OBJECTIVE: Evaluation of systematic epidemiological data regarding clinical characteristics, sex distribution and autoantibody pattern in Caucasian children with autoimmune hepatitis (AIH). STUDY DESIGN: Data of 142 children presenting with AIH (97 girls and 45 boys) have been analysed for their clinical, serological, and histological profile. RESULTS: Clinical findings were jaundice (58%), unspecific weakness (57%), anorexia (47%), abdominal pain (38%) and paleness (26%). One hundred and three children (73%) (68 girls, 35 boys, 1.9:1) had AIH type 1 and 35 patients (25%) (27 girls, 8 boys, 3.4:1) type 2 due to specific autoantibodies. Four children could not be classified. Histology of 122 children revealed active hepatitis in 64 (52%), cirrhosis in 46 (38%), and mild inflammatory activity in 12 individuals (10%). The most prevalent HLA type was B8. CONCLUSION: In our cohort the prevalence of AIH was half as frequent in boys as in girls. Type 1 was the most frequent diagnosis (73%) and was more prevalent in older children. Type 2 was equally age distributed. The clinical presentation of AIH in children was unspecific and type I and type II could only be differentiated by the determination of the specific autoantibodies. Ninety percent of patients presented with high inflammatory activity or liver cirrhosis.
OBJECTIVE: Evaluation of systematic epidemiological data regarding clinical characteristics, sex distribution and autoantibody pattern in Caucasian children with autoimmune hepatitis (AIH). STUDY DESIGN: Data of 142 children presenting with AIH (97 girls and 45 boys) have been analysed for their clinical, serological, and histological profile. RESULTS: Clinical findings were jaundice (58%), unspecific weakness (57%), anorexia (47%), abdominal pain (38%) and paleness (26%). One hundred and three children (73%) (68 girls, 35 boys, 1.9:1) had AIH type 1 and 35 patients (25%) (27 girls, 8 boys, 3.4:1) type 2 due to specific autoantibodies. Four children could not be classified. Histology of 122 children revealed active hepatitis in 64 (52%), cirrhosis in 46 (38%), and mild inflammatory activity in 12 individuals (10%). The most prevalent HLA type was B8. CONCLUSION: In our cohort the prevalence of AIH was half as frequent in boys as in girls. Type 1 was the most frequent diagnosis (73%) and was more prevalent in older children. Type 2 was equally age distributed. The clinical presentation of AIH in children was unspecific and type I and type II could only be differentiated by the determination of the specific autoantibodies. Ninety percent of patients presented with high inflammatory activity or liver cirrhosis.
Authors: Yun Ma; Haibin Su; Marianne Samyn; Diego Vergani; Giorgina Mieli-Vergani; Muhammed Yuksel; Maria Serena Longhi; Mark J McPhail; Pengyun Wang; Sanjay Bansal; Guan-Wee Wong; Jonathon Graham; Li Yang; Richard J Thompson; Derek G Doherty; Nedim Hadzic; Yoh Zen; Alberto Quaglia; Michael A Heneghan Journal: Hepatology Date: 2021-06-21 Impact factor: 17.425
Authors: Mohammed A Khedr; Tahany A Salem; Ghada M Boghdadi; Ahmed S Elharoun; Allia A El-Shahaway; Hany R Atallah; Mostafa M Sira Journal: Wien Klin Wochenschr Date: 2021-07-20 Impact factor: 1.704