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Immunologic Deficiency Syndromes (12):
Recent article
Immunologic Deficiency Syndromes
Abstract
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Abstract
The mucosal secretory immune system provides an important primary defence against disease, as studies of humans with mucosal humoral immunodeficiencies suggest that the absence of secretory immunoglobulin A leads to an increase in mucosal infections. However, the infection risks pose...
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PMID: 20694027
PDF is available here.
PDF is available here.
Southern Medical Journal (103)9 2010
Abstract
Southern Medical Journal (103)9 2010
Abstract
Home SCIG therapy was safe and led to improved perceptions of general health, higher serum IgG levels, and very low rates of infections and days missed from work/school....
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PMID: 20689467
PDF is available here.
PDF is available here.
Abstract
Yearly influenza vaccination is recommended for patients with humoral primary immunodeficiency (hPID). However, humoral responses following vaccination can be expected to be reduced in these patients. The efficacy of influenza vaccination in patients with hPID, anti-influenza antibody responses was...
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PMID: 20421178
PDF is available here.
PDF is available here.
Abstract
PID should be considered in children who suffer from recurrent infections and autoimmune diseases or do not respond to long-term use of antibiotics. Immunologic tests should be done as early as possible for the children....
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PMID: 20704795
PDF is available here.
PDF is available here.
Abstract
Prevalence data from different geographical regions have been supplemented by more detailed incidence data on primary antibody deficiencies, revealing trends in diagnosis and management. Continued exploration of the role of genetic variations in TACI in CVID populations has improved our understandin...
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PMID: 20442656
PDF is available here.
PDF is available here.
Abstract
We reviewed all documented cases caused by immunodeficiency-associated vaccine-derived polioviruses in Iran from 1995 through 2008. Changing to an inactivated polio vaccine vaccination schedule and introduction of screening of neonates for immunodeficiencies could reduce the risk for VAPP infection....
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PMID: 20587188
PDF is available here.
PDF is available here.
Abstract
We have studied a mut/mut mouse model of leaky SCID with a homozygous Rag1 S723C mutation that impairs, but does not abrogate, V(D)J recombination activity. In spite of a severe block at the pro-B cell stage and profound B cell lymphopenia, significant serum levels of immunoglobulin (Ig) G, IgM, IgA...
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PMID: 20547827
PDF is available here.
PDF is available here.
Abstract
We report a novel homozygous missense mutation of Rab27A, i.e. K22R, in a Persian GS-2 patient and the results of analysis of the impact of the K22R mutation and the previously reported I44T mutation on protein function. Both mutations completely abolish Slac2-a/melanophilin binding activity but the...
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PMID: 20370853
PDF is available here.
PDF is available here.
Abstract
Primary immunodeficiency disease (PID) is a rare disorder in adults. Most often, serious forms are detected during infancy or childhood. However, mild forms of PID may not be diagnosed until later in life, and some types of humoral immunodeficiency may occur in adulthood. The purpose of this study w...
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PMID: 20514300
PDF is available here.
PDF is available here.
Abstract
On the last stage of Global Polio Eradication Initiative (GPEI), a number of cases with Primary Immunodeficiency (PID) disorder were detected excreting the Vaccine-derived Poliovirus (VDPV). Due to these cases can be prolonged or chronic excretion of the VDPV in the environment, all countries have t...
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PMID: 20726276
PDF is available here.
PDF is available here.
Deutsche Medizinische Wochenschrift (135)25-26 2010
Abstract
Abstract
We report three patients with Kostmann disease who, besides recurrent infections, have developmental delay....
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PMID: 20177699
PDF is available here.
PDF is available here.
Abstract
Abstract
We report the case of a 79-year-old woman with GS who developed subacute motor deficits and cognitive changes. Magnetic resonance imaging (MRI) of the brain disclosed white- and gray-matter lesions, mostly in the right frontal and parietal areas. Polyoma virus JC, the agent of progressive multifocal...
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PMID: 19695918
PDF is available here.
PDF is available here.
Abstract
We assess the evidence on the efficacy of IVIG in certain infectious diseases....
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PMID: 20447982
PDF is available here.
PDF is available here.
Abstract
BACKGROUND: In Sri Lanka, cutaneous leishmaniasis is a well-established disease caused by Leishmania donovani. Only a few cases of visceral disease and mucosal localization have been reported to date. CASE REPORT: A 52-year-old man presented with severe local destruction of his upper and lower lip a...
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PMID: 20534090
PDF is available here.
PDF is available here.
Pediatric Research (67)5 2010
Abstract
Our study on T lymphocyte subpopulations in DS shows that the normal expansion of naive helper (CD4CD45RA) and cytotoxic (CD8CD45RACD27) T lymphocytes is lacking in the first years of life; this is more logically explainable with an intrinsic T lymphocyte defect. Furthermore, memory cell numbers are...
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PMID: 20098345
PDF is available here.
PDF is available here.
Pediatric Research (67)5 2010
Abstract
We determined B-lymphocyte subpopulations in 95 children with DS compared with 33 age-matched control (AMC) children. DS serum immunoglobulin levels were compared with 962 non-DS children with recurrent infections. The results were combined with clinical data. Transitional and naive B lymphocytes ar...
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PMID: 20098344
PDF is available here.
PDF is available here.
MMW Fortschritte der Medizin (152)16 2010
Abstract
Abstract
Thirty primary immunodeficient patients aged 5-55 years of age (21 males and 9 females) were enrolled in this study. Half of the patients (50%) were found to have bronchiectasis in HRCT imaging. In echocardiographic examination, 20 patients (67%) had at least one abnormality; among which pulmonary h...
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PMID: 19918187
PDF is available here.
PDF is available here.
La Tunisie medicale (88)4 2010
Abstract
Our patients were 2 men and one woman. All these patients presented clinical symptoms of septic meningitides. Meningococcal orign was confirmed in one case. C5 level varies between 0 and 0.4%. Levels of other complement components: Clq, C3, C4, properdine, C6, C8 and C9 were normal. Antigenic C7 lev...
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PMID: 20446263
PDF is available here.
PDF is available here.
Giornale italiano di dermatologia e venereologia : organo ufficiale, Societa italiana di dermatologia e sifilografia (145)2 2010
Abstract
We hope that this effort will help specialists to facilitate the recognition of primary immunodeficiencies and therefore early diagnosis and management....
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PMID: 20467400
PDF is available here.
PDF is available here.
Abstract
Our case report presents 2 full siblings to nonconsanguineous parents with pyloric atresia, multiple small bowel and colonic atresias, and severe immune dysfunction. Care was withdrawn within 3 months of life on both siblings after multiple bouts of sepsis. Data suggest that the immune defect may no...
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PMID: 20385266
PDF is available here.
PDF is available here.
Pediatric Allergy and Immunology (21)2 Pt 2 2010
Abstract
Abstract
Abstract
We report the first case of disseminated disease caused by M. scrofulaceum in a child with inherited interferon-gamma receptor 1 (IFN-gammaR1) complete deficiency. As in this case, mycobacterial bone infections in IFN-gammaR1 deficiency can sometimes mimic the clinical picture of chronic recurrent m...
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PMID: 19880337
PDF is available here.
PDF is available here.
médecine/sciences (26)2 2010
Abstract
Abstract
Since the early 1980s T-cell depletion has allowed haploidentical bone marrow transplantation to be performed in patients with primary immunodeficiency for whom a matched sibling donor was not available, without causing severe graft versus host disease (GVHD). This review article presents the availa...
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PMID: 20113886
PDF is available here.
PDF is available here.
Abstract
Many advances have been made since the first successful hematopoietic cell transplants (HCT) in children with primary immunodeficiency disorders (PID) were reported 40 years ago, and many children with PID can now be cured from their otherwise lethal disorders through well-matched HCT procedures. Pr...
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PMID: 20113889
PDF is available here.
PDF is available here.
Abstract
The last 40 years has seen the emergence of hematopoietic stem cell transplantation as a therapeutic modality for fatal diseases and as a curative option for individuals born with inherited disorders that carry limited life expectancy and poor quality of life. Despite the rarity of many primary immu...
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PMID: 20113883
PDF is available here.
PDF is available here.
Abstract
Pediatric Research (67)2 2010
Abstract
T-cell immunodeficiency may pose a diagnostic challenge to clinicians, especially when the basic T-cell immune workup is not sufficiently informative. An intensive assessment of thymus capabilities that involves either measuring the recent thymic emigrant cells or analyzing the T-cell receptor (TCR)...
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PMID: 19858778
PDF is available here.
PDF is available here.
Abstract
We have reviewed herein the complications of different PIDs focusing on gastrointestinal and hepatic manifestation....
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PMID: 20339173
PDF is available here.
PDF is available here.
Abstract
We describe the mechanisms involved in the development of adaptive immunity relating to DNA repair, and the clinical consequences and treatment of the primary immunodeficiency resulting from such defects....
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PMID: 20298636
PDF is available here.
PDF is available here.
Biologie Aujourd hui (204)4 2010
Abstract
We review here CXCR4 dysfunctions unveiled in two rare human immunodeficiency disorders, one characterized by a gain of CXCR4 function, the WHIM syndrome, and the other by a loss of CXCR4 function, the idiopathic CD4(+) T-cell lymphocytopenia.
© Société de Biologie, 2011....
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PMID: 21215244
PDF is available here.
PDF is available here.
Abstract
We examine adult PIDs and their clinical presentation and provide a sequential and directed framework for their diagnosis. Finally, we present a brief review of the most important adult PIDs, common variable immunodeficiency, including diagnosis, pathogenesis, clinical signs, and disease management....
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PMID: 20635783
PDF is available here.
PDF is available here.
Pneumonologia i alergologia polska : organ Polskiego Towarzystwa Ftyzjopneumonologicznego, Polskiego Towarzystwa Alergologicznego, i Instytutu Gruzlicy i Chorob Pluc (78)3 2010
Abstract
The interstitial lung disease in children is a group of disorders heterogeneous in its etiology and pathogenesis. In differential diagnosis infectious, immunological and metabolic factors must be taken into consideration, as well as a genetically determined surfactant protein C deficiency. In the pa...
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PMID: 20461694
PDF is available here.
PDF is available here.
Tuberkuloz ve toraks (58)1 2010
Abstract
We describe clinical features, laboratory findings and therapeutic outcome of children with CID who had tuberculosis disease. Medical reports of 10 patients were reviewed. Three patients had chronic granulomatous disease, two had common variable immuno deficiency, the others had cyclic neutropenia,...
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PMID: 20517730
PDF is available here.
PDF is available here.
Abstract
We are hereby reporting one such case we encountered at our centre who presented with monoparesis and subsequently developed multiple cranial nerve palsy and deteriorating sensorium unresponsive to therapy which eventually turned out to be a case of "Idiopathic CD4+ T-Lymphocytopenia" with cryptococ...
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PMID: 20649100
PDF is available here.
PDF is available here.
Abstract
We evaluated the number of deaths among the children and the effects of different variables on their LPPS scores and mortality. We examined 98 pediatric patients with a mean delay in diagnosis of 21.2 months. Antimicrobial prophylaxis was administered to 57.2% of the patients, whereas intravenous im...
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PMID: 21038783
PDF is available here.
PDF is available here.
Przeglad lekarski (67)1 2010
Abstract
The basic mechanisms of specific immune response in children to pathogens with characteristics of cells involved in the process were described. The characteristics of commonly used stimulators (thymus-derived peptides, bacterial lysates and ribosoms, glukans) and the mechanisms of action, target cel...
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PMID: 20509582
PDF is available here.
PDF is available here.
Biomedical Research (31)1 2010
Contiguous gene deletion of Ptprk and Themis causes T-helper immunodeficiency (thid) in the LEC rat.
Abstract
We identified the contiguous gene deletion of Ptprk and Themis genes in the thid locus that causes the simultaneous loss of expression of both genes, and the exogenous Ptprk expression partially rescued this phenotype. To determine whether the deletion of Themis influences thid phenotype, bone marro...
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PMID: 20203423
PDF is available here.
PDF is available here.
Abstract
The investigation of an inherited primary immunodeficiency, the immunoglobulin class switch recombination deficiency, has allowed the delineation of complex molecular events that underlie antibody maturation in humans. The Activation-induced cytidine deaminase (AID)-deficiency, characterized by a de...
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PMID: 20687504
PDF is available here.
PDF is available here.
American Family Physician (80)11 2009
Abstract
Abstract
We summarize our research findings on PIDs in Chinese population in the past 20 years. Close collaboration among various immunology centers, cross-referrals and systematic data analysis constitute the foundation for research on PIDs. Future directions include establishment of a national PID registry...
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PMID: 20003815
PDF is available here.
PDF is available here.
Pediatric Annals (38)11 2009
Abstract
Abstract
Primary immunodeficiency diseases comprise a genetically heterogeneous group of disorders that affect distinct components of the innate and adaptive immune system, such as neutrophils, macrophages, dendritic cells, complement proteins and natural killer cells, as well as T and B lymphocytes. The stu...
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PMID: 19918634
PDF is available here.
PDF is available here.
MMW Fortschritte der Medizin (151 Suppl 3) 2009
Abstract
Abstract
We discuss here, human predisposition to infectious diseases seems to cover a continuous spectrum from monogenic to polygenic inheritance. Although many studies have provided proof of principle that infectious diseases may result from various types of inborn errors of immunity, the genetic determini...
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PMID: 19729848
PDF is available here.
PDF is available here.
Abstract
Primary immunodeficiency disorders are associated with increased patient susceptibility to recurrent infections. Since the 1950s, intramuscular, intravenous and subcutaneous immunoglobulin products have been used to replace functionally deficient or absent immunoglobulins, reduce the incidence of in...
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PMID: 19670998
PDF is available here.
PDF is available here.
Current Gene Therapy (9)4 2009
Abstract
Primary immunodeficiencies (PID) are caused by mutations in genes that impair the development or activity of the immune system. Although bone marrow transplants achieve long time restoration in up to 90% of treated patients, morbidity and mortality are still high for some PID and ade...
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PMID: 19534652
PDF is available here.
PDF is available here.
Orvosi Hetilap (150)31 2009
Abstract
Voenno-meditsinskii zhurnal (330)8 2009
Abstract
The article suggests the observed phylactic and non-phylactic mechanisms of defense from mycotic infection, primary and secondary immunodeficiency (ID) and causes of appearance. There were analyzed different forms of course of candidiasis, were given their specific manifestations by the patients wit...
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PMID: 19916315
PDF is available here.
PDF is available here.
Abstract
Bloody stools, diarrhea and perianal abscesses were observed from the age of two months infant. The boy received a BCG vaccination at the age of four months. The patient was diagnosed as having Crohn's disease at the age of six months by intestinal endoscopy. Based on the diagnosis, he was treated w...
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PMID: 19764466
PDF is available here.
PDF is available here.
Clinical Immunology (132)1 2009
Abstract
We therefore sought to examine serologic markers of systemic inflammation and intestinal pathology in a kindred of patients with the NEMO syndrome. We observed persistent elevation of erythrocyte sedimentation rates in five patients, and two were symptomatic, with a chronic but atypical enterocoliti...
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PMID: 19375390
PDF is available here.
PDF is available here.
Abstract
Primary antibody deficiencies are the most common primary immunodeficiency diseases. They are a heterogeneous group of disorders with various degrees of dysfunctional antibody production resulting from a disruption of B-cell differentiation at different stages. While there has been tremendous recent...
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PMID: 19597006
PDF is available here.
PDF is available here.
Abstract
We report the first successful hematopoietic stem cell transplantation with an unrelated donor, performed in a schoolgirl with severe interferon gamma receptor 1deficiency caused by a novel mutation....
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PMID: 19451859
PDF is available here.
PDF is available here.
Abstract
Hematopoietic stem cell transplantation is the optimal treatment for patients with primary immunodeficiencies. Best results are achieved with stem cells from a HLA-identical donor, but it is only possible in a small number of patients. Until recently, HLA-mismatched/haploidentical hematopoietic stem...
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PMID: 19827355
PDF is available here.
PDF is available here.