The utility of liver transplantation to treat acute liver failure caused by adult-onset Still's disease : case reports
PURPOSE: Adult-onset Still's disease (AOSD) is an inflammatory condition commonly complicated by mild liver dysfunction. However, severe liver failure is rarely reported. We report three cases of severe acute hepatic failure (ALF) associated with AOSD. We encountered three cases of acute liver failure (ALF) with encephalopathy.
RESULTS: Case 1 was a 75-year-old female, who was started on a steroid (prednisolone, PSL) to treat AOSD; this was gradually tapered. Two months later, severe ALF developed. She died despite an increase in the PSL dose and artificial liver support. Case 2 was a 26-year-old-female taking PSL 30 mg/day to treat subacute thyroiditis. PSL was tapered, and she received methyl PSL pulse therapy and artificial liver support, but this did not cure the ALF. Liver transplantation (LT) was performed 25 days later. Three years later, the same symptoms were observed and we diagnosed AOSD. Case 3 was a 56-year-old-female who met the AOSD criteria. PSL 50 mg/day was started and then tapered. Methyl PSL pulse therapy was prescribed to treat hemophagocytic syndrome, but she required LT on hospital day 13.
CONCLUSION: In AOSD cases, ALF is rarely complicated; urgent LT should be considered only for patients with AOSD-related severe ALF.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2021 |
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| Erschienen: |
2021 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:14 |
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| Enthalten in: |
Clinical journal of gastroenterology - 14(2021), 3 vom: 01. Juni, Seite 866-875 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Ogasawara, Yuri [VerfasserIn] |
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| Links: |
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| Themen: |
9PHQ9Y1OLM |
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| Anmerkungen: |
Date Completed 25.06.2021 Date Revised 25.06.2021 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1007/s12328-021-01398-3 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM323525415 |
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| 245 | 1 | 4 | |a The utility of liver transplantation to treat acute liver failure caused by adult-onset Still's disease |b case reports |
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| 500 | |a Citation Status MEDLINE | ||
| 520 | |a PURPOSE: Adult-onset Still's disease (AOSD) is an inflammatory condition commonly complicated by mild liver dysfunction. However, severe liver failure is rarely reported. We report three cases of severe acute hepatic failure (ALF) associated with AOSD. We encountered three cases of acute liver failure (ALF) with encephalopathy | ||
| 520 | |a RESULTS: Case 1 was a 75-year-old female, who was started on a steroid (prednisolone, PSL) to treat AOSD; this was gradually tapered. Two months later, severe ALF developed. She died despite an increase in the PSL dose and artificial liver support. Case 2 was a 26-year-old-female taking PSL 30 mg/day to treat subacute thyroiditis. PSL was tapered, and she received methyl PSL pulse therapy and artificial liver support, but this did not cure the ALF. Liver transplantation (LT) was performed 25 days later. Three years later, the same symptoms were observed and we diagnosed AOSD. Case 3 was a 56-year-old-female who met the AOSD criteria. PSL 50 mg/day was started and then tapered. Methyl PSL pulse therapy was prescribed to treat hemophagocytic syndrome, but she required LT on hospital day 13 | ||
| 520 | |a CONCLUSION: In AOSD cases, ALF is rarely complicated; urgent LT should be considered only for patients with AOSD-related severe ALF | ||
| 650 | 4 | |a Case Reports | |
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Acute liver failure (ALF) | |
| 650 | 4 | |a Adult-onset Still’s disease (AOSD) | |
| 650 | 4 | |a Liver transplantation (LT) | |
| 650 | 7 | |a Glucocorticoids |2 NLM | |
| 650 | 7 | |a Prednisolone |2 NLM | |
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| 700 | 1 | |a Omori, Akiko |e verfasserin |4 aut | |
| 700 | 1 | |a Konda, Naoko |e verfasserin |4 aut | |
| 700 | 1 | |a Sugano, Eri |e verfasserin |4 aut | |
| 700 | 1 | |a Sagawa, Takaomi |e verfasserin |4 aut | |
| 700 | 1 | |a Taniai, Makiko |e verfasserin |4 aut | |
| 700 | 1 | |a Harigai, Masayoshi |e verfasserin |4 aut | |
| 700 | 1 | |a Egawa, Hiroto |e verfasserin |4 aut | |
| 700 | 1 | |a Yamamoto, Masakazu |e verfasserin |4 aut | |
| 700 | 1 | |a Tokushige, Katsutoshi |e verfasserin |4 aut | |
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