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Cholestasis, intrahepatic, of pregnancy, 3(ICP3)

MedGen UID:
767155
Concept ID:
C3554241
Disease or Syndrome
Synonyms: CHOLESTASIS, INTRAHEPATIC, OF PREGNANCY 3; ICP3
 
Gene (location): ABCB4 (7q21.12)
 
Monarch Initiative: MONDO:0013995
OMIM®: 614972

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. Women with ICP are also susceptible to oral contraceptive-induced cholestasis (OCIC). Ursodeoxycholic acid (UDCA) is an effective treatment for conditions caused by ABCB4 mutations (summary by Pasmant et al., 2012). Mutation in the ABCB4 gene accounts for about 15% of ICP cases (summary by Ziol et al., 2008). For a discussion of genetic heterogeneity of ICP, see ICP1 (147480). [from OMIM]

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.
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

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
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
Bechtel MA
Dermatol Clin 2018 Jul;36(3):259-265. Epub 2018 Apr 26 doi: 10.1016/j.det.2018.02.012. PMID: 29929597

Recent clinical studies

Etiology

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
Birkness-Gartman JE, Oshima K
Pathol Int 2022 Jan;72(1):1-13. Epub 2021 Nov 24 doi: 10.1111/pin.13186. PMID: 34818440
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
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
Bechtel MA
Dermatol Clin 2018 Jul;36(3):259-265. Epub 2018 Apr 26 doi: 10.1016/j.det.2018.02.012. PMID: 29929597

Diagnosis

Birkness-Gartman JE, Oshima K
Pathol Int 2022 Jan;72(1):1-13. Epub 2021 Nov 24 doi: 10.1111/pin.13186. PMID: 34818440
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
Ma K, Berger D, Reau N
Clin Liver Dis 2019 May;23(2):345-361. Epub 2019 Feb 26 doi: 10.1016/j.cld.2018.12.013. PMID: 30947881
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
Bechtel MA
Dermatol Clin 2018 Jul;36(3):259-265. Epub 2018 Apr 26 doi: 10.1016/j.det.2018.02.012. PMID: 29929597

Therapy

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
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
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
Wijarnpreecha K, Thongprayoon C, Sanguankeo A, Upala S, Ungprasert P, Cheungpasitporn W
Clin Res Hepatol Gastroenterol 2017 Feb;41(1):39-45. Epub 2016 Aug 16 doi: 10.1016/j.clinre.2016.07.004. PMID: 27542514

Prognosis

Malarkiewicz P, Nowacka U, Januszaniec A, Mankiewicz A, Kozłowski S, Issat T
Medicina (Kaunas) 2024 Apr 22;60(4) doi: 10.3390/medicina60040676. PMID: 38674322Free PMC Article
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
Zhu D, Song Y, Ding Q, Duan C, Wu W, Xu J
Arch Gynecol Obstet 2021 Sep;304(3):695-701. Epub 2021 May 23 doi: 10.1007/s00404-021-06100-z. PMID: 34027618
Hämäläinen ST, Turunen K, Mattila KJ, Kosunen E, Sumanen M
Acta Obstet Gynecol Scand 2019 Dec;98(12):1534-1539. Epub 2019 Aug 14 doi: 10.1111/aogs.13695. PMID: 31355915
O'Leary JG, Pratt DS
Curr Opin Gastroenterol 2007 May;23(3):232-6. doi: 10.1097/MOG.0b013e3280d942d8. PMID: 17414837

Clinical prediction guides

Malarkiewicz P, Nowacka U, Januszaniec A, Mankiewicz A, Kozłowski S, Issat T
Medicina (Kaunas) 2024 Apr 22;60(4) doi: 10.3390/medicina60040676. PMID: 38674322Free 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
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
Vasavan T, Ferraro E, Ibrahim E, Dixon P, Gorelik J, Williamson C
Biochim Biophys Acta Mol Basis Dis 2018 Apr;1864(4 Pt B):1345-1355. Epub 2018 Jan 6 doi: 10.1016/j.bbadis.2017.12.039. PMID: 29317337

Recent systematic reviews

Daneshvar M, Yadegari A, Ribaldone DG, Hasanzadeh M, Djafarian K
J Obstet Gynaecol 2022 Oct;42(7):2621-2628. Epub 2022 Sep 12 doi: 10.1080/01443615.2022.2114822. PMID: 36094006
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
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
Wijarnpreecha K, Thongprayoon C, Sanguankeo A, Upala S, Ungprasert P, Cheungpasitporn W
Clin Res Hepatol Gastroenterol 2017 Feb;41(1):39-45. Epub 2016 Aug 16 doi: 10.1016/j.clinre.2016.07.004. PMID: 27542514
Pongcharoen P, Fleischer AB Jr
Eur J Pain 2016 Jan;20(1):24-31. Epub 2015 Sep 28 doi: 10.1002/ejp.766. PMID: 26416344

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