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Common variable immunodeficiency

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https://www.readbyqxmd.com/read/28931519/beta-cell-replacement-in-mice-using-human-type-1-diabetes-nuclear-transfer-embryonic-stem-cells
#1
Lina Sui, Nichole Danzl, Sean R Campbell, Ryan Viola, Damian Williams, Yuan Xing, Yong Wang, Neil Phillips, Greg Poffenberger, Bjarki Johannesson, Jose Oberholzer, Alvin C Powers, Rudolph L Leibel, Xiaojuan Chen, Megan Sykes, Dieter Egli
Beta cells derived from stem cells hold great promise for cell replacement therapy for diabetes. Here we examine the ability of nuclear transfer embryonic stem cells (NT-ES) derived from a type 1 diabetes patient to differentiate into beta cells, and provide a source of autologous islets for cell replacement. NT-ES cells differentiate in vitro with an average efficiency of 55% into C-peptide-positive cells, expressing markers of mature beta cells, including MAFA and NKX6.1. Upon transplantation in immunodeficient mice, grafted cells form vascularized islet-like structures containing MAFA/C-peptide-positive cells...
September 20, 2017: Diabetes
https://www.readbyqxmd.com/read/28929596/hypogammaglobulinemia-in-children-a-warning-sign-to-look-deeply
#2
Karina Mescouto de Melo, Maria Isabel de Moraes-Pinto, Luís E C Andrade, Reinaldo Salomão, Milena K C Brunialti, Vanessa S Ferreira, Beatriz T Costa-Carvalho
This study investigated phenotypic and functional characteristics of lymphocytes in children with common variable immunodeficiency (CVID) and unclassified hypogammaglobulinemia (UH), as well as B-cell subsets in non-consanguineous parents. Blood samples of 30 children, CVID (n = 9), UH (n = 9), healthy donors HD (n = 12), and 19 adults (parents and controls) were labeled by a combination of surface markers to identify CD4, CD8 T-cell and B-cell subpopulations. T-cell cytokine production in children was analyzed in vitro after stimulation with phytohemagglutinin (PHA) and tetanus toxoid...
October 2017: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
https://www.readbyqxmd.com/read/28927200/epidemiology-of-invasive-fungal-disease-in-children
#3
Zoi Dorothea Pana, Emmanuel Roilides, Adilia Warris, Andreas H Groll, Theoklis Zaoutis
Considerable progress has been made in the prevention, diagnosis, and management of pediatric patients with invasive fungal disease (IFD). The reported decreasing trend in the incidence of invasive candidiasis (IC) over the past 15 years in both neonates and children has been encouraging. Nevertheless, due to the growing number of immunocompromised children at risk for IFD, this disease continues to be associated with significant morbidity and death and with increased financial burden to the health care system...
September 1, 2017: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/28925897/role-of-apoptosis-in-the-pathogenesis-of-common-variable-immunodeficiency-cvid
#4
Mazdak Ganjalikhani-Hakemi, Reza Yazdani, Mohammad Esmaeili, Hassan Abolhassani, Wiliam Rae, Gholamreza Azizi, Majid Zaki Dizaji, Mohammadreza Shaghaghi, Faezeh Abbasi-Rad, Abbas Rezaei, Sanaz Afshar-Qasemloo, Saeed Mohammadi, Nima Rezaei, Asghar Aghamohammadi
BACKGROUND: Common variable immunodeficiency (CVID) is a heterogeneous immune deficiency characterized by hypogammaglobulinemia. Since B cell maturation and differentiation is defective in this disorder, we evaluated apoptosis in B cells of patients with CVID compared with healthy donors (HD). METHODS: Determination of peripheral blood B-cell subsets in CVID and HDs, was performed using flow cytometry. We compared total apoptosis, early apoptosis and late apoptosis/necrosis in unstimulated and stimulated B-cells of patients with CVID and HDs...
September 19, 2017: Endocrine, Metabolic & Immune Disorders Drug Targets
https://www.readbyqxmd.com/read/28924447/passive-blood-anaphylaxis-subcutaneous-immunoglobulins-are-a-cause-of-ongoing-passive-anaphylactic-reaction
#5
Przemyslaw Zdziarski, Andrzej Gamian, Jacek Majda, Agnieszka Korzeniowska-Kowal
BACKGROUND: Allergic, especially anaphylactic, reactions during immunoglobulin replacement therapy are rare, but their pathophysiology and classification remain ambiguous. Recent findings show positive results of skin tests with commercially available immunoglobulins, but target antigens and responsible compounds of the tested immunoglobulins have not been strictly identified. CASE DESCRIPTION AND FINDINGS: Four adult patients with recently diagnosed common variable immunodeficiency qualified for standard subcutaneous immunoglobulin replacement therapy regimen...
2017: Allergy, Asthma, and Clinical Immunology
https://www.readbyqxmd.com/read/28924106/intravenous-immunoglobulin-monotherapy-for-granulomatous-lymphocytic-interstitial-lung-disease-in-common-variable-immunodeficiency
#6
Mizue Hasegawa, Fumikazu Sakai, Asako Okabayashi, Akitoshi Sato, Naoko Yokohori, Hideki Katsura, Chihiro Asano, Toshiko Kamata, Eitetsu Koh, Yasuo Sekine, Kenzo Hiroshima, Takashi Ogura, Tamiko Takemura
Common variable immunodeficiency (CVID) is a heterogeneous subset of immunodeficiency disorders. Recurrent bacterial infection is the main feature of CVID, but various non-infectious complications can occur. A 42-year-old woman presented with cough and abnormal chest X-ray shadows. Laboratory tests showed remarkable hypogammaglobulinemia. Computed tomography revealed multiple consolidation and nodules on the bilateral lung fields, systemic lymphadenopathy, and splenomegaly. A surgical lung biopsy specimen provided the final diagnosis of lymphoproliferative disease in CVID, which was grouped under the term granulomatous lymphocytic interstitial lung disease...
September 15, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28910817/the-lung-in-dysregulated-states-of-humoral-immunity
#7
Yurdagül Uzunhan, Florence Jeny, Marianne Kambouchner, Morgane Didier, Diane Bouvry, Hilario Nunes, Jean-François Bernaudin, Dominique Valeyre
In common variable immunodeficiency, lung manifestations are related to different mechanisms: recurrent pneumonias due to encapsulated bacteria responsible for diffuse bronchiectasis, diffuse infiltrative pneumonia with various patterns, and lymphomas, mostly B cell extranodal non-Hodgkin type. The diagnosis relies on significant serum Ig deficiency and the exclusion of any primary or secondary cause. Histopathology may be needed. Immunoglobulin (IgG) replacement is crucial to prevent infections and bronchiectasis...
September 15, 2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28904698/hiv-related-knowledge-attitude-and-practices-of-healthy-adults-in-cross-river-state-nigeria-a-population-based-survey
#8
Uchenna Okonkwo, Soter Ameh, Akaninyene Otu, Henry Okpara
INTRODUCTION: Human Immunodeficiency Virus (HIV) remains a global health problem disproportionately distributed across Nigeria. Cross river state (CRS), a tourist state, located in the Niger delta, has one of the highest prevalence rates. There is evidence that poor knowledge and stigmatization are obstacles to achieving universal access to HIV prevention programs. The objective of this study was to determine the Knowledge, Attitude and Practice (KAP) of HIV among adults resident in CRS, Nigeria...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28891536/urine-osmolality-in-treatment-na%C3%A3-ve-hiv-positive-subjects-in-southeast-nigeria
#9
E N Anyabolu, I I Chukwuonye, I Ezeani
BACKGROUND AND OBJECTIVES: Urine osmolality varies over a wide range of values in a healthy state. Dilute urine or concentrated urine may be observed in many environmental, physiologic, and disease conditions. Urine osmolality is not commonly evaluated in routine clinical practice and in human immunodeficiency virus (HIV) subjects. The factors that influence urine osmolality have not been completely identified. The aim of this study was to evaluate urine osmolality in treatment-naïve HIV subjects and to identify the factors that may influence dilute and concentrated urine in this group of patients...
August 2017: Nigerian Journal of Clinical Practice
https://www.readbyqxmd.com/read/28890017/an-unusual-cause-of-fever-in-a-patient-with-common-variable-immunodeficiency
#10
Andrew T Dang, Gene Schwartz, LaQuita Jones, Michael J Absalon, Richard L McMasters, Amal Assa'ad
No abstract text is available yet for this article.
September 2017: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/28879211/common-variable-immunodeficiency-with-several-gastrointestinal-manifestations
#11
Catarina Atalaia-Martins, Sandra Barbeiro, Pedro Marcos, Isabel Cotrim, Helena Vasconcelos
Common variable immunodeficiency (CVID) is an immunodeficiency disorder with a high incidence of gastrointestinal (GI) manifestations and an increased risk of gastric malignancy. We report a case of a CVID with mild anemia presenting with multiple GI manifestations: gastric low-grade dysplasia (LGD), enteropathy with villous atrophy, refractory Giardia infection, nodular lymphoid hyperplasia, and inflammatory bowel-like disease. The differential diagnosis with celiac sprue could be challenging because of CVID enteropathy with villous flattening...
2017: ACG Case Reports Journal
https://www.readbyqxmd.com/read/28876552/breast-cancer-and-hiv-a-south-african-perspective-and-a-critical-review-of-the-literature
#12
P Reddy, S Ebrahim, B Singh, S Ramklass, I Buccimazza
BACKGROUND: The diagnosis of breast cancer and concurrent HIV in South Africa is common. The limited current evidence on this subject suggests that the patients thus afflicted appear to be younger, have a more advanced stage of breast cancer, have a higher treatment related complications and poorer outcomes. This paper reviews the literature related to HIV and breast cancer, with a view to improving the standard and quality of care of HIV positive breast cancer patients. METHOD: Pubmed, EBSCOhost, Google scholar and Science Direct electronic databases were searched from 2001 and 2015...
March 2017: South African Journal of Surgery. Suid-Afrikaanse Tydskrif Vir Chirurgie
https://www.readbyqxmd.com/read/28871669/facilitated-subcutaneous-immunoglobulin-fscig-in-autoimmune-cytopenias-associated-with-common-variable-immunodeficiency
#13
Veronica Pedini, Isabella Savore, Giovanna Maria Danieli
BACKGROUND: Common variable immunodeficiency (CVID) is the most common symptomatic primary immune deficiency of adulthood. Besides recurrent infections, autoimmune disorders-mainly cytopenias-affect 30% of patients with CVID. OBJECTIVES: To describe the efficacy and safety of facilitated subcutaneous immunoglobulin (fSCIg), which is a combination of 10% [human] SCIg with recombinant human hyaluronidase for the treatment of CVID-linked cytopenias. METHODS: We describe four women (mean age 54 years) with CVID associated with idiopathic thrombocytopenic purpura (ITP) (n=3) and autoimmune hemolytic anemia (AIHA) (n=1)...
July 2017: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28861081/14%C3%A2-years-after-discovery-clinical-follow-up-on-15-patients-with-inducible-co-stimulator-deficiency
#14
Johanna Schepp, Janet Chou, Andrea Skrabl-Baumgartner, Peter D Arkwright, Karin R Engelhardt, Sophie Hambleton, Tomohiro Morio, Ekkehard Röther, Klaus Warnatz, Raif Geha, Bodo Grimbacher
BACKGROUND: Inducible co-stimulator (ICOS) deficiency was the first monogenic defect reported to cause common variable immunodeficiency (CVID)-like disease in 2003. Since then, 16 patients have been reported worldwide with an increasing range of clinical phenotypes. OBJECTIVE: We sought to compare the clinical and immunological phenotype and provide clinical follow-up and therapeutic approaches for treating ICOS-deficient patients. METHODS: We describe the clinical and laboratory data of 15 patients with available clinical data...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28860769/factors-associated-with-depressive-symptoms-in-people-living-with-hiv-attending-antiretroviral-clinic-at-fitche-zonal-hospital-central-ethiopia-cross-sectional-study-conducted-in-2012
#15
Tebikew Yeneabat, Asres Bedaso, Tadele Amare
BACKGROUND: Depression is one of the most common psychiatric disorders with the prevalence rate ranging from 5% to 10% in the general population and about 60% in people living with human immunodeficiency virus (PLHIV). It has been reported to be more common among women living with HIV. In HIV patients, depression can have negative impacts on their quality of life. OBJECTIVE: This study was aimed at identifying the prevalence of depressive symptoms and associated factors among PLHIV attending the antiretroviral therapy clinic at Fitche Zonal Hospital...
2017: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/28842786/neutropenia-in-patients-with-common-variable-immunodeficiency-a-rare-event-associated-with-severe-outcome
#16
Aurélien Guffroy, Rachel Mourot-Cottet, Laurence Gérard, Vincent Gies, Chantal Lagresle, Aurore Pouliet, Patrick Nitschké, Sylvain Hanein, Boris Bienvenu, Valérie Chanet, Jean Donadieu, Martine Gardembas, Marina Karmochkine, Raphaele Nove-Josserand, Thierry Martin, Vincent Poindron, Pauline Soulas-Sprauel, Fréderic Rieux-Laucat, Claire Fieschi, Eric Oksenhendler, Isabelle André-Schmutz, Anne-Sophie Korganow
BACKGROUND: Common variable immunodeficiency (CVID) is characterized by infections and hypogammaglobulinemia. Neutropenia is rare during CVID. METHODS: The French DEFI study enrolled patients with primary hypogammaglobulinemia. Patients with CVID and neutropenia were retrospectively analyzed. RESULTS: Among 473 patients with CVID, 16 patients displayed neutropenia (lowest count [0-1400]*10(6)/L). Sex ratio (M/F) was 10/6. Five patients died during the follow-up (11 years) with an increased percentage of deaths compared to the whole DEFI group (31...
August 26, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28826735/rapid-cd4-decline-prior-to-antiretroviral-therapy-predicts-subsequent-failure-to-reconstitute-despite-hiv-viral-suppression
#17
Majid Darraj, Leigh Anne Shafer, Shanna Chan, Ken Kasper, Yoav Keynan
HIV-1 infection is characterized by loss of CD4T cells, leading to immunodeficiency. Initiation of antiretroviral therapy (ART) results in suppression of the viral load and increased CD4 counts. Both viral and host factors determine CD4 cell responses to ART with approximately 15-30% of individuals having suboptimal increase of CD4T cell count, most commonly due to lack of compliance to ART. A smaller fraction of patients will have immune reconstitution failure and suboptimal CD4 increase despite suppression of HIV replication, and these individuals are at risk for adverse health outcomes...
August 18, 2017: Journal of Infection and Public Health
https://www.readbyqxmd.com/read/28805315/a-comparison-of-clinical-and-immunologic-phenotypes-in-familial-and-sporadic-forms-of-common-variable-immunodeficiency
#18
Amir Valizadeh, Reza Yazdani, Gholamreza Azizi, Hassan Abolhassani, Asghar Aghamohammadi
Common variable immunodeficiency (CVID) is the most frequent symptomatic primary immunodeficiency disease and its prevalence varies significantly among different population. Minority of CVID patients present a familial aggregation suggesting a higher probability of heritable genetic defects. A total of 235 registered CVID patients were evaluated in this cohort study. Familial and sporadic patients were stratified and demographic information, clinical records, laboratory and molecular data were compared among these two groups of patients...
August 11, 2017: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/28791687/skin-signs-of-primary-immunodeficiencies-how-to-find-the-genes-to-check
#19
REVIEW
M Ettinger, J Schreml, K Wirsching, M Berneburg, S Schreml
Primary immunodeficiencies (PIDs) are a heterogeneous group of rare diseases that result from defects in immune system development and/or function. The clinical manifestations of PIDs are highly variable, but most disorders involve at least an increased susceptibility to infection. Furthermore, cutaneous manifestations are very common in PIDs. As an easy accessible organ, skin canbecrucial for early diagnosis and treatment. This is relevant for preventing significant disease-associated morbidity and mortality...
August 9, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28781847/management-of-common-variable-immunodeficiency-by-subcutaneous-igg-self-administration-during-pregnancy-a-case-report
#20
Carolina Marasco, Alberta Venturelli, Luigia Rao, Angelo Vacca, Maria Rosaria Carratù
Patients with common variable immunodeficiency are prone to infections, and this poses a particular challenge during pregnancy, when the requirement for immunoglobulin (Ig) replacement therapy is even more demanding so as to achieve an effective protection also of the fetus. This case report highlights the benefits observed with subcutaneous IgG self-administration in the management of common variable immunodeficiency (CVID) during pregnancy, in terms of efficacy and safety.
August 2017: Clinical Case Reports
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