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Mesangial hypercellularity

MedGen UID:
924090
Concept ID:
C4281741
Cell or Molecular Dysfunction
Synonym: Mesangial proliferation
 
HPO: HP:0012574

Definition

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. [from HPO]

Term Hierarchy

Conditions with this feature

Lipoprotein glomerulopathy
MedGen UID:
382034
Concept ID:
C2673196
Disease or Syndrome
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.
LAMB2-related infantile-onset nephrotic syndrome
MedGen UID:
481743
Concept ID:
C3280113
Disease or Syndrome
Nephrotic syndrome type 5 (NPHS5) is an autosomal recessive disorder characterized by very early onset of progressive renal failure manifest as proteinuria with consecutive edema starting in utero or within the first 3 months of life. A subset of patients may develop mild ocular anomalies, such as myopia, nystagmus, and strabismus (summary by Hasselbacher et al., 2006). For a general phenotypic description and a discussion of genetic heterogeneity of nephrotic syndrome, see NPHS1 (256300).
C3 glomerulonephritis
MedGen UID:
884569
Concept ID:
C4055342
Disease or Syndrome
C3 glomerulopathy-3 (C3G3) is an autosomal dominant kidney disease characterized by the onset of microscopic or macroscopic hematuria in the first 3 decades of life, followed by variable progression of renal disease. After age 30, about half of patients continue to have episodic hematuria while maintaining normal renal function, whereas the other half develop proteinuria and progressive renal failure or end-stage renal disease. In some cases, renal dysfunction may be triggered or exacerbated by an infectious disease, often an upper respiratory infection or pharyngitis. Some patients may also develop hypertension. Renal biopsy shows glomerular C3 deposition and mesangial proliferation with glomerulonephritis. Membranoproliferative glomerulonephritis (MPGN) may also be observed on renal biopsy. Males tend to have a more severe phenotype than females and are more likely to develop end-stage renal disease, often necessitating dialysis or renal transplant (summary by Athanasiou et al., 2011). For a general description and discussion of genetic heterogeneity of C3G, see C3G1 (609814).
IgA nephropathy, susceptibility to, 3
MedGen UID:
897340
Concept ID:
C4225194
Finding
Any IgA glomerulonephritis in which the cause of the disease is a mutation in the SPRY2 gene.
Nephrotic syndrome 14
MedGen UID:
1617660
Concept ID:
C4540559
Disease or Syndrome
Sphingosine phosphate lyase insufficiency syndrome (SPLIS) is characterized by varying combinations of steroid-resistant nephrotic syndrome (ranging from nonimmune fetal hydrops to adolescent onset), primary adrenal insufficiency (with or without mineralocorticoid deficiency), testicular insufficiency, hypothyroidism, ichthyosis, lymphopenia/immunodeficiency, and neurologic abnormalities that can include developmental delay, regression / progressive neurologic involvement, cranial nerve deficits, and peripheral motor and sensory neuropathy.
Autoimmune interstitial lung disease-arthritis syndrome
MedGen UID:
1800821
Concept ID:
C5243948
Disease or Syndrome
Systemic autoinflammation and autoimmunity with immune dysregulation (AIAISD) is an autosomal dominant systemic autoinflammatory disorder with autoimmunity and immune dysregulation. Affected individuals present in the first decade of life with variable features that may include interstitial lung disease, alveolar hemorrhage, inflammatory arthritis, neuromyelitis optica, livedo reticularis, dysautonomia, recurrent infections, and renal disease. Laboratory studies usually show high-titer autoantibodies and features of inflammation, including a type I interferon (e.g., 147660) signature and elevation of inflammatory cytokines. The disorder shows significant incomplete penetrance; most carrier parents are unaffected (summary by Watkin et al., 2015; Delafontaine et al., 2024).
Nephrotic syndrome, type 23
MedGen UID:
1787011
Concept ID:
C5543092
Disease or Syndrome
Nephrotic syndrome type 23 (NPHS23) is an autosomal recessive renal disorder characterized by the onset of proteinuria in the first or second decade of life. The outcome is variable: some patients have normal renal function after many years, whereas others may progress to chronic kidney disease. Renal biopsy shows mesangial hypercellularity, consistent with minimal change disease, focal segmental glomerulosclerosis, and effacement of podocyte foot processes (summary by Solanki et al., 2019). For a general phenotypic description and a discussion of genetic heterogeneity of nephrotic syndrome and FSGS, see NPHS1 (256300).
Cataracts, hearing impairment, nephrotic syndrome, and enterocolitis 2
MedGen UID:
1841226
Concept ID:
C5830590
Disease or Syndrome
Cataracts, hearing impairment, nephrotic syndrome, and enterocolitis-2 (CHINE2) is an autosomal recessive syndromic disorder characterized by onset of this constellation of features in infancy, resulting in death in early childhood. Telomeres are shortened, but classic mucocutaneous features of DKCB1 are not typically observed. CHINE2 is due to a ribosomal pseudouridylation defect (Balogh et al., 2020). See also CHINE1 (301108), caused by mutation in the DKC1 gene (300126).

Professional guidelines

PubMed

El Karoui K, Fervenza FC, De Vriese AS
J Am Soc Nephrol 2024 Jan 1;35(1):103-116. Epub 2023 Sep 29 doi: 10.1681/ASN.0000000000000242. PMID: 37772889Free PMC Article
Zee J, McNulty MT, Hodgin JB, Zhdanova O, Hingorani S, Jefferson JA, Gibson KL, Trachtman H, Fornoni A, Dell KM, Reich HN, Bagnasco S, Greenbaum LA, Lafayette RA, Gipson DS, Brown E, Kretzler M, Appel G, Sambandam KK, Tuttle KR, Chen D, Atkinson MA, Hogan MC, Kaskel FJ, Meyers KE, O'Toole J, Srivastava T, Sethna CB, Hladunewich MA, Lin JJ, Nast CC, Derebail VK, Patel J, Vento S, Holzman LB, Athavale AM, Adler SG, Lemley KV, Lieske JC, Hogan JJ, Gadegbeku CA, Fervenza FC, Wang CS, Matar RB, Singer P, Kopp JB, Barisoni L, Sampson MG
Pediatr Nephrol 2021 Sep;36(9):2747-2757. Epub 2021 Mar 1 doi: 10.1007/s00467-021-04990-4. PMID: 33646395Free PMC Article
Bajema IM, Wilhelmus S, Alpers CE, Bruijn JA, Colvin RB, Cook HT, D'Agati VD, Ferrario F, Haas M, Jennette JC, Joh K, Nast CC, Noël LH, Rijnink EC, Roberts ISD, Seshan SV, Sethi S, Fogo AB
Kidney Int 2018 Apr;93(4):789-796. Epub 2018 Feb 16 doi: 10.1016/j.kint.2017.11.023. PMID: 29459092

Recent clinical studies

Etiology

El Karoui K, Fervenza FC, De Vriese AS
J Am Soc Nephrol 2024 Jan 1;35(1):103-116. Epub 2023 Sep 29 doi: 10.1681/ASN.0000000000000242. PMID: 37772889Free PMC Article
Hassler JR
Semin Diagn Pathol 2020 May;37(3):143-147. Epub 2020 Mar 16 doi: 10.1053/j.semdp.2020.03.001. PMID: 32241578
Bajema IM, Wilhelmus S, Alpers CE, Bruijn JA, Colvin RB, Cook HT, D'Agati VD, Ferrario F, Haas M, Jennette JC, Joh K, Nast CC, Noël LH, Rijnink EC, Roberts ISD, Seshan SV, Sethi S, Fogo AB
Kidney Int 2018 Apr;93(4):789-796. Epub 2018 Feb 16 doi: 10.1016/j.kint.2017.11.023. PMID: 29459092
Trimarchi H, Barratt J, Cattran DC, Cook HT, Coppo R, Haas M, Liu ZH, Roberts IS, Yuzawa Y, Zhang H, Feehally J; IgAN Classification Working Group of the International IgA Nephropathy Network and the Renal Pathology Society; Conference Participants
Kidney Int 2017 May;91(5):1014-1021. Epub 2017 Mar 22 doi: 10.1016/j.kint.2017.02.003. PMID: 28341274
Weening JJ, D'Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB, Balow JE, Bruijn JA, Cook T, Ferrario F, Fogo AB, Ginzler EM, Hebert L, Hill G, Hill P, Jennette JC, Kong NC, Lesavre P, Lockshin M, Looi LM, Makino H, Moura LA, Nagata M; International Society of Nephrology Working Group on the Classification of Lupus Nephritis; Renal Pathology Society Working Group on the Classification of Lupus Nephritis
Kidney Int 2004 Feb;65(2):521-30. doi: 10.1111/j.1523-1755.2004.00443.x. PMID: 14717922

Diagnosis

Kademani SP, Nelaturi P, Sathyasagar K, Ravikumar S
J Nephrol 2023 Apr;36(3):911-923. Epub 2022 Dec 10 doi: 10.1007/s40620-022-01498-4. PMID: 36495425
Said SM, Rocha AB, Royal V, Valeri AM, Larsen CP, Theis JD, Vrana JA, McPhail ED, Bandi L, Safabakhsh S, Barnes C, Cornell LD, Fidler ME, Alexander MP, Leung N, Nasr SH
Am J Kidney Dis 2021 Mar;77(3):454-458. Epub 2020 Jul 23 doi: 10.1053/j.ajkd.2020.04.015. PMID: 32711071
Hassler JR
Semin Diagn Pathol 2020 May;37(3):143-147. Epub 2020 Mar 16 doi: 10.1053/j.semdp.2020.03.001. PMID: 32241578
Bajema IM, Wilhelmus S, Alpers CE, Bruijn JA, Colvin RB, Cook HT, D'Agati VD, Ferrario F, Haas M, Jennette JC, Joh K, Nast CC, Noël LH, Rijnink EC, Roberts ISD, Seshan SV, Sethi S, Fogo AB
Kidney Int 2018 Apr;93(4):789-796. Epub 2018 Feb 16 doi: 10.1016/j.kint.2017.11.023. PMID: 29459092
Weening JJ, D'Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB, Balow JE, Bruijn JA, Cook T, Ferrario F, Fogo AB, Ginzler EM, Hebert L, Hill G, Hill P, Jennette JC, Kong NC, Lesavre P, Lockshin M, Looi LM, Makino H, Moura LA, Nagata M; International Society of Nephrology Working Group on the Classification of Lupus Nephritis; Renal Pathology Society Working Group on the Classification of Lupus Nephritis
Kidney Int 2004 Feb;65(2):521-30. doi: 10.1111/j.1523-1755.2004.00443.x. PMID: 14717922

Therapy

Vasconcelos AS, Mazzali M, de Sousa MV
J Bras Nefrol 2023 Jul-Sep;45(3):350-356. doi: 10.1590/2175-8239-JBN-2022-0051en. PMID: 36626310Free PMC Article
Shoji J, Mii A, Terasaki M, Shimizu A
Nephron 2020;144 Suppl 1:65-70. Epub 2020 Dec 1 doi: 10.1159/000510748. PMID: 33260184
Trimarchi H, Barratt J, Cattran DC, Cook HT, Coppo R, Haas M, Liu ZH, Roberts IS, Yuzawa Y, Zhang H, Feehally J; IgAN Classification Working Group of the International IgA Nephropathy Network and the Renal Pathology Society; Conference Participants
Kidney Int 2017 May;91(5):1014-1021. Epub 2017 Mar 22 doi: 10.1016/j.kint.2017.02.003. PMID: 28341274
Soares MF, Roberts IS
Curr Opin Nephrol Hypertens 2017 May;26(3):165-171. doi: 10.1097/MNH.0000000000000312. PMID: 28221174
Shimizu A, Higo S, Fujita E, Mii A, Kaneko T
Clin Transplant 2011 Jul;25 Suppl 23:6-14. doi: 10.1111/j.1399-0012.2011.01452.x. PMID: 21623907

Prognosis

El Karoui K, Fervenza FC, De Vriese AS
J Am Soc Nephrol 2024 Jan 1;35(1):103-116. Epub 2023 Sep 29 doi: 10.1681/ASN.0000000000000242. PMID: 37772889Free PMC Article
Bajema IM, Wilhelmus S, Alpers CE, Bruijn JA, Colvin RB, Cook HT, D'Agati VD, Ferrario F, Haas M, Jennette JC, Joh K, Nast CC, Noël LH, Rijnink EC, Roberts ISD, Seshan SV, Sethi S, Fogo AB
Kidney Int 2018 Apr;93(4):789-796. Epub 2018 Feb 16 doi: 10.1016/j.kint.2017.11.023. PMID: 29459092
Trimarchi H, Barratt J, Cattran DC, Cook HT, Coppo R, Haas M, Liu ZH, Roberts IS, Yuzawa Y, Zhang H, Feehally J; IgAN Classification Working Group of the International IgA Nephropathy Network and the Renal Pathology Society; Conference Participants
Kidney Int 2017 May;91(5):1014-1021. Epub 2017 Mar 22 doi: 10.1016/j.kint.2017.02.003. PMID: 28341274
Soares MF, Roberts IS
Curr Opin Nephrol Hypertens 2017 May;26(3):165-171. doi: 10.1097/MNH.0000000000000312. PMID: 28221174
Working Group of the International IgA Nephropathy Network and the Renal Pathology Society, Cattran DC, Coppo R, Cook HT, Feehally J, Roberts IS, Troyanov S, Alpers CE, Amore A, Barratt J, Berthoux F, Bonsib S, Bruijn JA, D'Agati V, D'Amico G, Emancipator S, Emma F, Ferrario F, Fervenza FC, Florquin S, Fogo A, Geddes CC, Groene HJ, Haas M, Herzenberg AM, Hill PA, Hogg RJ, Hsu SI, Jennette JC, Joh K, Julian BA, Kawamura T, Lai FM, Leung CB, Li LS, Li PK, Liu ZH, Mackinnon B, Mezzano S, Schena FP, Tomino Y, Walker PD, Wang H, Weening JJ, Yoshikawa N, Zhang H
Kidney Int 2009 Sep;76(5):534-45. Epub 2009 Jul 1 doi: 10.1038/ki.2009.243. PMID: 19571791

Clinical prediction guides

El Karoui K, Fervenza FC, De Vriese AS
J Am Soc Nephrol 2024 Jan 1;35(1):103-116. Epub 2023 Sep 29 doi: 10.1681/ASN.0000000000000242. PMID: 37772889Free PMC Article
Hassler JR
Semin Diagn Pathol 2020 May;37(3):143-147. Epub 2020 Mar 16 doi: 10.1053/j.semdp.2020.03.001. PMID: 32241578
Bajema IM, Wilhelmus S, Alpers CE, Bruijn JA, Colvin RB, Cook HT, D'Agati VD, Ferrario F, Haas M, Jennette JC, Joh K, Nast CC, Noël LH, Rijnink EC, Roberts ISD, Seshan SV, Sethi S, Fogo AB
Kidney Int 2018 Apr;93(4):789-796. Epub 2018 Feb 16 doi: 10.1016/j.kint.2017.11.023. PMID: 29459092
Trimarchi H, Barratt J, Cattran DC, Cook HT, Coppo R, Haas M, Liu ZH, Roberts IS, Yuzawa Y, Zhang H, Feehally J; IgAN Classification Working Group of the International IgA Nephropathy Network and the Renal Pathology Society; Conference Participants
Kidney Int 2017 May;91(5):1014-1021. Epub 2017 Mar 22 doi: 10.1016/j.kint.2017.02.003. PMID: 28341274
Working Group of the International IgA Nephropathy Network and the Renal Pathology Society, Cattran DC, Coppo R, Cook HT, Feehally J, Roberts IS, Troyanov S, Alpers CE, Amore A, Barratt J, Berthoux F, Bonsib S, Bruijn JA, D'Agati V, D'Amico G, Emancipator S, Emma F, Ferrario F, Fervenza FC, Florquin S, Fogo A, Geddes CC, Groene HJ, Haas M, Herzenberg AM, Hill PA, Hogg RJ, Hsu SI, Jennette JC, Joh K, Julian BA, Kawamura T, Lai FM, Leung CB, Li LS, Li PK, Liu ZH, Mackinnon B, Mezzano S, Schena FP, Tomino Y, Walker PD, Wang H, Weening JJ, Yoshikawa N, Zhang H
Kidney Int 2009 Sep;76(5):534-45. Epub 2009 Jul 1 doi: 10.1038/ki.2009.243. PMID: 19571791

Recent systematic reviews

Luo X, Tan J, Wan D, Chen J, Hu Y
Int Urol Nephrol 2022 Jan;54(1):99-109. Epub 2021 Apr 27 doi: 10.1007/s11255-021-02861-3. PMID: 33905044
Khairwa A
Afr Health Sci 2021 Mar;21(1):159-165. doi: 10.4314/ahs.v21i1.21. PMID: 34394293Free PMC Article
Jellouli M, Charfi R, Maalej B, Mahfoud A, Trabelsi S, Gargah T
J Pediatr 2018 Jun;197:191-197.e1. Epub 2018 Apr 18 doi: 10.1016/j.jpeds.2018.01.008. PMID: 29680473
Shao X, Li B, Cao L, Liang L, Yang J, Wang Y, Feng S, Wang C, Weng C, Shen X, Jiang H, Chen J
Oncotarget 2017 Jul 11;8(28):46436-46448. doi: 10.18632/oncotarget.17502. PMID: 28526805Free PMC Article
Lv J, Shi S, Xu D, Zhang H, Troyanov S, Cattran DC, Wang H
Am J Kidney Dis 2013 Nov;62(5):891-9. Epub 2013 Jun 29 doi: 10.1053/j.ajkd.2013.04.021. PMID: 23820066

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