Severe Neonatal and Obstetric Outcomes in Recurrent Early Intrahepatic Cholestasis of Pregnancy in a Patient with Pre-Existing Myasthenia Gravis: A Case Report of Two Consecutive Pregnancies
Article Sidebar
Main Article Content
Abstract
Intrauterine fetal demise (IUFD) is a recognized risk in intrahepatic cholestasis of pregnancy (ICP), particularly when bile acids exceed 40 µmol/l.
Therefore, many obstetric societies recommend considering induction of labor at or before term, depending on bile acid levels. The Danish guidelines recommend induction of labor after gestational week 34+0 if bile acids exceed 100 µmol/l.
This report details a case of a woman experiencing recurrent early ICP, characterized by initially elevated bile acid levels, leading to IUFD at 33+6 weeks of gestation in her first pregnancy and a preterm subacute delivery at 32+1 weeks of gestation in her second pregnancy. Comprehensive medical evaluations during and after the first pregnancy identified no underlying hepatic disorders. Despite an initial decline in bile acid levels following treatment in both pregnancies, pruritic symptoms intensified and bile acid levels began to rise again at beginning of the third trimester. This case suggests that an early onset of ICP, characterized by increasing bile acids despite treatment and reduced fetal movements, may warrant consideration for preterm delivery even before 34 weeks of gestation; however, the decision to deliver is complex.
Abbreviations
IUFD - Intrauterine fetal demise
ICP – Intrahepatic cholestasis of pregnancy
ALT - alanine-aminotransferase
MRCP - magnetic resonance cholangiopancreatography
CT - scan - computed tomography scan
MRI - magnetic resonance scan
Article Details
This work is licensed under a Creative Commons Attribution 4.0 International License.
CC BY. The paper and it's contents belong to the author, and can be used by others when giving appropriate credit to the authors and the creator (DJOG).
How to Cite
References
1. Bergman H, Melamed N, Koren G. Pruritus in pregnancy: treatment of dermatoses unique to pregnancy. Can Fam Physician Med Fam Can. 2013 Dec;59(12):1290–4.
2. Bacq Y, Sentilhes L, Reyes HB, Glantz A, Kondrackiene J, Binder T, et al. Efficacy of ursodeoxycholic acid in treating intrahepatic cholestasis of pregnancy: a meta-analysis. Gastroenterology. 2012 Dec;143(6):1492–501. DOI: https://doi.org/10.1053/j.gastro.2012.08.004
3. Chiang JYL. Bile acids: regulation of synthesis. J Lipid Res. 2009 Oct;50(10):1955–66. DOI: https://doi.org/10.1194/jlr.R900010-JLR200
4. Lazaridis KN, Gores GJ, Lindor KD. Ursodeoxycholic acid ‘mechanisms of action and clinical use in hepatobiliary disorders.’ J Hepatol. 2001 Jul;35(1):134–46. DOI: https://doi.org/10.1016/S0168-8278(01)00092-7
5. Chappell LC, Bell JL, Smith A, Linsell L, Juszczak E, Dixon PH, et al. Ursodeoxycholic acid versus placebo in women with intrahepatic cholestasis of pregnancy (PITCHES): a randomised controlled trial. Lancet Lond Engl. 2019 Sep 7;394(10201):849–60.
6. Ovadia C, Seed PT, Sklavounos A, Geenes V, Di Ilio C, Chambers J, et al. Association of adverse perinatal outcomes of intrahepatic cholestasis of pregnancy with biochemical markers: results of aggregate and individual patient data meta-analyses. The Lancet. 2019 Mar;393(10174):899–909.
7. Gonzalez MC, Reyes H, Arrese M, Figueroa D, Lorca B, Andresen M, et al. Intrahepatic cholestasis of pregnancy in twin pregnancies. J Hepatol. 1989 Jul;9(1):84–90. DOI: https://doi.org/10.1016/0168-8278(89)90079-2
8. Wånggren K, Sparre LS, Wramsby H. Severe jaundice in early IVF pregnancy. Eur J Obstet Gynecol Reprod Biol. 2004 Feb 10;112(2):228–9. DOI: https://doi.org/10.1016/S0301-2115(03)00339-7
9. Mutlu MF, Aslan K, Guler I, Mutlu I, Erdem M, Bozkurt N, et al. Two cases of first onset intrahepatic cholestasis of pregnancy associated with moderate ovarian hyperstimulation syndrome after IVF treatment and review of the literature. J Obstet Gynaecol J Inst Obstet Gynaecol. 2017 Jul;37(5):547–9. DOI: https://doi.org/10.1080/01443615.2017.1286302
10. Andersen BR, Bergholt T, Erdberg P, Hvidman L, Sharif H, Weber T. DSOG Guideline: Leverbetinget graviditetskløe Intrahepatic cholestasis of Pregnancy; ICP [Internet]. 2022. Available from: https://static1.squarespace.com/static/5467abcce4b056d72594db79/t/633f32954490d22306debc35/1665086103359/Cholestase-guideline+-+2022.pdf
11. Geenes V, Williamson C. Intrahepatic cholestasis of pregnancy. World J Gastroenterol. 2009 May 7;15(17):2049–66. DOI: https://doi.org/10.3748/wjg.15.2049
12. Reyes H, Gonzalez MC, Ribalta J, Aburto H, Matus C, Schramm G, et al. Prevalence of intrahepatic cholestasis of pregnancy in Chile. Ann Intern Med. 1978 Apr;88(4):487–93. DOI: https://doi.org/10.7326/0003-4819-88-4-487
13. Wikström Shemer E, Marschall H. Decreased 1,25‐dihydroxy vitamin D levels in women with intrahepatic cholestasis of pregnancy. Acta Obstet Gynecol Scand. 2010 Nov;89(11):1420–3. DOI: https://doi.org/10.3109/00016349.2010.515665
14. Perez MJ, Macias RIR, Marin JJG. Maternal cholestasis induces placental oxidative stress and apoptosis. Protective effect of ursodeoxycholic acid. Placenta. 2006 Jan;27(1):34–41. DOI: https://doi.org/10.1016/j.placenta.2004.10.020
15. Williamson C, Gorelik J, Eaton BM, Lab M, de Swiet M, Korchev Y. The bile acid taurocholate impairs rat cardiomyocyte function: a proposed mechanism for intra-uterine fetal death in obstetric cholestasis. Clin Sci Lond Engl 1979. 2001 Apr;100(4):363–9. DOI: https://doi.org/10.1042/cs1000363
16. Zhang L, Ding D, Yu L, Qi H, Han C, Jiang J, et al. Primary biliary cirrhosis associated with myasthenia gravis after postpartum: a case report. J Med Case Reports. 2021 Dec;15(1):498. DOI: https://doi.org/10.1186/s13256-021-03092-x
17. Pope C, Karanth S, Liu J. Pharmacology and toxicology of cholinesterase inhibitors: uses and misuses of a common mechanism of action. Environ Toxicol Pharmacol. 2005 May;19(3):433–46. DOI: https://doi.org/10.1016/j.etap.2004.12.048
18. Hubschmann AG, Orzechowski KM, Berghella V. Severe First Trimester Recurrent Intrahepatic Cholestasis of Pregnancy: A Case Report and Literature Review. AJP Rep. 2016 Mar;6(1):e38-41. DOI: https://doi.org/10.1055/s-0035-1565922
19. He J, Chen L, Liang C. [Clinical analysis of fetal death cases in intrahepatic cholestasis of pregnancy]. Zhonghua Fu Chan Ke Za Zhi. 2011 May;46(5):333–7.
20. Williamson C, Hems LM, Goulis DG, Walker I, Chambers J, Donaldson O, et al. Clinical outcome in a series of cases of obstetric cholestasis identified via a patient support group. BJOG Int J Obstet Gynaecol. 2004 Jul;111(7):676–81. DOI: https://doi.org/10.1111/j.1471-0528.2004.00167.x