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Treatment of CVID

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https://www.readbyqxmd.com/read/29881587/improvement-of-common-variable-immunodeficiency-using-embryonic-stem-cell-therapy-in-a-patient-with-lyme-disease-a-clinical-case-report
#1
Richard Horowitz, Phyllis R Freeman
Bone marrow transplantation and stem cell therapies have been used for the treatment of common variable immunodeficiency (CVID) and other life-threatening medical disorders. This is the first known case report in the medical literature describing improvement of both Lyme disease and CVID with human embryonic stem cell therapy.
June 2018: Clinical Case Reports
https://www.readbyqxmd.com/read/29867917/delayed-diagnosis-and-complications-of-predominantly-antibody-deficiencies-in-a-cohort-of-australian-adults
#2
Charlotte A Slade, Julian J Bosco, Tran Binh Giang, Elizabeth Kruse, Robert G Stirling, Paul U Cameron, Fiona Hore-Lacy, Michael F Sutherland, Sara L Barnes, Stephen Holdsworth, Samar Ojaimi, Gary A Unglik, Joseph De Luca, Mittal Patel, Jeremy McComish, Kymble Spriggs, Yang Tran, Priscilla Auyeung, Katherine Nicholls, Robyn E O'Hehir, Philip D Hodgkin, Jo A Douglass, Vanessa L Bryant, Menno C van Zelm
Background: Predominantly antibody deficiencies (PADs) are the most common type of primary immunodeficiency in adults. PADs frequently pass undetected leading to delayed diagnosis, delayed treatment, and the potential for end-organ damage including bronchiectasis. In addition, PADs are frequently accompanied by comorbid autoimmune disease, and an increased risk of malignancy. Objectives: To characterize the diagnostic and clinical features of adult PAD patients in Victoria, Australia...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29849668/evaluation-of-clinical-and-immunological-characteristics-of-children-with-common-variable-immunodeficiency
#3
Gülsüm Alkan, Sevgi Keles, İsmail Reisli
Background: Common variable immunodeficiency (CVID) is a primary immunodeficiency disorder (PID) that typically presents with hypogammaglobulinemia and impaired antibody production. Objectives: This study aimed to promote the awareness of CVID, whose clinical spectrum is quite broad. Methods: The demographic, clinical, and laboratory characteristics of 12 children (seven males and five females) with CVID were analyzed retrospectively. The patients were diagnosed using the diagnostic criteria of the European Society for Primary Immunodeficiencies...
2018: International Journal of Pediatrics
https://www.readbyqxmd.com/read/29780906/hla-a29-negative-birdshot-like-chorioretinopathy-associated-with-common-variable-immunodeficiency
#4
Razek Georges Coussa, Fares Antaki, David E Lederer
Purpose: To report the longest ophthalmic follow-up and the associated ocular complications of HLA-A29 negative Birdshot-like chorioretinopathy (BLCR) associated with common variable immunodeficiency (CVID). Observations: A 22-year-old man known for CVID presented with a 3-month history of decreased visual acuity OS. Funduscopy revealed significant cystoid macular edema OS, as well as optic disk edema and chorioretinal infiltrates without signs of vitritis OU. No infectious, inflammatory or neoplastic etiologies were identified...
June 2018: American Journal of Ophthalmology Case Reports
https://www.readbyqxmd.com/read/29709555/genetic-screening-of-male-patients-with-primary-hypogammaglobulinemia-can-guide-diagnosis-and-clinical-management
#5
Nicolas Vince, Gaël Mouillot, Marion Malphettes, Sophie Limou, David Boutboul, Angélique Guignet, Véronique Bertrand, Philippe Pellet, Pierre-Antoine Gourraud, Patrice Debré, Eric Oksenhendler, Ioannis Théodorou, Claire Fieschi
The precise diagnosis of an immunodeficiency is sometimes difficult to assess, especially due to the large spectrum of phenotypic variation reported among patients. Common variable immunodeficiency disorders (CVID) do not have, for a large part, an identified genetic cause. The identification of a causal genetic mutation is important to confirm, or in some cases correct, the diagnosis. We screened >150 male patients with hypogammaglobulinemia for mutations in three genes involved in pediatric X-linked primary immunoglobulin deficiency: CD40LG, SH2D1A and BTK...
July 2018: Human Immunology
https://www.readbyqxmd.com/read/29675923/latent-therapeutic-demand-model-for-the-immunoglobulin-replacement-therapy-of-primary-immune-deficiency-disorders-in-the-usa
#6
J S Stonebraker, J Hajjar, J S Orange
BACKGROUND AND OBJECTIVES: Our research aim is to model latent therapeutic demand (LTD) for the immunoglobulin replacement therapy (IgGRT) of primary immune deficiency disorders (PIDDs) in the USA. Given the high level of variability of IgGRT use and major differences among American and European practices in the management of patients with PIDDs, we develop a USA-specific LTD model for common variable immune deficiency (CVID), hyper IGM syndrome, severe combined immune deficiency, Wiskott-Aldrich syndrome and X-linked agammaglobulinemia (XLA)...
April 20, 2018: Vox Sanguinis
https://www.readbyqxmd.com/read/29618830/evaluation-of-the-tlr-negative-regulatory-network-in-cvid-patients
#7
Roozbeh Sanaei, Nima Rezaei, Asghar Aghamohammadi, Ali-Akbar Delbandi, Shahram Teimourian, Reza Yazdani, Parsova Tavasolian, Fatemeh Kiaee, Nader Tajik
Common variable immunodeficiency (CVID), a clinically symptomatic primary immunodeficiency disease (PID), is characterized by hypogammaglobulinemia leading to recurrent infections and various complications. Recently, some defects in the signaling of TLRs have been identified in CVID patients which led us to investigate the expression of TLR4 and 9 negative regulatory molecules and their upregulation status following their activation. Using TaqMan real-time PCR, SOCS1, TNFAIP3, RFN216, and IRAK-M transcripts among peripheral blood mononuclear cells (PBMCs) were measured with/without TLR4 and 9 activations...
April 5, 2018: Genes and Immunity
https://www.readbyqxmd.com/read/29618687/-successful-cord-blood-transplantation-in-a-patient-with-adult-onset-common-variable-immunodeficiency
#8
Yuhei Harutani, Shinobu Tamura, Hiroshi Kobata, Takehiro Oiwa, Yusuke Yamashita, Toshiki Mushino, Hiroki Hosoi, Shogo Murata, Akinori Nishikawa, Kohsuke Imai, Tomohiro Morio, Takashi Sonoki
Common variable immunodeficiency (CVID) is the most frequently diagnosed congenital immunodeficiency and is characterized by dysfunctional antibody production. It often occurs at the age of ≥10 years. Here we reported a case of a 46-year-old man confirmed with adult-onset CVID. He was effectively treated with cord blood transplant (CBT). The patient was observed with repeated upper respiratory infection a few years back and was referred to our department owing to a marked decrease in neutrophil counts and progression of anemia...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29614902/intravenous-immunoglobulin-ivig-efficiency-in-women-with-common-variable-immunodeficiency-cvid-decreases-significantly-during-pregnancy
#9
Makiko Egawa, Hirokazu Kanegane, Kohsuke Imai, Tomohiro Morio, Naoyuki Miyasaka
Immunoglobulin replacement therapy, including intravenous immunoglobulin (IVIG), is essential for pregnant women with common variable immunodeficiency (CVID) since it prevents infection and improves the health of the newborn. There are no established IVIG treatment protocols for pregnant women with CVID, and the relationship between IVIG treatment and maternal serum IgG changes during pregnancy remains unclear. Therefore, we reviewed the medical charts of four CVID patients, including one receiving subcutaneous immunoglobulin (SCIG), for IVIG dose and frequency, maternal serum IgG changes, obstetrical findings, and perinatal outcomes...
April 3, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29549228/the-significance-of-b-cell-subsets-in-patients-with-unclassified-hypogammaglobulinemia-and-association-with-intravenous-immunoglobulin-replacement-requirement
#10
Sait Karaman, Semiha Bahçeci Erdem, Nesrin Gülez, Ferah Genel
BACKGROUND: Patients with unclassified hypogammaglobulinemia (UCH) constitute a diagnostic and therapeutic dilemma, because information concerning the clinical and immunological characteristics of these patients is insufficient. OBJECTIVE: To evaluate B-cell subsets in cases with UCH and common variable immunodeficiency (CVID) and their association with treatment requirement in UCH patients. METHODS: The study included 41 UCH, 25 CVID, and 36 healthy individuals between the ages of 4-18 years...
March 2018: Iranian Journal of Immunology: IJI
https://www.readbyqxmd.com/read/29532571/sirolimus-as-an-alternative-treatment-in-patients-with-granulomatous-lymphocytic-lung-disease-and-humoral-immunodeficiency-with-impaired-regulatory-t-cells
#11
Angela Deyà-Martínez, Ana Esteve-Solé, Natalia Vélez-Tirado, Veronica Celis, Jordi Costa, Maria Cols, Cristina Jou, Alexandru Vlagea, Ana María Plaza-Martin, Manel Juan, Laia Alsina
BACKGROUND: One of the most frequent non-infectious complications of humoral immunodeficiencies with a CVID-like pattern is a particular form of inflammatory lung disease which is called granulomatous-lymphocytic interstitial lung disease (GLILD). Its development worsens patient prognosis, with a significant decrease in survival. Currently, there are no unified guidelines regarding its management, and different combinations of immunosuppressants have been used with variable success. METHODS: Clinical and radiological data were collected from patient's medical charts...
March 12, 2018: Pediatric Allergy and Immunology
https://www.readbyqxmd.com/read/29368942/autoimmune-hepatitis-as-a-complication-of-common-variable-immunodeficiency
#12
Patrícia de Oliveira Queirós, José Manuel Sousa Martín
In common variable immunodeficiency (CVID) there is a deregulation of the immune system, which frequently leads to an increased risk of infections, but also to autoimmunity phenomena. Autoimmune hepatitis may develop at any time of CVID's evolution, but it is difficult to diagnose due to the frequent absence of autoantibodies and low levels of IgG. Early diagnosis is important because targeted treatment may allow disease improvement. We present a case of autoimmune hepatitis in a patient with CVID.
March 2018: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/29325751/a-difficult-diagnosis-of-coeliac-disease-repeat-duodenal-histology-increases-diagnostic-yield-in-patients-with-concomitant-causes-of-villous-atrophy
#13
Gaetano Cristian Morreale, Luigi Maria Montalbano, Maria Cappello, Emanuele Sinagra, Aroldo Rizzo, Antonio Carroccio
Villous atrophy in absence of coeliac disease (CD)-specific antibodies represents a diagnostic dilemma. We report a case of a woman with anaemia, weight loss and diarrhoea with an initial diagnosis of seronegative CD and a histological documented villous atrophy who did not improve on gluten-free diet due to the concomitant presence of common variable immunodeficiency (CVID) and Giardia lamblia infection. This case report confirms that CD diagnosis in CVID patients is difficult; the combination of anti-endomysial antibodies (EmA-IgA), anti-tissue transglutaminase antibodies (tTG-IgAb) antibodies and total IgA is obligatory in basic diagnostic of CD but in CVID are negative...
December 2017: Arab Journal of Gastroenterology: the Official Publication of the Pan-Arab Association of Gastroenterology
https://www.readbyqxmd.com/read/29277461/intra-cranial-granulomatous-disease-in-common-variable-immunodeficiency-case-series-and-review-of-the-literature
#14
REVIEW
Catherine E Najem, Jason Springer, Richard Prayson, Daniel A Culver, James Fernandez, Jinny Tavee, Rula A Hajj-Ali
BACKGROUND/PURPOSE: Common variable immunodeficiency (CVID) is typically characterized by hypogammaglobulinemia and often but not always recurrent infections. Paradoxically, 8-22% of patients with CVID develop granulomatous disease. Granulomata have been described in many organs including the lungs, skin, liver, spleen, kidneys, eyes, lymph nodes, and intestines. Data about central nervous system (CNS) involvement in CVID are extremely rare. We aim to describe a case series and include an extensive literature review of CNS involvement in CVID to understand the different features and patterns of the disease...
October 20, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29249107/-common-immunodeficiency-variable-in-adults
#15
Patricia María O'Farrill-Romanillos, Diana Andrea Herrera-Sánchez, Cecilia Hernández-Fernández, Eunice Giselle López-Rocha
Primary immunodeficiencies (PIDs) are low-incidence diseases caused by defects in genes involved in the development, maintenance, and regulation of the immune system. Common variable immunodeficiency (CVID) is the most common symptomatic immunodeficiency of adulthood. It has an approximate prevalence of 1 in 25 000-50 000 in the general population, with a delay in diagnosis between 6-7 years. The clinical manifestations of CVID constitute six main categories: infections, pulmonary complications, granulomatous or polyclonal lymphocytic disease, autoimmunity, gastrointestinal diseases and malignancy Most patients must have at least one of the following clinical manifestations (infection, autoimmunity, lymphoproliferation)...
October 2017: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
https://www.readbyqxmd.com/read/29225631/initial-intravenous-immunoglobulin-doses-should-be-based-on-adjusted-body-weight-in-obese-patients-with-primary-immunodeficiency-disorders
#16
Rohan Ameratunga
Background: Immunoglobulin therapy plays a critical role in the treatment of immunodeficiency disorders as well as autoimmune and inflammatory conditions. In immunodeficient patients, there has been controversy whether initial loading doses of intravenous (IVIG) should be based on actual body weight or a calculated parameter such as adjusted body weight in obese patients. Case presentation: I describe a patient with Common Variable Immunodeficiency disorder (CVID) who underwent bariatric surgery for morbid obesity...
2017: Allergy, Asthma, and Clinical Immunology
https://www.readbyqxmd.com/read/29184600/-common-variable-immune-deficiency-lately-revealed-by-gastrointestinal-problems-about-a-case
#17
Fatima Ezzaitouni, Youssef Thiyfa, Mohamed Tahiri, Fouad Haddad, Wafaa Hliwa, Ahmed Bellabah, Wafaa Badre
Common Variable Immune Deficiency (CVID) is rare. It is a constitutional deficit of humoral immunity characterized by recurrent bacterial infections and by increased frequency of tumors, autoimmune or granulomatous diseases. Gastrointestinal manifestations are very variable and sometimes reveal common variable immune deficiency. We report the case of a 31-year old patient with a history of childhood recurrent respiratory infections complicated by bronchiectasis and with a 3-year history of recurrent glairy diarrhea...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/29114494/giardiasis-mimicking-celiac-disease-in-a-patient-of-common-variable-immunodeficiency
#18
Kumar Saurabh, Vijaya Lakshmi Nag, Daisy Khera, Poonam Elhence
An adolescent boy presented to pediatrics outpatient department with complaints of recurrent diarrhea, nausea, vomiting, and pedal edema since 3-4 months, with no relief even after taking treatment. His investigation revealed decreased serum IgA, IgG, and IgM levels with hypoproteinemia. Duodenal biopsy showed features of celiac disease (CD), but tissue transglutaminase IgA was negative. In stool examination, plenty of Giardia lamblia cysts and eggs of Hymenolepis nana were present. He improved on treatment and remained asymptomatic for 4 months...
July 2017: Tropical Parasitology
https://www.readbyqxmd.com/read/28973009/tlr9-stimulation-of-b-cells-induces-transcription-of-p53-and-prevents-spontaneous-and-irradiation-induced-cell-death-independent-of-dna-damage-responses-implications-for-common-variable-immunodeficiency
#19
Kristine Lillebø Holm, Randi Gussgard Syljuåsen, Grete Hasvold, Lene Alsøe, Hilde Nilsen, Kristina Ivanauskiene, Philippe Collas, Sergey Shaposhnikov, Andrew Collins, Randi Larsen Indrevær, Pål Aukrust, Børre Fevang, Heidi Kiil Blomhoff
In the present study, we address the important issue of whether B-cells protected from irradiation-induced cell death, may survive with elevated levels of DNA damage. If so, such cells would be at higher risk of gaining mutations and undergoing malignant transformation. We show that stimulation of B-cells with the TLR9 ligands CpG-oligodeoxynucleotides (CpG-ODN) prevents spontaneous and irradiation-induced death of normal peripheral blood B-cells, and of B-cells from patients diagnosed with Common variable immunodeficiency (CVID)...
2017: PloS One
https://www.readbyqxmd.com/read/28937520/evans-syndrome-as-first-manifestation-of-primary-immunodeficiency-in-clinical-practice
#20
Libny Martínez-Valdez, Angela Deyà-Martínez, María T Giner, Rubén Berrueco, Ana Esteve-Solé, Manel Juan, Ana M Plaza-Martín, Laia Alsina
BACKGROUND: Evans syndrome (ES) is a rare immune disorder in children, manifested by simultaneous or sequential autoimmune cytopenias (ACs) of unknown cause and having a chronic course with periods of exacerbation and remission. Some primary immunodeficiencies (PIDs) may present with autoimmune manifestations without infections, masking suspicion of them. The PIDs that can typically manifest as ES are autoimmune lymphoproliferative syndrome and common variable immunodeficiency (CVID)...
October 2017: Journal of Pediatric Hematology/oncology
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