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Clinical features of hemolysis, elevated liver enzymes, and low platelet count syndrome in undiagnosed Wilson disease: report of two cases.
Arch Gynecol Obstet. 2010 Jan; 281(1):129-34.AG

Abstract

INTRODUCTION

Wilson's disease (WD) is an autosomal recessive disorder characterized by toxic accumulation of copper mainly in the liver and brain. The hepatic manifestation of WD is diverse and may include asymptomatic elevation of aminotransferase, chronic hepatitis, cirrhosis, or acute/fulminant hepatic failure. Characteristic of acute hepatic failure in WD is concomitance of acute intravascular hemolytic anemia that in some patients may represent a first clinical symptom of WD. The diagnosis of acute Wilsonian liver failure is difficult, as similar signs may be observed in other clinical conditions. In pregnant patients with unrecognized WD, liver failure with hemolysis may be interpreted as the low platelet count (HELLP) syndrome.

PATIENTS

We describe two women, who developed the clinical features of hemolysis, elevated liver enzymes, and HELLP syndrome. In both, further diagnostics confirmed WD.

CONCLUSION

WD should be remembered in the differential diagnostics of HELLP syndrome.

Authors+Show Affiliations

Second Department of Neurology, Institute of Psychiatry and Neurology, Sobieskiego 9, Warsaw, Poland. czlonkow@ipin.edu.plNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

19381668

Citation

Członkowska, Anna, et al. "Clinical Features of Hemolysis, Elevated Liver Enzymes, and Low Platelet Count Syndrome in Undiagnosed Wilson Disease: Report of Two Cases." Archives of Gynecology and Obstetrics, vol. 281, no. 1, 2010, pp. 129-34.
Członkowska A, Gromadzka G, Büttner J, et al. Clinical features of hemolysis, elevated liver enzymes, and low platelet count syndrome in undiagnosed Wilson disease: report of two cases. Arch Gynecol Obstet. 2010;281(1):129-34.
Członkowska, A., Gromadzka, G., Büttner, J., & Chabik, G. (2010). Clinical features of hemolysis, elevated liver enzymes, and low platelet count syndrome in undiagnosed Wilson disease: report of two cases. Archives of Gynecology and Obstetrics, 281(1), 129-34. https://doi.org/10.1007/s00404-009-1080-6
Członkowska A, et al. Clinical Features of Hemolysis, Elevated Liver Enzymes, and Low Platelet Count Syndrome in Undiagnosed Wilson Disease: Report of Two Cases. Arch Gynecol Obstet. 2010;281(1):129-34. PubMed PMID: 19381668.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Clinical features of hemolysis, elevated liver enzymes, and low platelet count syndrome in undiagnosed Wilson disease: report of two cases. AU - Członkowska,Anna, AU - Gromadzka,Grażyna, AU - Büttner,Janine, AU - Chabik,Grzegorz, Y1 - 2009/04/19/ PY - 2009/01/13/received PY - 2009/03/26/accepted PY - 2009/4/22/entrez PY - 2009/4/22/pubmed PY - 2011/12/13/medline SP - 129 EP - 34 JF - Archives of gynecology and obstetrics JO - Arch. Gynecol. Obstet. VL - 281 IS - 1 N2 - INTRODUCTION: Wilson's disease (WD) is an autosomal recessive disorder characterized by toxic accumulation of copper mainly in the liver and brain. The hepatic manifestation of WD is diverse and may include asymptomatic elevation of aminotransferase, chronic hepatitis, cirrhosis, or acute/fulminant hepatic failure. Characteristic of acute hepatic failure in WD is concomitance of acute intravascular hemolytic anemia that in some patients may represent a first clinical symptom of WD. The diagnosis of acute Wilsonian liver failure is difficult, as similar signs may be observed in other clinical conditions. In pregnant patients with unrecognized WD, liver failure with hemolysis may be interpreted as the low platelet count (HELLP) syndrome. PATIENTS: We describe two women, who developed the clinical features of hemolysis, elevated liver enzymes, and HELLP syndrome. In both, further diagnostics confirmed WD. CONCLUSION: WD should be remembered in the differential diagnostics of HELLP syndrome. SN - 1432-0711 UR - https://www.unboundmedicine.com/medline/citation/19381668/Clinical_features_of_hemolysis_elevated_liver_enzymes_and_low_platelet_count_syndrome_in_undiagnosed_Wilson_disease:_report_of_two_cases_ L2 - https://dx.doi.org/10.1007/s00404-009-1080-6 DB - PRIME DP - Unbound Medicine ER -