Dec;98(12):1934-1950. Epub 2023 Sep 21 doi: 10.1002/ajh.27081. PMID: 37732822
Broadening horizons in mechanisms, management, and treatment of diabetic kidney disease.
Petrazzuolo A, Sabiu G, Assi E, Maestroni A, Pastore I, Lunati ME, Montefusco L, Loretelli C, Rossi G, Ben Nasr M, Usuelli V, Xie Y, Balasubramanian HB, Zocchi M, El Essawy B, Yang J, D'Addio F, Fiorina P
Pharmacol Res 2023 Apr;190:106710. Epub 2023 Mar 4 doi: 10.1016/j.phrs.2023.106710. PMID: 36871895
Endothelial factors in the pathogenesis and treatment of chronic kidney disease Part I: General mechanisms: a joint consensus statement from the European Society of Hypertension Working Group on Endothelin and Endothelial Factors and The Japanese Society of Hypertension.
Rossi GP, Seccia TM, Barton M, Danser AHJ, de Leeuw PW, Dhaun N, Rizzoni D, Rossignol P, Ruilope LM, van den Meiracker AH, Ito S, Hasebe N, Webb DJ
J Hypertens 2018 Mar;36(3):451-461. doi: 10.1097/HJH.0000000000001599. PMID: 29120962

Recent clinical studies

Etiology

LPS/TLR4 Pathway Regulates IgA1 Secretion to Induce IgA Nephropathy.
He H, Shen M, Tang Y, Sun W, Xu X
Altern Ther Health Med 2024 Jan;30(1):419-425. PMID: 37820669
POEMS syndrome: Update on diagnosis, risk-stratification, and management.
Dispenzieri A
Am J Hematol 2023 Dec;98(12):1934-1950. Epub 2023 Sep 21 doi: 10.1002/ajh.27081. PMID: 37732822
Glomerular endothelial cell-podocyte stresses and crosstalk in structurally normal kidney transplants.
Menon R, Otto EA, Berthier CC, Nair V, Farkash EA, Hodgin JB, Yang Y, Luo J, Woodside KJ, Zamani H, Norman SP, Wiggins RC, Kretzler M, Naik AS
Kidney Int 2022 Apr;101(4):779-792. Epub 2021 Dec 22 doi: 10.1016/j.kint.2021.11.031. PMID: 34952098Free PMC Article
Endothelial factors in the pathogenesis and treatment of chronic kidney disease Part I: General mechanisms: a joint consensus statement from the European Society of Hypertension Working Group on Endothelin and Endothelial Factors and The Japanese Society of Hypertension.
Rossi GP, Seccia TM, Barton M, Danser AHJ, de Leeuw PW, Dhaun N, Rizzoni D, Rossignol P, Ruilope LM, van den Meiracker AH, Ito S, Hasebe N, Webb DJ
J Hypertens 2018 Mar;36(3):451-461. doi: 10.1097/HJH.0000000000001599. PMID: 29120962
A glimpse of the glomerular milieu: from endothelial cell to thrombotic disease in nephrotic syndrome.
Chen G, Liu H, Liu F
Microvasc Res 2013 Sep;89:1-6. Epub 2013 Jul 11 doi: 10.1016/j.mvr.2013.06.011. PMID: 23851046

Diagnosis

POEMS syndrome: Update on diagnosis, risk-stratification, and management.
Dispenzieri A
Am J Hematol 2023 Dec;98(12):1934-1950. Epub 2023 Sep 21 doi: 10.1002/ajh.27081. PMID: 37732822
Endocan levels in children with renal hypodysplasia.
Cayci FS, Aydin Z, Aygar IS, Avci B, Inozu M, Aksoy OY, Bayrakci US
Clin Nephrol 2021 Jun;95(6):332-337. doi: 10.5414/CN110402. PMID: 33704056
Corneal endothelial cell abnormalities in X-linked Alport syndrome.
Nicklason E, Mack H, Beltz J, Jacob J, Farahani M, Colville D, Savige J
Ophthalmic Genet 2020 Feb;41(1):13-19. Epub 2020 Mar 11 doi: 10.1080/13816810.2019.1709126. PMID: 32159412
A glimpse of the glomerular milieu: from endothelial cell to thrombotic disease in nephrotic syndrome.
Chen G, Liu H, Liu F
Microvasc Res 2013 Sep;89:1-6. Epub 2013 Jul 11 doi: 10.1016/j.mvr.2013.06.011. PMID: 23851046
Clinical and biochemical evidence of endothelial cell dysfunction in the pregnancy syndrome preeclampsia.
Roberts JM, Taylor RN, Goldfien A
Am J Hypertens 1991 Aug;4(8):700-8. doi: 10.1093/ajh/4.8.700. PMID: 1930853

Therapy

The mineralocorticoid receptor in diabetic kidney disease.
Jia G, Lastra G, Bostick BP, LahamKaram N, Laakkonen JP, Ylä-Herttuala S, Whaley-Connell A
Am J Physiol Renal Physiol 2024 Sep 1;327(3):F519-F531. Epub 2024 Jul 18 doi: 10.1152/ajprenal.00135.2024. PMID: 39024357Free PMC Article
Renal intrinsic cells remodeling in diabetic kidney disease and the regulatory effects of SGLT2 Inhibitors.
Guo W, Li H, Li Y, Kong W
Biomed Pharmacother 2023 Sep;165:115025. Epub 2023 Jun 28 doi: 10.1016/j.biopha.2023.115025. PMID: 37385209
Kidney Biopsy Features Most Predictive of Clinical Outcomes in the Spectrum of Minimal Change Disease and Focal Segmental Glomerulosclerosis.
Zee J, Liu Q, Smith AR, Hodgin JB, Rosenberg A, Gillespie BW, Holzman LB, Barisoni L, Mariani LH; Nephrotic Syndrome Study Network (NEPTUNE); NEPTUNE Members
J Am Soc Nephrol 2022 Jul;33(7):1411-1426. Epub 2022 May 17 doi: 10.1681/ASN.2021101396. PMID: 35581011Free PMC Article
Glomerular endothelial cell-podocyte stresses and crosstalk in structurally normal kidney transplants.
Menon R, Otto EA, Berthier CC, Nair V, Farkash EA, Hodgin JB, Yang Y, Luo J, Woodside KJ, Zamani H, Norman SP, Wiggins RC, Kretzler M, Naik AS
Kidney Int 2022 Apr;101(4):779-792. Epub 2021 Dec 22 doi: 10.1016/j.kint.2021.11.031. PMID: 34952098Free PMC Article
The glomerulopathy of sickle cell disease.
Ataga KI, Derebail VK, Archer DR
Am J Hematol 2014 Sep;89(9):907-14. Epub 2014 Jun 19 doi: 10.1002/ajh.23762. PMID: 24840607Free PMC Article

Prognosis

Endocan levels in children with renal hypodysplasia.
Cayci FS, Aydin Z, Aygar IS, Avci B, Inozu M, Aksoy OY, Bayrakci US
Clin Nephrol 2021 Jun;95(6):332-337. doi: 10.5414/CN110402. PMID: 33704056
Corneal endothelial cell abnormalities in X-linked Alport syndrome.
Nicklason E, Mack H, Beltz J, Jacob J, Farahani M, Colville D, Savige J
Ophthalmic Genet 2020 Feb;41(1):13-19. Epub 2020 Mar 11 doi: 10.1080/13816810.2019.1709126. PMID: 32159412
Ultrastructural Characterization of Proteinuric Patients Predicts Clinical Outcomes.
Royal V, Zee J, Liu Q, Avila-Casado C, Smith AR, Liu G, Mariani LH, Hewitt S, Holzman LB, Gillespie BW, Hodgin JB, Barisoni L
J Am Soc Nephrol 2020 Apr;31(4):841-854. Epub 2020 Feb 21 doi: 10.1681/ASN.2019080825. PMID: 32086276Free PMC Article
The Players: Cells Involved in Glomerular Disease.
Kitching AR, Hutton HL
Clin J Am Soc Nephrol 2016 Sep 7;11(9):1664-1674. Epub 2016 Apr 12 doi: 10.2215/CJN.13791215. PMID: 27073196Free PMC Article
Glomerulonephritis.
Remuzzi G, Zoja C, Bertani T
Curr Opin Nephrol Hypertens 1993 May;2(3):465-74. doi: 10.1097/00041552-199305000-00015. PMID: 7922207

Clinical prediction guides

Kidney Biopsy Features Most Predictive of Clinical Outcomes in the Spectrum of Minimal Change Disease and Focal Segmental Glomerulosclerosis.
Zee J, Liu Q, Smith AR, Hodgin JB, Rosenberg A, Gillespie BW, Holzman LB, Barisoni L, Mariani LH; Nephrotic Syndrome Study Network (NEPTUNE); NEPTUNE Members
J Am Soc Nephrol 2022 Jul;33(7):1411-1426. Epub 2022 May 17 doi: 10.1681/ASN.2021101396. PMID: 35581011Free PMC Article
Ultrastructural Characterization of Proteinuric Patients Predicts Clinical Outcomes.
Royal V, Zee J, Liu Q, Avila-Casado C, Smith AR, Liu G, Mariani LH, Hewitt S, Holzman LB, Gillespie BW, Hodgin JB, Barisoni L
J Am Soc Nephrol 2020 Apr;31(4):841-854. Epub 2020 Feb 21 doi: 10.1681/ASN.2019080825. PMID: 32086276Free PMC Article
The Players: Cells Involved in Glomerular Disease.
Kitching AR, Hutton HL
Clin J Am Soc Nephrol 2016 Sep 7;11(9):1664-1674. Epub 2016 Apr 12 doi: 10.2215/CJN.13791215. PMID: 27073196Free PMC Article
Glomerulonephritis.
Remuzzi G, Zoja C, Bertani T
Curr Opin Nephrol Hypertens 1993 May;2(3):465-74. doi: 10.1097/00041552-199305000-00015. PMID: 7922207
Nonimmunologic mechanisms of glomerular injury.
Olson JL, Heptinstall RH
Lab Invest 1988 Nov;59(5):564-78. PMID: 3054312

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