Journal ArticleJ Clin Immunol · April 27, 2024
PURPOSE: Patients with adenosine deaminase 1 deficient severe combined immunodeficiency (ADA-SCID) are initially treated with enzyme replacement therapy (ERT) with polyethylene glycol-modified (PEGylated) ADA while awaiting definitive treatment with hemato ...
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Journal ArticleFront Pediatr · 2024
We report the case of a 1-week-old male born full-term, who had two inconclusive severe combined immunodeficiency (SCID) newborn screens and developed scalp cellulitis and Escherichia coli bacteremia. He did not pass early confirmatory hearing screens. Ini ...
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Journal ArticleFront Pediatr · 2024
A 20-year-old male patient with a history of celiac disease came to medical attention after developing profound fatigue and pancytopenia. Evaluation demonstrated pan-hypogammaglobulinemia. There was no history of significant clinical infections. Bone marro ...
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Journal ArticleFront Immunol · 2023
INTRODUCTION: Factors influencing vaccine immune priming in the first year of life involve both innate and adaptive immunity but there are gaps in understanding how these factors sustain vaccine antibody levels in healthy infants. The hypothesis was that b ...
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Journal ArticleAutoimmunity · December 2022
Antagonism of the neonatal Fc receptor (FcRn) by efgartigimod has been studied in several autoimmune diseases mediated by immunoglobulin G (IgG) as a therapeutic approach to remove pathogenic IgGs. Whereas reduction of pathogenic titres has demonstrated ef ...
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Journal ArticleNature immunology · August 2022
The recombination-activating genes (RAG) 1 and 2 are indispensable for diversifying the primary B cell receptor repertoire and pruning self-reactive clones via receptor editing in the bone marrow; however, the impact of RAG1/RAG2 on peripheral tolerance is ...
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Journal ArticleRetrovirology · May 31, 2022
BACKGROUND: Marijuana's putative anti-inflammatory properties may benefit HIV-associated comorbidities. How recreational marijuana use affects gene expression in peripheral blood cells (PBC) among youth with HIV-1 (YWH) is unknown. APPROACH: YWH with defin ...
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Journal ArticleJ Clin Immunol · October 2021
PURPOSE: Adenosine deaminase (ADA) deficiency causes severe combined immunodeficiency (SCID) through an accumulation of toxic metabolites within lymphocytes. Recently, ADA deficiency has been successfully treated using lentiviral-transduced autologous CD34 ...
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Journal ArticleJ Acquir Immune Defic Syndr · June 1, 2021
BACKGROUND: Depression and neurocognitive impairment are highly prevalent among persons living with HIV and associated with poorer clinical outcomes; however, longitudinal studies of depression-neurocognition relationships in youth living with HIV (YLWH), ...
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Journal ArticleNat Commun · April 1, 2021
Mucosal-associated invariant T (MAIT) cells have important functions in immune responses against pathogens and in diseases, but mechanisms controlling MAIT cell development and effector lineage differentiation remain unclear. Here, we report that IL-2/IL-1 ...
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Journal ArticleAmyotroph Lateral Scler Frontotemporal Degener · February 2021
Amyotrophic lateral sclerosis (ALS) can have marked phenotypic variability. To date, no biomarker explains this variability. This study tested the hypothesis that immunoglobulin A (IgA) levels might help explain the variability seen in ALS clinical present ...
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Journal ArticleFront Immunol · 2021
Expression of tissue-restricted antigens (TRAs) in thymic epithelial cells (TECs) ensures negative selection of highly self-reactive T cells to establish central tolerance. Whether some of these TRAs could exert their canonical biological functions to shap ...
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Journal ArticleFront Immunol · 2021
T Follicular helper (Tfh) cells promote germinal center (GC) B cell responses to develop effective humoral immunity against pathogens. However, dysregulated Tfh cells can also trigger autoantibody production and the development of autoimmune diseases. We r ...
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Journal ArticleEur J Immunol · November 2020
Diacylglycerol kinases (DGKs) play important roles in restraining diacylglycerol (DAG)-mediated signaling. Within the DGK family, the ζ isoform appears to be the most important isoform in T cells for controlling their development and function. DGKζ has bee ...
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Journal ArticleFront Immunol · 2020
Immune tolerance induction (ITI) with a short-course of rituximab, methotrexate, and/or IVIG in the enzyme replacement therapy (ERT)-naïve setting has prolonged survival and improved clinical outcomes in patients with infantile Pompe disease (IPD) lacking ...
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Journal ArticleFront Immunol · 2020
Vasculitis can be a life-threatening complication associated with high mortality and morbidity among patients with primary immunodeficiencies (PIDs), including variants of severe and combined immunodeficiencies ((S)CID). Our understanding of vasculitis in ...
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Journal ArticleImmunotherapy · November 2019
Aim: This prospective, Phase III study assessed the pharmacokinetics (PK), safety and tolerability of immune globulin subcutaneous, human - klhw 20% solution (IGSC-C 20%) in participants with primary humoral immunodeficiency (PI), compared with immune glob ...
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Journal ArticleAm J Hum Genet · September 5, 2019
FOXN1 is the master regulatory gene of thymic epithelium development. FOXN1 deficiency leads to thymic aplasia, alopecia, and nail dystrophy, accounting for the nude/severe combined immunodeficiency (nu/SCID) phenotype in humans and mice. We identified sev ...
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Journal ArticleAIDS · September 2, 2019
OBJECTIVE: HIV-associated neurocognitive disorders persist despite early antiretroviral therapy (ART) and optimal viral suppression. We examined the relationship between immunopathogenesis driven by various pathways of immune activation and discrete neuroc ...
Full textLink to itemCite
Journal ArticleJ Clin Immunol · April 27, 2024
PURPOSE: Patients with adenosine deaminase 1 deficient severe combined immunodeficiency (ADA-SCID) are initially treated with enzyme replacement therapy (ERT) with polyethylene glycol-modified (PEGylated) ADA while awaiting definitive treatment with hemato ...
Full textLink to itemCite
Journal ArticleFront Pediatr · 2024
We report the case of a 1-week-old male born full-term, who had two inconclusive severe combined immunodeficiency (SCID) newborn screens and developed scalp cellulitis and Escherichia coli bacteremia. He did not pass early confirmatory hearing screens. Ini ...
Full textOpen AccessLink to itemCite
Journal ArticleFront Pediatr · 2024
A 20-year-old male patient with a history of celiac disease came to medical attention after developing profound fatigue and pancytopenia. Evaluation demonstrated pan-hypogammaglobulinemia. There was no history of significant clinical infections. Bone marro ...
Full textLink to itemCite
Journal ArticleFront Immunol · 2023
INTRODUCTION: Factors influencing vaccine immune priming in the first year of life involve both innate and adaptive immunity but there are gaps in understanding how these factors sustain vaccine antibody levels in healthy infants. The hypothesis was that b ...
Full textLink to itemCite
Journal ArticleAutoimmunity · December 2022
Antagonism of the neonatal Fc receptor (FcRn) by efgartigimod has been studied in several autoimmune diseases mediated by immunoglobulin G (IgG) as a therapeutic approach to remove pathogenic IgGs. Whereas reduction of pathogenic titres has demonstrated ef ...
Full textLink to itemCite
Journal ArticleNature immunology · August 2022
The recombination-activating genes (RAG) 1 and 2 are indispensable for diversifying the primary B cell receptor repertoire and pruning self-reactive clones via receptor editing in the bone marrow; however, the impact of RAG1/RAG2 on peripheral tolerance is ...
Full textCite
Journal ArticleRetrovirology · May 31, 2022
BACKGROUND: Marijuana's putative anti-inflammatory properties may benefit HIV-associated comorbidities. How recreational marijuana use affects gene expression in peripheral blood cells (PBC) among youth with HIV-1 (YWH) is unknown. APPROACH: YWH with defin ...
Full textOpen AccessLink to itemCite
Journal ArticleJ Clin Immunol · October 2021
PURPOSE: Adenosine deaminase (ADA) deficiency causes severe combined immunodeficiency (SCID) through an accumulation of toxic metabolites within lymphocytes. Recently, ADA deficiency has been successfully treated using lentiviral-transduced autologous CD34 ...
Full textLink to itemCite
Journal ArticleJ Acquir Immune Defic Syndr · June 1, 2021
BACKGROUND: Depression and neurocognitive impairment are highly prevalent among persons living with HIV and associated with poorer clinical outcomes; however, longitudinal studies of depression-neurocognition relationships in youth living with HIV (YLWH), ...
Full textLink to itemCite
Journal ArticleNat Commun · April 1, 2021
Mucosal-associated invariant T (MAIT) cells have important functions in immune responses against pathogens and in diseases, but mechanisms controlling MAIT cell development and effector lineage differentiation remain unclear. Here, we report that IL-2/IL-1 ...
Full textOpen AccessLink to itemCite
Journal ArticleAmyotroph Lateral Scler Frontotemporal Degener · February 2021
Amyotrophic lateral sclerosis (ALS) can have marked phenotypic variability. To date, no biomarker explains this variability. This study tested the hypothesis that immunoglobulin A (IgA) levels might help explain the variability seen in ALS clinical present ...
Full textLink to itemCite
Journal ArticleFront Immunol · 2021
Expression of tissue-restricted antigens (TRAs) in thymic epithelial cells (TECs) ensures negative selection of highly self-reactive T cells to establish central tolerance. Whether some of these TRAs could exert their canonical biological functions to shap ...
Full textLink to itemCite
Journal ArticleFront Immunol · 2021
T Follicular helper (Tfh) cells promote germinal center (GC) B cell responses to develop effective humoral immunity against pathogens. However, dysregulated Tfh cells can also trigger autoantibody production and the development of autoimmune diseases. We r ...
Full textLink to itemCite
Journal ArticleEur J Immunol · November 2020
Diacylglycerol kinases (DGKs) play important roles in restraining diacylglycerol (DAG)-mediated signaling. Within the DGK family, the ζ isoform appears to be the most important isoform in T cells for controlling their development and function. DGKζ has bee ...
Full textLink to itemCite
Journal ArticleFront Immunol · 2020
Immune tolerance induction (ITI) with a short-course of rituximab, methotrexate, and/or IVIG in the enzyme replacement therapy (ERT)-naïve setting has prolonged survival and improved clinical outcomes in patients with infantile Pompe disease (IPD) lacking ...
Full textLink to itemCite
Journal ArticleFront Immunol · 2020
Vasculitis can be a life-threatening complication associated with high mortality and morbidity among patients with primary immunodeficiencies (PIDs), including variants of severe and combined immunodeficiencies ((S)CID). Our understanding of vasculitis in ...
Full textLink to itemCite
Journal ArticleImmunotherapy · November 2019
Aim: This prospective, Phase III study assessed the pharmacokinetics (PK), safety and tolerability of immune globulin subcutaneous, human - klhw 20% solution (IGSC-C 20%) in participants with primary humoral immunodeficiency (PI), compared with immune glob ...
Full textLink to itemCite
Journal ArticleAm J Hum Genet · September 5, 2019
FOXN1 is the master regulatory gene of thymic epithelium development. FOXN1 deficiency leads to thymic aplasia, alopecia, and nail dystrophy, accounting for the nude/severe combined immunodeficiency (nu/SCID) phenotype in humans and mice. We identified sev ...
Full textLink to itemCite
Journal ArticleAIDS · September 2, 2019
OBJECTIVE: HIV-associated neurocognitive disorders persist despite early antiretroviral therapy (ART) and optimal viral suppression. We examined the relationship between immunopathogenesis driven by various pathways of immune activation and discrete neuroc ...
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Journal ArticleAIDS Res Hum Retroviruses · August 2019
Both HIV infection and tenofovir disoproxil fumarate (TDF) treatment adversely impact bone metabolism and may lead to osteopenia, which has critical implications for youth with HIV (YWH). This study evaluates changes in the biomarkers of bone metabolism an ...
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Journal ArticleJ Allergy Clin Immunol Pract · 2019
BACKGROUND: Although autoimmunity and hyperinflammation secondary to recombination activating gene (RAG) deficiency have been associated with delayed diagnosis and even death, our current understanding is limited primarily to small case series. OBJECTIVE: ...
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Journal ArticleJ Clin Virol · May 2018
BACKGROUND: Human immunodeficiency virus (HIV) infection is associated with chronic immune activation, and concurrent sexually transmitted infections (STIs) may increase immune activation. OBJECTIVES: Because HIV-infected youth are at high risk of STIs and ...
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Journal ArticleJ Leukoc Biol · April 2018
OBJECTIVE: To define inflammatory pathways in youth living with HIV infection (YLWH), assessments of biomarkers associated with lymphocyte and macrophage activation, vascular injury, or bone metabolism were performed in YLWH in comparison with healthy cont ...
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Journal ArticleJAMA Pediatr · July 1, 2017
IMPORTANCE: Establishment of the infant microbiome has lifelong implications on health and immunity. Gut microbiota of breastfed compared with nonbreastfed individuals differ during infancy as well as into adulthood. Breast milk contains a diverse populati ...
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Journal ArticleAIDS Res Hum Retroviruses · July 2017
Macrophages play important roles in HIV-1 pathogenesis as targets for viral replication and mediators of chronic inflammation. Similar to IFNγ-priming, HIV-1 primes macrophages, resulting in hyperresponsiveness to subsequent toll-like receptor (TLR) stimul ...
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Journal ArticleJ Acquir Immune Defic Syndr · June 1, 2017
We compared antibody responses of HIV-infected young women to the human papillomavirus (HPV) 6, 11, 16, and 18 vaccine using total immunoglobulin (Ig) G Luminex immunoassay (LIA) and competitive Luminex immunoassay (cLIA) assays. HPV18 seropositivity after ...
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Journal ArticleAm J Med Genet A · May 2017
Immunodysregulation, Polyendocrinopathy, Enteropathy, X-linked (IPEX) syndrome is a rare, X-linked recessive disease that affects regulatory T cells (Tregs) resulting in diarrhea, enteropathy, eczema, and insulin-dependent diabetes mellitus. IPEX syndrome ...
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Journal ArticleJ Allergy Clin Immunol · April 2017
BACKGROUND: X-linked hyper-IgM syndrome (XHIGM) is a primary immunodeficiency with high morbidity and mortality compared with those seen in healthy subjects. Hematopoietic cell transplantation (HCT) has been considered a curative therapy, but the procedure ...
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Journal ArticleArthritis Rheumatol · January 2017
OBJECTIVE: Regulatory B cells that inhibit immune responses through interleukin-10 (IL-10) secretion (B10 cells) have been characterized in adult subjects with autoimmune disease. The aim of this study was to characterize B10 cells in individuals across th ...
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Journal ArticleJ Acquir Immune Defic Syndr · August 1, 2016
Accumulating evidence suggests that rates of low bone mass are greater in HIV-infected males than females. Of 11 biomarkers assessed by sex and HIV-status, HIV-infected males had increased levels of soluble CD14 which inversely correlated with bone mineral ...
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Journal ArticleJ Clin Immunol · August 2016
PURPOSE: The previous studies with Flebogamma(®) 5 % DIF intravenous immunoglobulin (IVIG) contained insufficient numbers of pediatric subjects to fully warrant a pediatric indication by the FDA. The objective of this study was to evaluate the efficacy, sa ...
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Journal ArticleJ Neurovirol · April 2016
Although youth living with behaviorally acquired HIV (YLWH) are at risk for cognitive impairments, the relationship of impairments to HIV and potential to improve with antiretroviral therapy (ART) are unclear. This prospective observational study was desig ...
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Journal ArticleClin Exp Immunol · September 2015
Multiple subcutaneous immunoglobulin (SCIG) products are available to treat primary antibody deficiency (PAD). The efficacy and tolerability of 16% SCIG (Vivaglobin(®) ) was compared with 20% SCIG (Hizentra(®) ) in PAD subjects. The study was a prospective ...
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Journal ArticleBMC Immunol · July 24, 2015
BACKGROUND: Systemic immune activation (inflammation) and immunosenescence develop in some people with advancing age. This process, known as "inflamm-aging," is associated with physical frailty and sarcopenia. Meanwhile, successful antiretroviral therapy h ...
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Journal ArticleJournal of Inflammation (United Kingdom) · June 12, 2015
Background: Immunomodulatory effects in humans of Δ9-Tetrahydrocannabinol (THC), the psychoactive component of marijuana are controversial. Tissue factor (TF), the activator of the extrinsic coagulation cascade, is increased on circulating activated monocy ...
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Journal ArticleJ Acquir Immune Defic Syndr · May 1, 2015
BACKGROUND: Measures of immune outcomes in youth who initiate combination antiretroviral therapy (cART) early in HIV infection are limited. DESIGN: Adolescent Trials Network 061 examined changes over 48 weeks of cART in T-cell subsets and markers of T-cell ...
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Journal ArticleAnn Otol Rhinol Laryngol · February 2015
BACKGROUND: Mannose-binding lectin (MBL) is a protein produced by the liver that participates in innate immunity by tagging the surface of microbes for opsonization. Mannose-binding lectin deficiency is present in 7% of the population and has been implicat ...
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Journal ArticleJ Neuroimmune Pharmacol · June 2014
The major psychoactive component of marijuana, Δ(9)-tetrahydrocannabinol (THC), also acts to suppress inflammatory responses. Receptors for THC, CB1, CB2, and GPR55, are differentially expressed on multiple cell types including monocytes and macrophages, w ...
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Journal ArticleJ Clin Immunol · May 2014
Primary immunodeficiencies are intrinsic defects in the immune system that result in a predisposition to infection and are frequently accompanied by a propensity to autoimmunity and/or immunedysregulation. Primary immunodeficiencies can be divided into inn ...
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Journal ArticleDrug Alcohol Depend · January 1, 2014
BACKGROUND: Substance use by youth living with HIV (YLWH) is a concern, given potential interactions with virus-associated immune suppression and adverse effects on risk behaviors, neurocognition, and adherence. Self-report substance use measures provide e ...
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Journal ArticleJ Allergy Clin Immunol Pract · 2014
In the United States, newborn screening (NBS) is currently recommended for identification of 31 debilitating and potentially fatal conditions. However, individual states determine which of the recommended conditions are screened. The addition of severe com ...
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Journal ArticlePediatr Res · January 2014
BACKGROUND: Lipopolysaccharide (LPS), an endotoxin of Gram-negative bacteria, causes preterm birth in animals and has been implicated as a factor triggering preterm labor and systemic complications in humans. Little is known regarding LPS in the cord blood ...
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Journal ArticleJ Clin Immunol · January 2014
A 16-year old boy with chronic granulomatous disease (CGD) developed Psychrobacter immobilis septicemia during a course of fulminant hepatic failure. The patient died despite aggressive management with antimicrobials and corticosteroids. While Psychrobacte ...
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Journal ArticleFrontiers in Immunology · December 19, 2013
Background: HIV-1 infection or systemic lupus erythematosus (SLE) disrupt B cell homeostasis, reduce memory B cells, and impair function of IgG and IgM antibodies. Objective: To determine how disturbances in B cell populations producing polyclonal antibodi ...
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Journal ArticleJ Virol · September 2013
Anti-human immunodeficiency virus (HIV) cytotoxic T lymphocyte (CTL)-associated epitopes, evolutionarily conserved on both HIV type 1 (HIV-1) and feline immunodeficiency virus (FIV) reverse transcriptases (RT), were identified using gamma interferon (IFN-γ ...
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Journal ArticleJ Clin Immunol · July 2013
PURPOSE: US licensing studies of subcutaneous IgG (SCIG) calculate dose adjustments necessary to achieve area under the curve (AUC) of serum IgG vs. time on SCIG that is non-inferior to that on intravenous IgG (IVIG), within the FDA-set limit of ±20%. The ...
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Journal ArticleAIDS Res Hum Retroviruses · March 2013
Cardiovascular disease (CVD) biomarkers were examined in a cohort of HIV-infected and HIV-uninfected adolescents who participated in Adolescent Trials Network study 083 utilizing samples from the Reaching for Excellence in Adolescent Care cohort, a longitu ...
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Journal ArticleRetrovirology · December 17, 2012
BACKGROUND: Deep sequencing provides the basis for analysis of biodiversity of taxonomically similar organisms in an environment. While extensively applied to microbiome studies, population genetics studies of viruses are limited. To define the scope of HI ...
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Journal ArticleJ Leukoc Biol · October 2012
The HIV-1 PI NFV has off-target effects upon host enzymes, including inhibition of the 20S proteasome, resulting in activation of PP1. HIV-1-associated monocyte/macrophage activation, in part a result of systemically elevated levels of microbial products i ...
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Journal ArticleClin Infect Dis · August 2012
BACKGROUND: Peak bone mass is achieved in adolescence/early adulthood and is the key determinant of bone mass in adulthood. We evaluated the association of bone mass with human immunodeficiency virus (HIV) infection and antiretroviral therapy (ART) during ...
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Journal ArticleCardiol Young · December 2011
Highly sensitised children in need of cardiac transplantation have overall poor outcomes because of increased risk for dysfunction of the cardiac allograft, acute cellular and antibody-mediated rejection, and vasculopathy of the cardiac allograft. Cardiopu ...
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Journal ArticleJ Leukoc Biol · October 2011
The thymus harbors HIV-1 and supports its replication. Treatment with PI-containing ART restores thymic output of naïve T cells. This study demonstrates that CXCR4-using WT viruses are more sensitive to PI in fetal thymcocytes than mature T cells with aver ...
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Journal ArticleAdv Ther · July 2011
To reduce the risk of infection in adults and children with primary immunodeficiencies, replacement therapy with IgG, which can be administered to patients intravenously or subcutaneously, is required. Although intravenous administration of IgG (IVIG) has ...
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Journal ArticleJ Allergy Clin Immunol · June 2011
BACKGROUND: Common variable immunodeficiency (CVID) is a heterogeneous immune defect characterized by hypogammaglobulinemia, failure of specific antibody production, susceptibility to infections, and an array of comorbidities. OBJECTIVE: To address the und ...
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Journal ArticleImmunol Lett · August 16, 2010
We previously described a patient with a history of frequent life-threatening pneumonias, infections with bacterial pathogens, interleukin 1 receptor-associated kinase 4 deficiency, and failure to maintain antibody titers to polysaccharide antigens or to a ...
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Journal ArticleAIDS · June 1, 2010
OBJECTIVE: HIV-1 replication and microbial translocation occur concomitant with systemic immune activation. This study delineates mechanisms of immune activation and CD4 T-cell decline in pediatric HIV-1 infection. DESIGN: Cross-sectional and longitudinal ...
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Journal ArticleJ Clin Immunol · May 2010
PURPOSE: The tolerability of L-proline-stabilized Privigen, a new 10% liquid immunoglobulin for intravenous administration, was assessed at high infusion rates in a Phase III, open-label, single-arm, multicenter study in 45 patients with primary immune def ...
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Journal ArticleCell Immunol · 2010
The objective was to assess outcomes of IFNgamma-priming upon macrophage activation by the synovial macromolecule high molecular weight hyaluronan [HMW-HA] in the context of rheumatoid arthritis inflammation. Human macrophages primed by IFNgamma and activa ...
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Journal Article · January 1, 2010
Significant progress has been made towards the understanding of the clinical implications of the immature neonatal immune system. With the increasing survival of extremely premature infants, neonatologists and other physicians caring for these patients mus ...
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Journal ArticleClin Infect Dis · January 1, 2010
BACKGROUND: The goal of this study was to determine the nature and prevalence of abnormalities in lipids, glucose metabolism, and body composition in behaviorally human immunodeficiency virus (HIV)-infected young women and the relationship of these abnorma ...
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Journal ArticleClin Vaccine Immunol · September 2009
Human immunodeficiency virus (HIV) type 1 infection perturbs the T-cell receptor (TCR) Vbeta repertoire. The TCR CDR3 length diversity of individual Vbeta families was examined within CD45RA and CD45RO CD4 T cells to assess the impact of the virus on clona ...
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Journal ArticleAIDS · August 24, 2009
OBJECTIVE: To identify novel viral determinants in HIV-1 protease, Gag, and envelope V3 that relate to outcomes to initial protease inhibitor-based antiretroviral therapy. DESIGN: A longitudinal cohort study of protease inhibitor-naive, HIV-infected indivi ...
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Journal ArticleEur J Pharm Sci · June 28, 2009
Intravenous immunoglobulin (IVIg) is used in treating immunodeficiencies and autoimmune or inflammatory disorders. As manufacturing processes and storage can alter IgG molecules, pharmacokinetic assessments are important for new preparations. Thus, we stud ...
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Journal ArticlePediatric Asthma, Allergy and Immunology · June 1, 2009
The objective of this prospective, open-label, single-arm, Phase III study was to assess the efficacy and safety of Privigen ®, the first 10% liquid intravenous immunoglobulin stabilized with L-proline, in patients with primary immunodeficiency. As part of ...
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Journal ArticleAIDS Res Hum Retroviruses · June 2009
This was a proof-of-principle study to evaluate the impact of short cycle therapy (SCT; 4 days on/3 days off) in adolescents and young adults with good viral suppression on a protease inhibitor-based antiretroviral regimen. Subjects were recruited by the A ...
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Journal ArticleClin Pharmacol Ther · April 2009
The pharmacokinetics of abacavir and its metabolites were investigated in 30 human immunodeficiency virus (HIV)-infected adolescents and young adults 13-25 years of age, equally divided into two groups: <18 years of age and >or=18 years of age. All the sub ...
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Journal ArticleJ Pediatr Psychol · March 2009
OBJECTIVE: To evaluate the relationship between cognitive functioning and medication adherence in children and adolescents with perinatally acquired HIV infection. METHODS: Children and adolescents, ages 3-18 (N = 1,429), received a cognitive evaluation an ...
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Journal ArticleImmunol Res · 2009
The objective of this study is to determine if immune reconstitution of FOXP3+ T regulatory cells correlates with clinical improvement of IPEX syndrome following allogeneic hematopoietic stem cell transplant. An 8-months-old male infant with a mutation in ...
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Journal ArticleVirology · September 1, 2008
Regions of HIV-1 gag between p2 and p6(Gag)/p6(Pol), in addition to protease (PR), develop genetic diversity in HIV-1 infected individuals who fail to suppress virus replication by combination protease inhibitor (PI) therapy. To elucidate functional conseq ...
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Journal ArticleJ Allergy Clin Immunol · July 2008
BACKGROUND: HIV infection decreases thymic output and induces chronic T-cell activation. OBJECTIVE: To examine the reconstitution of naive and activated T cells. METHODS: Extended immune phenotyping of CD4(+) and CD8(+) T-cell subsets was combined with T-c ...
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Journal ArticleMar Drugs · June 10, 2008
Harmful algal blooms (HABs) of the toxic dinoflagellate, Karenia brevis, produce red tide toxins, or brevetoxins. Significant health effects associated with red tide toxin exposure have been reported in sea life and in humans, with brevetoxins documented w ...
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Journal ArticleImmunol Res · 2008
Many HIV-infected children treated with protease inhibitors (PI) reconstitute immunity despite viral breakthrough predicting disease progression. We studied a unique cohort of PI treated children with advanced disease who demonstrated sustained CD4 T cell ...
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Journal ArticlePLoS One · 2008
BACKGROUND: Macrophages provide an interface between innate and adaptive immunity and are important long-lived reservoirs for Human Immunodeficiency Virus Type-1 (HIV-1). Multiple genetic networks involved in regulating signal transduction cascades and imm ...
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Journal ArticlePLoS One · September 26, 2007
BACKGROUND: During HIV-1 infection coreceptor switch from CCR5- (R5)- to CXCR4 (X4)-using viruses is associated with disease progression. X4 strains of HIV-1 are highly cytopathic to immature thymocytes. Virtually no studies have evaluated the HIV-1 quasis ...
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Chapter · January 1, 2007
Immunodeficiency diseases are a major cause of mortality and morbidity worldwide. Immune deficient states can be subdivided into primary immune deficiency diseases due to a hereditary or intrinsic defect in the immune system, or secondary immune deficiency ...
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Chapter · January 1, 2007
Primary immunodeficiencies can be classified by a variety of criteria, such as the main limb of the immune system affected, the spectrum of infections, their primary or secondary nature, and, in the case of hereditary primary immunodeficiencies, their mech ...
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Chapter · January 1, 2007
Many factors influencing the function of the immune system can lead to variable degrees of immunoincompetence. Infections, exposure to toxic environmental factors, physical trauma, and therapeutic interventions can all be associated with immune dysfunction ...
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Chapter · January 1, 2007
The immune system has evolved to ensure constant surveillance of “nonself” structures. Both T and B lymphocytes have cell-surface receptors that are able to recognize structures not normally presented or expressed by the organism. Once that recognition tak ...
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Journal ArticleJ Allergy Clin Immunol · December 2006
BACKGROUND: Measuring antibody responses to 23-valent pneumococcal polysaccharide vaccines (PPV) is crucial to evaluation of humoral immune function. However, data are limited comparing responses in immunodeficient subjects. OBJECTIVE: A case-controlled st ...
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Journal ArticleJ Pediatr Psychol · October 2006
OBJECTIVE: Examine the 24-hr Recall Interview (24RI) for assessing children's antiretroviral medication adherence. METHODS: Caregivers of 54 children with HIV (aged 2-12 years) completed a clinical adherence interview and the 24RI by telephone. Children's ...
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Journal ArticleAIDS Res Ther · March 31, 2006
BACKGROUND: Co-infections of human immunodeficiency virus (HIV) and Mycobacterium tuberculosis (M. Tb) are steadily increasing and represent a major health crisis in many developing countries. Both pathogens individually stimulate tumor necrosis factor-alp ...
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Journal ArticleJ Virol · November 2005
Host cell range, or tropism, combined with coreceptor usage defines viral phenotypes as macrophage tropic using CCR5 (M-R5), T-cell-line tropic using CXCR4 (T-X4), or dually lymphocyte and macrophage tropic using CXCR4 alone or in combination with CCR5 (D- ...
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Journal ArticleAnn Allergy Asthma Immunol · March 2005
BACKGROUND: Acrodermatitis enteropathica-like eruption (AE) is a distinct rash associated with profound zinc deficiency. It is seen in a variety of conditions but has not been reported as a presentation of food allergy. OBJECTIVE: To report AE as an unusua ...
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Journal Article · January 1, 2005
Introduction Significant progress has been made towards the understanding of the clinical implications of the neonatal immune system and its immaturity. With the increasing survival of extremely premature infants, neonatologists and other physicians caring ...
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Journal ArticleJ Virol · November 2004
Human immunodeficiency virus type 1 (HIV-1) impacts multiple lineages of hematopoietic cells, including lymphocytes and macrophages, either by direct infection or indirectly by perturbations of cell networks, leading to generalized immune deficiency. We de ...
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Journal ArticlePediatrics · November 2004
OBJECTIVE: To evaluate 96-week clinical and immune outcomes to protease inhibitor-containing antiretroviral therapy. METHODS: A prospective study was conducted of 40 human immunodeficiency virus (HIV)-infected children who displayed viral suppression (VS) ...
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Journal ArticleAnn Allergy Asthma Immunol · September 2004
BACKGROUND: Administration of influenza vaccine to human immunodeficiency virus (HIV)-infected children can lead to increased viral load. CCR5 and CXCR4 are known to play an important role in HIV cell entry and viral replication. OBJECTIVE: To determine th ...
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Journal ArticleJ Gerontol Nurs · July 2004
As the human immunodeficiency virus (HIV) epidemic enters its third decade, nurses are caring for increasing numbers of older adults with HIV who are on complicated medication regimens or highly active antiretroviral therapy (HAART). Although HAART has rev ...
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Journal ArticleAnn Allergy Asthma Immunol · May 2004
BACKGROUND: The 23-valent pneumococcal polysaccharide vaccine (23PSV) has been recommended for children infected with human immunodeficiency virus (HIV); however, the efficacy of this vaccination in HIV-infected children undergoing highly active antiretrov ...
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Journal ArticleAIDS Care · April 2004
This paper presents findings of a multi-site study designed to document: (1) caregivers' regimen knowledge; (2) barriers to adherence; and (3) the relationships between adherence, regimen knowledge and barriers. Fifty-one predominantly female, African Amer ...
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Journal ArticleJ Allergy Clin Immunol · April 2004
BACKGROUND: Exacerbation of opportunistic infections in HIV-infected patients shortly after initiation of highly active antiretroviral therapy (HAART) has been named immune restoration disease (IRD). Thus far, IRD has not been reported in children. OBJECTI ...
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Journal ArticleAIDS Res Hum Retroviruses · March 2004
HIV-1 infection alters expression of CCR5 and CXCR4 on CD4 T cells in adults, although an effect by virus on expression of coreceptor genes in pediatric subjects is unknown. We designed an exploratory study to evaluate surface expression of CXCR4 and CCR5 ...
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Journal ArticleJ Leukoc Biol · November 2003
HIV-1 comprises a collection of closely related, but not identical, viruses or quasispecies. Fitness represents a selective advantage for propagation among populations of organisms competing in a particular environment and is an important characteristic of ...
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Journal ArticlePediatr Diabetes · March 2003
METHODS: We examined monocyte prostaglandin synthase 2 (PGS2/COX2) expression in individuals at risk for or with type 1 diabetes including: (i) 58 established type 1 and 2 diabetic patients; (ii) 34 autoantibody positive (AA+) children and adults; (iii) 16 ...
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Journal ArticleJ Leukoc Biol · March 2003
Human immunodeficiency virus type 1 (HIV-1) impacts the activation state of multiple lineages of hematopoietic cells. Chronic HIV-1 infection among individuals with progressive disease can be associated with increased levels of activated signal transducers ...
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Journal ArticleJ Allergy Clin Immunol · February 2003
This review is intended to provide a fundamental perspective on the dynamic interplay between HIV-1 and the immune system, an essential aspect in defining the pathogenesis and treatment of AIDS. HIV-1 infection, the cause of AIDS, is a worldwide pandemic w ...
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Journal ArticleJ Infect Dis · February 1, 2003
Human immunodeficiency virus (HIV) type 1 disrupts the T cell receptor (TCR) variable region (V) beta repertoire in CD8 T cells by impairing thymic capacity and skewing postthymic cellular maturation. The TCR repertoire was examined using spectratyping of ...
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Journal ArticleJ Pediatr · January 2003
OBJECTIVE: To determine zidovudine pharmacokinetics and tolerance in premature human human immunodeficiency virus-exposed infants. STUDY DESIGN: Pediatric AIDS Clinical Trials Group Study 331 was a multicentered prospective, open-label study of the use of ...
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Journal ArticleJ Infect Dis · July 15, 2002
Pediatric AIDS Clinical Trials Group protocol 316 was an international, multicenter, placebo-controlled trial comparing single-dose oral nevirapine (200 mg to mother and 2 mg/kg to infant) with placebo in human immunodeficiency virus (HIV)-infected pregnan ...
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Journal ArticleClin Infect Dis · April 1, 2002
The relative potency and tolerability of multidrug regimens used to treat infants and children infected with human immunodeficiency virus type 1 (HIV-1) are largely unknown. In Pediatric AIDS Clinical Trials Group (PACTG) Protocol 377, 181 infants and chil ...
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Journal ArticleTransplantation · March 27, 2002
Humoral or antibody-mediated rejection in cardiac transplant recipients is mediated by donor-specific cytotoxic antibodies and is histologically defined by linear deposits of immunoglobulin and complement in the myocardial capillaries. Antibody-mediated re ...
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Journal ArticleAIDS Res Hum Retroviruses · March 20, 2002
Non-syncytium-inducing (NSI) strains of HIV-1 prevail among most infected children, including pediatric patients who develop advanced disease, severe immune suppression, and die. A study was designed to address the hypothesis that genotypic and/or phenotyp ...
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Journal ArticleVirology · January 5, 2002
Human immunodeficiency virus type 1 (HIV-1) protease activity is targeted at nine cleavage sites comprising different amino acid sequences in the viral Gag-Pol polyprotein. Amino acid polymorphisms in protease and in regions of Gag, particularly p7(NC) and ...
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Journal ArticleInternational Journal of Pediatric Hematology/Oncology · October 4, 2001
Purpose. Obtain preliminary response, toxicity, and survival data in patients with non-Hodgkin's lymphomas (NHLs) and immunodeficiency syndromes using short-duration chemotherapy, granulocyte colony-stimulating factor (G-CSF), intravenous (IV) immunoglobul ...
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Journal ArticleClin Diagn Lab Immunol · September 2001
Evaluation of the T-cell immune response following primary antigenic challenge with a neoantigen is a critical aspect of assessment of the cellular immune response. While many antigens can be used to accurately assess in vitro T-cell proliferation to a rec ...
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Journal ArticlePediatrics · August 2001
OBJECTIVE: During the 2 decades in which effective antiviral therapies have been available for neonatal herpes simplex virus (HSV) disease, changes have been documented not only in the outcomes of infected infants, but also in the natural history of the di ...
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Journal ArticlePediatrics · August 2001
OBJECTIVE: The objective of this investigation was to establish the safety of high-dose (HD) acyclovir for the treatment of neonatal herpes simplex virus (HSV) disease. In addition, an estimate of therapeutic efficacy was sought, both with respect to morta ...
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Journal ArticleJ Infect Dis · April 1, 2001
Virologic and immunologic responses were examined for 33 human immunodeficiency virus (HIV)-infected children who participated for > or = 96 weeks in a phase 1/2 protocol of 16 weeks of indinavir monotherapy, followed by the addition of zidovudine and lami ...
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Journal ArticleJ Infect Dis · February 15, 2001
Protease genotype, as a variable in outcome to combination therapy for human immunodeficiency virus (HIV) type 1 infection, was evaluated among protease inhibitor-naive children and adolescents who had received extensive treatment with reverse-transcriptas ...
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Journal ArticlePediatrics · January 2001
OBJECTIVES: Abacavir (ABC) is a potent inhibitor of human immunodeficiency virus type 1 (HIV-1) reverse transcriptase. We compared the efficacy, safety, and tolerability of combination therapy with ABC, lamivudine (3TC), and zidovudine (ZDV) versus 3TC and ...
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Journal ArticleClin Diagn Lab Immunol · November 2000
The T-cell receptor (TCR) CDR3 length heterogeneity is formed during recombination of individual Vbeta gene families. We hypothesized that CDR3 length diversity could be used to assess the fundamental differences within the TCR repertoire of CD45RA and CD4 ...
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Journal ArticleJ Med Virol · June 2000
The objective of this study was to identify phenotypic parameters that could distinguish among seemingly homogeneous non-syncytium-inducing (NSI) viruses and that might provide a surrogate marker for clinical progression in pediatric human immunodeficiency ...
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Journal ArticleJ Pediatr · May 1999
OBJECTIVE: To evaluate lymphocyte reconstitution after protease inhibitor therapy in children with human immunodeficiency virus (HIV) infection. STUDY DESIGN: Forty-four HIV-infected children receiving ritonavir monotherapy followed by the addition of zido ...
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Journal ArticlePediatr Infect Dis J · May 1999
BACKGROUND: Dapsone is an alternative drug for Pneumocystis carinii pneumonia (PCP) prophylaxis in individuals intolerant to trimethoprim-sulfamethoxazole (T/S). There are, however, few data on the pharmacokinetics, toxicity or efficacy of dapsone in child ...
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Journal ArticleBlood · May 1, 1999
Telomere shortening may reflect the total number of divisions experienced by a somatic cell and is associated with replicative senescence. We found that the average rate of telomere shortening in peripheral blood mononuclear cells (PBMCs) obtained longitud ...
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Journal ArticlePediatrics · July 1998
BACKGROUND: Indinavir, an inhibitor of the human immunodeficiency virus type 1 (HIV-1) protease, is approved for the treatment of HIV infection in adults when antiretroviral therapy is indicated. We evaluated the safety and pharmacokinetic profile of the i ...
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Journal ArticleJ Virol · June 1998
The stage of differentiation and the lineage of CD4+ cells profoundly affect their susceptibility to infection by human immunodeficiency virus type 1 (HIV-1). While CD4(+) T lymphocytes in patients are readily susceptible to HIV-1 infection, peripheral blo ...
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Journal ArticlePediatrics · March 1998
BACKGROUND: Ritonavir, a potent antiretroviral protease inhibitor, has been approved for the treatment of adults and children with human immunodeficiency virus (HIV) infection. In a phase I/II study, we assessed the safety, tolerability, and pharmacokineti ...
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Journal ArticleAm J Kidney Dis · March 1998
Autoimmune systemic lupus erythematosus (SLE) nephritis is characterized by the influx of mononuclear inflammatory cell infiltrates within the glomeruli and renal interstitium. To evaluate the possibility that intrarenal T cells result from the expansion o ...
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Journal ArticleFASEB Journal · December 1, 1997
Ten symptomatic HIV infected children (CDC stage B or C) with a mean age of 76 months (range 22-142 months) and with normal status in all monitored nutritional parameters consumed 1 can daily of a nutrition supplement drink (Peptamen Junior) for 6 months. ...
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Journal ArticleJ Pediatr · November 1997
Glutamine, described as a "conditionally essential" amino acid for critically ill patients, has not been routinely added to parenteral amino acid formulations for critically ill neonates and is provided in only small quantities by the enteral route when en ...
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Journal ArticleClin Diagn Lab Immunol · November 1997
Cryopreservation is a method commonly used to store human blood samples. We sought to determine if cryopreserved peripheral blood mononuclear cells (PBMC) could be separated effectively into distinct populations by using monoclonal antibodies and immunomag ...
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Journal ArticleJ Pediatr · August 1997
BACKGROUND: Children with human immunodeficiency virus (HIV) infection have an increased susceptibility to severe and unusual infections, malignancies, and disorders characterized by abnormal lymphoproliferation (e.g., lymphoid interstitial pneumonitis). W ...
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Journal ArticleJ Pediatr · June 1997
OBJECTIVE: To determine whether zidovudine, administered to reduce vertical transmission of human immunodeficiency virus type 1 (HIV-1), impacts the level of maternal viral DNA within the lymphocytes of infected pregnant women. STUDY DESIGN: A prospective, ...
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Journal ArticleAm J Kidney Dis · March 1997
Antiphospholipid antibodies (aPL) of various isotypes are known to occur in systemic lupus erythematosus (SLE), but the significance of this finding in the pediatric population remains unclear. Our aim was to determine whether children with lupus nephritis ...
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Journal ArticleAIDS · November 1996
BACKGROUND: CD4+ memory T cells express CD45RO and are the principal viral reservoir in HIV-infected adults. In infants and children, CD45RO T cells comprise the minority of the CD4+ T-cell population. The majority of blood CD4+ T cells are naive, expressi ...
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Journal ArticleSouth Med J · November 1996
Aminoglycosides have been reported to cause tetany by inducing renal wasting of magnesium and secondary hypocalcemia. A 9-year-old girl with AIDS was brought to the emergency department because of tetany. She had just finished a 3-week course of intravenou ...
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Journal ArticleAm J Med Genet · September 6, 1996
We report on two patients with velo-cardio-facial syndrome (VCFS) and juvenile rheumatoid arthritis (JRA). The first, a 9-year-old girl, presented with microcephaly, characteristic face, congenital heart disease, and velopharyngeal insufficiency. Fluoresce ...
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Journal ArticleAIDS Res Hum Retroviruses · August 10, 1996
HIV-1 env gene encodes a multifunctional glycoprotein that is involved in virus infectivity, interactions between the virus and the host immune system, and phenotypic characteristics of virus isolates in culture. A number of Env functions map by genetic an ...
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Journal ArticleJournal of the Florida Medical Association · August 6, 1996
Pediatric HIV-1 infection is acquired most commonly by transmission from mother to child. Transmission varies geographically and ranges between 15% to 50%. Several factors are involved. The mother's immune status and disease stage, characteristics of the i ...
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Journal ArticleVirology · May 15, 1996
Reduced sensitivity of human immunodeficiency virus type 1 (HIV-1) to protease inhibitors is associated with multiple amino acid substitutions in the virus-encoded protease. The combination of changes that contribute to drug resistance is dependent in part ...
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Journal ArticleAdv Dent Res · April 1996
There is a paradoxical relationship between immunodeficiency diseases and autoimmunity. While not all individuals with immunodeficiency develop autoimmunity, nor are all individuals with autoimmunity immunodeficient, defects within certain components of th ...
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Journal ArticleJournal of Investigative Medicine · January 1, 1996
Combined Immunodeficiency due to the selective absence of CD4+ T lymphocytes is a rare form of immunodeficiency disease characterized by low CD4+ T cell number at birth, decreased production of IL-2, elevated CD8+ T cell numbers, poor mitogen induced lymph ...
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Journal ArticleClin Diagn Lab Immunol · November 1995
CD4+ T lymphocytes and monocytes/macrophages are important components of the immune system. Blood monocytes are usually targeted for studies of the human macrophage lineage cells because of their accessibility through blood sampling. Most separation techni ...
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Journal ArticleSouth Med J · October 1995
Hyponatremia has been recognized as a complication in adults with acquired immunodeficiency syndrome (AIDS). We did a retrospective study evaluating the medical records of 86 children (age 4 months to 21 years) with human immunodeficiency virus (HIV-1) inf ...
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Journal ArticleJ Immunol · July 1, 1994
Human X-linked severe combined immunodeficiency disease (SCID) is an immunodeficiency disorder in which T cell development is arrested in the thymic cortex. B lymphocytes in children with X-linked SCID seem to differentiate normally. X-linked SCID is assoc ...
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Journal ArticleJ Clin Invest · January 1994
The extent of nucleotide variation within the HIV-1 env hypervariable domains serves as a marker of virus genotypes within infected individuals and as a means to track transmission of the virus between individuals. We analyzed env V1 and V2 sequences in lo ...
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Journal ArticleJ Virol · July 1993
Multiple targets for immune recognition and cellular tropism are localized to the V1 and V2 hypervariable regions in the amino portion of human immunodeficiency virus type 1 (HIV-1) gp120env. We have assessed genetic diversity in env V1 and V2 hypervariabl ...
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Journal ArticleAm J Dis Child · January 1993
OBJECTIVE: To determine if corticosteroids administered in addition to antimicrobials improve survival in children with acquired immunodeficiency syndrome and severe Pneumocystis carinii pneumonia (PCP). DESIGN: Before-after, nonrandomized, case-comparison ...
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Journal ArticleCell Immunol · August 1992
To determine whether human antibody responses to T cell-independent pneumococcal polysaccharide antigens are derived from CD5+ or CD5- B cells, we utilized an ELISPOT assay to detect individual anti-polysaccharide antibody-secreting cells. Human anti-type ...
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Journal ArticleDiabetes · October 1991
Human CD5+ B lymphocytes produce autoantibodies that bind to self- and exogenous antigens. Extremely high percentages of CD5+ B lymphocytes are present in the fetal and newborn periods, whereas they constitute only a minority of B lymphocytes in healthy ad ...
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Journal ArticleJ Fla Med Assoc · October 1991
Acquired immune deficiency syndrome (AIDS) is caused by infection with the human immunodeficiency virus (HIV), a human retrovirus. The virus infects cells of the immune system by attachment of a glycoprotein viral envelope (gp 120) to a molecule expressed ...
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Journal ArticleJ Fla Med Assoc · October 1991
Human immunodeficiency virus infection is a leading cause of immunodeficiency in children. The epidemic in children parallels that in women since most infected women are in the child-bearing age groups. The risk of vertical transmission of HIV from an infe ...
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Journal ArticleJ Immunol · June 1, 1991
Cytochalasins are known to inhibit or enhance the proliferation of T cells induced by mitogens in a concentration-dependent fashion. To clarify the mechanism by which cytochalasins enhance T cell proliferation, we examined which activation pathways and eve ...
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Journal ArticleCell Immunol · April 1, 1991
Human T cells incubated for 48 hr with Con A suppress B cell Ig production. We sought to define the interactions between CD4+ T cells and CD8+ T cells in the generation of suppressor cell activity following Con A stimulation. Con A-activated CD4+ inducer T ...
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Journal ArticleClin Immunol Immunopathol · June 1990
Selective congenital deficiency of the CD4 inducer T lymphocyte subset is a recently described variant of combined immunodeficiency. To further characterize the cellular and molecular mechanisms which lead to the profound T and B cell immunodeficiency in t ...
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Journal ArticleEur J Immunol · June 1990
Human helper T lymphocytes can be dissected into two functionally distinct subpopulations based on expression of the CD45RA (2H4) or CD45R0 (UCHL-1) surface antigens. While both subpopulations are able to induce equivalent levels of B cell activation and p ...
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Journal ArticleThromb Haemost · October 28, 1987
The use of plasma thrombospondin (TSP) concentration was investigated as an indicator of intravascular platelet activation. Patients (n = 20) with diseases that have known vasculitis were included in the study. The range and the mean of plasma TSP concentr ...
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Journal ArticleBone Marrow Transplant · August 1987
It has previously been demonstrated that graft-versus-host disease can be overcome in patients receiving HLA-mismatched bone marrow transplants by prior in vitro depletion of T lymphocytes from the marrow. In this report we describe the use of monoclonal a ...
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Journal ArticleEur J Pediatr · October 1986
This study was done to determine whether intravenous methylprednisolone therapy given concomitantly with low-dose daily, oral prednisone would be as effective as high-dose daily prednisone in the treatment of patients with active systemic lupus erythematos ...
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