17:00 Uhr
Mikrobiomanalysen bei gastrointestinalen Erkrankungen: nur Hype oder hilfreich?
C. Sokollik (Bern, CH)
18:20 Uhr
DGKJ-FV 06:
Severe acute non-A-E hepatitis (NAEH) of unknown origin: A 13-year single-center retrospective analysis in children
S. Tsaka (Essen, DE)
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Autor:innen:
S. Tsaka (Essen, DE)
S. Kathemann (Essen, DE)
D. Pilic (Essen, DE)
B. Prusinskas (Essen, DE)
H. Baba (Essen, DE)
E. Lainka (Essen, DE)
Introduction: Pediatric acute liver failure (PALF) is a rare, but potentially life-threating condition. 30-50% of PALF remains without detectable cause,especially in children under 4 years even up to 60%. NAEH(known as indeterminate, non-A-non-E hepatitis) refers to hepatitis of unknown origin. Many of the pediatric patients with NAEH develop bone marrow hypoplasia-aplasia.Material-methods:We retrospectively collected all pediatric patients with presentation of severe acute NAEH between 2009-2022. The patients enrolled in the study met the following criteria: 1) age 3 months-18 years old, 2) no prior evidence of chronic liver disease, 3) GOT and GPT >500 IU/ml, 4) acute hepatitis causes such as viral-bacterial infections, drugs, metabolic diseases, toxins, ischemia and rare miscellaneous causes have been excluded. Results:A total of 39 children with severe acute NAEH were included in our study. Median age was 6.5 years (range 0,8-17). In 17 (44%) patients a potentially triggering unspecific viral respiratory or gastrointestinal infection was found.20/39 (51%) patients had developed by the time of the presentation a PALF, whereas the rest of the patients with an acute hepatitis did not fulfill the criteria for PALF. 8/20 (40%) patients with PALF underwent urgent liver transplantation. 27/39 (69%) patients with NAEH presented with icterus. Additional symptoms included fatigue, weight loss, abdominal pain, and emesis. In all patients, initial laboratory results showed remarkably high transaminases and relatively low GGT.12/39 (30%) children developed an hepatitis associated aplastic anemia (HAAA) and received later bone marrow transplantation or other therapy. Patients who later developed HAAA had a slight low count of lymphocytes (mean lymphocyte-count 1100/μl) upon presentation and over time they developed a severe lymphopenia (minimal mean lymphocyte-count 340\μl) in contrast to the other ones that had a normal lymphocytes count. 1/12 patients with HAAA underwent a liver transplantation, 9/12 received steroid treatment, and 2/12 received neither steroids nor liver transplantation. In comparison to patients who received steroid treatment, the 2 patients who were not treated with steroids showed a similar hepatic recovery (mean 12 weeks). 10/27 patients with NAEH (included the NAEH with PALF) without HAAA were also treated with steroids. 7/27 patients underwent liver transplantation, and 10/27 patients received neither steroids nor liver transplantation. 10/27 patients treated with steroids showed a hepatic recovery in 9.3 weeks whereas the others without steroid treatment nor liver transplantation showed a recovery in 5.4 weeks, leading to the conclusion that the effect of steroid treatment remains uncertain.Conclusion:Severe acute NAEH makes up a big proportion of fulminant hepatitis and ALF in children. Furthermore, HAAA could develop 0-3 months after NAEH. Low lymphocytes could be an early marker for HAAA.