Rapid subcutaneous IgG replacement therapy is effective and safe in children and adults with primary immunodeficiencies--a prospective, multi-national study.

Abstract:

:Sixty patients (16 children, 44 adults) participated in the study aiming at evaluating: (i) IgG levels when switching patients from intravenous IgG (IVIG) infusions in hospital to subcutaneous (SCIG) self-infusions at home using the same cumulative monthly dose, (ii) protections against infections, and (iii) safety of a new, ready-to-use 16% IgG preparation. All children and 33 adults had received IVIG therapy for >6 months at enrolment. Ten adults who had been on SCIG therapy for many years served as controls. Mean serum IgG trough levels increased in the pre-IVIG children from 7.8 to 9.2 g/L (non-inferiority: p < 0.001) and in the adults from 8.6 to 8.9 g/L (non-inferiority: p < 0.001). Totally 114 respiratory tract infections occurred, 90% of them mild. One serious bacterial infection (pneumonia) was reported for one adult. The annualized rate of serious infections was 0.04 episodes/patient. In total 2297 infusions were given and 28 (1%) systemic adverse reactions occurred, none of them severe. Local tissue reactions declined over time, this being particularly distinct after 8 to 10 weeks. In conclusion, the SCIG administration route was safe. High IgG levels were easily maintained resulting in a very good protection against infections.

journal_name

J Clin Immunol

authors

Gardulf A,Nicolay U,Asensio O,Bernatowska E,Böck A,Carvalho BC,Granert C,Haag S,Hernández D,Kiessling P,Kus J,Pons J,Niehues T,Schmidt S,Schulze I,Borte M

doi

10.1007/s10875-006-9002-x

subject

Has Abstract

pub_date

2006-03-01 00:00:00

pages

177-85

issue

2

eissn

0271-9142

issn

1573-2592

journal_volume

26

pub_type

临床试验,杂志文章,多中心研究
  • Correction to: Unusual Severe Seborrheic Dermatitis in Two Siblings with Autosomal Recessive Chronic Granulomatous Disease.

    abstract::The original version of this article contained an error in Fig. 1. The incomplete heredogram mistakenly appeared in panel d. ...

    journal_title:Journal of clinical immunology

    pub_type: 已发布勘误

    doi:10.1007/s10875-019-00701-9

    authors: Zurro NB,de Oliveira Junior EB,França TT,Dantas VM,Tavares de Albuquerque JA,Condino-Neto A

    更新日期:2019-11-01 00:00:00

  • Effect of hyperoxia on pulmonary SIgA and its components, IgA and SC.

    abstract:PURPOSE:Oxygen therapy (hyperoxia) is essential for the treatment of some neonatal critical care conditions. The lung is the primary target for the changes induced by hyperoxia. Secretory immunoglobulin A (SIgA), IgA and secretory component (SC) reflect the local immunity in the respiratory tract induced by hyperoxia. ...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-013-9891-4

    authors: Liu DY,Jiang T,Wang S,Cao X

    更新日期:2013-07-01 00:00:00

  • Primary immunodeficiency diseases in Latin America: first report from eight countries participating in the LAGID. Latin American Group for Primary Immunodeficiency Diseases.

    abstract::The Latin American Group for Primary Immunodeficiencies, formed in 1993, presently includes 12 countries. One goal was to study the frequency of primary immunodeficiencies in various regions of the American continent and to enhance knowledge about these diseases among primary-care physicians, as well as allergist-immu...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1023/a:1023255102170

    authors: Zelazko M,Carneiro-Sampaio M,Cornejo de Luigi M,Garcia de Olarte D,Porras Madrigal O,Berrón Perez R,Cabello A,Rostan MV,Sorensen RU

    更新日期:1998-03-01 00:00:00

  • Early lymphocyte transformation abnormalities in human immunodeficiency virus infection.

    abstract::Two hundred twelve patients with enzyme immunoassay and Western blot confirmation of human immunodeficiency virus (HIV) infection were evaluated with anergy panel, lymphocyte cell surface phenotyping, lymphocyte transformation, and serum immunoglobulins. Mitogen responses were used to develop a lymphocyte transformati...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/BF00916939

    authors: Ridley DJ,Houk RW,Reid MJ,Boswell RN

    更新日期:1989-03-01 00:00:00

  • The transfer of antigen-specific humoral immunity from marrow donors to marrow recipients.

    abstract::This study shows that specific humoral immunity could be transferred from marrow donors to marrow recipients. Peripheral blood lymphocytes from long-term human marrow recipients produced IgG anti-tetanus toxoid antibody after in vitro tetanus toxoid stimulation. Antitetanus toxoid antibody biosynthesis was induced usi...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/BF00915378

    authors: Lum LG,Seigneuret MC,Storb R

    更新日期:1986-09-01 00:00:00

  • The effects of indomethacin and interleukin-2 on the proliferation of lymphocytes from patients with lung cancer.

    abstract::The effects of the addition of indomethacin and interleukin-2 (IL-2) to phytohemagglutinin (PHA)-stimulated lymphocytes from patients with untreated squamous-cell carcinoma of the lung and with chronic obstructive pulmonary disease and from normal individuals were studied. In 13 of 21 patients with lung carcinoma, the...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/BF00915506

    authors: Maca RD,Burford JG,Taylor RT

    更新日期:1985-05-01 00:00:00

  • ANCA-GBM dot-blot: evaluation of an assay in the differential diagnosis of patients presenting with rapidly progressive glomerulonephritis.

    abstract::Rapidly progressive glomerulonephritis (RPGN) is characterized by rapid and progressive loss of renal function and the presence of crescentic glomerulonephritis (CGN). Early diagnosis and appropriate treatment is mandatory to prevent death and/or renal failure. We have evaluated an ANCA-GBM dot-blot diagnostic test in...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1023/B:JOCI.0000029115.79266.ae

    authors: Rutgers A,Damoiseaux J,Roozendaal C,Limburg PC,Stegeman CA,Tervaert JW

    更新日期:2004-07-01 00:00:00

  • How I Manage Natural Killer Cell Deficiency.

    abstract::Natural killer (NK) cell deficiency (NKD) is a subset of primary immunodeficiency disorders (PID) in which an abnormality of NK cells represents a major immunological defect resulting in the patient's clinical immunodeficiency. This is distinct from a much larger group of PIDs that include an NK cell abnormality as a ...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-019-00711-7

    authors: Orange JS

    更新日期:2020-01-01 00:00:00

  • Diagnosis, Treatment and Long-Term Follow Up of Patients with ADA Deficiency: a Single-Center Experience.

    abstract:PURPOSE:We carried out a retrospective analysis of 27 patients with Adenosine Deaminase (ADA) deficiency diagnosed in a single center from 1997 to the 2013, for evaluating whether data regarding types of disease-inducing mutations, biochemical and immunological features as well as clinical outcomes of patients treated ...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-015-0191-z

    authors: Baffelli R,Notarangelo LD,Imberti L,Hershfield MS,Serana F,Santisteban I,Bolda F,Porta F,Lanfranchi A

    更新日期:2015-10-01 00:00:00

  • Complexes of serum amyloid P component and DNA in serum from healthy individuals and systemic lupus erythematosus patients.

    abstract::Serum amyloid P component (SAP) binds in vitro to DNA; based on findings in SAP-deficient mice it was proposed that SAP's role is to handle chromatin and DNA, thereby preventing formation of anti-DNA antibodies. For the first time we have shown the presence of Ca2+-dependent SAP-DNA complexes, measured by ELISA, in se...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1023/a:1026478914129

    authors: Sørensen IJ,Holm Nielsen E,Schrøder L,Voss A,Horváth L,Svehag SE

    更新日期:2000-11-01 00:00:00

  • TLR-mediated B cell defects and IFN-α in common variable immunodeficiency.

    abstract::Common variable immune deficiency (CVID) B cells have impaired responses to TLR7 and TLR9 agonists including poor cell proliferation, loss of cytokine production, and failure to produce IgG or IgA. We show that TLR7- or 9-activated B cells from CVID subjects with >0.5% peripheral isotype-switched CD27(+) B cells (grou...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-011-9602-y

    authors: Yu JE,Zhang L,Radigan L,Sanchez-Ramon S,Cunningham-Rundles C

    更新日期:2012-02-01 00:00:00

  • A novel missense mutation in the nuclear factor-κB essential modulator (NEMO) gene resulting in impaired activation of the NF-κB pathway and a unique clinical phenotype presenting as MRSA subdural empyema.

    abstract:INTRODUCTION:We describe a previously unreported 437 T→G missense mutation producing a V146G substitution in the first coiled-coil (CC1) domain of nuclear factor-κB essential modulator (NEMO) in a 9-month-old boy with ectodermal dysplasia with immunodeficiency who presented with methicillin-resistant Staphylococcus aur...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-010-9445-y

    authors: Devora GA,Sun L,Chen Z,van Oers NS,Hanson EP,Orange JS,de la Morena MT

    更新日期:2010-11-01 00:00:00

  • Proteolytically inactive per a 10 allergen of Periplaneta americana modulates Th2 response and enhances IL-10 in mouse model.

    abstract:BACKGROUND:Purified allergens with reduced IgE reactivity are required to improve the safety and efficacy of allergen-specific immunotherapy (IT). OBJECTIVE:The present study investigates the efficacy of purified cockroach allergen immunotherapy with proteolytically active and inactive Per a 10 in allergic mouse model...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-009-9362-0

    authors: Srivastava D,Mehta AK,Arora N,Gaur SN,Singh BP

    更新日期:2010-05-01 00:00:00

  • Studies on the production of IL-15 in HIV-infected/AIDS patients.

    abstract::IL-15 is essential for the development and differentiation of NK cells. It selectively induces proliferation of CD8+ memory T lymphocytes. Despite its importance in both innate and adaptive immune responses, little is known about its production in HIV-infected persons. We report here that IL-15 levels are significantl...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1023/a:1022568626500

    authors: Ahmad R,Sindhu ST,Toma E,Morisset R,Ahmad A

    更新日期:2003-03-01 00:00:00

  • A portrait of the Bcl-2 protein family: life, death, and the whole picture.

    abstract::The Bcl-2 family of proteins are important regulators of cell death. They are comprised of two opposing factions, the proapoptotic versus the antiapoptotic members. Both are required for normal development and cellular homeostasis of the immune system and other tissues. However, in certain circumstances they may parti...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章,评审

    doi:10.1023/a:1020598632068

    authors: Pellegrini M,Strasser A

    更新日期:1999-11-01 00:00:00

  • Circulating CD3+ CD4+ CD8+ T lymphocytes in multiple sclerosis.

    abstract::Triple-antibody flow cytometry was used to search for distinctive populations of peripheral blood lymphocyte immunophenotypes in multiple sclerosis (MS). Using monoclonal antibodies to the cell surface markers CD3, CD4, and CD8, T cell subsets were quantified on a cohort of 31 MS patients (not treated with corticoster...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/BF00919267

    authors: Munschauer FE,Stewart C,Jacobs L,Kaba S,Ghorishi Z,Greenberg SJ,Cookfair D

    更新日期:1993-03-01 00:00:00

  • Identification of an HLA-A0201-restricted CTL epitope generated by a tumor-specific frameshift mutation in a coding microsatellite of the OGT gene.

    abstract::Deficient DNA mismatch repair results in microsatellite instability and might induce shifts of translational reading frames of genes encompassing coding microsatellites. These may be translated in truncated proteins, including neo-peptide tails functioning as tumor rejection antigens, when presented in the context of ...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1023/a:1025329819121

    authors: Ripberger E,Linnebacher M,Schwitalle Y,Gebert J,von Knebel Doeberitz M

    更新日期:2003-09-01 00:00:00

  • Infectious and immunologic phenotype of MECP2 duplication syndrome.

    abstract::MECP2 (methyl CpG binding protein 2) duplication causes syndromic intellectual disability. Patients often suffer from life-threatening infections, suggesting an additional immunodeficiency. We describe for the first time the detailed infectious and immunological phenotype of MECP2 duplication syndrome. 17/27 analyzed ...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-015-0129-5

    authors: Bauer M,Kölsch U,Krüger R,Unterwalder N,Hameister K,Kaiser FM,Vignoli A,Rossi R,Botella MP,Budisteanu M,Rosello M,Orellana C,Tejada MI,Papuc SM,Patat O,Julia S,Touraine R,Gomes T,Wenner K,Xu X,Afenjar A,Toutain

    更新日期:2015-02-01 00:00:00

  • Natural autoantibody to galectin-9 in normal human sera.

    abstract::Antibodies to certain self-antigens are detected in normal individuals as well as in patients with autoimmune diseases. Natural autoantibodies found in normal human sera are thought to act as an immune regulator, a functional controller of specific proteins, or the first-line defense against pathogenic microorganisms....

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1023/a:1020599526249

    authors: Suk K,Hwang DY,Lee MS

    更新日期:1999-05-01 00:00:00

  • RAG Deficiency: Two Genes, Many Diseases.

    abstract:PURPOSE:To review the clinical and laboratory spectrum of RAG gene defects in humans, and discuss the mechanisms underlying phenotypic heterogeneity, the basis of immune dysregulation, and the current and perspective treatment modalities. METHODS:Literature review and analysis of medical records RESULTS: RAG gene defe...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章,评审

    doi:10.1007/s10875-018-0537-4

    authors: Delmonte OM,Schuetz C,Notarangelo LD

    更新日期:2018-08-01 00:00:00

  • Curcumin protects against collagen-induced arthritis via suppression of BAFF production.

    abstract:PURPOSE:The aim of the present study was to evaluate whether the anti-Rheumatoid arthritis (RA) effect of curcumin is associated with the regulation of B cell-activating factor belonging to the TNF family (BAFF) production. METHODS:Collagen-induced arthritis (CIA) was induced in DBA/1 J mice by immunization with bovin...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-012-9839-0

    authors: Huang G,Xu Z,Huang Y,Duan X,Gong W,Zhang Y,Fan J,He F

    更新日期:2013-04-01 00:00:00

  • Primary immunodeficiency diseases in Latin America: the second report of the LAGID registry.

    abstract::This is the second report on the continuing efforts of LAGID to increase the recognition and registration of patients with primary immunodeficiency diseases in 12 Latin American countries: Argentina, Brazil, Chile, Colombia, Costa Rica, Honduras, Mexico, Panama, Paraguay, Peru, Uruguay, and Venezuela. This report reve...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-006-9052-0

    authors: Leiva LE,Zelazco M,Oleastro M,Carneiro-Sampaio M,Condino-Neto A,Costa-Carvalho BT,Grumach AS,Quezada A,Patiño P,Franco JL,Porras O,Rodríguez FJ,Espinosa-Rosales FJ,Espinosa-Padilla SE,Almillategui D,Martínez C,Tafur JR,

    更新日期:2007-01-01 00:00:00

  • Case Report: Acute Thrombotic Microangiopathy in a Patient with STING-Associated Vasculopathy with Onset in Infancy (SAVI).

    abstract::Stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI) is a rare disorder that is associated with extensive inflammation throughout the body due to a high interferon state. Common clinical manifestations of this disorder include chronic lung disease, digital necrosis, recurrent low...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-020-00850-2

    authors: Ma M,Mazumder S,Kwak H,Adams M,Gregory M

    更新日期:2020-11-01 00:00:00

  • Analysis of the costimulatory requirements for generating human virus-specific in vitro T helper and effector responses.

    abstract::The present study analyzes the role of CD28-B7-mediated costimulation during in vitro human peripheral blood memory T cell activation by influenza A virus. Inhibition studies using the B7-binding fusion protein CTLA4Ig and antibodies against CD80 and CD86 demonstrate that CTLA4Ig and anti-CD86 inhibited influenza-spec...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1023/a:1010987426835

    authors: Blazevic V,Trubey CM,Shearer GM

    更新日期:2001-07-01 00:00:00

  • Enhancement of membrane B7-H3 costimulatory molecule but reduction of its soluble form in multiple sclerosis.

    abstract:PURPOSE:Multiple sclerosis (MS) is an autoimmune disease of the central nervous system mediated by T cells. B7-H3 plays a diverse role in regulating T cell responses. However, its expression and clinical significance in MS are not well known. This study analyzed the expression of membrane B7-H3 (mB7-H3) and levels of s...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-012-9800-2

    authors: Jiang J,Jiang J,Liu C,Zhang G,Gao L,Chen Y,Zhu R,Wang T,Wang F,Zhang X,Xue Q

    更新日期:2013-01-01 00:00:00

  • Inhibition of IFN regulatory factor-1 down-regulate Th1 cell function in patients with acute coronary syndrome.

    abstract:BACKGROUND:The crucial role of T helper (Th) cells and chronic inflammation in atherosclerosis and coronary artery disease is no longer controversial. Evidence has revealed that Th cell type 1 (Th1) is closely associated with the pathogenesis of acute coronary syndrome (ACS). But the mechanisms involved in the generati...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-010-9367-8

    authors: Guo M,Mao X,Ji Q,Lang M,Li S,Peng Y,Zhou W,Xiong B,Zeng Q

    更新日期:2010-03-01 00:00:00

  • 15-deoxy-Delta(12,14)-prostaglandin J(2) and curcumin modulate the expression of toll-like receptors 4 and 9 in autoimmune T lymphocyte.

    abstract:INTRODUCTION:Experimental allergic encephalomyelitis (EAE) is a T cell-mediated autoimmune disease model for multiple sclerosis (MS). We have shown earlier that 15-deoxy-Delta(12,14)-prostaglandin J(2) (15d-PGJ(2)) and curcumin ameliorate EAE by modulating inflammatory signaling pathways in T lymphocytes. Toll-like rec...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-008-9202-7

    authors: Chearwae W,Bright JJ

    更新日期:2008-09-01 00:00:00

  • Severe combined immunodeficiency (SCID) in Canadian children: a national surveillance study.

    abstract:PURPOSE:Severe Combined Immune Deficiency (SCID) is universally fatal unless treated with hematopoietic stem cell transplantation (HSCT). Following the identification of disseminated Bacille Calmette-Guérin (BCG) infections in Canadian First Nations, Métis and Inuit (FNMI) children with unrecognized primary immune defi...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-013-9952-8

    authors: Rozmus J,Junker A,Thibodeau ML,Grenier D,Turvey SE,Yacoub W,Embree J,Haddad E,Langley JM,Ramsingh RM,Singh VA,Long R,Schultz KR

    更新日期:2013-11-01 00:00:00

  • Diagnostic Delay of Primary Immunodeficiencies at a Tertiary Care Hospital in Peru- Brief Report.

    abstract:OBJECTIVE:The aim of the study was to assess the diagnostic delay in pediatric patients with primary immunodeficiencies (PID) at a tertiary care hospital in Peru. METHODS:A descriptive study was carried out in which patients from a third-level referral center in Peru were included. Those without a specific diagnosis o...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-017-0398-2

    authors: Veramendi-Espinoza LE,Zafra-Tanaka JH,Pérez-Casquino GA,Córdova-Calderón WO

    更新日期:2017-05-01 00:00:00

  • Molecular mechanism of NLRP3 inflammasome activation.

    abstract::The inflammasome is an intracellular multimolecular complex that controls caspase-1 activity in the innate immune system. NLRP3, a member of the NLR family of cytosolic pattern recognition receptors, along with the adaptor protein ASC, mediates caspase-1 activation via assembly of the inflammasome in response to vario...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章,评审

    doi:10.1007/s10875-010-9440-3

    authors: Jin C,Flavell RA

    更新日期:2010-09-01 00:00:00