An Unusual Occurrence of Hepatic Granulomas and Secondary Sitosterolemia in Turner Syndrome
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...
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| Format: | Article |
| Language: | English |
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Wiley
2015-01-01
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| Series: | Case Reports in Medicine |
| Online Access: | http://dx.doi.org/10.1155/2015/186718 |
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| author | JayaKrishna Chintanaboina Pragnesh R. Shah Thomas R. Riley |
| author_facet | JayaKrishna Chintanaboina Pragnesh R. Shah Thomas R. Riley |
| author_sort | JayaKrishna Chintanaboina |
| collection | DOAJ |
| description | 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 ingested β-sitosterol supplement and had worsening liver function tests and lipid panel. She had remarkably abnormal lipid panel that responded to ezetimibe and by stopping the β-sitosterol supplement. |
| format | Article |
| id | doaj-art-c1573b2c28c04ca3b35d3355a40468bf |
| institution | Kabale University |
| issn | 1687-9627 1687-9635 |
| language | English |
| publishDate | 2015-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Medicine |
| spelling | doaj-art-c1573b2c28c04ca3b35d3355a40468bf2025-02-03T06:01:17ZengWileyCase Reports in Medicine1687-96271687-96352015-01-01201510.1155/2015/186718186718An Unusual Occurrence of Hepatic Granulomas and Secondary Sitosterolemia in Turner SyndromeJayaKrishna Chintanaboina0Pragnesh R. Shah1Thomas R. Riley2Penn State Hershey Medical Center, Penn State College of Medicine, Division of General Internal Medicine, 500 University Drive, Hershey, PA 17033, USAMemorial Hermann Sugar Land Hospital, 17500 West Grand Parkway, Sugar Land, TX 77479, USAPenn State Hershey Medical Center, Penn State College of Medicine, Division of Gastroenterology and Hepatology, 500 University Drive, Hershey, PA 17033, USAAlthough 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 ingested β-sitosterol supplement and had worsening liver function tests and lipid panel. She had remarkably abnormal lipid panel that responded to ezetimibe and by stopping the β-sitosterol supplement.http://dx.doi.org/10.1155/2015/186718 |
| spellingShingle | JayaKrishna Chintanaboina Pragnesh R. Shah Thomas R. Riley An Unusual Occurrence of Hepatic Granulomas and Secondary Sitosterolemia in Turner Syndrome Case Reports in Medicine |
| title | An Unusual Occurrence of Hepatic Granulomas and Secondary Sitosterolemia in Turner Syndrome |
| title_full | An Unusual Occurrence of Hepatic Granulomas and Secondary Sitosterolemia in Turner Syndrome |
| title_fullStr | An Unusual Occurrence of Hepatic Granulomas and Secondary Sitosterolemia in Turner Syndrome |
| title_full_unstemmed | An Unusual Occurrence of Hepatic Granulomas and Secondary Sitosterolemia in Turner Syndrome |
| title_short | An Unusual Occurrence of Hepatic Granulomas and Secondary Sitosterolemia in Turner Syndrome |
| title_sort | unusual occurrence of hepatic granulomas and secondary sitosterolemia in turner syndrome |
| url | http://dx.doi.org/10.1155/2015/186718 |
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