Liver Transplantation in Progressive Familial Intrahepatic Cholestasis with Normal Gamma-Glutamyl Transferase: Evaluation of Post-transplant Steatosis and Steatohepatitis
| Author | Mohammad Hossein Anbardar | en |
| Author | Seyed Mohsen Dehghani | en |
| Author | Maryam Poostkar | en |
| Author | Seyed Ali Malek-Hosseini | en |
| Orcid | Mohammad Hossein Anbardar [0000-0002-2317-0737] | en |
| Orcid | Seyed Mohsen Dehghani [0000-0001-5930-0110] | en |
| Issued Date | 2021-10-31 | en |
| Abstract | Background: Progressive familial intrahepatic cholestasis is a disease presenting with severe cholestasis and progressing to the end-stage liver disease later. Liver transplantation is a treatment modality available for progressive familial intrahepatic cholestasis, especially in patients with end-stage liver disease or those who are unsuitable for or have failed biliary diversion. Objectives: To evaluate clinical and pathological characteristics of progressive familial intrahepatic cholestasis patients who had undergone liver transplantation and to determine post-transplant steatosis and steatohepatitis. Methods: We evaluated 111 progressive familial intrahepatic cholestasis patients with normal gamma-glutamyl transferase that performed liver transplantation in Shiraz Transplant Center in Iran between March 2000 and March 2017. Results: The most common clinical manifestations were jaundice and pruritus. Growth retardation and diarrhea were detected in 76.6% and 42.5% of the patients. After transplantation, growth retardation was seen in 31.5% of the patients, and diarrhea in 36.9% of them. Besides, 29.1% of the patients died post-transplant. Post-transplant liver biopsies were taken from 50 patients, and 15 (30%) patients had steatosis or steatohepatitis, five of whom (10%) had macrovesicular steatosis alone, and 10 (20%) had steatohepatitis. Only one patient showed moderate bridging fibrosis (stage III), and none of them showed severe fibrosis. Conclusions: Liver transplantation is the final treatment option for these patients, and it can relieve most clinical manifestations. However, post-transplant mortality rate was relatively high in our center. Diarrhea, growth retardation, and steatosis are unique post-transplant complications in these patients. The rate of post-transplant steatosis and steatohepatitis in patients with liver biopsy in our study was 30%, with a significant difference from previous studies. | en |
| DOI | https://doi.org/10.5812/ijp.117380 | en |
| Keyword | Intrahepatic Cholestasis | en |
| Keyword | Liver | en |
| Keyword | Transplantation | en |
| Keyword | Steatosis | en |
| Keyword | Steatohepatitis | en |
| Keyword | Diarrhea | en |
| Keyword | Growth Retardation | en |
| Keyword | Normal Gamma-Glutamyl Transferase | en |
| Publisher | Brieflands | en |
| Title | Liver Transplantation in Progressive Familial Intrahepatic Cholestasis with Normal Gamma-Glutamyl Transferase: Evaluation of Post-transplant Steatosis and Steatohepatitis | en |
| Type | Research Article | en |
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