scholarly journals Characteristics and Long-term Outcome of 535 Patients with Histologically Confirmed Autoimmune Hepatitis (AIH) - A Single Center Experience of 18 Years

2021 ◽  
Author(s):  
Matthias Buechter ◽  
Johannes Kessener ◽  
Birte Möhlendick ◽  
Hideo A. Baba ◽  
Guido Gerken ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Autoimmune Hepatitis ◽  
Smooth Muscle Actin ◽  
Primary Biliary Cholangitis ◽  
Type I ◽  
Long Term Outcome ◽  
Number Of Patients ◽  
Almost All ◽  
Long Term Experience

Abstract Introduction: Autoimmune hepatitis (AIH) is a rare liver disease with a favorable prognosis following immunosuppression. In this work-up, we present our long-term experience with this patient collective. Patients and Methods: Overall, we were able to retrospectively evaluate data from 535 patients with histologically confirmed AIH over a period of 18 years between 2002 and 2020. Results: As expected, almost ¾ of the patients were women (74.5 %) of middle age (47.0 ± 14.9 years). Type I AIH was diagnosed in almost all patients (97.5 %). However, 88 patients (16.4 %) revealed an overlap to primary biliary cholangitis (PBC). In contrast, overlap to primary sclerosing cholangitis (PSC) was detected in only 22 patients (3.7 %). Regarding auto-antibody profile, positivity for anti-nuclear antibodies (ANA) was found in 388 patients (72.5 %); smooth-muscle actin (SMA)-titer was positive in 90 patients (16.8 %), anti-mitochondrial antibodies (AMA) were detected in 43 individuals (8.0 %), and we found positive p-anti-neutrophil cytoplasmic antibodies (p-ANCA) in 12 patients (2.2 %). More than ¾ of the patients (n = 417 ≈ 77.9 %) were initially treated with corticosteroids (1 mg/kg/day) and this therapy was continued in almost all subjects (97.4 %) in low dose (5 - 7.5 mg/day) as maintenance therapy. Steroid-saving and remission-maintaining therapy with azathioprine was carried out in 380 patients (71.0 %). Mycophenolate mofetil (MMF) or calcineurin-inhibitors (CNI) were used as second- or third-line immunosuppression. Acute liver failure (ALF) - as the first manifestation of AIH - was diagnosed in 101 patients (18.8 %). Hepatocellular carcinoma (HCC) was detected in at least 6 patients (1.1 %). Liver transplantation (LT) was performed in 51 patients (9.5 %) who progressed to cirrhosis despite immunosuppression. Unfortunately, a total of 45 patients (8.4 %) died of cirrhosis-related complications (infected ascites, bleeding, encephalopathy) without chance for adequate organ offer.Conclusion: We here present our long-term experience with a significant number of patients diagnosed with AIH over a long observation period of 18 years. In general, patients with AIH can adequately be managed with good clinical outcome at a liver center requiring immunosuppressive therapy. However, HCC-screening, acute-on-chronic (AOC) liver decompensation, or liver transplantation have to be taken into consideration carefully.

Modifiable Factors Affecting Renal Preservation in Type I Glycogen Storage Disease After Liver Transplantation: A Single-center Propensity-match Cohort Study

2021 ◽  
Author(s):  
Yi-Chia Chan ◽  
Kai-Min Liu ◽  
Chao-Long Chen ◽  
Aldwin D. Ong ◽  
Chih-Che Lin ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Renal Function ◽  
Storage Disease ◽  
Glycogen Storage ◽  
Renal Outcome ◽  
Control Group ◽  
Type I ◽  
Long Term Outcome ◽  
Modifiable Factors

Abstract Background and aimsGlycogen storage disease type I (GSD-I) is an autosomal recessive disorder of carbohydrate metabolism, resulting in limited production of glucose and excessive glycogen storage in the liver and kidneys. These patients are characterized by life-threatening hypoglycemia, metabolic derangements, hepatomegaly, chronic kidney disease, and failure to thrive. Liver transplantation (LT) has been performed for poor metabolic control, delayed growth, and complications including liver cirrhosis or failure. However, renal outcome was diverse in pediatric GSD patients after LT. The aim of this study was to investigate the long-term outcome of renal function in pediatric GSD-I patients after living donor LT (LDLT), and to identify modifiable variables that potentially permits LT to confer native renal preservation.Methods The study included eight GSD-Ia and one GSD-Ib children with a median age of 9.0 (range 4.2-15.7) years at the time of LT. Using propensity score matching, 20 children with biliary atresia (BA) receiving LT were selected as the control group by matching for age, sex, pre-operative creatinine (Cr) and pediatric end-stage liver disease (PELD) score. Renal function was evaluated based on the serum Cr, estimated glomerular filtration rate (eGFR), microalbuminuria, and morphological changes in the kidneys. Comparability in long-term renal outcome in terms of anatomic and functional parameters will help to identify pre-LT factors of GSD-I that affect renal prognosis.Results The clinical and biochemical characteristics of the GSD and BA groups were similar, including immunosuppressive regimens and duration of follow-up (median 15 years) after LT. Overall, renal function, including eGFR and microalbuminuria was comparable in the GSD-I and BA groups (median eGFR: 111 vs. 123 ml/min/1.73m2, P=0.268; median urine microalbuminuria to creatinine ratio: 16.0 vs 7.2 mg/g, P=0.099, respectively) after LT. However, in the subgroups of the GSD cohort, patients starting cornstarch therapy at an older age (≥ 6-year-old) before transplantation demonstrated a worse renal outcome in terms of eGFR change over years (P<0.001). In addition, the enlarged kidney in GSD-I returned to within normal range after LT.Conclusions Post-LT renal function was well-preserved in most GSD-I patients. Early initiation of cornstarch therapy before preschool age, followed by LT, achieved a good renal prognosis.

scholarly journals Long term outcome of acquired food allergy in pediatric liver recipients: a single center experience

2012 ◽  
Vol 4 (1) ◽  
pp. 6 ◽  
Author(s):  
Antigoni Mavroudi ◽  
Ioannis Xinias ◽  
Aristidis Deligiannidis ◽  
Efthimia Parapanissiou ◽  
George Imvrios
Keyword(s):  
Liver Transplantation ◽  
Food Allergy ◽  
Food Allergies ◽  
Food Allergens ◽  
Long Term Outcome ◽  
The Third ◽  
Pediatric Liver ◽  
Number Of Patients ◽  
Long Term Prognosis

Food induced sensitization has been reported in pediatric liver recipients. However long term follow up has not been established so far. We report here our experience regarding 3 pediatric patients who developed acquired food allergy after liver transplantation. The first patient suffered from persistent diarrhea and eczema. The second one presented with abdominal pain with no signs of rejection, abdominal discomfort, vomiting when ingesting milk proteins and responded well to the elimination diet. The third patient presented with facial angioedema and hoarseness of voice. She had multiple food allergies and reacted to milk, egg and sesame. All the patients had elevated total Immunoglobulin E (<em>IgE</em>) and elevated specific <em>IgE</em> antibodies to the implicated food allergens. The first patient presented clinical manifestations of allergy when she was 19 months old. The second patient became allergic at the age of 16 and the third patient at the age of 3. The symptoms of food allergy persisted for 8 years in the first case and for 2 years in the other two cases. Low levels of specific <em>IgE</em> antibodies to the implicated food allergens and an enhanced T-helper 1 cell immune response toward interferon-gamma production were markers of tolerance acquisition. The long term prognosis in our cases was excellent. Food allergy resolved in all the patients. The long term prognosis of acquired food allergy after liver transplantation is currently obscure. More studies would be needed including greater number of patients to determine whether acquired food allergy is transient in pediatric liver recipients.

Long-term outcome of liver transplantation for autoimmune hepatitis

2004 ◽  
Vol 18 (1) ◽  
pp. 62-69 ◽  
Author(s):  
Arndt Vogel ◽  
Eyk Heinrich ◽  
Matthias J Bahr ◽  
Kinan Rifai ◽  
Peer Flemming ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Autoimmune Hepatitis ◽  
Term Outcome ◽  
Long Term Outcome

scholarly journals Modifiable factors affecting renal preservation in type I glycogen storage disease after liver transplantation: a single-center propensity-match cohort study

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Yi-Chia Chan ◽  
Kai-Min Liu ◽  
Chao-Long Chen ◽  
Aldwin D. Ong ◽  
Chih-Che Lin ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Renal Function ◽  
Glycogen Storage Disease ◽  
Storage Disease ◽  
Glycogen Storage ◽  
Renal Outcome ◽  
Type I ◽  
Long Term Outcome ◽  
Renal Prognosis

Abstract Background and aims Glycogen storage disease type I (GSD-I) is an autosomal recessive disorder of carbohydrate metabolism, resulting in limited production of glucose and excessive glycogen storage in the liver and kidneys. These patients are characterized by life-threatening hypoglycemia, metabolic derangements, hepatomegaly, chronic kidney disease, and failure to thrive. Liver transplantation (LT) has been performed for poor metabolic control and delayed growth. However, renal outcome was diverse in pediatric GSD patients after LT. The aim of this study was to investigate the long-term outcome of renal function in pediatric GSD-I patients after living donor LT (LDLT), and to identify modifiable variables that potentially permits LT to confer native renal preservation. Methods The study included eight GSD-Ia and one GSD-Ib children with a median age of 9.0 (range 4.2–15.7) years at the time of LT. Using propensity score matching, 20 children with biliary atresia (BA) receiving LT were selected as the control group by matching for age, sex, pre-operative serum creatinine (SCr) and pediatric end-stage liver disease (PELD) score. Renal function was evaluated based on the SCr, estimated glomerular filtration rate (eGFR), microalbuminuria, and morphological changes in the kidneys. Comparability in long-term renal outcome in terms of anatomic and functional parameters will help to identify pre-LT factors of GSD-I that affect renal prognosis. Results The clinical and biochemical characteristics of the GSD and BA groups were similar, including immunosuppressive regimens and duration of follow-up (median 15 years) after LT. Overall, renal function, including eGFR and microalbuminuria was comparable in the GSD-I and BA groups (median eGFR: 111 vs. 123 ml/min/1.73m2, P = 0.268; median urine microalbuminuria to creatinine ratio: 16.0 vs. 7.2 mg/g, P = 0.099, respectively) after LT. However, in the subgroups of the GSD cohort, patients starting cornstarch therapy at an older age (≥ 6-year-old) before transplantation demonstrated a worse renal outcome in terms of eGFR change over years (P < 0.001). In addition, the enlarged kidney in GSD-I returned to within normal range after LT. Conclusions Post-LT renal function was well-preserved in most GSD-I patients. Early initiation of cornstarch therapy before preschool age, followed by LT, achieved a good renal prognosis.

Long‐term outcome of liver transplantation for congenital extrahepatic portosystemic shunt

2021 ◽  
Author(s):  
Valérie McLin ◽  
Maurice Beghetti ◽  
Lorenzo D’Antiga ◽  
Stéphanie Franchi‐Abella ◽  
Keyword(s):  
Liver Transplantation ◽  
Portosystemic Shunt ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Congenital Extrahepatic Portosystemic Shunt

1029 LONG-TERM OUTCOME OF CIRRHOTIC PATIENTS WITH SINGLE HCC ≤ 5CM. DOES LIVER RESECTION HAVE THE SAME RESULTS AS LIVER TRANSPLANTATION?

2012 ◽  
Vol 56 ◽  
pp. S402-S403
Author(s):  
G. Sapisochin ◽  
C. Dopazo ◽  
I. Bilbao ◽  
L. Castells ◽  
J.L. Lazaro ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Liver Resection ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Cirrhotic Patients

scholarly journals Long-Term Aspartame Administration Leads to Fibrosis, Inflammasome Activation, and Gluconeogenesis Impairment in the Liver of Mice

Biology ◽  
2021 ◽  
Vol 10 (2) ◽  
pp. 82
Author(s):  
Isabela A. Finamor ◽  
Caroline A. Bressan ◽  
Isabel Torres-Cuevas ◽  
Sergio Rius-Pérez ◽  
Marcelo da Veiga ◽  
...  
Keyword(s):  
Liver Fibrosis ◽  
Liver Damage ◽  
Transforming Growth Factor ◽  
Smooth Muscle Actin ◽  
Elevated Serum ◽  
Inflammasome Activation ◽  
Related Factor ◽  
Type I ◽  
Artificial Sweetener

Background: Aspartame is an artificial sweetener used in foods and beverages worldwide. However, it is linked to oxidative stress, inflammation, and liver damage through mechanisms that are not fully elucidated yet. This work aimed to investigate the effects of long-term administration of aspartame on the oxidative and inflammatory mechanisms associated with liver fibrosis progression in mice. Methods: Mice were divided into two groups with six animals each: control and aspartame. Aspartame (80 mg/kg, via oral) or vehicle was administrated for 12 weeks. Results: Aspartame caused liver damage and elevated serum transaminase levels. Aspartame also generated liver fibrosis, as evidenced by histology analysis, and pro-fibrotic markers’ upregulation, including transforming growth factor β 1, collagen type I alpha 1, and alpha-smooth muscle actin. Furthermore, aspartame reduced nuclear factor erythroid 2-related factor 2 (Nrf2) activation and enzymatic antioxidant activity and increased lipid peroxidation, which triggered NOD-like receptor containing protein 3 (NLRP3) inflammasome activation and p53 induction. Furthermore, aspartame reduced peroxisome proliferator-activated receptor gamma coactivator 1 alpha (PGC-1α) levels, possibly through p53 activation. This PGC-1α deficiency could be responsible for the changes in lipid profile in serum, total lipid accumulation, and gluconeogenesis impairment in liver, evidenced by the gluconeogenic enzymes’ downregulation, thus causing hypoglycemia. Conclusions: This work provides new insights to understand the mechanisms related to the adverse effects of aspartame on liver tissue.

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