Morphology of transplanted liver in recurrent progressive familial intrahepatic cholestasis type 2
Progressive familial intrahepatic cholestasis type 2 (PFIC-2), formerly known as Byler’s syndrome, is an autosomal recessive disorder. In infancy or early childhood, this disease leads to end-stage hepatic disease, in which liver transplantation is the only radical treatment. In general, liver trans...
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| Language: | Russian |
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Federal Research Center of Transplantology and Artificial Organs named after V.I.Shumakov
2021-01-01
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| Series: | Вестник трансплантологии и искусственных органов |
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| Online Access: | https://journal.transpl.ru/vtio/article/view/1283 |
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| author | I. M. Iljinsky N. P. Mozheiko O. M. Tsirulnikova |
| author_facet | I. M. Iljinsky N. P. Mozheiko O. M. Tsirulnikova |
| author_sort | I. M. Iljinsky |
| collection | DOAJ |
| description | Progressive familial intrahepatic cholestasis type 2 (PFIC-2), formerly known as Byler’s syndrome, is an autosomal recessive disorder. In infancy or early childhood, this disease leads to end-stage hepatic disease, in which liver transplantation is the only radical treatment. In general, liver transplantation outcomes are good, but in the long term, PFIC-2 may reoccur. We present a case where a girl, aged 28 months, who suffered from cirrhosis resulting from PFIC-2, underwent a related transplantation of the left lateral sector of the liver (her grandmother as the donor). Punch biopsy was performed 8 years after the liver transplant due to graft dysfunction. Histopathology revealed a recurrent PFIC-2. F4. Increased liver failure was the reason for retransplantation of the left lobe of the liver also from a related donor (mother). Pathological pictures in the biopsy specimen and in the liver removed during retransplantation were identical, which once again confirmed PFIC-2 recurrence. |
| format | Article |
| id | doaj-art-b52e4faa406d4507bf06f1d057c111b0 |
| institution | DOAJ |
| issn | 1995-1191 |
| language | Russian |
| publishDate | 2021-01-01 |
| publisher | Federal Research Center of Transplantology and Artificial Organs named after V.I.Shumakov |
| record_format | Article |
| series | Вестник трансплантологии и искусственных органов |
| spelling | doaj-art-b52e4faa406d4507bf06f1d057c111b02025-08-20T03:01:38ZrusFederal Research Center of Transplantology and Artificial Organs named after V.I.ShumakovВестник трансплантологии и искусственных органов1995-11912021-01-0122419219610.15825/1995-1191-2020-4-192-196937Morphology of transplanted liver in recurrent progressive familial intrahepatic cholestasis type 2I. M. Iljinsky0N. P. Mozheiko1O. M. Tsirulnikova2Shumakov National Medical Research Center of Transplantology and Artificial OrgansShumakov National Medical Research Center of Transplantology and Artificial OrgansShumakov National Medical Research Center of Transplantology and Artificial Organs; Sechenov UniversityProgressive familial intrahepatic cholestasis type 2 (PFIC-2), formerly known as Byler’s syndrome, is an autosomal recessive disorder. In infancy or early childhood, this disease leads to end-stage hepatic disease, in which liver transplantation is the only radical treatment. In general, liver transplantation outcomes are good, but in the long term, PFIC-2 may reoccur. We present a case where a girl, aged 28 months, who suffered from cirrhosis resulting from PFIC-2, underwent a related transplantation of the left lateral sector of the liver (her grandmother as the donor). Punch biopsy was performed 8 years after the liver transplant due to graft dysfunction. Histopathology revealed a recurrent PFIC-2. F4. Increased liver failure was the reason for retransplantation of the left lobe of the liver also from a related donor (mother). Pathological pictures in the biopsy specimen and in the liver removed during retransplantation were identical, which once again confirmed PFIC-2 recurrence.https://journal.transpl.ru/vtio/article/view/1283recurrent progressive familial intrahepatic cholestasispfic-2hepatocyte dystrophymultinucleated cells |
| spellingShingle | I. M. Iljinsky N. P. Mozheiko O. M. Tsirulnikova Morphology of transplanted liver in recurrent progressive familial intrahepatic cholestasis type 2 Вестник трансплантологии и искусственных органов recurrent progressive familial intrahepatic cholestasis pfic-2 hepatocyte dystrophy multinucleated cells |
| title | Morphology of transplanted liver in recurrent progressive familial intrahepatic cholestasis type 2 |
| title_full | Morphology of transplanted liver in recurrent progressive familial intrahepatic cholestasis type 2 |
| title_fullStr | Morphology of transplanted liver in recurrent progressive familial intrahepatic cholestasis type 2 |
| title_full_unstemmed | Morphology of transplanted liver in recurrent progressive familial intrahepatic cholestasis type 2 |
| title_short | Morphology of transplanted liver in recurrent progressive familial intrahepatic cholestasis type 2 |
| title_sort | morphology of transplanted liver in recurrent progressive familial intrahepatic cholestasis type 2 |
| topic | recurrent progressive familial intrahepatic cholestasis pfic-2 hepatocyte dystrophy multinucleated cells |
| url | https://journal.transpl.ru/vtio/article/view/1283 |
| work_keys_str_mv | AT imiljinsky morphologyoftransplantedliverinrecurrentprogressivefamilialintrahepaticcholestasistype2 AT npmozheiko morphologyoftransplantedliverinrecurrentprogressivefamilialintrahepaticcholestasistype2 AT omtsirulnikova morphologyoftransplantedliverinrecurrentprogressivefamilialintrahepaticcholestasistype2 |