Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 20 de 21
Filter
2.
Brain Dev ; 45(8): 456-461, 2023 Sep.
Article in English | MEDLINE | ID: mdl-37246116

ABSTRACT

BACKGROUND: A few case reports have described patients with myelin oligodendrocyte glycoprotein antibody (MOG-Ab)-associated demyelinating syndrome who presented with symptoms of aseptic meningitis. All such patients required immunotherapy. We report a patient with MOG-Ab-associated disorder (MOGAD) who presented with symptoms of aseptic meningitis and improved without treatment. CASE: A 13-year-old girl presented with fever, headache, decreased appetite, and neck stiffness. Cerebrospinal fluid (CSF) analysis revealed pleocytosis and magnetic resonance imaging (MRI) showed leptomeningeal enhancement. The patient was diagnosed with aseptic meningitis at admission. However, there were no signs of recovery 4 days after admission (i.e., 8 days after disease onset). Therefore, we performed extensive investigations to identify the cause of the underlying infection and inflammation. On day 14 after admission, the serum MOG-Ab test performed at admission came back positive (1:128) and she was diagnosed with MOGAD. She was discharged on day 18 after admission, because her symptoms, CSF pleocytosis, and MRI findings had improved. About 6 weeks after discharge, MRI revealed hyperintensity without gadolinium enhancement. However, her serum MOG-Ab test was negative. We did follow-ups for 11 months but found no new neurological symptoms. DISCUSSION AND CONCLUSION: To the best of our knowledge, this is the first ever report of a pediatric patient with MOGAD experiencing spontaneous remission with no demyelinating symptoms during an extended follow-up period.


Subject(s)
Meningitis, Aseptic , Female , Humans , Autoantibodies , Contrast Media , Gadolinium , Leukocytosis , Myelin-Oligodendrocyte Glycoprotein , Remission, Spontaneous , Adolescent
3.
Arerugi ; 71(9): 1129-1135, 2022.
Article in Japanese | MEDLINE | ID: mdl-36372424

ABSTRACT

BACKGROUND: Allergenicity to heated egg yolks is known to be low in hen's egg allergy. However, there are few reports concerning the safety of an oral food challenge (OFC) with boiled egg yolks. This study aimed to determine the safety of a boiled egg yolk OFC and the clinical characteristics of patients who were OFC-positive. METHODS: We retrospectively examined the data of patients who underwent an OFC with one boiled egg yolk at Miyagi Children's Hospital between January 2013 and December 2020. Patients were included if they had a history of immediate symptoms due to hen's egg or positive specific IgE levels to egg yolk or egg white. RESULTS: Among 600 patients, 15.0% were positive for OFC, only one patient required an intramuscular adrenaline injection, and 70% of OFC-positive patients had gastrointestinal symptoms. CONCLUSION: Boiled egg yolk OFC had a low symptom induction rate; however, when symptoms were induced, gastrointestinal symptoms were most commonly observed. Given the low risk of developing severe symptoms, we conclude that the likelihood of safely performing an OFC is high.


Subject(s)
Egg Hypersensitivity , Egg Yolk , Female , Animals , Chickens , Retrospective Studies , Egg Hypersensitivity/diagnosis , Allergens
4.
Transplant Proc ; 54(10): 2803-2806, 2022 Dec.
Article in English | MEDLINE | ID: mdl-36376106

ABSTRACT

Recessive gene mutations in ABCA3 cause lethal neonatal respiratory distress, and pediatric and adult interstitial lung disease. The effectiveness of medical treatments is limited and a subset of such patients will eventually require lung transplantation. A 20 months old boy developed interstitial lung disease and was treated with hydroxychloroquine, which had a significant effect. Sequence analysis of ABCA3 gene revealed newly discovered compound heterozygous mutations. His respiratory dysfunction gradually progressed over years and he underwent living-donor lobar lung transplantation (LDLLT) at 8 years of age with his parents serving as bilateral lobar donors. The parents had been genetically examined beforehand and found to be carriers who had one allele with an ABCA3 gene mutation and the other with no mutation. The recipient has been well without chronic lung allograft dysfunction and his parents have been enjoying healthy social lives for 7 years since the operations. LDLLT appears to be a valid option for selected children with ABCA3 gene mutations who are too ill to wait for cadaveric lung transplantation. When relatives of the recipient with ABCA3 gene mutation are deemed potential donors for LDLLT, sequence analyses of the donors are indispensable to exclude the possibility that they are late-onset patients of this recessive hereditary disease.


Subject(s)
Lung Diseases, Interstitial , Lung Transplantation , Adult , Male , Infant, Newborn , Humans , Child , Infant , Living Donors , Lung Diseases, Interstitial/genetics , Lung Diseases, Interstitial/surgery , Lung Transplantation/adverse effects , Heterozygote , Lung , ATP-Binding Cassette Transporters/genetics
6.
Allergol Int ; 69(1): 104-110, 2020 Jan.
Article in English | MEDLINE | ID: mdl-31421989

ABSTRACT

BACKGROUND: There have been no studies of dual administration of sublingual immunotherapy (SLIT) tablets for perennial and seasonal allergic rhinitis. This trial (JapicCTI-184014) was conducted to investigate the safety profile and immunological response during dual therapy with SQ house dust mite (HDM) and Japanese cedar pollen (JCP) SLIT tablets. METHODS: This was a multicenter, open-label, randomized trial of 109 Japanese patients with coexisting HDM and JCP allergic rhinitis who had positive tests for HDM- and JCP specific IgE (≥0.7 kU/L). Patients were allocated to receive HDM (N = 54) or JCP (N = 55) SLIT tablets alone for 4 weeks followed by 8 weeks of dual therapy with both SLIT tablets administered within 5 min of each other. Adverse events (AEs), adverse drug reactions (ADRs), and serum IgE and IgG4 specific for HDM (Dermatophagoides farinae, Dermatophagoides pteronyssinus) and JCP were recorded. RESULTS: The percentage of subjects with AEs and ADRs was similar between the two groups and between the two periods of monotherapy and dual therapy. Most AEs and ADRs were mild in severity, and no serious events were observed. The most common ADRs were local events in the oral cavity. Levels of IgE and IgG4 specific for HDM (D. farinae, D. pteronyssinus) and JCP were increased after treatment with HDM and JCP SLIT tablets, respectively. CONCLUSIONS: Dual therapy with both SLIT tablets administered within 5 min after 4 weeks of monotherapy with HDM or JCP tablet was well tolerated and induced the expected immunological responses.


Subject(s)
Rhinitis, Allergic/drug therapy , Sublingual Immunotherapy/adverse effects , Sublingual Immunotherapy/methods , Adolescent , Adult , Animals , Antigens, Dermatophagoides/administration & dosage , Child , Cryptomeria/immunology , Female , Humans , Male , Middle Aged , Pollen/immunology , Pyroglyphidae/immunology , Rhinitis, Allergic/etiology , Tablets , Young Adult
8.
Allergy Asthma Immunol Res ; 11(3): 433-437, 2019 May.
Article in English | MEDLINE | ID: mdl-30912331

ABSTRACT

Hen's egg is the most common allergen in IgE-mediated food allergy among children in Japan. Although the majority of patients with egg allergy can eat heated egg yolk safely because of its low allergenicity, severely allergic patients show an immediate-type reaction to heated egg yolk. We hypothesized that patients with hyperresponsiveness to boiled egg yolk may have difficulty in acquiring tolerance to egg. The purpose of this study was to examine the prognosis of patients with hyperresponsiveness to boiled egg yolk. Data from 121 patients with egg allergy who underwent oral food challenge (OFC) with boiled egg yolk between January 2012 and December 2013 were analyzed retrospectively. The proportion of patients who could consume heated whole egg 3 years after OFC was 15.4% in the OFC-positive group and 75.8% in the OFC-negative group. Hyperresponsiveness to boiled egg yolk in early life might lead to prolonged egg allergy in children. This finding might aid in the selection of an appropriate population requiring practical immunotherapy.

9.
J Epidemiol ; 29(12): 478-486, 2019 Dec 05.
Article in English | MEDLINE | ID: mdl-30643099

ABSTRACT

BACKGROUND: Metal exposures could possibly affect allergic responses in pregnant women, although no studies have yet shown a clear relationship between the two, and such exposures might also affect the development of allergic diseases in children. METHODS: We investigated the relationship between metal concentrations in whole blood and immunoglobulin E (IgE; total and specific) in 14,408 pregnant women who participated in the Japan Environment and Children's Study. The subjects submitted self-administered questionnaires, and blood samples were collected from them twice, specifically, during the first trimester and again during the second/third trimester. Concentrations of the metals Cd, Pb, Hg, Se, and Mn, as well as serum total and allergen-specific IgEs for egg white, house dust-mites (HDM), Japanese cedar pollen (JCP), animal dander, and moth, were measured. Allergen-specific IgE(s) were divided based on concentrations <0.35 or ≥0.35 UA/mL, and the metal levels were divided into quartiles. RESULTS: Multivariable logistic regression analysis showed that there was a significant negative correlation between HDM- and animal dander-specific IgEs and Hg and Mn concentrations. Conversely, there was a significant positive relationship between JCP-specific IgE and Hg and Se concentrations. CONCLUSIONS: Metal exposures may be related to both increases and decreases in allergen-specific IgEs in pregnant women.


Subject(s)
Immunoglobulin E/blood , Metals/blood , Adult , Female , Humans , Japan , Pregnancy
10.
J Allergy Clin Immunol ; 138(5): 1395-1403.e6, 2016 11.
Article in English | MEDLINE | ID: mdl-27056266

ABSTRACT

BACKGROUND: Although platelets play a key role in allergic inflammation in addition to their well-established role in hemostasis, the precise mechanisms of how platelets modulate allergic inflammation are not fully understood. IL-33 is an essential regulator of innate immune responses and allergic inflammation. OBJECTIVE: We sought to determine the expression of IL-33 protein by platelets and its functional significance in airway inflammation. METHODS: IL-33 protein in human platelets, the human megakaryocyte cell line MEG-01, and bone marrow-derived mouse megakaryocytes was detected by using Western blot analysis and fluorescent immunostaining. We examined the functional relevance of IL-33 protein in platelets by comparing platelet-intact and platelet-depleted groups in a murine model of IL-33-dependent airway eosinophilia elicited by intranasal administration of papain. We further compared the additive effect of administration of platelets derived from wild-type versus IL-33-deficient mice on the papain-induced eosinophilia. RESULTS: Platelets and their progenitor cells, megakaryocytes, constitutively expressed IL-33 protein (31 kDa). Papain-induced IL-33-dependent airway eosinophilia in mice was significantly attenuated by platelet depletion. Conversely, concomitant administration of platelets derived from wild-type mice but not IL-33-deficient mice enhanced the papain-induced airway eosinophilia. CONCLUSIONS: Our novel findings suggest that platelets might be important cellular sources of IL-33 protein in vivo and that platelet-derived IL-33 might play a role in airway inflammation. Therefore platelets might become an attractive novel therapeutic target for asthma and probably allergic inflammation.


Subject(s)
Blood Platelets/immunology , Cytokines/immunology , Pulmonary Eosinophilia/immunology , Animals , Bronchoalveolar Lavage Fluid/cytology , Bronchoalveolar Lavage Fluid/immunology , COS Cells , Cell Count , Cell Line , Cytokines/genetics , Female , Humans , Lung/immunology , Mice, Inbred C57BL , Mice, Knockout , Papain , Pulmonary Eosinophilia/chemically induced , RNA, Messenger/metabolism
11.
Community Ment Health J ; 52(8): 983-988, 2016 11.
Article in English | MEDLINE | ID: mdl-26308837

ABSTRACT

Maternal depression has been widely studied but paternal depression is often overlooked. Depression in men is generally more difficult to detect as the symptoms are not apparent. Furthermore, Japanese couples tend to suppress their real emotions to avoid confrontation. We aimed to investigate the reliability and validity of the K6, K10 and PHQ-9 in assessing the mental health status of men when used by their pregnant partners, as well as the prevalence of paternal prenatal depression in a Japanese study sample. A total of 136 couples participated in this study. The prevalence of paternal prenatal depression reported by the men themselves was higher compared to that reported by their female partners (K6, 10.3 %; K10, 6.6 %; PHQ-9, 3.7 % vs. K6-FP, 2.2 %; K10-FP, 1.5 %; PHQ-9-FP, 0 %, respectively). Mental health issues in men may not be accurately rated by their female partners, suggesting the importance of self-rating and direct consultation.


Subject(s)
Depression/epidemiology , Parents/psychology , Adult , Fathers/psychology , Female , Humans , Japan/epidemiology , Male , Pregnancy , Prevalence , Psychometrics , Reproducibility of Results , Self Report , Surveys and Questionnaires
12.
Pediatr Int ; 57(5): 1044-7, 2015 Oct.
Article in English | MEDLINE | ID: mdl-26508194

ABSTRACT

Pain management for needle-related procedures is poor in Japan. In many countries the use of lidocaine/prilocaine cream for the relief of pain associated with venipuncture has been approved. In children, a psychological approach has also been shown to be effective in reducing pain with venipuncture. We developed a multidisciplinary procedure that combines a cream (2.5% lidocaine and 2.5% prilocaine) and pharmacological approaches such as preparation, education, positioning and distraction. We evaluated the feasibility and acceptability of the procedure for young children. Among 132 pediatric participants, 58.3% did not cry during venipuncture. According to caregiver questionnaire, 71.9% felt that the multidisciplinary procedure eliminated the fear of needle-related procedures in the children; 90.9% were satisfied with it and 75.8% thought it should be applied to all children undergoing venipuncture. The present results suggest that the multidisciplinary procedure is feasible, acceptable and suitable for use in children undergoing venipuncture.


Subject(s)
Pain Management/methods , Pain Measurement/methods , Pain/diagnosis , Child, Preschool , Feasibility Studies , Female , Follow-Up Studies , Humans , Incidence , Japan/epidemiology , Male , Pain/epidemiology , Pilot Projects , Prognosis , Retrospective Studies
13.
Clin Med Insights Circ Respir Pulm Med ; 9(Suppl 1): 57-68, 2015.
Article in English | MEDLINE | ID: mdl-26512209

ABSTRACT

Interstitial lung disease (ILD) in childhood is a heterogeneous group of rare pulmonary conditions presenting chronic respiratory disorders. Many clinical features of ILD still remain unclear, making the treatment strategies mainly investigative. Guidelines may provide physicians with an overview on the diagnosis and therapeutic directions. However, the criteria used in different clinical studies for the classification and diagnosis of ILDs are not always the same, making the development of guidelines difficult. Advances in genetic testing have thrown light on some etiologies of ILD, which were formerly classified as ILDs of unknown origins. The need of genetic testing for unexplained ILD is growing, and new classification criteria based on the etiology should be adopted to better understand the disease. The purpose of this review is to give an overview of the clinical and genetic aspects of ILD in children.

14.
Arerugi ; 64(5): 714-20, 2015 May.
Article in Japanese | MEDLINE | ID: mdl-26108754

ABSTRACT

BACKGROUND: Some caregivers of children with food allergy may eliminate specific foods from the diet of these children without first consulting doctors. The aim of this study was to investigate the prevalence of such practice and the sensitization of the offending foods. METHODS: All participants were patients aged one year or over who visited our outpatients unit with complaints of food allergy. Caregivers of the patients were asked to fill out a questionnaire to state the eliminated food items and the grounds for avoidance ("doctor's instructions", "family's judgment", and "not consumed in daily diet") of nine food items including chicken egg, cow's milk, wheat, soy bean, rice, buckwheat, peanut, sesame and shrimp. Data on food-specific IgE titers were extracted from the patients' electronic medical charts. RESULTS: A total of 463 subjects were included in the analyses. Prevalence of patients with specific food avoidance of each of the food item was higher in the lower age group than the higher age group. More than 83% of the patients who avoided chicken egg, cow's milk or wheat were advised by their doctors to do so, while less than 49% of patients who avoided buckwheat did so for the same reason. The percentage of the patients who showed positive sensitization to buckwheat, peanut or shrimp, and avoided it without doctor's instructions, was 46%, 48%, and 34%, respectively. CONCLUSION: While the majority of caregivers of the children who visited our outpatient unit eliminated specific foods according to doctor's instructions, a considerable proportion of them do so inappropriately without first consulting a doctor.


Subject(s)
Food Hypersensitivity/prevention & control , Allergens/immunology , Child , Child, Preschool , Female , Food Hypersensitivity/immunology , Humans , Immunoglobulin E/immunology , Infant , Male
15.
J Allergy Clin Immunol ; 134(4): 824-830.e6, 2014 Oct.
Article in English | MEDLINE | ID: mdl-25282564

ABSTRACT

BACKGROUND: Recent studies have suggested that epidermal barrier dysfunction contributes to the development of atopic dermatitis (AD) and other allergic diseases. OBJECTIVE: We performed a prospective, randomized controlled trial to investigate whether protecting the skin barrier with a moisturizer during the neonatal period prevents development of AD and allergic sensitization. METHODS: An emulsion-type moisturizer was applied daily during the first 32 weeks of life to 59 of 118 neonates at high risk for AD (based on having a parent or sibling with AD) who were enrolled in this study. The onset of AD (eczematous symptoms lasting >4 weeks) and eczema (lasting >2 weeks) was assessed by a dermatology specialist on the basis of the modified Hanifin and Rajka criteria. The primary outcome was the cumulative incidence of AD plus eczema (AD/eczema) at week 32 of life. A secondary outcome, allergic sensitization, was evaluated based on serum levels of allergen-specific IgE determined by using a high-sensitivity allergen microarray of diamond-like carbon-coated chips. RESULTS: Approximately 32% fewer neonates who received the moisturizer had AD/eczema by week 32 than control subjects (P = .012, log-rank test). We did not show a statistically significant effect of emollient on allergic sensitization based on the level of IgE antibody against egg white at 0.34 kUA/L CAP-FEIA equivalents. However, the sensitization rate was significantly higher in infants who had AD/eczema than in those who did not (odds ratio, 2.86; 95% CI, 1.22-6.73). CONCLUSION: Daily application of moisturizer during the first 32 weeks of life reduces the risk of AD/eczema in infants. Allergic sensitization during this time period is associated with the presence of eczematous skin but not with moisturizer use.


Subject(s)
Dermatitis, Atopic/prevention & control , Egg Hypersensitivity/prevention & control , Emulsions/administration & dosage , Epidermis/drug effects , Adult , Allergens/immunology , Dermatitis, Atopic/immunology , Egg Hypersensitivity/immunology , Egg Proteins/immunology , Emulsions/adverse effects , Epidermis/immunology , Epidermis/pathology , Female , Humans , Immunoglobulin E/blood , Infant, Newborn , Japan , Male , Microarray Analysis , Risk
16.
Eur J Haematol ; 91(3): 242-248, 2013 Sep.
Article in English | MEDLINE | ID: mdl-23734904

ABSTRACT

BACKGROUND: Epstein-Barr virus (EBV)-infected T or NK cells cause chronic active EBV infection (CAEBV). Allogeneic hematopoietic stem cell transplantation (HSCT) is curative treatment for CAEBV patients. However, chemotherapy prior to HSCT and optimal conditioning regimen for allogeneic HSCT are still controversial. PATIENTS AND METHODS: We retrospectively analyzed five patients with CAEBV treated with reduced-intensity conditioning (RIC) consisted of fludarabine, cyclophosphamide, and low-dose total-body irradiation followed by allogeneic bone marrow transplantation in a single institute. Only one of five patients received chemotherapy prior to transplantation. We analyzed EBV-infected cells in a patient whose EBV load increased after HSCT by T-cell repertoire assay, separation of T-cell subpopulations, in situ hybridization and microsatellite analysis. RESULTS: All five patients achieved engraftment, complete chimera, and eradication of EBV load. All patients have been alive without any serious regimen-related toxicity for more than 16 months following HSCT. However, one patient transplanted from HLA-matched sibling donor developed clonal proliferation of CD4+ Vß3+ T cells caused by monoclonal EBV infection on day 99 after transplantation. Further analysis revealed that the CD4+ Vß3+ T cells selectively harbored EBV genome, and these infected cells were derived from donor T cells. CONCLUSIONS: Allogeneic HSCT with RIC is a safe and effective treatment for better overall survival and less regimen-related toxicity in patients with CAEBV. Our first pediatric case reported in the literature suggests that we should consider the possibility of persistent EBV infection in donor T cells as well as the relapse in recipient cells if EBV load increases after allogeneic HSCT.


Subject(s)
Epstein-Barr Virus Infections/therapy , Hematopoietic Stem Cell Transplantation , Herpesvirus 4, Human , Lymphocyte Subsets/virology , Transplantation Conditioning , Adolescent , CD4 Antigens/metabolism , Child , Epstein-Barr Virus Infections/immunology , Epstein-Barr Virus Infections/transmission , Epstein-Barr Virus Infections/virology , Female , Herpesvirus 4, Human/genetics , Herpesvirus 4, Human/immunology , Humans , Lymphocyte Activation , Lymphocyte Subsets/immunology , Lymphocyte Subsets/metabolism , Male , Receptors, Antigen, T-Cell, alpha-beta/metabolism , Tissue Donors , Transplantation, Homologous , Treatment Outcome , Young Adult
17.
Eur J Pediatr ; 172(7): 953-7, 2013 Jul.
Article in English | MEDLINE | ID: mdl-23443156

ABSTRACT

Mutations in genes critical for surfactant metabolism, including surfactant protein C (SP-C) and ABCA3, are well-recognized causes of interstitial lung disease. Recessive mutations in ABCA3 were first attributed to fatal respiratory failure in full-term neonates, but they are also increasingly being recognized as a cause of respiratory disorders with less severe phenotypes in older children and also adults. Here, we report a 20-month-old boy with interstitial lung disease caused by two distinct ABCA3 mutations. Initial treatment with methylprednisolone was unsuccessful, but the additional administration of hydroxychloroquine was effective. The family history revealed that the patient's older brother had died of idiopathic interstitial lung disease at 6 months of age, suggesting a genetic etiology of the disease. Sequence analyses of SP-C and ABCA3 genes were performed using DNA samples from the patient himself, his parents, and his brother. These analyses revealed novel compound heterozygous mutations in the coding exons of ABCA3 in both the patient and his brother: c.2741A > G, of paternal origin, and c.3715_3716insGGGGGG, of maternal origin. Conclusion Since ABCA3 mutations seem to be a heterogeneous entity with various phenotypes, we recommend genetic testing for mutations in SP-C and ABCA3 genes to be considered in children with unexplained interstitial lung disease.


Subject(s)
ATP-Binding Cassette Transporters/genetics , Hydroxychloroquine/therapeutic use , Lung Diseases, Interstitial/genetics , Pulmonary Surfactant-Associated Proteins/genetics , Fatal Outcome , Heterozygote , Humans , Infant , Lung Diseases, Interstitial/drug therapy , Male , Mutation , Phenotype , Respiratory Distress Syndrome, Newborn/genetics , Sequence Analysis, DNA , Siblings
19.
Arch Immunol Ther Exp (Warsz) ; 58(1): 37-44, 2010 Feb.
Article in English | MEDLINE | ID: mdl-20049647

ABSTRACT

Signaling lymphocytic activation molecule (SLAM)-associated protein (SAP) is an adaptor molecule containing a Src homology 2 (SH2) domain. SAP is expressed in T cells and natural killer (NK) cells and binds to the cytoplasmic domains of SLAM family receptors, resulting in the subsequent recruitment of Fyn. The SAP (SH2D1A) gene is located on the X chromosome and is responsible for X-linked lymphoproliferative disease, characterized by higher susceptibility to Epstein-Barr virus infection. The SAP-mediated signal is not only essential for the development of NKT cells, i.e. unconventional CD1d-restricted T cells with invariant Valpha14 T cell receptors, but also for the regulation of the function of NK cells and conventional T cells. The role of SAP-mediated signaling in the induction of autoimmune diseases has been analyzed using animal models such as lupus, hepatitis, and graft-versus-host disease and is considered important in their pathogenesis in humans. In this review we highlight the current findings on SAP-mediated signaling in hematopoietic cells and discuss its importance in autoimmune diseases and immunological disorders.


Subject(s)
Antigens, CD/metabolism , Autoimmune Diseases/immunology , Epstein-Barr Virus Infections/immunology , Intracellular Signaling Peptides and Proteins/immunology , Lymphoproliferative Disorders/immunology , Receptors, Cell Surface/metabolism , Animals , Antigens, CD/immunology , Autoimmune Diseases/etiology , Humans , Intracellular Signaling Peptides and Proteins/genetics , Intracellular Signaling Peptides and Proteins/metabolism , Killer Cells, Natural/immunology , Lymphoproliferative Disorders/etiology , Lymphoproliferative Disorders/genetics , Mice , Natural Killer T-Cells/immunology , Receptors, Cell Surface/immunology , Signal Transduction , Signaling Lymphocytic Activation Molecule Associated Protein , Signaling Lymphocytic Activation Molecule Family Member 1 , T-Lymphocytes/immunology
20.
Immunology ; 128(1 Suppl): e151-8, 2009 Sep.
Article in English | MEDLINE | ID: mdl-18800987

ABSTRACT

Concanavalin A (Con A)-induced hepatitis is a T-cell-mediated murine experimental model of autoimmune hepatitis. Mice lacking Valpha14 NKT cells were found to be less sensitive to this hepatitis and the MRL/Mp-Fas(lpr/lpr) (MRL/lpr; i.e. Fas deficient) mice were also less sensitive. We report herein that MRL/Mp-Fas(lpr/lpr)-Sap(rpl/-) (MRL/lpr/rpl) mice lack Valpha14 NKT cells and are deficient in the Fas antigen but sensitive to Con A-induced hepatitis. The signaling lymphocytic activation molecule (SLAM)-associated protein (SAP) is an adaptor molecule containing a Src homology 2 (SH2) domain. We previously reported new mutant mice found among MRL/lpr mice and revealed that SAP deficiency led to the regression of autoimmune phenotypes in mutant MRL/lpr/rpl mice. It was also revealed that CD4(+) and CD8(+) T cells were effector cells and that blockade of 2B4, one of the SLAM family receptors, inhibited the induction of hepatitis in MRL/lpr/rpl mice. These data suggest that signals mediated by molecules other than SAP from 2B4 in T cells played important roles in the induction of hepatitis in MRL/lpr/rpl mice.


Subject(s)
Antigens, CD/immunology , Hepatitis, Autoimmune/immunology , Natural Killer T-Cells/immunology , Receptors, Immunologic/immunology , fas Receptor/immunology , Animals , Antibodies, Monoclonal/immunology , CD4-Positive T-Lymphocytes/drug effects , CD4-Positive T-Lymphocytes/immunology , CD8-Positive T-Lymphocytes/drug effects , CD8-Positive T-Lymphocytes/immunology , Concanavalin A/administration & dosage , Concanavalin A/pharmacology , Disease Models, Animal , Immunologic Factors/pharmacology , Interleukin-4/immunology , Interleukin-4/metabolism , Mice , Mice, Inbred MRL lpr , Mice, Mutant Strains , Mitogens/administration & dosage , Mitogens/pharmacology , Signal Transduction/immunology , Signaling Lymphocytic Activation Molecule Family , fas Receptor/genetics
SELECTION OF CITATIONS
SEARCH DETAIL
...