Abstract:
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.
journal_name
J Autoimmunjournal_title
Journal of autoimmunityauthors
Oettinger R,Brunnberg A,Gerner P,Wintermeyer P,Jenke A,Wirth Sdoi
10.1016/j.jaut.2004.11.009subject
Has Abstractpub_date
2005-02-01 00:00:00pages
79-84issue
1eissn
0896-8411issn
1095-9157pii
S0896-8411(04)00136-2journal_volume
24pub_type
杂志文章abstract::Interleukin-2 (IL-2) gene regulation was investigated in primary cultures of highly purified human peripheral blood CD28+ T cells. Two discrete mechanisms for induction of T-cell proliferation could be distinguished by examining cell cycle progression and the expression of the IL-2 gene. Stimulation of cells by CD3 Mo...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1016/0896-8411(89)90117-0
更新日期:1989-06-01 00:00:00
abstract::T cell clones reactive to beta-cell antigens prepared from different species were established in order to identify putative pathogenic T cells in human IDDM. We were able to generate T cell clones from patients, but not from controls, reactive specifically to the insulin secretory enriched fraction (ISG) of a rat insu...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1006/jaut.1995.0017
更新日期:1995-04-01 00:00:00
abstract::Costimulatory molecules are cell-surface glycoproteins that can direct, modulate and fine tune immune responses. B7-2(CD86) costimulatory molecules are considered as major regulators of T cell responses, acting by appropriate interactions with the stimulatory CD28 or inhibitory CTLA-4 receptors found on T cells. Altho...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1016/j.jaut.2010.06.006
更新日期:2010-11-01 00:00:00
abstract::Various specific and non-specific environmental factors have been associated with the induction and/or exacerbation of disease activity in patients with Crohn's disease and ulcerative colitis. One such factor is the potential role of ingested ultrafine particles. In fact, based on a Western diet, recent data suggest t...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1006/jaut.1999.0342
更新日期:2000-02-01 00:00:00
abstract::Natural autoantibodies (autoNAb) recognize self antigens and are an important component of the immune system, as species ranging from invertebrates to vertebrates have polyreactive IgM NAbs. In higher vertebrates, different polyreactive autoNAbs isotypes are also frequently encountered and autopolyreactive IgG NAbs ar...
journal_title:Journal of autoimmunity
pub_type: 杂志文章,评审
doi:10.1016/j.jaut.2013.01.006
更新日期:2013-03-01 00:00:00
abstract::A common epitope on S-antigen (arrestin), a potent autoantigen inducing experimental autoimmune uveoretinitis (EAU), and on human tumor necrosis factor alpha (hTNF alpha) was revealed using two monoclonal antibodies to S-antigen which inhibit EAU induction. The minimal common sequence for monoclonal antibody recogniti...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1016/s0896-8411(05)80048-4
更新日期:1992-02-01 00:00:00
abstract:OBJECTIVE:To identify potential causative markers involved in the development of early-onset myasthenia gravis (EOMG) in the MHC and non-MHC regions that may interact with the HLA-B*08:01 allele. METHODS:We analyzed 583 MG patients and identified 5 patients homozygous for the disease-associated ancestral haplotype 8.1...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1016/j.jaut.2017.10.002
更新日期:2018-03-01 00:00:00
abstract::Sjögren's syndrome (SS) is a chronic autoimmune disorder that is characterized by dysfunction and destruction of the exocrine glands. Exocrinopathy is associated with periductal mononuclear cell infiltrates in the affected exocrine glands and B-cell hyperreactivity. Epithelial cells are thought to play an important pa...
journal_title:Journal of autoimmunity
pub_type: 杂志文章,评审
doi:10.1016/j.jaut.2010.06.011
更新日期:2010-11-01 00:00:00
abstract::Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disease of the peripheral nerves evolving with diffuse sensory and motor symptoms. Although it is claimed that in neurodegenerative pathologies, a common feature is the failure of proteolytic systems to adequately eliminate aggregated or...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1016/j.jaut.2018.05.009
更新日期:2018-08-01 00:00:00
abstract::Coronavirus disease 2019 (COVID-19) can progress to cytokine storm that is associated with organ dysfunction and death. The purpose of the present study is to determine clinical characteristics associated with 28 day in-hospital survival in patients with coronavirus disease 2019 (COVID-19) that received tocilizumab. T...
journal_title:Journal of autoimmunity
pub_type: 杂志文章,多中心研究
doi:10.1016/j.jaut.2020.102512
更新日期:2020-11-01 00:00:00
abstract::The adrenocortical response is central to recovery from experimental allergic encephalomyelitis (EAE) in the Lewis rat, as reflected by the increased severity of the disease in adrenalectomized animals. The protection conferred by glucocorticoids is related to the immunosuppressive effects of the steroid, which may in...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1006/jaut.1996.0020
更新日期:1996-04-01 00:00:00
abstract::Clinical course and changes in serum levels of antibodies to the acetylcholine receptor (a-AChR) were followed in 82 patients with myasthenia gravis during a period of 1-8 years after thymectomy. Decrease in a-AChR immediately after thymectomy was influenced by changes in a total IgG. Immunosuppressive medication affe...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1016/0896-8411(91)90018-8
更新日期:1991-04-01 00:00:00
abstract::Many mechanisms can explain the mode of action of IVIg in immune disorders. Macrophage blockade and interference in the idiotypic network are supported by some experimental data. Among the other mechanisms, two are considered in greater detail. Firstly, in some disorders, the patients could improve simply because the ...
journal_title:Journal of autoimmunity
pub_type: 杂志文章,评审
doi:10.1006/jaut.1993.1057
更新日期:1993-12-01 00:00:00
abstract::Autoimmunity is a field that has only been around for a little over a century. Initially, it was thought that autoimmunity could not happen, that the body would never turn on itself (i.e. "horror autotoxicus"). It was only around the First World War that autoimmunity was recognized as the pathogenesis of various disea...
journal_title:Journal of autoimmunity
pub_type: 社论,历史文章
doi:10.1016/j.jaut.2013.12.019
更新日期:2014-02-01 00:00:00
abstract::Dermatomyositis (DM) is an idiopathic inflammatory myopathy (IIM) characterized by an inflammatory infiltrate primarily affecting the skeletal muscle and skin. Most common and peculiar cutaneous lesions include Gottron's papules, Gottron's sign and heliotrope rash. Different DM subsets have been identified until now e...
journal_title:Journal of autoimmunity
pub_type: 杂志文章,评审
doi:10.1016/j.jaut.2013.11.005
更新日期:2014-02-01 00:00:00
abstract::The probability that epitope spreading occurs in multiple sclerosis (MS) and the fact that patients have been shown to respond to multiple myelin epitopes concurrently makes the use of peptide-specific tolerance therapies targeting single epitopes problematic. To attempt to overcome this limitation, we have employed c...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1016/j.jaut.2006.12.002
更新日期:2006-12-01 00:00:00
abstract::Peptidomimetic compounds that bind to major histocompatibility complex class II molecules and are resistant to cathepsins can competitively inhibit the presentation of processed protein antigens. Therefore, compounds that bind to autoimmune disease-associated class II molecules are expected to compete with autoantigen...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1016/j.jaut.2006.09.005
更新日期:2006-11-01 00:00:00
abstract::Sera from autoimmune patients and normal volunteers were tested for antibodies to the A1 core protein of heteronuclear ribonucleoprotein (hnRNP) particles by ELISA and Western blot assays. The A1 protein used in these studies was produced by recombinant DNA technology. Thirty-seven per cent of patients with systemic l...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1016/0896-8411(88)90078-9
更新日期:1988-02-01 00:00:00
abstract::Beta(2)-glycoprotein I (beta(2)GPI) is known as a major autoantigen for antiphospholipid antibodies. Our recent data show that binding of beta(2)GPI to oxidized low-density lipoprotein (oxLDL) or to liposomes containing anionic phospholipid(s) may facilitate the presentation of beta(2)GPI's epitope by macrophages/dend...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1016/j.jaut.2007.07.003
更新日期:2007-09-01 00:00:00
abstract::Neonatal lupus erythematosus (NLE) is associated with the transplacental passage of maternal anti-Ro and anti-La antibodies. In order to better determine the risk of delivery of a child with NLE, we examined the frequency of anti-Ro and anti-La antibody secreting cells in mothers of children with NLE and in mothers at...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1006/jaut.1993.1040
更新日期:1993-08-01 00:00:00
abstract::The standard treatment of thrombosis related to antiphospholipid antibodies is lifelong high-intensity oral anticoagulation. This standard is primarily based on retrospective evaluations of such patients. In this paper we give a summary of current treatment of deep leg vein thrombosis, pulmonary embolism, ischaemic st...
journal_title:Journal of autoimmunity
pub_type: 杂志文章,评审
doi:10.1006/jaut.2000.0395
更新日期:2000-09-01 00:00:00
abstract::Antiphospholipid syndrome (APS) is characterized by thrombosis and pregnancy loss in the presence of antiphospholipid antibodies (aPL), mainly anticardiolipin, anti-beta2-glycoprotein I and lupus anticoagulant. However, similar to systemic lupus erythematosus, APS is also characterized by multiple other autoantibodies...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1016/j.jaut.2007.11.011
更新日期:2008-02-01 00:00:00
abstract::Nasal tolerance has recently been used to modulate immune responses in animal models of autoimmunity. We have compared immunogenic collagen type II (CII) peptides for induction of nasal tolerance in DBA/1 mice to collagen-induced arthritis (CIA). Three synthetic peptides corresponding to T cell-stimulating sequences o...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1006/jaut.1998.0255
更新日期:1999-02-01 00:00:00
abstract::To investigate patterns of autoreactivity in Sjögren's syndrome, the epitope specificity of anti-La antibodies was determined using recombinant antigens bearing sequences of the amino, middle, and carboxyl portions of the La molecule. Sera from patients with primary as well as secondary Sjögren's syndrome reacted with...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1016/0896-8411(89)90161-3
更新日期:1989-08-01 00:00:00
abstract::Immune cell infiltration to the brain's territory was considered for decades to reflect a pathological process in which immune cells attack the central nervous system (CNS); such a process is observed in the inflammatory autoimmune disease, multiple sclerosis (MS). As neuroinflammatory processes within the CNS parench...
journal_title:Journal of autoimmunity
pub_type: 杂志文章,评审
doi:10.1016/j.jaut.2014.08.002
更新日期:2014-11-01 00:00:00
abstract::Oxidative stress makes an important contribution to the development of autoimmune diabetes. We therefore tested the possible therapeutic value of two anti-oxidants, butylated hydroxyanisole (BHA) and pyrrolidine dithiocarbamate (PDTC), in the animal model of diabetes induced in susceptible DA rats by multiple low dose...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1016/j.jaut.2004.01.005
更新日期:2004-06-01 00:00:00
abstract::Common variable immunodeficiency (CVID) is associated with low serum immunoglobulin concentrations and an increased susceptibility to infections and autoimmune diseases. The treatment of choice for CVID patients is replacement intravenous immunoglobulin (IVIg) therapy. IVIg has been beneficial in preventing or allevia...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1016/j.jaut.2010.09.006
更新日期:2011-02-01 00:00:00
abstract::Autoimmune thyroid diseases (AITDs), i.e., Graves' disease (GD) and Hashimoto thyroiditis (HT), are the most prevalent organ-specific autoimmune diseases, but their pathogenesis is still incompletely understood. The PD-1/PD-L1 pathway is an important mechanism of peripheral tolerance that has not been investigated in ...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1016/j.jaut.2019.05.013
更新日期:2019-09-01 00:00:00
abstract::The costimulatory interaction between CD28 on T cells and B7-related molecules on antigen presenting cells plays an important role in a broad range of functions of the immune system, including protective immunity, tolerance induction, allograft rejection, and the development of autoimmune diseases. Monoclonal antibodi...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1006/jaut.1998.0258
更新日期:1999-03-01 00:00:00
abstract::Malathion administration at non-cholinergical doses was shown to elevate macrophage, proliferative and humoral immune responses. This study examined the effects of malathion on autoimmunity, autoantibody formation, macrophage function and mitogenic responses in MRL-lpr mice (genetically predisposed to autoimmune disea...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1006/jaut.1997.0145
更新日期:1997-08-01 00:00:00