Skip to main content Skip to main navigation menu Skip to site footer

Gastrointestinal bleeding as a life-threatening complication of liver abnormality in a Turner syndrome patient

  • Soffy Enggar ,
  • Budi Widodo ,

Abstract

Background: Turner syndrome is a rare condition with high morbidity and mortality rates, it leads to multiple organ damage when left untreated. Meanwhile, liver involvement in this condition ranges from asymptomatic to severe such as cirrhosis with massive gastrointestinal bleeding. This study presents a case of gastrointestinal bleeding due to cirrhosis in Turner syndrome where the patient showed third-grade esophageal varices as a sign of portal hypertension.

Case Presentation: A 26-years old female came to the Emergency Department of Dr. Soetomo General Hospital in Surabaya with massive gastrointestinal bleeding. The patient was a Turner syndrome, diagnosed ten years ago. The patient was negative for hepatitis B surface antigen (HbsAg) and anti-hepatitis C virus (anti-HCV). The gastric lavage was performed to reduce active bleeding with blood transfusion. The upper gastrointestinal endoscopy indicated portal hypertension with third-grade esophageal varices, fibro scan indicated cirrhosis hepatic and abdominal CT-scan showed early cirrhosis and splenomegaly supporting the presence of portal hypertension. Since the patient refused to endoscopic varices ligation, hematemesis repeated three times after the first visit resulting death of the patient.

Conclusion: This is a Turner syndrome patient who experienced life-threatening gastrointestinal bleeding as complication of liver abnormality. This case highlights that liver cirrhosis should be evaluated in individuals with Turner syndrome to prevent such complications.

References

  1. Gravholt CH, Viuff MH, Brun S, Stochholm K, Andersen NH. Turner syndrome: mechanisms and management. Nat Rev Endocrinol. 2019;15(10):601–14. Available from: http://dx.doi.org/10.1038/s41574-019-0224-4
  2. Macdonald G. Harrison’s Internal Medicine, 17th edition. - by A. S. Fauci, D. L. Kasper, D. L. Longo, E. Braunwald, S. L. Hauser, J. L. Jameson and J. Loscalzo. Intern Med J. 2008;38(12):932. Available from: http://dx.doi.org/10.1111/j.1445-5994.2008.01837.x
  3. Kawabata S, Sakamoto S, Honda M, Hayashida S, Yamamoto H, Mikami Y, et al. Liver transplantation for a patient with Turner syndrome presenting severe portal hypertension: a case report and literature review. Surg case reports. 2016/06/29. 2016;2(1):68. Available from: https://pubmed.ncbi.nlm.nih.gov/27358061
  4. Sylvén L, Hagenfeldt K, Bröndum-Nielsen K, von Schoultz B. Middle-aged women with Turner’s syndrome. Medical status, hormonal treatment and social life. Acta Endocrinol (Copenh). 1991;125(4):359–65. Available from: http://dx.doi.org/10.1530/acta.0.1250359
  5. Roulot D, Degott C, Chazouillères O, Oberti F, Calès P, Carbonell N, et al. Vascular involvement of the liver in Turner’s syndrome. Hepatology. 2004;39(1):239–47. Available from: http://dx.doi.org/10.1002/hep.20026
  6. Gravholt CH, Juul S, Naeraa RW, Hansen J. Morbidity in Turner Syndrome. J Clin Epidemiol. 1998;51(2):147–58. Available from: http://dx.doi.org/10.1016/s0895-4356(97)00237-0
  7. Boregowda U, Umapathy C, Halim N, Desai M, Nanjappa A, Arekapudi S, et al. Update on the management of gastrointestinal varices. World J Gastrointest Pharmacol Ther. 2019;10(1):1–21. Available from: https://pubmed.ncbi.nlm.nih.gov/30697445
  8. Roulot D. Liver involvement in Turner syndrome. Liver Int. 2012;33(1):24–30. Available from: http://dx.doi.org/10.1111/liv.12007
  9. El‐Mansoury M, Berntorp K, Bryman I, Hanson C, Innala E, Karlsson A, et al. Elevated liver enzymes in Turner syndrome during a 5‐year follow‐up study. Clin Endocrinol (Oxf). 2007;68(3):485–90. Available from: http://dx.doi.org/10.1111/j.1365-2265.2007.03166.x
  10. Koulouri O, Ostberg J, Conway GS. Liver dysfunction in Turner’s syndrome: prevalence, natural history and effect of exogenous oestrogen. Clin Endocrinol (Oxf). 2008;69(2):306–10. Available from: http://dx.doi.org/10.1111/j.1365-2265.2008.03203.x
  11. Larizza D, Locatelli M, Vitali L, Viganò C, Calcaterra V, Tinelli C, et al. Serum liver enzymes in Turner syndrome. Eur J Pediatr. 2000;159(3):143–8. Available from: http://dx.doi.org/10.1007/s004310050038
  12. Roulot D, Valla D. Hepatic disease in Turner syndrome. Int Congr Ser. 2006;1298:146–51. Available from: http://dx.doi.org/10.1016/j.ics.2006.06.006
  13. Gravholt CH, Poulsen HE, Ott P, Christiansen JS, Vilstrup H. Quantitative liver functions in Turner syndrome with and without hormone replacement therapy. Eur J Endocrinol. 2007;156(6):679–86. Available from: http://dx.doi.org/10.1530/eje-07-0070
  14. Elsheikh M, Hodgson HJF, Wass JAH, Conway GS. Hormone replacement therapy may improve hepatic function in women with Turner’s syndrome. Clin Endocrinol (Oxf). 2001;55(2):227–31. Available from: http://dx.doi.org/10.1046/j.1365-2265.2001.01321.x
  15. Mariadi IK, Sudjana K, Wibawa IDN. High c-reactive protein level as risk factors of complications in upper gastrointestinal bleeding. Bali Med J. 2020;9(3):698-701. Available from: http://dx.doi.org/10.15562/bmj.v9i3.2015

How to Cite

Enggar, S., & Widodo, B. (2022). Gastrointestinal bleeding as a life-threatening complication of liver abnormality in a Turner syndrome patient. Bali Medical Journal, 11(1), 246–249. https://doi.org/10.15562/bmj.v11i1.3259

HTML
0

Total
0

Share

Search Panel