TY  - JOUR
AB  - OBJECTIVES: Paediatric acute liver failure (PALF) is a rare but life-threatening condition, yet comprehensive epidemiological data in Germany are lacking. Our study aimed to systematically analyse incidence, aetiology, and outcome of PALF in Germany. METHODS: In a nationwide, population-based surveillance study, cases of PALF (defined following the PALF study group inclusion criteria) were queried from 2016 to 2018 through the German Paediatric Surveillance Unit (ESPED). Demographic, clinical, laboratory, therapeutic, and outcome data were collected and analysed. In case of unexplained aetiology, whole exome and virus sequencing was offered as a complementary diagnostic. RESULTS: Over the 3-year period, 148 cases were reported, yielding an estimated incidence of 3.7 per 1 million children per year. Neonates and infants were predominantly affected (45% of the cases); median age at PALF was 1.2 years (range: 0-17.9 years). Metabolic/genetic diseases were the most common cause (23%), followed by infectious causes (17%). The overall diagnostic yield was 73%, diagnosis remained unknown in 40 cases. Clinical outcome was age-dependent: new-borns showed a significant higher lethality (42%), followed by infants (29%), toddlers (15%), and school children (12%). Liver transplantation was reported in 22% of cases. CONCLUSIONS: This study provides comprehensive insights into PALF epidemiology in Germany. Metabolic/genetic causes and infectious diseases were most common. Advances in standardised diagnostic work-up and genetic analysis have enhanced diagnostic yield, yet mortality remains substantial, particularly among neonates. Further research is warranted to improve diagnostic accuracy, therapeutic outcomes, and overall management of PALF.
AU  - Lenz, D.*
AU  - Abdulaziz, M.*
AU  - Peters, B.*
AU  - Wagner, M.
AU  - Schlieben, L.D.
AU  - Corman, V.M.*
AU  - Baumann, U.*
AU  - Bufler, P.*
AU  - Dattner, T.*
AU  - Ganschow, R.*
AU  - Genzel, K.*
AU  - Hammann, N.*
AU  - Hartleif, S.*
AU  - Hegen, B.*
AU  - Henning, S.*
AU  - Hoerning, A.*
AU  - Jankofsky, M.*
AU  - Junge, N.*
AU  - Kathemann, S.*
AU  - Knoppke, B.*
AU  - Kohl-Sobania, M.*
AU  - Laass, M.W.*
AU  - Lainka, E.*
AU  - Lurz, E.*
AU  - Melter, M.*
AU  - Müller, H.*
AU  - Pilic, D.*
AU  - Ries, M.*
AU  - Schiefele, L.*
AU  - Schwerd, T.*
AU  - Sturm, E.*
AU  - Wegner, M.*
AU  - Urschitz, M.S.*
AU  - Garbade, S.F.*
AU  - Wenning, D.*
AU  - Drosten, C.*
AU  - Fichtner, A.*
AU  - Kölker, S.*
AU  - Hoffmann, G.F.*
AU  - Prokisch, H.
AU  - Staufner, C.*
C1  - 75083
C2  - 57793
CY  - 111 River St, Hoboken 07030-5774, Nj Usa
TI  - Paediatric acute liver failure: A prospective, nationwide, population-based surveillance study in Germany.
JO  - J. Pediatr. Gastroenterol. Nutr.
PB  - Wiley
PY  - 2025
SN  - 0277-2116
ER  - 

TY  - JOUR
AB  - OBJECTIVE: Cesarean section (C-section) is associated with various immune-mediated diseases in the offspring. We investigated the relationship between mode of delivery and celiac disease (CD) and CD autoimmunity (CDA) in a multinational birth cohort. METHODS: From 2004 to 2010, infants from the general population who tested positive for HLA DR3-DQ2 or DR4-DQ8 were enrolled in The Environmental Determinants for Diabetes in the Young (TEDDY) study. Children were annually screened for transglutaminase autoantibodies, if positive, they are retested after 3 to 6 months and those persistently positive defined as CDA. Associations of C-section with maternal (age, education level, parity, pre-pregnancy weight, diabetes, smoking, weight gain during pregnancy) and child characteristics (gestational age, birth weight) were examined by Fisher exact test or Wilcoxon rank-sum test. Hazard ratios (HRs) for CDA or CD were calculated by Cox proportional hazard regression models. RESULTS: Of 6087 analyzed singletons, 1600 (26%) were born by C-section (Germany 38%, United States 37%, Finland 18%, Sweden 16%), and the remaining were born vaginally without instrumental support; 979 (16%) had developed CDA and 343 (6%) developed CD. C-section was associated with lower risk for CDA (hazard ratio [HR] = 0.85; 95% confidence interval [CI] 0.73, 0.99 P = 0.032) and CD (HR = 0.75; 95% CI 0.58, 0.98; P = 0.034). After adjusting for country, sex, HLA-genotype, CD in family, maternal education, and breast-feeding duration, significance was lost for CDA (HR = 0.91; 95% CI 0.78, 1.06; P = 0.20) and CD (HR = 0.85; 95% CI 0.65, 1.11; P = 0.24). Presurgical ruptured membranes had no influence on CDA or CD development. CONCLUSION: C-section is not associated with increased risk for CDA or CD in the offspring.
AU  - Koletzko, S.*
AU  - Lee, H.S.*
AU  - TEDDY Study Group (Beyerlein, A.
AU  - Hummel, M.
AU  - Ziegler, A.-G.
AU  - Hummel, S.
AU  - Janz, N.
AU  - Knopff, A.
AU  - Peplow, C.
AU  - Roth, R.
AU  - Scholz, M.
AU  - Stock, J.
AU  - Strauss, E.
AU  - Warncke, K.
AU  - Wendel, L.
AU  - Winkler, C.)
AU  - Aronsson, C.A.*
AU  - Liu, E.*
AU  - Simell, V.*
AU  - Kurppa, K.*
AU  - Lernmark, A.*
AU  - Hagopian, W.*
AU  - Rewers, M.*
AU  - She, J.X.*
AU  - Simell, O*
AU  - Toppari, J.*
AU  - Krischer, J.*
AU  - Agardh, D.*
C1  - 51621
C2  - 43317
SP  - 417-424
TI  - Cesarean section on the risk of celiac disease in the offspring: The Teddy Study.
JO  - J. Pediatr. Gastroenterol. Nutr.
VL  - 66
IS  - 3
PY  - 2017
SN  - 0277-2116
ER  -