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IgA nephropathy, susceptibility to, 1(IGAN1)

MedGen UID:
463619
Concept ID:
C3160719
Finding
Synonym: IGAN1
 
Monarch Initiative: MONDO:0008072
OMIM®: 161950

Clinical features

From HPO
Arthralgia
MedGen UID:
13917
Concept ID:
C0003862
Sign or Symptom
Joint pain.
Hematuria
MedGen UID:
5488
Concept ID:
C0018965
Disease or Syndrome
The presence of blood in the urine. Hematuria may be gross hematuria (visible to the naked eye) or microscopic hematuria (detected by dipstick or microscopic examination of the urine).
Proteinuria
MedGen UID:
10976
Concept ID:
C0033687
Finding
Increased levels of protein in the urine.
Stage 5 chronic kidney disease
MedGen UID:
384526
Concept ID:
C2316810
Disease or Syndrome
A degree of kidney failure severe enough to require dialysis or kidney transplantation for survival characterized by a severe reduction in glomerular filtration rate (less than 15 ml/min/1.73 m2) and other manifestations including increased serum creatinine.
IgA deposition in the glomerulus
MedGen UID:
892365
Concept ID:
C4025827
Finding
The presence of immunoglobulin A deposits in the glomerulus.
Hypertensive disorder
MedGen UID:
6969
Concept ID:
C0020538
Disease or Syndrome
The presence of chronic increased pressure in the systemic arterial system.
Nephritis
MedGen UID:
14328
Concept ID:
C0027697
Disease or Syndrome
The presence of inflammation affecting the kidney.
Purpura
MedGen UID:
19584
Concept ID:
C0034150
Disease or Syndrome
Purpura (from Latin

Professional guidelines

PubMed

Smeijer JD, Kohan DE, Webb DJ, Dhaun N, Heerspink HJL
Curr Opin Nephrol Hypertens 2021 Jul 1;30(4):456-465. doi: 10.1097/MNH.0000000000000716. PMID: 33990507
Couser WG
J Bras Nefrol 2016 Mar;38(1):107-22. doi: 10.5935/0101-2800.20160016. PMID: 27049372
Boyd JK, Cheung CK, Molyneux K, Feehally J, Barratt J
Kidney Int 2012 May;81(9):833-43. Epub 2012 Feb 8 doi: 10.1038/ki.2011.501. PMID: 22318424

Recent clinical studies

Etiology

Márquez-Tirado B, Gutiérrez-Tenorio J, Tortajada A, Lucientes Continente L, Caravaca-Fontán F, Malik TH, Roldán Montero R, Elías S, Saiz Gonzalez A, Fernández-Juarez G, Sánchez-Corral P, Pickering MC, Praga M, Rodríguez de Córdoba S, Goicoechea de Jorge E
J Am Soc Nephrol 2022 Jun;33(6):1137-1153. Epub 2022 May 11 doi: 10.1681/ASN.2021101318. PMID: 35545301Free PMC Article
Currie EG, Coburn B, Porfilio EA, Lam P, Rojas OL, Novak J, Yang S, Chowdhury RB, Ward LA, Wang PW, Khaleghi K, An J, Crome SQ, Hladunewich MA, Barbour SJ, Cattran DC, Parekh RS, Licht C, John R, Kaul R, Croitoru K, Gray-Owen SD, Guttman DS, Gommerman JL, Reich HN
JCI Insight 2022 Mar 8;7(5) doi: 10.1172/jci.insight.141289. PMID: 35133979Free PMC Article
Shi M, Ouyang Y, Yang M, Yang M, Zhang X, Huang W, Wang W, Wang Z, Zhang W, Chen X, Pan X, Ren H, Chen N, Xie J
Clin J Am Soc Nephrol 2018 Sep 7;13(9):1330-1338. Epub 2018 Jul 24 doi: 10.2215/CJN.13701217. PMID: 30042224Free PMC Article
Couser WG
J Bras Nefrol 2016 Mar;38(1):107-22. doi: 10.5935/0101-2800.20160016. PMID: 27049372
Suzuki H, Kiryluk K, Novak J, Moldoveanu Z, Herr AB, Renfrow MB, Wyatt RJ, Scolari F, Mestecky J, Gharavi AG, Julian BA
J Am Soc Nephrol 2011 Oct;22(10):1795-803. Epub 2011 Sep 23 doi: 10.1681/ASN.2011050464. PMID: 21949093Free PMC Article

Diagnosis

In JW, Jung K, Shin S, Park KU, Lee H, Song EY
Ann Lab Med 2022 Jan 1;42(1):54-62. doi: 10.3343/alm.2022.42.1.54. PMID: 34374349Free PMC Article
Wang YN, Zhou XJ, Chen P, Yu GZ, Zhang X, Hou P, Liu LJ, Shi SF, Lv JC, Zhang H
J Am Soc Nephrol 2021 Mar;32(3):545-552. Epub 2021 Feb 16 doi: 10.1681/ASN.2020060823. PMID: 33593824Free PMC Article
Sallustio F, Curci C, Di Leo V, Gallone A, Pesce F, Gesualdo L
Int J Mol Sci 2019 Dec 26;21(1) doi: 10.3390/ijms21010189. PMID: 31888082Free PMC Article
Limou S, Vince N, Parsa A
Clin J Am Soc Nephrol 2018 Jan 6;13(1):140-152. Epub 2017 Dec 14 doi: 10.2215/CJN.09030817. PMID: 29242368Free PMC Article
Wada J, Sugiyama H, Makino H
Semin Nephrol 2003 Nov;23(6):556-63. doi: 10.1053/s0270-9295(03)00134-7. PMID: 14631563

Therapy

Liu L, Khan A, Sanchez-Rodriguez E, Zanoni F, Li Y, Steers N, Balderes O, Zhang J, Krithivasan P, LeDesma RA, Fischman C, Hebbring SJ, Harley JB, Moncrieffe H, Kottyan LC, Namjou-Khales B, Walunas TL, Knevel R, Raychaudhuri S, Karlson EW, Denny JC, Stanaway IB, Crosslin D, Rauen T, Floege J, Eitner F, Moldoveanu Z, Reily C, Knoppova B, Hall S, Sheff JT, Julian BA, Wyatt RJ, Suzuki H, Xie J, Chen N, Zhou X, Zhang H, Hammarström L, Viktorin A, Magnusson PKE, Shang N, Hripcsak G, Weng C, Rundek T, Elkind MSV, Oelsner EC, Barr RG, Ionita-Laza I, Novak J, Gharavi AG, Kiryluk K
Nat Commun 2022 Nov 11;13(1):6859. doi: 10.1038/s41467-022-34456-6. PMID: 36369178Free PMC Article
Smeijer JD, Kohan DE, Webb DJ, Dhaun N, Heerspink HJL
Curr Opin Nephrol Hypertens 2021 Jul 1;30(4):456-465. doi: 10.1097/MNH.0000000000000716. PMID: 33990507
Mao S, Huang S
Ren Fail 2015 Feb;37(1):16-21. Epub 2014 Oct 28 doi: 10.3109/0886022X.2014.977142. PMID: 25350836
Li Z, Li Y, Chen L
Ren Fail 2014 Nov;36(10):1473-80. Epub 2014 Aug 12 doi: 10.3109/0886022X.2014.947517. PMID: 25112155
Boyd JK, Cheung CK, Molyneux K, Feehally J, Barratt J
Kidney Int 2012 May;81(9):833-43. Epub 2012 Feb 8 doi: 10.1038/ki.2011.501. PMID: 22318424

Prognosis

Márquez-Tirado B, Gutiérrez-Tenorio J, Tortajada A, Lucientes Continente L, Caravaca-Fontán F, Malik TH, Roldán Montero R, Elías S, Saiz Gonzalez A, Fernández-Juarez G, Sánchez-Corral P, Pickering MC, Praga M, Rodríguez de Córdoba S, Goicoechea de Jorge E
J Am Soc Nephrol 2022 Jun;33(6):1137-1153. Epub 2022 May 11 doi: 10.1681/ASN.2021101318. PMID: 35545301Free PMC Article
Fu D, Zhong Z, Shi D, Peng Y, Li B, Wang D, Guo L, Li Z, Mao H, Yu X, Li M
Immunobiology 2020 Jul;225(4):151973. Epub 2020 Jun 8 doi: 10.1016/j.imbio.2020.151973. PMID: 32747022
Shi M, Ouyang Y, Yang M, Yang M, Zhang X, Huang W, Wang W, Wang Z, Zhang W, Chen X, Pan X, Ren H, Chen N, Xie J
Clin J Am Soc Nephrol 2018 Sep 7;13(9):1330-1338. Epub 2018 Jul 24 doi: 10.2215/CJN.13701217. PMID: 30042224Free PMC Article
Limou S, Vince N, Parsa A
Clin J Am Soc Nephrol 2018 Jan 6;13(1):140-152. Epub 2017 Dec 14 doi: 10.2215/CJN.09030817. PMID: 29242368Free PMC Article
Brenchley PE, Poulton K, Morton M, Picton ML
Transplant Rev (Orlando) 2014 Jul;28(3):140-4. Epub 2014 Jan 27 doi: 10.1016/j.trre.2014.01.004. PMID: 24630158

Clinical prediction guides

Liu L, Khan A, Sanchez-Rodriguez E, Zanoni F, Li Y, Steers N, Balderes O, Zhang J, Krithivasan P, LeDesma RA, Fischman C, Hebbring SJ, Harley JB, Moncrieffe H, Kottyan LC, Namjou-Khales B, Walunas TL, Knevel R, Raychaudhuri S, Karlson EW, Denny JC, Stanaway IB, Crosslin D, Rauen T, Floege J, Eitner F, Moldoveanu Z, Reily C, Knoppova B, Hall S, Sheff JT, Julian BA, Wyatt RJ, Suzuki H, Xie J, Chen N, Zhou X, Zhang H, Hammarström L, Viktorin A, Magnusson PKE, Shang N, Hripcsak G, Weng C, Rundek T, Elkind MSV, Oelsner EC, Barr RG, Ionita-Laza I, Novak J, Gharavi AG, Kiryluk K
Nat Commun 2022 Nov 11;13(1):6859. doi: 10.1038/s41467-022-34456-6. PMID: 36369178Free PMC Article
Shi M, Ouyang Y, Yang M, Yang M, Zhang X, Huang W, Wang W, Wang Z, Zhang W, Chen X, Pan X, Ren H, Chen N, Xie J
Clin J Am Soc Nephrol 2018 Sep 7;13(9):1330-1338. Epub 2018 Jul 24 doi: 10.2215/CJN.13701217. PMID: 30042224Free PMC Article
Limou S, Vince N, Parsa A
Clin J Am Soc Nephrol 2018 Jan 6;13(1):140-152. Epub 2017 Dec 14 doi: 10.2215/CJN.09030817. PMID: 29242368Free PMC Article
Brenchley PE, Poulton K, Morton M, Picton ML
Transplant Rev (Orlando) 2014 Jul;28(3):140-4. Epub 2014 Jan 27 doi: 10.1016/j.trre.2014.01.004. PMID: 24630158
Wada J, Sugiyama H, Makino H
Semin Nephrol 2003 Nov;23(6):556-63. doi: 10.1053/s0270-9295(03)00134-7. PMID: 14631563

Recent systematic reviews

Ding X, Mei Y, Mao Z, Long L, Han Q, You Y, Zhu H
Front Immunol 2021;12:683913. Epub 2021 Jun 30 doi: 10.3389/fimmu.2021.683913. PMID: 34354705Free PMC Article

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