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3.
J Allergy Clin Immunol ; 139(3S): S1-S46, 2017 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-28041678

RESUMO

Human immunoglobulin preparations for intravenous or subcutaneous administration are the cornerstone of treatment in patients with primary immunodeficiency diseases affecting the humoral immune system. Intravenous preparations have a number of important uses in the treatment of other diseases in humans as well, some for which acceptable treatment alternatives do not exist. We provide an update of the evidence-based guideline on immunoglobulin therapy, last published in 2006. Given the potential risks and inherent scarcity of human immunoglobulin, careful consideration of its indications and administration is warranted.


Assuntos
Imunoglobulinas Intravenosas/uso terapêutico , Imunoglobulinas/uso terapêutico , Síndromes de Imunodeficiência/terapia , Animais , Medicina Baseada em Evidências , Humanos , Imunidade Humoral , Síndromes de Imunodeficiência/imunologia , Imunomodulação , Injeções Subcutâneas , Guias de Prática Clínica como Assunto
4.
Allergy Rhinol (Providence) ; 7(2): 69-73, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-27470901

RESUMO

BACKGROUND: We reported on six infants between 5 and 11 months old, with transient hypogammaglobulinemia of infancy and severe refractory atopic dermatitis, who were treated with open-label immunoglobulin (Ig) after conventional therapy failed. All six infants had an IgG level of <225 mg/dL, elevated eosinophil and IgE levels, and no urine or stool protein losses, but they did exhibit hypoalbuminemia. OBJECTIVE: To evaluate the utility of open-label immunoglobulin in infants with severe atopic dermatitis for whom conventional therapy failed. We reviewed the clinical utility of intravenous immunoglobulin in the treatment of severe atopic dermatitis, the most recent research in the field, and suggested mechanisms for its benefit. METHODS: The six infants were identified from a retrospective chart review at the University of California Los Angeles Allergy and Immunology outpatient pediatric clinic. RESULTS: All six patients were treated with 400 mg/kg/month of intravenous immunoglobulin and had normalization of their IgG and albumin levels, and all but one had clinically improved atopic dermatitis. CONCLUSION: Infants with severe atopic dermatitis who did not respond to conventional therapy avoidance may benefit from intravenous immunoglobulin therapy.

6.
J Allergy Clin Immunol ; 133(4): 961-6, 2014 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24582311

RESUMO

The present uncertainty of which live viral or bacterial vaccines can be given to immunodeficient patients and the growing neglect of societal adherence to routine immunizations has prompted the Medical Advisory Committee of the Immune Deficiency Foundation to issue recommendations based on published literature and the collective experience of the committee members. These recommendations address the concern for immunodeficient patients acquiring infections from healthy subjects who have not been immunized or who are shedding live vaccine-derived viral or bacterial organisms. Such transmission of infectious agents can occur within the hospital, clinic, or home or at any public gathering. Collectively, we define this type of transmission as close-contact spread of infectious disease that is particularly relevant in patients with impaired immunity who might have an infection when exposed to subjects carrying vaccine-preventable infectious diseases or who have recently received a live vaccine. Immunodeficient patients who have received therapeutic hematopoietic stem transplantation are also at risk during the time when immune reconstitution is incomplete or while they are receiving immunosuppressive agents to prevent or treat graft-versus-host disease. This review recommends the general education of what is known about vaccine-preventable or vaccine-derived diseases being spread to immunodeficient patients at risk for close-contact spread of infection and describes the relative risks for a child with severe immunodeficiency. The review also recommends a balance between the need to protect vulnerable subjects and their social needs to integrate into society, attend school, and benefit from peer education.


Assuntos
Infecções Bacterianas/transmissão , Vacinas Bacterianas/efeitos adversos , Hospedeiro Imunocomprometido , Vacinas Vivas não Atenuadas/efeitos adversos , Vacinas Virais/efeitos adversos , Viroses/transmissão , Infecções Bacterianas/imunologia , Infecções Bacterianas/prevenção & controle , Vacinas Bacterianas/imunologia , Criança , Pré-Escolar , Humanos , Síndromes de Imunodeficiência , Vacinas Vivas não Atenuadas/imunologia , Vacinas Virais/imunologia , Viroses/imunologia , Viroses/prevenção & controle
7.
J Allergy Clin Immunol ; 132(1): 140-50, 2013 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-23810098

RESUMO

BACKGROUND: Assay of T-cell receptor excision circles (TRECs) in dried blood spots obtained at birth permits population-based newborn screening (NBS) for severe combined immunodeficiency (SCID). OBJECTIVE: We sought to report the first 2 years of TREC NBS in California. METHODS: Since August 2010, California has conducted SCID NBS. A high-throughput TREC quantitative PCR assay with DNA isolated from routine dried blood spots was developed. Samples with initial low TREC numbers had repeat DNA isolation with quantitative PCR for TRECs and a genomic control, and immunophenotyping was performed within the screening program for infants with incomplete or abnormal results. Outcomes were tracked. RESULTS: Of 993,724 infants screened, 50 (1/19,900 [0.005%]) had significant T-cell lymphopenia. Fifteen (1/66,250) required hematopoietic cell or thymus transplantation or gene therapy; these infants had typical SCID (n = 11), leaky SCID or Omenn syndrome (n = 3), or complete DiGeorge syndrome (n = 1). Survival to date in this group is 93%. Other T-cell lymphopenic infants had variant SCID or combined immunodeficiency (n = 6), genetic syndromes associated with T-cell impairment (n = 12), secondary T-cell lymphopenia (n = 9), or preterm birth (n = 8). All T-cell lymphopenic infants avoided live vaccines and received appropriate interventions to prevent infections. TREC test specificity was excellent: only 0.08% of infants required a second test, and 0.016% required lymphocyte phenotyping by using flow cytometry. CONCLUSIONS: TREC NBS in California has achieved early diagnosis of SCID and other conditions with T-cell lymphopenia, facilitating management and optimizing outcomes. Furthermore, NBS has revealed the incidence, causes, and follow-up of T-cell lymphopenia in a large diverse population.


Assuntos
Linfopenia/diagnóstico , Triagem Neonatal , Imunodeficiência Combinada Severa/diagnóstico , Linfócitos T/imunologia , California , Feminino , Humanos , Recém-Nascido , Masculino
8.
Transfus Med Rev ; 27(3): 171-8, 2013 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-23835249

RESUMO

Human immunoglobulin (IG) is used for IgG replacement therapy in primary and secondary immunodeficiency, for prevention and treatment of certain infections, and as an immunomodulatory agent for autoimmune and inflammatory disorders. IG has a wide spectrum of antibodies to microbial and human antigens. Several high-titered IGs are also available enriched in antibodies to specific viruses or bacterial toxins. IG can be given intravenously (IGIV), intramuscularly (IGIM) or by subcutaneous infusions (SCIG). Local adverse reactions such as persistent pain, bruising, swelling and erythema are rare with IGIV infusions but common (75%) with SCIG infusions. By contrast, adverse systemic reactions are rare with SCIG infusions but common with IGIV infusions, occurring as often as 20% to 50% of patients and 5% to 15% of all IGIV infusions. Systemic adverse reactions can be immediate (60% of reactions) occurring within 6 hours of an infusion, delayed (40% of reactions) occurring 6 hours-1 week after an infusion, and late (less than 1% of reactions), occurring weeks and months after an infusion. Immediate systemic reactions such as head and body aches, chills and fever are usually mild and readily treatable. Immediate anaphylactic and anaphylactoid reactions are uncommon. The most common delayed systemic reaction is persistent headache. Less common but more serious delayed reactions include aseptic meningitis, renal failure, thromboembolism, and hemolytic reactions. Late reactions are uncommon but often severe, and include lung disease, enteritis, dermatologic disorders and infectious diseases. The types, incidence, causes, prevention, and management of these reactions are discussed.


Assuntos
Imunização Passiva/efeitos adversos , Imunoglobulinas Intravenosas/efeitos adversos , Anticorpos Monoclonais/uso terapêutico , Calafrios/etiologia , Contusões/etiologia , Esquema de Medicação , Eritema/etiologia , Febre/etiologia , Humanos , Imunoglobulina G , Imunoglobulinas Intravenosas/administração & dosagem , Síndromes de Imunodeficiência/terapia , Dor/etiologia , Fatores de Tempo
10.
J Pediatr Hematol Oncol ; 35(1): e14-8, 2013 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-22510776

RESUMO

Hematopoietic stem-cell transplantation is the treatment of choice for severe combined immunodeficiency (SCID). Despite successful T-cell engraftment in transplanted patients, B-cell function is not always achieved; up to 58% of patients require immunoglobulin therapy after receiving haploidentical transplants. We report 2 half-sibling males with X-linked γ-chain SCID treated with different sources of stem cells. Sibling 1 was transplanted with T-cell-depleted haploidentical maternal bone marrow and sibling 2 was transplanted with 7/8 human leukocyte antigen-matched unrelated umbilical cord blood. Both patients received pretransplant conditioning and posttransplant graft-versus-host-disease prophylaxis. B-cell engraftment and function was achieved in sibling 1 but not in sibling 2. This disparate result is consistent with a review of 19 other SCID children who received cord blood transplants. B-cell function, as indicated by no need for immunoglobulin therapy, was restored in 42% of patients given haploidentical transplants and in 68% of patients given matched unrelated donor transplants compared with 80% of patients given cord blood transplants. Cord blood is an alternative source of stem cells for transplantation in children with SCID and has a higher likelihood of B-cell reconstitution.


Assuntos
Linfócitos B/imunologia , Transplante de Medula Óssea , Sangue Fetal/transplante , Doença Enxerto-Hospedeiro , Imunodeficiência Combinada Severa/terapia , Feminino , Teste de Histocompatibilidade , Humanos , Recém-Nascido , Depleção Linfocítica , Masculino , Prognóstico
11.
J Allergy Clin Immunol ; 130(3 Suppl): S1-24, 2012 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-22935624

RESUMO

A major diagnostic intervention in the consideration of many patients suspected to have primary immunodeficiency diseases (PIDDs) is the application and interpretation of vaccination. Specifically, the antibody response to antigenic challenge with vaccines can provide substantive insight into the status of human immune function. There are numerous vaccines that are commonly used in healthy individuals, as well as others that are available for specialized applications. Both can potentially be used to facilitate consideration of PIDD. However, the application of vaccines and interpretation of antibody responses in this context are complex. These rely on consideration of numerous existing specific studies, interpolation of data from healthy populations, current diagnostic guidelines, and expert subspecialist practice. This document represents an attempt of a working group of the American Academy of Allergy, Asthma & Immunology to provide further guidance and synthesis in this use of vaccination for diagnostic purposes in consideration of PIDD, as well as to identify key areas for further research.


Assuntos
Síndromes de Imunodeficiência/imunologia , Vacinação , Cápsulas Bacterianas/imunologia , Bacteriófago phi X 174/imunologia , Vacinas Anti-Haemophilus/imunologia , Humanos , Imunidade Humoral , Síndromes de Imunodeficiência/diagnóstico , Vacinas Pneumocócicas/imunologia , Vacinas Antirrábicas/imunologia , Vacinas contra Salmonella/imunologia
13.
Pediatr Transplant ; 15(7): 733-41, 2011 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-21883749

RESUMO

There are few reports of clinical presentation, genotype, and HCT outcomes for patients with T-B+NK+ SCID. Between 1981 and 2007, eight of 84 patients with SCID who received and/or were followed after HCT at UCSF had the T-B+NK+ phenotype. One additional patient with T-B+NK+ SCID was identified as the sibling of a patient treated at UCSF. Chart reviews were performed. Molecular analyses of IL7R, IL2RG, JAK3, and the genes encoding the CD3 T-cell receptor components δ (CD3D), ε (CD3E), and ζ (CD3Z) were carried out. IL7R mutations were documented in four patients and CD3D mutations in two others. Three patients had no defects found. Only two of nine patients had an HLA-matched related HCT donor. Both survived, and neither developed GVHD. Five of seven recipients of haploidentical grafts survived. Although the majority of reported cases of T-B+NK+ SCID are caused by defects in IL7R, CD3 complex defects were also found in this series and should be considered when evaluating patients with T-B+NK+ SCID. Additional genes, mutations in which account for T-B+NK+ SCID, remain to be found. Better approaches to early diagnosis and HCT treatment are needed for patients lacking an HLA-matched related donor.


Assuntos
Linfócitos B/citologia , Complexo CD3/biossíntese , Transplante de Células-Tronco Hematopoéticas/métodos , Interleucina-7/metabolismo , Imunodeficiência Combinada Severa/sangue , Linfócitos T/citologia , Complexo CD3/genética , Análise Mutacional de DNA , Feminino , Fibroblastos/metabolismo , Citometria de Fluxo/métodos , Genótipo , Antígenos HLA/metabolismo , Humanos , Lactente , Recém-Nascido , Interleucina-7/genética , Masculino , Mutação , Fenótipo , Imunodeficiência Combinada Severa/genética , Resultado do Tratamento
14.
Front Immunol ; 2: 61, 2011.
Artigo em Inglês | MEDLINE | ID: mdl-22566850

RESUMO

Ectodermal dysplasias (ED) are uncommon genetic disorders resulting in abnormalities in ectodermally derived structures. Many ED-associated genes have been described, of which ectodysplasin-A (EDA) is one of the more common. The NF-κB essential modulator (NEMO encoded by the IKBKG gene) is unique in that mutations result in severe humoral and cellular immunologic defects in addition to ED. We describe three unrelated kindreds with defects in both EDA and IKBKG resulting from X-chromosome crossover. This demonstrates the importance of thorough immunologic consideration of patients with ED even when an EDA etiology is confirmed, and raises the possibility of a specific phenotype arising from coincident mutations in EDA and IKBKG.

16.
J Allergy Clin Immunol ; 124(3): 536-43, 2009 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-19683336

RESUMO

BACKGROUND: Mutations in serine protease inhibitor Kazal-type 5 (SPINK5), encoding the serine protease inhibitor lympho-epithelial Kazal-type 5 related inhibitor (LEKTI), cause Comèl-Netherton syndrome, an autosomal-recessive disease characterized by congenital ichthyosis, bamboo hair, and atopic diathesis. Despite increased frequency of infections, the immunocompetence of patients with Comèl-Netherton syndrome has not been extensively investigated. OBJECTIVE: To define Comèl-Netherton syndrome as a primary immunodeficiency disorder and to explore the benefit of intravenous immunoglobulin replacement therapy. METHODS: We enrolled 9 patients with Comèl-Netherton syndrome, sequenced SPINK5, and analyzed LEKTI expression by immunohistochemistry. Immune function was assessed by measuring cognate immunity, serum cytokine levels, and natural killer cell cytotoxicity. RESULTS: All patients presented with recurrent skin infections caused predominantly by Staphylococcus aureus. All but 1 reported recurrent respiratory tract infections; 78% had sepsis and/or pneumonia; 67% had recurrent gastrointestinal disease and failure to thrive. Mutations in SPINK5-including 6 novel mutations-were identified in 8 patients. LEKTI expression was decreased or absent in all patients. Immunologic evaluation revealed reduced memory B cells and defective responses to vaccination with Pneumovax and bacteriophage phiX174, characterized by impaired antibody amplification and class-switching. Immune dysregulation was suggested by a skewed T(h)1 phenotype and elevated proinflammatory cytokine levels, whereas serum concentrations of the chemokine (C-C motif) ligand 5 and natural killer cell cytotoxicity were decreased. Treatment with intravenous immunoglobulin resulted in remarkable clinical improvement and temporarily increased natural killer cell cytotoxicity. CONCLUSION: These data provide new insights into the immunopathology of Comèl-Netherton syndrome and demonstrate that this multisystem disorder, characterized by lack of LEKTI expression in epithelial cells, is complicated by cognate and innate immunodeficiency that responds favorably to intravenous immunoglobulin therapy.


Assuntos
Síndromes de Imunodeficiência/genética , Síndromes de Imunodeficiência/imunologia , Proteínas Secretadas Inibidoras de Proteinases/genética , Infecções Cutâneas Estafilocócicas/genética , Infecções Cutâneas Estafilocócicas/imunologia , Staphylococcus aureus , Linfócitos B/imunologia , Linfócitos B/metabolismo , Criança , Pré-Escolar , Citocinas/sangue , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Síndromes de Imunodeficiência/tratamento farmacológico , Fatores Imunológicos/uso terapêutico , Masculino , Mutação , Células T Matadoras Naturais/imunologia , Células T Matadoras Naturais/metabolismo , Proteínas Secretadas Inibidoras de Proteinases/biossíntese , Proteínas Secretadas Inibidoras de Proteinases/metabolismo , Inibidor de Serinopeptidase do Tipo Kazal 5 , Infecções Cutâneas Estafilocócicas/tratamento farmacológico , Linfócitos T Reguladores/imunologia , Linfócitos T Reguladores/metabolismo
17.
Immunol Res ; 45(1): 37-45, 2009.
Artigo em Inglês | MEDLINE | ID: mdl-19238335

RESUMO

Adoptive transfer of mature T cells (ATMTC) through bone marrow (BM) transplantation, first attempted over 20 years ago, has recently emerged as a successful therapy for complete 22q deletion syndrome (22qDS). This provides a potential option to thymic transplantation (TT) for immune reconstitution in 22qDS. Compared to thymic transplant, ATMTC is an easier procedure to accomplish and is available at more centers. However, there are differences in the nature of the T-cell reconstitution that results. Predictably, more naïve T cells and recent thymic emigrants are present in patients treated with thymus transplant. There are no significant differences in mortality between the two procedures, but the number of patients is too limited to conclude that the procedures are equally effective. Adoptive transfer should be pursued as a reasonable treatment for 22qDS patients requiring immune reconstitution when thymus transplant is not available.


Assuntos
Cromossomos Humanos Par 22 , Síndrome de DiGeorge/imunologia , Imunoterapia , Linfócitos T/transplante , Timo/transplante , Transferência Adotiva , Antígenos CD/biossíntese , Transplante de Medula Óssea/mortalidade , Contagem de Células , Síndrome de DiGeorge/genética , Síndrome de DiGeorge/patologia , Síndrome de DiGeorge/terapia , Seguimentos , Humanos , Análise de Sobrevida , Linfócitos T/imunologia , Linfócitos T/patologia , Timo/imunologia , Timo/patologia , Resultado do Tratamento
19.
Immunol Allergy Clin North Am ; 28(4): 715-36, vii, 2008 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-18940571

RESUMO

This article reviews the major syndromic immunodeficiencies with significant antibody defects, many of which may require intravenous immunogammaglobulin therapy. The authors define syndromic immunodeficiency as an illness associated with a characteristic group of phenotypic abnormalities or laboratory features that comprise a recognizable syndrome. Many are familial with a defined inheritance pattern. Immunodeficiency may not be a major part of the illness and may not be present in all patients; thus, these conditions differ from primary immunodeficiency syndromes, in which immune abnormalities are a consistent and prominent feature of their disease.


Assuntos
Tamanho Corporal/imunologia , Anormalidades Congênitas/imunologia , Síndromes de Imunodeficiência/imunologia , Tamanho Corporal/genética , Anormalidades Congênitas/genética , Humanos , Imunização Passiva , Imunoglobulinas/deficiência , Imunoglobulinas/genética , Imunoglobulinas Intravenosas/uso terapêutico , Síndromes de Imunodeficiência/diagnóstico , Síndromes de Imunodeficiência/genética , Síndromes de Imunodeficiência/fisiopatologia , Síndromes de Imunodeficiência/terapia , Mutação , Especificidade de Órgãos , Linhagem
20.
Biologicals ; 36(6): 363-74, 2008 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-18789721

RESUMO

Therapeutic antibodies include polyclonal immunoglobulins isolated from regular or high-titered human plasma, sera from immunized animals, and monoclonal antibodies. This array of therapeutic antibodies is used for the prevention and treatment of many infectious diseases, antibody immunodeficiencies, autoimmune and inflammatory diseases, neurological disorders, and cancers. Polyclonal human immunoglobulins are available for intramuscular injection (IGIM), intravenous infusion (IGIV) and subcutaneous infusion (SCIG). We review these products and detail the therapeutic use of polyclonal human antibodies in the treatment of antibody immunodeficiencies, including their occasional local side effects (tenderness, sterile abscesses), minor systemic side effects (chills, muscle aches, malaise, headaches) and major side effects (aseptic meningitis, nephropathy, thrombosis).


Assuntos
Anticorpos Monoclonais/uso terapêutico , Anticorpos/uso terapêutico , Formação de Anticorpos/imunologia , Doenças do Sistema Imunitário/terapia , Imunoterapia/métodos , Animais , Desenho de Drogas , Humanos , Soros Imunes/imunologia , Imunoglobulina G/uso terapêutico , Imunoglobulinas Intravenosas/uso terapêutico , Imunoterapia/instrumentação , Modelos Teóricos
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