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Liddle syndrome 1(LIDLS1)

MedGen UID:
1684617
Concept ID:
CN031472
Disease or Syndrome
Synonyms: Liddle Syndrome, SCNN1B-Related; LIDLS1; Pseudoaldosteronism
 
Gene (location): SCNN1B (16p12.2)
 
Monarch Initiative: MONDO:0020607
OMIM®: 177200

Definition

Liddle syndrome is an autosomal dominant disorder characterized by early-onset salt-sensitive hypertension, hypokalemia, metabolic alkalosis, and suppression of plasma renin activity and aldosterone secretion (summary by Yang et al., 2014). Genetic Heterogeneity of Liddle Syndrome Liddle syndrome-2 (618114) is caused by mutation in the SCNN1G gene (600761), which encodes the ENaC gamma subunit. Liddle syndrome-3 (618126) is caused by mutation in the SCNN1A gene (600228), which encodes the ENaC alpha subunit. Hanukoglu and Hanukoglu (2016) provided a detailed review of the ENaC gene family, including structure, function, tissue distribution, and associated inherited diseases. [from OMIM]

Professional guidelines

PubMed

Charoensri S, Auchus RJ
Eur J Endocrinol 2023 Nov 8;189(5):R11-R22. doi: 10.1093/ejendo/lvad140. PMID: 37847213
Guo W, Ji P, Xie Y
J Nephrol 2023 Mar;36(2):575-591. Epub 2022 Aug 22 doi: 10.1007/s40620-022-01428-4. PMID: 35994232
Assadi F
Iran J Kidney Dis 2008 Jul;2(3):115-22. PMID: 19377223

Recent clinical studies

Etiology

Singh V, Van Why SK
Med Clin North Am 2024 Jan;108(1):157-172. Epub 2023 Jul 27 doi: 10.1016/j.mcna.2023.06.005. PMID: 37951648
Charoensri S, Auchus RJ
Eur J Endocrinol 2023 Nov 8;189(5):R11-R22. doi: 10.1093/ejendo/lvad140. PMID: 37847213
Pappachan JM, Buch HN
Adv Exp Med Biol 2017;956:215-237. doi: 10.1007/5584_2016_26. PMID: 27864805
Valsan D, Burhan U, Teehan G
Adv Exp Med Biol 2017;956:181-189. doi: 10.1007/5584_2016_38. PMID: 27864800
Aziz DA, Memon F, Rahman A, Ali M
J Ayub Med Coll Abbottabad 2016 Oct-Dec;28(4):809-811. PMID: 28586600

Diagnosis

Singh V, Van Why SK
Med Clin North Am 2024 Jan;108(1):157-172. Epub 2023 Jul 27 doi: 10.1016/j.mcna.2023.06.005. PMID: 37951648
Athimulam S, Lazik N, Bancos I
Endocrinol Metab Clin North Am 2019 Dec;48(4):701-715. Epub 2019 Sep 25 doi: 10.1016/j.ecl.2019.08.003. PMID: 31655771
Pappachan JM, Buch HN
Adv Exp Med Biol 2017;956:215-237. doi: 10.1007/5584_2016_26. PMID: 27864805
Valsan D, Burhan U, Teehan G
Adv Exp Med Biol 2017;956:181-189. doi: 10.1007/5584_2016_38. PMID: 27864800
Assadi F
Iran J Kidney Dis 2008 Jul;2(3):115-22. PMID: 19377223

Therapy

Athimulam S, Lazik N, Bancos I
Endocrinol Metab Clin North Am 2019 Dec;48(4):701-715. Epub 2019 Sep 25 doi: 10.1016/j.ecl.2019.08.003. PMID: 31655771
Cui Y, Tong A, Jiang J, Wang F, Li C
J Clin Hypertens (Greenwich) 2017 May;19(5):524-529. Epub 2016 Nov 29 doi: 10.1111/jch.12949. PMID: 27896928Free PMC Article
Valsan D, Burhan U, Teehan G
Adv Exp Med Biol 2017;956:181-189. doi: 10.1007/5584_2016_38. PMID: 27864800
Aziz DA, Memon F, Rahman A, Ali M
J Ayub Med Coll Abbottabad 2016 Oct-Dec;28(4):809-811. PMID: 28586600
Assadi F
Iran J Kidney Dis 2008 Jul;2(3):115-22. PMID: 19377223

Prognosis

Khandelwal P, Deinum J
Pediatr Nephrol 2022 Jul;37(7):1495-1509. Epub 2021 Aug 20 doi: 10.1007/s00467-021-05246-x. PMID: 34414500
Kemp SA, Collier DA, Datir RP, Ferreira IATM, Gayed S, Jahun A, Hosmillo M, Rees-Spear C, Mlcochova P, Lumb IU, Roberts DJ, Chandra A, Temperton N; CITIID-NIHR BioResource COVID-19 Collaboration; COVID-19 Genomics UK (COG-UK) Consortium, Sharrocks K, Blane E, Modis Y, Leigh KE, Briggs JAG, van Gils MJ, Smith KGC, Bradley JR, Smith C, Doffinger R, Ceron-Gutierrez L, Barcenas-Morales G, Pollock DD, Goldstein RA, Smielewska A, Skittrall JP, Gouliouris T, Goodfellow IG, Gkrania-Klotsas E, Illingworth CJR, McCoy LE, Gupta RK
Nature 2021 Apr;592(7853):277-282. Epub 2021 Feb 5 doi: 10.1038/s41586-021-03291-y. PMID: 33545711Free PMC Article
Levanovich PE, Diaczok A, Rossi NF
Curr Hypertens Rev 2020;16(2):91-107. doi: 10.2174/1573402115666190409115330. PMID: 30963979Free PMC Article
Pappachan JM, Buch HN
Adv Exp Med Biol 2017;956:215-237. doi: 10.1007/5584_2016_26. PMID: 27864805
Khosla N, Hogan D
Semin Nephrol 2006 Nov;26(6):434-40. doi: 10.1016/j.semnephrol.2006.10.004. PMID: 17275580

Clinical prediction guides

Chen M, Lv X, Li J, Guo M, Ma S
Scand J Clin Lab Invest 2022 Nov-Dec;82(7-8):576-580. Epub 2022 Nov 6 doi: 10.1080/00365513.2022.2140454. PMID: 36336351
Kemp SA, Collier DA, Datir RP, Ferreira IATM, Gayed S, Jahun A, Hosmillo M, Rees-Spear C, Mlcochova P, Lumb IU, Roberts DJ, Chandra A, Temperton N; CITIID-NIHR BioResource COVID-19 Collaboration; COVID-19 Genomics UK (COG-UK) Consortium, Sharrocks K, Blane E, Modis Y, Leigh KE, Briggs JAG, van Gils MJ, Smith KGC, Bradley JR, Smith C, Doffinger R, Ceron-Gutierrez L, Barcenas-Morales G, Pollock DD, Goldstein RA, Smielewska A, Skittrall JP, Gouliouris T, Goodfellow IG, Gkrania-Klotsas E, Illingworth CJR, McCoy LE, Gupta RK
Nature 2021 Apr;592(7853):277-282. Epub 2021 Feb 5 doi: 10.1038/s41586-021-03291-y. PMID: 33545711Free PMC Article
Pappachan JM, Buch HN
Adv Exp Med Biol 2017;956:215-237. doi: 10.1007/5584_2016_26. PMID: 27864805
Bubien JK
J Biol Chem 2010 Jul 30;285(31):23527-31. Epub 2010 May 11 doi: 10.1074/jbc.R109.025049. PMID: 20460373Free PMC Article
Barbry P, Hofman P
Am J Physiol 1997 Sep;273(3 Pt 1):G571-85. doi: 10.1152/ajpgi.1997.273.3.G571. PMID: 9316461

Recent systematic reviews

Granhøj J, Nøhr TK, Hinrichs GR, Rasmussen M, Svenningsen P
Clin J Am Soc Nephrol 2024 May 1;19(5):610-619. Epub 2024 Jan 24 doi: 10.2215/CJN.0000000000000430. PMID: 38265765Free PMC Article
Lu Y, Liu X, Sun L, Zhang D, Fan P, Yang K, Zhang L, Liu Y, Zhou X
Mol Med Rep 2024 Feb;29(2) Epub 2023 Dec 15 doi: 10.3892/mmr.2023.13142. PMID: 38099339Free PMC Article
Fan P, Pan XC, Zhang D, Yang KQ, Zhang Y, Tian T, Luo F, Ma WJ, Liu YX, Wang LP, Zhang HM, Song L, Cai J, Zhou XL
Am J Hypertens 2020 Jul 18;33(7):670-675. doi: 10.1093/ajh/hpaa037. PMID: 32161960Free PMC Article
Yang KQ, Lu CX, Fan P, Zhang Y, Meng X, Dong XQ, Luo F, Liu YX, Zhang HM, Wu HY, Cai J, Zhang X, Zhou XL
Clin Exp Hypertens 2018;40(2):107-111. Epub 2017 Jul 18 doi: 10.1080/10641963.2017.1334799. PMID: 28718682

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