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https://www.readbyqxmd.com/read/27836054/clinical-phenotypes-of-hyper-igm-syndromes
#1
M Teresa de la Morena
The primary immunodeficiency (PID) diseases comprise a heterogeneous group of inherited disorders of immune function. Technical advancements in whole-genome, whole-exome, and RNA-sequencing have seen the explosion of genetic discoveries in the field of PIDs. The present review aims to focus on a group of immunodeficiency disorders associated with elevated levels of IgM (hyper IgM; HIGM) and provides a clinical differential diagnosis. Most patients present for evaluation of immunodeficiency due to recurrent infections, and laboratory studies show either a clear isolated elevation of serum immunoglobulin M (IgM) with low or absent IgG, IgA, and IgE...
November 2016: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/27393102/effect-of-partial-splenic-embolization-on-the-immune-function-of-cirrhosis-patients-with-hypersplenism
#2
Gui-Yun Jin, Chuan-Zhu Lv, Deng Tang, Shao-Wen Cheng, Chao-Qian Li
OBJECTIVE: To discover the effect of partial splenic embolization on the immune function of cirrhotic patients with hypersplenism. METHODS: Patients involved in the study were enrolled and divided into three groups, including control group, experimental group, and complication group. Numbers of CD3(+), CD4(+) and CD8(+) T cells and CD4(+)CD25(+)CDl27(low/-) Treg cells in the peripheral blood of patients before surgery, 1 month, 6 months, 1 year, and 2 years after surgery were analyzed by fluorescence active cell sorting (FACS)...
July 2016: Asian Pacific Journal of Tropical Medicine
https://www.readbyqxmd.com/read/26779698/detection-of-specific-immunoglobulin-e-antibodies-toward-common-airborne-allergens-peanut-wheat-and-latex-in-solvent-detergent-treated-pooled-plasma
#3
Torunn O Apelseth, Venny L Kvalheim, Einar K Kristoffersen
BACKGROUND: Allergic transfusion reactions (ATRs) present with a broad range of symptoms probably caused by mediators released from mast cells and basophil granulocytes upon activation. Passive immunoglobulin (Ig)E sensitization may yield clinical symptoms and positive allergy tests. Unexpected findings of IgE antibodies in pooled solvent/detergent (S/D)-treated plasma (Octaplas, Octapharma) during routine analysis initiated an investigation of serum proteins. STUDY DESIGN AND METHODS: Consecutive batches of S/D-plasma transfused during September 2014 through March 2015 were investigated for IgE, IgG, IgA IgM, C3, C4, haptoglobin, anti-nuclear antibodies (ANAs), and red blood cell (RBC) antibodies...
May 2016: Transfusion
https://www.readbyqxmd.com/read/25876637/-effects-of-astragalus-polysaccharide-on-intestinal-immune-function-of-rats-with-severe-scald-injury
#4
Cuilan Huang, Jianhua Zhan, Jinhua Luo
OBJECTIVE: To observe the effects of astragalus polysaccharide (AP) on the intestinal mucosal morphology, level of secretory IgA (s-IgA) in intestinal mucus, and distribution of T lymphocyte subsets in Peyer's patch in rats with severe scald injury. METHODS: One hundred and thirty SD rats were divided into sham injury group (SI, sham injured, n = 10), scald group (S, n = 30), low dosage group (LD, n = 30), moderate dosage group (MD, n = 30), and high dosage group (HD, n = 30) according to the random number table...
February 2015: Zhonghua Shao Shang za Zhi, Zhonghua Shaoshang Zazhi, Chinese Journal of Burns
https://www.readbyqxmd.com/read/25493386/downregulation-of-tim-3-mrna-expression-in-peripheral-blood-mononuclear-cells-from-patients-with-systemic-lupus-erythematosus
#5
X Z Cai, W Y Huang, Y Qiao, Y Chen, S Y Du, D Chen, S Yu, N Liu, L Y Dou, Y Jiang
The T-cell immunoglobulin and mucin domain (TIM) family is associated with autoimmune diseases, but its expression level in the immune cells of systemic lupus erythematosus (SLE) patients is not known. The aim of this study was to investigate whether the expression of TIM-3 mRNA is associated with pathogenesis of SLE. Quantitative real-time reverse transcription-polymerase chain reaction analysis (qRT-PCR) was used to determine TIM-1, TIM-3, and TIM-4 mRNA expression in peripheral blood mononuclear cells (PBMCs) from 132 patients with SLE and 62 healthy controls...
January 2015: Brazilian Journal of Medical and Biological Research, Revista Brasileira de Pesquisas Médicas e Biológicas
https://www.readbyqxmd.com/read/25370723/reversal-of-immunoglobulin-a-deficiency-in-children
#6
Che Kang Lim, Charlotte Dahle, Kerstin Elvin, Bengt A Andersson, Johan Rönnelid, Erik Melén, Anna Bergström, Lennart Truedsson, Lennart Hammarström
PURPOSE: Immunoglobulin A deficiency (IgAD) is the most common primary immunodeficiency in the general population. It is defined as a serum IgA level below or equal to 0.07 g/l with normal IgM and IgG levels in children over the age of 4. However, a few cases of reversal of IgAD at later ages have been observed previously, especially in pediatric patients. This study aimed at investigating the frequency of reversal in a large cohort of children and young adults in order to evaluate the present definition of IgAD...
January 2015: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/25337827/downregulation-of-tim-3-mrna-expression-in-peripheral-blood-mononuclear-cells-from-patients-with-systemic-lupus-erythematosus
#7
X Z Cai, W Y Huang, Y Qiao, Y Chen, S Y Du, D Chen, S Yu, N Liu, L Y Dou, Y Jiang
The T-cell immunoglobulin and mucin domain (TIM) family is associated with autoimmune diseases, but its expression level in the immune cells of systemic lupus erythematosus (SLE) patients is not known. The aim of this study was to investigate whether the expression of TIM-3 mRNA is associated with pathogenesis of SLE. Quantitative real-time reverse transcription-polymerase chain reaction analysis (qRT-PCR) was used to determine TIM-1, TIM-3, and TIM-4 mRNA expression in peripheral blood mononuclear cells (PBMCs) from 132 patients with SLE and 62 healthy controls...
October 17, 2014: Brazilian Journal of Medical and Biological Research, Revista Brasileira de Pesquisas Médicas e Biológicas
https://www.readbyqxmd.com/read/24652773/investigation-of-the-prevalence-of-antibody-immunodeficiency-in-a-polio-endemic-area-in-india
#8
Madhu C Mohanty, Jagadish M Deshpande
BACKGROUND: Protection against paralytic poliomyelitis is provided mainly by antibody mediated host defense. Despite intensive oral polio vaccine (OPV) immunization campaigns wild poliovirus transmission could not be stopped in Uttar Pradesh (UP) and Bihar states of India by the end of 2010. The objective of our study was to quantitate serum IgG and IgA in children of western UP, India, to determine the prevalence of antibody immunodeficiency. METHODS: A cross-sectional survey for IgG and IgA concentrations in serum samples from healthy children and children with acute flaccid paralysis (AFP), up to the age of 5 years, was performed using sandwich ELISA...
May 2014: Transactions of the Royal Society of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/24584924/abatacept-reduces-levels-of-switched-memory-b-cells-autoantibodies-and-immunoglobulins-in-patients-with-rheumatoid-arthritis
#9
Mirko Scarsi, Lucia Paolini, Doris Ricotta, Antonio Pedrini, Silvia Piantoni, Luigi Caimi, Angela Tincani, Paolo Airò
OBJECTIVE: Abatacept (ABA) is a chimeric molecule, able to block the CD28-mediated costimulatory pathway. To evaluate the hypothesis that, through this mechanism of action, ABA may down-modulate the immune responses of B lymphocytes in rheumatoid arthritis (RA), we investigated the serum levels of immunoglobulins (Ig), free light chains (FLC), anticitrullinated protein antibodies (ACPA), and rheumatoid factor (RF), as well as the number of B lymphocytes differentiated into post-switch memory cells in patients treated with ABA...
April 2014: Journal of Rheumatology
https://www.readbyqxmd.com/read/24463867/-persistent-microhaematuria-with-negative-or-low-proteinuria
#10
REVIEW
Eduardo Gutiérrez, Juan A Moreno, Manuel Praga
The level of proteinuria continues to be the clinical parameter that is best related to the development of long-term renal failure in glomerular pathologies. This quantity is particularly important when we analyse the progression of patients with IgA nephropathy. As such, the natural progression of patients with IgA who clinically present with normal kidney function, microhaematuria and low proteinuria had not been analysed comprehensively until the Spanish multicentre study herein analysed. After studying 141 Caucasian patients with biopsied IgA nephropathy and a "benign" clinical profile and after they were classified histologically in accordance with the new Oxford classification, it could be concluded that the renal prognosis of these patients was excellent...
2014: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
https://www.readbyqxmd.com/read/23414395/maternal-anemia-induces-changes-in-immunological-and-nutritional-components-of-breast-milk
#11
Eduardo Luzía França, Veridiana Assêncio Silva, Rosa Maria Jacinto Volpato, Priscila Assêncio Silva, Maria Fernanda Spegiorini Salla Brune, Adenilda Cristina Honorio-França
OBJECTIVE: The effects of low maternal hemoglobin levels on the immunological and nutritional components of breast milk at different maturation stages were investigated. METHODS: Colostrum, transitional and mature milk were collected from 25 mothers with normal hemoglobin levels (control group) and 18 mothers with hemoglobin levels below 11 g/dL (anemia group). Total protein, antibodies, complement proteins, fat and calorie, lipase, iron, transferrin levels, total iron-binding capacity, latent iron-binding capacity (LIBC) and transferrin saturation index (TSI) were determined...
August 2013: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/23082703/seroprevalence-of-celiac-disease-in-belgian-children-and-adolescents
#12
S Vijgen, P Alliet, P Gillis, P Declercq, A Mewis
BACKGROUND: Celiac Disease (CD) has a high prevalence worldwide. Because the disease may be underdiagnosed in Belgium, we wanted to evaluate the seroprevalence of CD in children and adolescents. PATIENTS AND METHODS: Serum samples from 1159 apparently healthy children and adolescents aged between 1-19 years, were prospectively collected in 2006 by 15 Belgian diagnostic laboratories. In September 2009, all samples were analyzed for human tissue transglutaminase IgA antibodies (IgA tTG) and total IgA levels by, respectively a commercial ELISA and immunonephelometry...
September 2012: Acta Gastro-enterologica Belgica
https://www.readbyqxmd.com/read/21924385/assessment-of-plasmatic-immunoglobulin-g-a-and-m-levels-in-septic-shock-patients
#13
Fabienne Venet, Rémi Gebeile, Julien Bancel, Caroline Guignant, Françoise Poitevin-Later, Christophe Malcus, Alain Lepape, Guillaume Monneret
Polyvalent immunoglobulin (Ig) therapy has been tested as adjunctive treatment in sepsis and septic shock, but its efficacy is still a matter of debate. This has been explained because clinical trials were mostly performed on small numbers of patients. Moreover, the endogenous level of circulating Ig in patients was never taken into account. In this study, plasmatic Ig classes and protein concentrations were measured at Days (D) 1-2, D3-4 and D5-7 in 62 septic shock patients. At D1-2 as well as at D3-4, patients presented with a significant reduction of plasmatic IgG concentrations...
December 2011: International Immunopharmacology
https://www.readbyqxmd.com/read/21122815/immunologic-parameters-and-viral-infections-in-patients-desensitized-with-intravenous-immunoglobulin-and-rituximab
#14
Shili Ge, Andy Pao, Ashley Vo, Nathan Deer, Artur Karasyov, Anna Petrosyan, Joseph Kahwaji, Marina Lukovsky, Ning-Ning Chai, Angela Aguiluz, James Mirocha, Stanley C Jordan, Mieko Toyoda
Desensitization with IVIG and rituximab followed by transplantation with alemtuzumab or daclizumab induction is an effective clinical protocol. Here, we examined the effects of this protocol on immune cell number, T cell function by Cylex ImmuKnow®, CMV-specific CD8+ T cell (CMV-Tc) activity, total and viral-specific immunoglobulin levels and viral infections. In 17 highly HLA-sensitized (HS) patients who received desensitization, CD19+ cells were undetectable immediately after desensitization, while other immune cells were unchanged...
April 15, 2011: Transplant Immunology
https://www.readbyqxmd.com/read/21120559/b-cell-depletion-for-autoimmune-diseases-in-paediatric-patients
#15
Annette F Jansson, Claudia Sengler, Jasmin Kuemmerle-Deschner, Bernd Gruhn, A Birgitta Kranz, Hartwig Lehmann, Daniela Kleinert, Lars Pape, Hermann J Girschick, Ivan Foeldvari, Dieter Haffner, Johannes P Haas, Dagmar Moebius, Dirk Foell, Joachim Peitz, Veit Grote
Data on B cell depletion therapy in severe autoimmune diseases in paediatric patients are very limited. We conducted a retrospective cohort study and recruited patients who were treated with rituximab (RTX) and followed up for at least 6 months through the German societies of paediatric rheumatology and nephrology. The aim was to describe the spectrum of autoimmune disorders for which RTX was used and to describe the applied therapeutic regimens, the observed efficacy, as well as potential immunological side effects...
January 2011: Clinical Rheumatology
https://www.readbyqxmd.com/read/20934629/infants-with-low-immunoglobulin-levels-isolated-low-iga-level-vs-other-immunoglobulin-abnormalities
#16
Alexandra Horwitz, Shiang-Ju Kung, Stephen J McGeady
BACKGROUND: The International Union of Immunological Societies defined transient hypogammaglobulinemia of infancy as decreased IgG and IgA levels. Some others, however, include decreased IgA level alone. We compared infants with decreased levels of IgG and IgA, all isotypes, and IgA alone. OBJECTIVE: To determine whether infants presenting with diminished IgA only differ clinically and in time of immunoglobulin recovery, from those with decreased levels of IgG and IgA, or of all major isotypes...
October 2010: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/20628891/thrombotic-thrombocytopenic-purpura-in-a-patient-with-rapidly-progressive-glomerulonephritis-with-both-anti-glomerular-basement-membrane-antibodies-and-myeloperoxidase-anti-neutrophil-cytoplasmic-antibodies
#17
Hitoshi Watanabe, Wataru Kitagawa, Keisuke Suzuki, Masabumi Yoshino, Ryuhei Aoyama, Naoto Miura, Kazuhiro Nishikawa, Hirokazu Imai
A 61-year-old Japanese woman with rapidly progressive glomerulonephritis exhibited both anti-glomerular basement membrane (GBM) antibodies (920 EU) and myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA; 66 EU). Multiple plasma exchanges with fresh frozen plasma preceded by 500 mg/day intravenous methylprednisolone and 30 mg/day oral prednisolone decreased anti-GBM antibody and MPO-ANCA antibody titers to 106 EU and below 10 EU (normal ranges), respectively. Thrombotic thrombocytopenic purpura (TTP) manifests itself as a moderate decrease in the activity of disintegrin-like and metalloproteinase with thrombospondin type 1 motif, 13 (ADAMTS13) protein levels to 35% of normal; ADAMTS13 deficiency is only symptomatic when levels are less than 50%...
December 2010: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/20367263/recovery-of-humoral-and-cellular-immunities-to-vaccine-preventable-infectious-diseases-in-pediatric-oncology-patients
#18
Frankie Wai Tsoi Cheng, Ting Fan Leung, Paul Kay Sheung Chan, Wing Kwan Leung, Vincent Lee, Ming Kong Shing, Patrick Man Pan Yuen, Chi Kong Li
The recovery of antibodies to various vaccine-preventable infectious diseases, humoral and cellular immunity in pediatric oncology patients were evaluated by a prospective longitudinal study for 18 months. Lymphocyte subset (CD3+, CD4+, CD8+, CD16/56+, CD19+), CD4/CD8 ratio, immunoglobulin levels, antibodies to diphtheria, pertussis, tetanus, hepatitis B, measles, mumps, and rubella were measured serially at 6 months till 18 months after stopping all chemotherapy (including maintenance chemotherapy). Twenty-eight children (hematological malignancies, n = 14; solid tumors, n = 14) were studied...
April 2010: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/20217285/severe-hypogammaglobulinemia-persisting-for-6-years-after-treatment-with-rituximab-combined-chemotherapy-due-to-arrest-of-b-lymphocyte-differentiation-together-with-alteration-of-t-lymphocyte-homeostasis
#19
Erika Irie, Yuko Shirota, Chihiro Suzuki, Yumi Tajima, Kenichi Ishizawa, Junichi Kameoka, Hideo Harigae, Tomonori Ishii
We report a case of prolonged severe hypogammaglobulinemia after rituximab combined chemotherapy for follicular lymphoma. Although the patient's globulin level was within the normal limits before treatment, the level of IgG dropped below 100 mg/dL, and both IgA and IgM became undetectable after treatment, and the levels have shown no changes for 6 years despite recovery of peripheral B cell counts. Phenotypic analysis of B cells revealed a reduction of class-switched CD27+IgM-IgD- memory B cells below 0.5% and overexpression of CD95...
April 2010: International Journal of Hematology
https://www.readbyqxmd.com/read/19781364/clinical-features-and-follow-up-of-chinese-patients-with-symptomatic-hypogammaglobulinemia-in-infancy
#20
Ji-hong Qian, Jian-xing Zhu, Xiao-dong Zhu, Tong-xin Chen
BACKGROUND: Hypogammaglobulinemia is common in infant humoral immunodeficiencies and has complicated causes and outcomes. We aimed to determine the clinical manifestations, immunological changes and outcomes of Shanghai infants with hypogammaglobulinemia. METHODS: Patients under 2 years old, having one or more warning signs of primary immunodeficiency disorders, serum immunoglobulin levels below the lower limit of reference range per age, and with normal numbers for lymphocyte subsets, were analyzed and followed up for 2 to 3 years...
August 20, 2009: Chinese Medical Journal
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