An Unusual Occurrence of Hepatic Granulomas and Secondary Sitosterolemia in Turner Syndrome

Author:

Chintanaboina JayaKrishna1ORCID,Shah Pragnesh R.2,Riley Thomas R.3

Affiliation:

1. Penn State Hershey Medical Center, Penn State College of Medicine, Division of General Internal Medicine, 500 University Drive, Hershey, PA 17033, USA

2. Memorial Hermann Sugar Land Hospital, 17500 West Grand Parkway, Sugar Land, TX 77479, USA

3. Penn State Hershey Medical Center, Penn State College of Medicine, Division of Gastroenterology and Hepatology, 500 University Drive, Hershey, PA 17033, USA

Abstract

Although abnormal liver function tests occur in 50–80% of cases with Turner syndrome, there are no previous reports of overt hepatic disease or hepatic granulomas associated with Turner’s syndrome. We report three cases of Turner syndrome associated with hepatic granulomas with a wide range of liver dysfunction. Of the three patients, first patient underwent liver transplantation; second patient remained stable on immunosuppressants; and third patient died from complications of decompensated liver cirrhosis as she declined liver transplantation due to multiple comorbidities. One patient had sitosterolemia, a rare inherited autosomal recessive disorder of cholesterol metabolism, after she ingestedβ-sitosterol supplement and had worsening liver function tests and lipid panel. She had remarkably abnormal lipid panel that responded to ezetimibe and by stopping theβ-sitosterol supplement.

Publisher

Hindawi Limited

Subject

General Medicine

Cited by 8 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. Turner syndrome with isochromosome Xq as a cause of granulomatous hepatitis: a case report;Annals of Medicine & Surgery;2024-04-15

2. Liver in Systemic Disease;MacSween's Pathology of the Liver;2024

3. Síndrome de Turner como causa de cirrosis hepática;Gastroenterología y Hepatología;2018-05

4. Turner syndrome as a cause of liver cirrhosis;Gastroenterología y Hepatología (English Edition);2018-05

5. Heart Transplantation in Children with Turner Syndrome: Analysis of a Linked Dataset;Pediatric Cardiology;2018-01-03

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