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Cholestasis, intrahepatic, of pregnancy, 1(ICP1)

MedGen UID:
762759
Concept ID:
C3549845
Disease or Syndrome
Synonyms: CHOLESTASIS, PREGNANCY-RELATED, 1; ICP1
 
Gene (location): ATP8B1 (18q21.31)
 
Monarch Initiative: MONDO:0007829
OMIM®: 147480

Definition

Intrahepatic cholestasis of pregnancy is a reversible form of cholestasis that occurs most often in the third trimester of pregnancy and recurs in 45 to 70% of subsequent pregnancies. Symptoms include pruritus, jaundice, increased serum bile salts, and abnormal liver enzymes, all of which resolve rapidly after delivery. However, the condition is associated with fetal complications, including placental insufficiency, premature labor, fetal distress, and intrauterine death. Some women with ICP may also be susceptible to oral contraceptive-induced cholestasis (OCIC) (summary by Pasmant et al., 2012). Genetic Heterogeneity of Intrahepatic Cholestasis of Pregnancy See also ICP3 (614972), caused by mutation in the ABCB4 gene (171060). [from OMIM]

Additional description

From MedlinePlus Genetics
Intrahepatic cholestasis of pregnancy is a liver disorder that occurs in pregnant women. Cholestasis is a condition that impairs the release of a digestive fluid called bile from liver cells. As a result, bile builds up in the liver, impairing liver function. Because the problems with bile release occur within the liver (intrahepatic), the condition is described as intrahepatic cholestasis. Intrahepatic cholestasis of pregnancy usually becomes apparent in the third trimester of pregnancy. Bile flow returns to normal after delivery of the baby, and the signs and symptoms of the condition disappear. However, they can return during later pregnancies.

This condition causes severe itchiness (pruritus) in the expectant mother. The itchiness usually begins on the palms of the hands and the soles of the feet and then spreads to other parts of the body. Occasionally, affected women have yellowing of the skin and whites of the eyes (jaundice). Some studies have shown that women with intrahepatic cholestasis of pregnancy are more likely to develop gallstones sometime in their life than women who do not have the condition.

Intrahepatic cholestasis of pregnancy can cause problems for the unborn baby. This condition is associated with an increased risk of premature delivery and stillbirth. Additionally, some infants born to mothers with intrahepatic cholestasis of pregnancy have a slow heart rate and a lack of oxygen during delivery (fetal distress).  https://medlineplus.gov/genetics/condition/intrahepatic-cholestasis-of-pregnancy

Clinical features

From HPO
Intrahepatic cholestasis
MedGen UID:
3042
Concept ID:
C0008372
Disease or Syndrome
Impairment of bile flow due to obstruction in the small bile ducts within the liver.
Jaundice
MedGen UID:
43987
Concept ID:
C0022346
Sign or Symptom
Yellow pigmentation of the skin due to bilirubin, which in turn is the result of increased bilirubin concentration in the bloodstream.
Elevated circulating hepatic transaminase concentration
MedGen UID:
338525
Concept ID:
C1848701
Finding
Elevations of the levels of SGOT and SGPT in the serum. SGOT (serum glutamic oxaloacetic transaminase) and SGPT (serum glutamic pyruvic transaminase) are transaminases primarily found in the liver and heart and are released into the bloodstream as the result of liver or heart damage. SGOT and SGPT are used clinically mainly as markers of liver damage.
Increased serum bile acid concentration during pregnancy
MedGen UID:
892830
Concept ID:
C4021882
Finding
Abnormal liver function tests during pregnancy
MedGen UID:
892927
Concept ID:
C4021883
Finding
Pruritus
MedGen UID:
19534
Concept ID:
C0033774
Sign or Symptom
Pruritus is an itch or a sensation that makes a person want to scratch. This term refers to an abnormally increased disposition to experience pruritus.
Fetal distress
MedGen UID:
5164
Concept ID:
C0015930
Pathologic Function
An intrauterine state characterized by suboptimal values in the fetal heart rate, oxygenation of fetal blood, or other parameters indicative of compromise of the fetus. Signs of fetal distress include repetitive variable decelerations, fetal tachycardia or bradycardia, late decelerations, or low biophysical profile.
Premature birth
MedGen UID:
57721
Concept ID:
C0151526
Pathologic Function
The birth of a baby of less than 37 weeks of gestational age.

Professional guidelines

PubMed

Society for Maternal-Fetal Medicine (SMFM). Electronic address: pubs@smfm.org, Lee RH, Mara Greenberg, Metz TD, Pettker CM
Am J Obstet Gynecol 2021 Feb;224(2):B2-B9. Epub 2020 Nov 13 doi: 10.1016/j.ajog.2020.11.002. PMID: 33197417
Italian Association for the Study of the Liver (AISF); Italian Association for the Study of the Liver AISF
Dig Liver Dis 2016 Feb;48(2):120-37. Epub 2015 Nov 14 doi: 10.1016/j.dld.2015.11.004. PMID: 26747754
Kremer AE, Bolier R, van Dijk R, Oude Elferink RP, Beuers U
Dig Dis 2014;32(5):637-45. Epub 2014 Jul 14 doi: 10.1159/000360518. PMID: 25034299

Recent clinical studies

Etiology

Terrault NA, Williamson C
Gastroenterology 2022 Jul;163(1):97-117.e1. Epub 2022 Mar 8 doi: 10.1053/j.gastro.2022.01.060. PMID: 35276220
Society for Maternal-Fetal Medicine (SMFM). Electronic address: pubs@smfm.org, Lee RH, Mara Greenberg, Metz TD, Pettker CM
Am J Obstet Gynecol 2021 Feb;224(2):B2-B9. Epub 2020 Nov 13 doi: 10.1016/j.ajog.2020.11.002. PMID: 33197417
Walker KF, Chappell LC, Hague WM, Middleton P, Thornton JG
Cochrane Database Syst Rev 2020 Jul 27;7(7):CD000493. doi: 10.1002/14651858.CD000493.pub3. PMID: 32716060Free PMC Article
Smith DD, Rood KM
Clin Obstet Gynecol 2020 Mar;63(1):134-151. doi: 10.1097/GRF.0000000000000495. PMID: 31764000
Williamson C, Geenes V
Obstet Gynecol 2014 Jul;124(1):120-133. doi: 10.1097/AOG.0000000000000346. PMID: 24901263

Diagnosis

Terrault NA, Williamson C
Gastroenterology 2022 Jul;163(1):97-117.e1. Epub 2022 Mar 8 doi: 10.1053/j.gastro.2022.01.060. PMID: 35276220
Society for Maternal-Fetal Medicine (SMFM). Electronic address: pubs@smfm.org, Lee RH, Mara Greenberg, Metz TD, Pettker CM
Am J Obstet Gynecol 2021 Feb;224(2):B2-B9. Epub 2020 Nov 13 doi: 10.1016/j.ajog.2020.11.002. PMID: 33197417
Walker KF, Chappell LC, Hague WM, Middleton P, Thornton JG
Cochrane Database Syst Rev 2020 Jul 27;7(7):CD000493. doi: 10.1002/14651858.CD000493.pub3. PMID: 32716060Free PMC Article
Smith DD, Rood KM
Clin Obstet Gynecol 2020 Mar;63(1):134-151. doi: 10.1097/GRF.0000000000000495. PMID: 31764000
Williamson C, Geenes V
Obstet Gynecol 2014 Jul;124(1):120-133. doi: 10.1097/AOG.0000000000000346. PMID: 24901263

Therapy

Beuers U, Wolters F, Oude Elferink RPJ
Nat Rev Gastroenterol Hepatol 2023 Jan;20(1):26-36. Epub 2022 Oct 28 doi: 10.1038/s41575-022-00687-7. PMID: 36307649
Ovadia C, Sajous J, Seed PT, Patel K, Williamson NJ, Attilakos G, Azzaroli F, Bacq Y, Batsry L, Broom K, Brun-Furrer R, Bull L, Chambers J, Cui Y, Ding M, Dixon PH, Estiú MC, Gardiner FW, Geenes V, Grymowicz M, Günaydin B, Hague WM, Haslinger C, Hu Y, Indraccolo U, Juusela A, Kane SC, Kebapcilar A, Kebapcilar L, Kohari K, Kondrackienė J, Koster MPH, Lee RH, Liu X, Locatelli A, Macias RIR, Madazli R, Majewska A, Maksym K, Marathe JA, Morton A, Oudijk MA, Öztekin D, Peek MJ, Shennan AH, Tribe RM, Tripodi V, Türk Özterlemez N, Vasavan T, Wong LFA, Yinon Y, Zhang Q, Zloto K, Marschall HU, Thornton J, Chappell LC, Williamson C
Lancet Gastroenterol Hepatol 2021 Jul;6(7):547-558. Epub 2021 Apr 27 doi: 10.1016/S2468-1253(21)00074-1. PMID: 33915090Free PMC Article
Society for Maternal-Fetal Medicine (SMFM). Electronic address: pubs@smfm.org, Lee RH, Mara Greenberg, Metz TD, Pettker CM
Am J Obstet Gynecol 2021 Feb;224(2):B2-B9. Epub 2020 Nov 13 doi: 10.1016/j.ajog.2020.11.002. PMID: 33197417
Walker KF, Chappell LC, Hague WM, Middleton P, Thornton JG
Cochrane Database Syst Rev 2020 Jul 27;7(7):CD000493. doi: 10.1002/14651858.CD000493.pub3. PMID: 32716060Free PMC Article
Ovadia C, Seed PT, Sklavounos A, Geenes V, Di Ilio C, Chambers J, Kohari K, Bacq Y, Bozkurt N, Brun-Furrer R, Bull L, Estiú MC, Grymowicz M, Gunaydin B, Hague WM, Haslinger C, Hu Y, Kawakita T, Kebapcilar AG, Kebapcilar L, Kondrackienė J, Koster MPH, Kowalska-Kańka A, Kupčinskas L, Lee RH, Locatelli A, Macias RIR, Marschall HU, Oudijk MA, Raz Y, Rimon E, Shan D, Shao Y, Tribe R, Tripodi V, Yayla Abide C, Yenidede I, Thornton JG, Chappell LC, Williamson C
Lancet 2019 Mar 2;393(10174):899-909. Epub 2019 Feb 14 doi: 10.1016/S0140-6736(18)31877-4. PMID: 30773280Free PMC Article

Prognosis

Mathur D, Morgan M, McKenzie J, Wakefield D, Janicki MB, Figueroa R
J Matern Fetal Neonatal Med 2022 Dec;35(25):8975-8981. Epub 2021 Nov 25 doi: 10.1080/14767058.2021.2008896. PMID: 34823422
Pataia V, Dixon PH, Williamson C
Am J Physiol Gastrointest Liver Physiol 2017 Jul 1;313(1):G1-G6. Epub 2017 Apr 27 doi: 10.1152/ajpgi.00028.2017. PMID: 28450276
Ozkan S, Ceylan Y, Ozkan OV, Yildirim S
World J Gastroenterol 2015 Jun 21;21(23):7134-41. doi: 10.3748/wjg.v21.i23.7134. PMID: 26109799Free PMC Article
Azzaroli F, Turco L, Lisotti A, Calvanese C, Mazzella G
Curr Clin Pharmacol 2011 Feb;6(1):12-7. doi: 10.2174/157488411794941313. PMID: 21352094
Pusl T, Beuers U
Orphanet J Rare Dis 2007 May 29;2:26. doi: 10.1186/1750-1172-2-26. PMID: 17535422Free PMC Article

Clinical prediction guides

Karjalainen MK, Karthikeyan S, Oliver-Williams C, Sliz E, Allara E, Fung WT, Surendran P, Zhang W, Jousilahti P, Kristiansson K, Salomaa V, Goodwin M, Hughes DA, Boehnke M, Fernandes Silva L, Yin X, Mahajan A, Neville MJ, van Zuydam NR, de Mutsert R, Li-Gao R, Mook-Kanamori DO, Demirkan A, Liu J, Noordam R, Trompet S, Chen Z, Kartsonaki C, Li L, Lin K, Hagenbeek FA, Hottenga JJ, Pool R, Ikram MA, van Meurs J, Haller T, Milaneschi Y, Kähönen M, Mishra PP, Joshi PK, Macdonald-Dunlop E, Mangino M, Zierer J, Acar IE, Hoyng CB, Lechanteur YTE, Franke L, Kurilshikov A, Zhernakova A, Beekman M, van den Akker EB, Kolcic I, Polasek O, Rudan I, Gieger C, Waldenberger M, Asselbergs FW; China Kadoorie Biobank Collaborative Group; Estonian Biobank Research Team; FinnGen, Hayward C, Fu J, den Hollander AI, Menni C, Spector TD, Wilson JF, Lehtimäki T, Raitakari OT, Penninx BWJH, Esko T, Walters RG, Jukema JW, Sattar N, Ghanbari M, Willems van Dijk K, Karpe F, McCarthy MI, Laakso M, Järvelin MR, Timpson NJ, Perola M, Kooner JS, Chambers JC, van Duijn C, Slagboom PE, Boomsma DI, Danesh J, Ala-Korpela M, Butterworth AS, Kettunen J
Nature 2024 Apr;628(8006):130-138. Epub 2024 Mar 6 doi: 10.1038/s41586-024-07148-y. PMID: 38448586Free PMC Article
Li C, Li N, Liu C, Yin S
BMC Pregnancy Childbirth 2023 Aug 5;23(1):568. doi: 10.1186/s12884-023-05889-8. PMID: 37543573Free PMC Article
Tsur A, Leonard SA, Kan P, Datoc IA, Girsen AI, Shaw GM, Stevenson DK, El-Sayed YY, Druzin ML, Blumenfeld YJ
Am J Perinatol 2023 Aug;40(11):1158-1162. Epub 2023 Apr 26 doi: 10.1055/a-2081-2573. PMID: 37100422
Li J, Chen J, Lee PMY, Zhang J, Li F, Ren T
Hepatology 2023 Aug 1;78(2):389-396. Epub 2023 Feb 24 doi: 10.1097/HEP.0000000000000328. PMID: 36815353
Walker KF, Chappell LC, Hague WM, Middleton P, Thornton JG
Cochrane Database Syst Rev 2020 Jul 27;7(7):CD000493. doi: 10.1002/14651858.CD000493.pub3. PMID: 32716060Free PMC Article

Recent systematic reviews

Zhan Y, Xu T, Chen T, Deng X, Kong Y, Li Y, Wang X
Am J Obstet Gynecol MFM 2023 Aug;5(8):100952. Epub 2023 Apr 5 doi: 10.1016/j.ajogmf.2023.100952. PMID: 37023984
Zhan Y, Xu T, Chen T, Wang X
Acta Obstet Gynecol Scand 2022 Jul;101(7):719-727. Epub 2022 May 22 doi: 10.1111/aogs.14380. PMID: 35599353Free PMC Article
Ovadia C, Sajous J, Seed PT, Patel K, Williamson NJ, Attilakos G, Azzaroli F, Bacq Y, Batsry L, Broom K, Brun-Furrer R, Bull L, Chambers J, Cui Y, Ding M, Dixon PH, Estiú MC, Gardiner FW, Geenes V, Grymowicz M, Günaydin B, Hague WM, Haslinger C, Hu Y, Indraccolo U, Juusela A, Kane SC, Kebapcilar A, Kebapcilar L, Kohari K, Kondrackienė J, Koster MPH, Lee RH, Liu X, Locatelli A, Macias RIR, Madazli R, Majewska A, Maksym K, Marathe JA, Morton A, Oudijk MA, Öztekin D, Peek MJ, Shennan AH, Tribe RM, Tripodi V, Türk Özterlemez N, Vasavan T, Wong LFA, Yinon Y, Zhang Q, Zloto K, Marschall HU, Thornton J, Chappell LC, Williamson C
Lancet Gastroenterol Hepatol 2021 Jul;6(7):547-558. Epub 2021 Apr 27 doi: 10.1016/S2468-1253(21)00074-1. PMID: 33915090Free PMC Article
Walker KF, Chappell LC, Hague WM, Middleton P, Thornton JG
Cochrane Database Syst Rev 2020 Jul 27;7(7):CD000493. doi: 10.1002/14651858.CD000493.pub3. PMID: 32716060Free PMC Article
Ovadia C, Seed PT, Sklavounos A, Geenes V, Di Ilio C, Chambers J, Kohari K, Bacq Y, Bozkurt N, Brun-Furrer R, Bull L, Estiú MC, Grymowicz M, Gunaydin B, Hague WM, Haslinger C, Hu Y, Kawakita T, Kebapcilar AG, Kebapcilar L, Kondrackienė J, Koster MPH, Kowalska-Kańka A, Kupčinskas L, Lee RH, Locatelli A, Macias RIR, Marschall HU, Oudijk MA, Raz Y, Rimon E, Shan D, Shao Y, Tribe R, Tripodi V, Yayla Abide C, Yenidede I, Thornton JG, Chappell LC, Williamson C
Lancet 2019 Mar 2;393(10174):899-909. Epub 2019 Feb 14 doi: 10.1016/S0140-6736(18)31877-4. PMID: 30773280Free PMC Article

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