PMID- 26567543
OWN - NLM
STAT- MEDLINE
DCOM- 20161223
LR  - 20181113
IS  - 1432-1076 (Electronic)
IS  - 0340-6199 (Linking)
VI  - 175
IP  - 4
DP  - 2016 Apr
TI  - Human leucocyte antigens and pediatric autoimmune liver disease: diagnosis and 
      prognosis.
PG  - 527-37
LID - 10.1007/s00431-015-2662-x [doi]
AB  - An association of human leukocyte antigen (HLA) class II alleles with autoimmune 
      conditions is increasingly being used for diagnostic purposes. The aim of our 
      study was to examine whether HLA class II alleles in pediatric-onset autoimmune 
      liver disease (pAILD) may serve as diagnostic markers and if they correlate with 
      clinical outcome parameters. HLA-DRB1 alleles of 76 children with pAILD 
      (autoimmune hepatitis [AIH], autoimmune sclerosing cholangitis [AISC], primary 
      sclerosing cholangitis [PSC]) and of 50 healthy blood donors as control group 
      were analyzed retrospectively. Diagnosis of these patients was confirmed by the 
      autoimmune hepatitis score including liver histology, which has been re-evaluated 
      by a blinded liver pathologist, and by bile duct imaging, as appropriate. Our 
      results showed significant association of HLA-DRB1*03 with AIH1 and AISC with 82 
      % specificity for AIH. For pAILD (excluding AIH2), HLA-DRB1*03 homozygosity had 
      specificity of 98 %, whereas sensitivity is low. Remission in 
      HLA-DRB1*03-positive patients appears to be less likely. HLA-DRB1*13 is 
      significantly associated with PSC and also with AIH1. CONCLUSION: HLA-DRB1 
      alleles provide supportive information for diagnostic workup in patients with 
      liver disease, but they were not suitable for differentiation within pAILD. Their 
      prognostic value could be helpful but needs to be evaluated further. WHAT IS 
      KNOWN: * HLA-DRB1*03 is NOT associated with pediatric AIH in a previous national 
      study. * In other studies, HLA-DRB1*03 is associated with AIH1. * HLA-DRB1*13 is 
      associated with PSC. * HLA-DRB1*04 is described as protective for AILD. What is 
      New: * HLA-DRB1 four-digit typing for all alleles and for all subgroups of pAILD 
      combined with re-assessment of liver histology * HLA-DRB1*03:01 is associated 
      with pediatric AIH1 and AISC. * HLA-DRB1*03:01 appears to be a prognostic marker. 
      * HLA-DRB1*13:01 is associated with pediatric AIH1 in mixed ethnicity cohort. * 
      HLA-DRB1*04 does not show any protective effect for pAILD.
FAU - Junge, Norman
AU  - Junge N
AD  - Pediatric Gastroenterology and Hepatology, Department of Paediatric Kidney, Liver 
      and Metabolic Diseases, Hannover Medical School, Carl- Neuberg-Str 1, 30625, 
      Hannover, Niedersachsen, Germany. Junge.Norman@mh-hannover.de.
FAU - Tiedau, Miriam
AU  - Tiedau M
AD  - Pediatric Gastroenterology and Hepatology, Department of Paediatric Kidney, Liver 
      and Metabolic Diseases, Hannover Medical School, Carl- Neuberg-Str 1, 30625, 
      Hannover, Niedersachsen, Germany.
FAU - Verboom, Murielle
AU  - Verboom M
AD  - Institute for Transfusion Medicine, Hannover Medical School, Carl- Neuberg-Str 1, 
      30625, Hannover, Niedersachsen, Germany.
FAU - Hallensleben, Michael
AU  - Hallensleben M
AD  - Institute for Transfusion Medicine, Hannover Medical School, Carl- Neuberg-Str 1, 
      30625, Hannover, Niedersachsen, Germany.
FAU - Blasczyk, Rainer
AU  - Blasczyk R
AD  - Institute for Transfusion Medicine, Hannover Medical School, Carl- Neuberg-Str 1, 
      30625, Hannover, Niedersachsen, Germany.
FAU - Schlue, Jerome
AU  - Schlue J
AD  - Institute for Pathology, Hannover Medical School, Carl- Neuberg-Str 1, 30625, 
      Hannover, Niedersachsen, Germany.
FAU - Goldschmidt, Imeke
AU  - Goldschmidt I
AD  - Pediatric Gastroenterology and Hepatology, Department of Paediatric Kidney, Liver 
      and Metabolic Diseases, Hannover Medical School, Carl- Neuberg-Str 1, 30625, 
      Hannover, Niedersachsen, Germany.
FAU - Pfister, Eva-Doreen
AU  - Pfister ED
AD  - Pediatric Gastroenterology and Hepatology, Department of Paediatric Kidney, Liver 
      and Metabolic Diseases, Hannover Medical School, Carl- Neuberg-Str 1, 30625, 
      Hannover, Niedersachsen, Germany.
FAU - Baumann, Ulrich
AU  - Baumann U
AD  - Pediatric Gastroenterology and Hepatology, Department of Paediatric Kidney, Liver 
      and Metabolic Diseases, Hannover Medical School, Carl- Neuberg-Str 1, 30625, 
      Hannover, Niedersachsen, Germany.
LA  - eng
PT  - Journal Article
DEP - 20151114
PL  - Germany
TA  - Eur J Pediatr
JT  - European journal of pediatrics
JID - 7603873
RN  - 0 (Biomarkers)
RN  - 0 (HLA-DRB1 Chains)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Alleles
MH  - Biomarkers
MH  - Child
MH  - Child, Preschool
MH  - Cholangitis, Sclerosing/*diagnosis/immunology
MH  - Female
MH  - HLA-DRB1 Chains/genetics/*immunology
MH  - Hepatitis, Autoimmune/*diagnosis/immunology
MH  - Humans
MH  - Infant
MH  - Liver/*pathology
MH  - Male
MH  - Middle Aged
MH  - Prognosis
MH  - Retrospective Studies
MH  - Sensitivity and Specificity
MH  - Young Adult
OTO - NOTNLM
OT  - Autoimmune hepatitis
OT  - Autoimmune sclerosing cholangitis
OT  - HLA-DRB1
OT  - Human leukocyte antigens
OT  - Liver
OT  - Liver transplantation
OT  - Pediatric autoimmune liver disease
OT  - Primary sclerosing cholangitis
EDAT- 2015/11/17 06:00
MHDA- 2016/12/24 06:00
CRDT- 2015/11/17 06:00
PHST- 2015/08/13 00:00 [received]
PHST- 2015/11/03 00:00 [accepted]
PHST- 2015/10/20 00:00 [revised]
PHST- 2015/11/17 06:00 [entrez]
PHST- 2015/11/17 06:00 [pubmed]
PHST- 2016/12/24 06:00 [medline]
AID - 10.1007/s00431-015-2662-x [pii]
AID - 10.1007/s00431-015-2662-x [doi]
PST - ppublish
SO  - Eur J Pediatr. 2016 Apr;175(4):527-37. doi: 10.1007/s00431-015-2662-x. Epub 2015 
      Nov 14.