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Hypogammaglobulinemia

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https://www.readbyqxmd.com/read/28543378/renal-transplantation-in-patients-with-pre-existing-hypogammaglobulinemia
#1
K P Lund, H Bruunsgaard, H V Marquart, S S Sørensen
Hypogammaglobulinemia is well-characterized as a common phenomenon after kidney transplantation. However, no reports of pre-existing hypogammaglobulinemia from kidney transplantation seem to be available. We have reviewed three patients who developed hypogammaglobulinemia prior to kidney transplantation, and all three were treated successfully with immunoglobulin-replacement therapy before and after kidney transplantation. The kidney grafts were functioning at follow-up 1.5 to 8 years (mean: 3.6 years) after transplantation, and there were no diagnosed episodes of clinical rejections and no severe infection complications post-transplantation...
May 22, 2017: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/28539185/frequency-of-untreated-hypogammaglobulinemia-in-bronchiectasis
#2
Melanie A Ruffner, Timothy R Aksamit, Byron Thomashow, Radmila Choate, Angela DiMango, Gerard M Turino, Anne E O'Donnell, Margaret M Johnson, Kenneth N Olivier, Kevin Fennelly, Charles L Daley, Kevin L Winthrop, Mark L Metersky, Matthias A Salathe, Michael R Knowles, M Leigh Anne Daniels, Peadar G Noone, Gregory Tino, David E Griffith, Kathleen E Sullivan
No abstract text is available yet for this article.
May 20, 2017: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/28532655/homozygous-tcf3-mutation-is-associated-with-severe-hypogammaglobulinemia-and-b-cell-acute-lymphoblastic-leukemia
#3
Meriem Ben-Ali, Jing Yang, Koon Wing Chan, Imen Ben-Mustapha, Najla Mekki, Chaouki Benabdesselem, Fethi Mellouli, Mohamed Bejaoui, WanLing Yang, Lamia Aissaoui, Yu Lung Lau, Mohamed-Ridha Barbouche
The identification of a homozygous TCF3 gene mutation in a patient presenting with severe hypogammaglobulinemia and acute lymphoblastic leukemia supports the crucial role of this transcription factor in normal B-lymphocyte development.
May 19, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28513979/identification-of-a-de-novo-variant-in-chuk-in-a-patient-with-an-eec-aec-syndrome-like-phenotype-and-hypogammaglobulinemia
#4
Kriti D Khandelwal, Charlotte W Ockeloen, Hanka Venselaar, Cécile Boulanger, Bénédicte Brichard, Etienne Sokal, Rolph Pfundt, Tuula Rinne, Ellen van Beusekom, Marjon Bloemen, Gerrit Vriend, Nicole Revencu, Carine E L Carels, Hans van Bokhoven, Huiqing Zhou
The cardinal features of Ectrodactyly, Ectodermal dysplasia, Cleft lip/palate (EEC), and Ankyloblepharon-Ectodermal defects-Cleft lip/palate (AEC) syndromes are ectodermal dysplasia (ED), orofacial clefting, and limb anomalies. EEC and AEC are caused by heterozygous mutations in the transcription factor p63 encoded by TP63. Here, we report a patient with an EEC/AEC syndrome-like phenotype, including ankyloblepharon, ED, cleft palate, ectrodactyly, syndactyly, additional hypogammaglobulinemia, and growth delay...
May 17, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28512785/clinical-immunological-molecular-analyses-and-outcomes-of-iranian-patients-with-lrba-deficiency-a-longitudinal-study
#5
Gholamreza Azizi, Hassan Abolhassani, Seyed Alireza Mahdaviani, Zahra Chavoshzadeh, Peyman Eshghi, Reza Yazdani, Fatemeh Kiaee, Mohammadreza Shaghaghi, Javad Mohammadi, Nima Rezaei, Lennart Hammarström, Asghar Aghamohammadi
BACKGROUND: LPS-responsive beige-like anchor protein (LRBA) deficiency is a combined immunodeficiency caused by mutation in LRBA gene. The patients have a variety of clinical symptoms including hypogammaglobulinemia, recurrent infections, autoimmunity and enteropathy. METHODS: A total of 17 LRBA-deficient patients were enrolled in this longitudinal study. For all patients, demographic information, clinical records, laboratory and molecular data were collected. RESULTS: Hypogammaglobulinemia were reported in 14 (82...
May 17, 2017: Pediatric Allergy and Immunology
https://www.readbyqxmd.com/read/28501028/multicentric-castleman-disease-of-hyaline-vascular-variant-presenting-with-unusual-systemic-manifestations-a-case-report
#6
B M D B Basnayake, A W M Wazil, T Kannangara, N V I Ratnatunga, S Hewamana, A M Ameer
BACKGROUND: Castleman disease is a rare lymphoproliferative disorder presenting with localized or disseminated lymphadenopathy and systemic manifestations. It can be categorized in numerous ways, such as unicentric versus multicentric, histopathological variants (hyaline-vascular, plasma cell, and mixed), or subtypes based on causative viral infections (human immunodeficiency virus, human herpesvirus-8, or Kaposi sarcoma herpesvirus). Presentation ranges from asymptomatic to symptoms involving multiple organs...
May 14, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28499783/cd21-deficiency-in-2-siblings-with-recurrent-respiratory-infections-and-hypogammaglobulinemia
#7
Jeremie Rosain, Charline Miot, Nathalie Lambert, Marie-Christine Rousselet, Isabelle Pellier, Capucine Picard
No abstract text is available yet for this article.
May 10, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28491220/a-primary-intestinal-lymphangiectasia-hiding-the-diagnosis-of-pleural-and-pericardial-tuberculosis-a-clinical-observation
#8
Sanaa Hammi, Hajar Berrani, Thami Benouchen, Naima Lamlami, Imane Elkhiyat, Jamal Eddine Bourkadi
Primary intestinal lymphangiectasia (Waldmann's disease) is an exudative enteropathy characterized by lymph leakage into the small bowel lumen leading to hypoalbuminemia, hypogammaglobulinemia and lymphopenia (particularly T-cell). The diagnosis is based on viewing the duodenal lymphangiectasia. A 20 years old female patient, treated for a primary intestinal lymphangiectasia, has consulted for anasarca. Etiological work-up reveals pleural and pericardial tuberculosis. The clinical aggravation of an enteropathy, particularly in adulthood, requires a search for a secondary etiology...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28488125/recurrent-infections-in-a-patient-with-psoriatic-arthritis-and-hypogammaglobulinemia-treated-with-conventional-and-biologic-disease-modifying-anti-rheumatic-drugs-a-primary-or-secondary-entity
#9
REVIEW
Ewa Więsik-Szewczyk, Aleksandra Kucharczyk, Katarzyna Świerkocka, Elżbieta Rutkowska, Karina Jahnz-Różyk
A 54-year-old man with confirmed psoriatic arthritis, treated with conventional and biologic disease-modifying anti-rheumatic drugs, suffered from severe, recurrent respiratory tract infections. He was found to have hypogammaglobulinemia. Further investigations confirmed the diagnosis of common variable immunodeficiency. Introduction of immunoglobulin G replacement therapy allowed for safe and effective treatment of psoriatic arthritis with etanercept and methotrexate. Patients with a history of recurrent infections on disease-modifying anti-rheumatic drugs and hypogammaglobulinemia should be assessed for primary antibody immunodeficiencies, even in adulthood...
May 9, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28482391/-two-families-of-x-linked-lymphoproliferative-disease-type-1-characterized-by-agammaglobulinemia
#10
W Y Li, J S Chen, Q Zhao, R X Dai, Y P Wang, H Y Zhao, X M Chen, X H Xue, X Y Sun, X M Tang, Y Zhang, Y Ding, X D Zhao, Z Y Zhang
Objective: To investigate the clinical and immunological laboratory features, mutations in SH2D1A gene and SAP protein expression in four children of two families with X-linked lymphoproliferative disease type 1(XLP-1). Method: Four patients (Family A including Patient 1 and Patient 2, Family B including Patient 3 and Patient 4) and their maternal relatives were enrolled in this study. The clinical manifestation, EBV infection status and chest CT scan were analyzed. The absolute and relative numbers of lymphocyte subsets, T lymphocyte proliferative response, SAP protein expression were assessed by flow cytometry...
May 4, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28476239/defective-il-4-signaling-in-t-cells-defines-severe-common-variable-immunodeficiency
#11
Eli Taraldsrud, Børre Fevang, Silje F Jørgensen, Kristine Moltu, Vera Hilden, Kjetil Taskén, Pål Aukrust, June H Myklebust, Johanna Olweus
Common variable immunodeficiency (CVID) is defined by hypogammaglobulinemia and B-cell dysfunction, with significant clinical and immunological heterogeneity. Severe non-infectious complications, such as autoimmunity, granulomatous disease and splenomegaly, constitute a major cause of morbidity in CVID patients. T cells are generally regarded important for development of these clinical features. However, while T-cell abnormalities have been found in CVID patients, functional characteristics of T cells corresponding to well-defined clinical subtypes have not been identified...
May 2, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28472507/a-case-report-of-hypoglycemia-and-hypogammaglobulinemia-david-syndrome-in-a-patient-with-a-novel-nfkb2-mutation
#12
Rayhan A Lal, Laura K Bachrach, Andrew R Hoffman, Jingga Inlora, Shannon Rego, Michael P Snyder, David B Lewis
Context: DAVID syndrome (Deficient Anterior pituitary with Variable Immune Deficiency) is a rare disorder in which children present with symptomatic ACTH deficiency preceded by hypogammaglobulinemia from B-cell dysfunction with recurrent infections, termed common variable immunodeficiency (CVID). Subsequent whole exome sequencing studies have revealed germline heterozygous C-terminal mutations of NFKB2 as either a cause of DAVID syndrome or of CVID without clinical hypopituitarism. However, to the best of our knowledge there have been no cases in which the endocrinopathy has presented in the absence of a prior clinical history of CVID...
May 3, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28456620/serum-immunoglobulin-g-and-risk-of-exacerbations-and-hospitalizations-in-chronic-obstructive-pulmonary-disease
#13
Fernando Sergio Leitao Filho, Seung Won Ra, Andre Mattman, Robert S Schellenberg, Nick Fishbane, Gerard J Criner, Prescott G Woodruff, Stephen C Lazarus, Richard Albert, John E Connett, Meilan K Han, Fernando J Martinez, Janice M Leung, S F Paul Man, Shawn D Aaron, Robert M Reed, Don D Sin
Hypogammaglobulinemia (total IgG levels < 7.0 g/L) is present in 1 in 4 patients with moderate to severe COPD and is associated with 50% to 100% increase in the risk of exacerbations and hospitalizations.
April 26, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28450687/thymoma-with-immunodeficiency-good-syndrome-associated-with-myasthenia-gravis
#14
Shunsuke Takai, Asako Tagawa, Tomoko Ogawa, Hiroyuki Kato, Noriko Saito, Shinya Okada
Good syndrome is a rare condition in which thymoma is associated with hypogammaglobulinemia; it is characterized by repeated respiratory or systemic infections caused by bacteria, viruses, and fungi, as well as with various autoimmune disorders such as pure red cell aplasia. A 65-year-old woman was admitted to our hospital with ptosis and abdominal muscle weakness. Based on the presence of anti-acetylcholine receptor (Ach-R) antibodies, she was diagnosed with myasthenia gravis (MG). At that time, invasive thymoma of Masaoka stage IVa was also detected...
April 27, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28449602/reference-values-for-b-cell-surface-markers-and-co-receptors-associated-with-primary-immune-deficiencies-in-healthy-turkish-children
#15
Elif Azarsiz, Neslihan Edeer Karaca, Guzide Aksu, Necil Kutukculer
In order to evaluate B-lymphocyte subsets of patients with primary immunodeficiencies, the normal values for national healthy children have to be used as a reference. Recently, B-cell co-receptor markers (CD19, CD21, and CD81) and CD20, CD22, and CD27 deficiencies have been reported in relation with different primary immunodeficiency diseases. The objective of this study was to establish national reference values for B-lymphocyte co-receptors and some surface markers, CD20, CD22, CD27, as well as classic lymphocyte subsets in the peripheral blood of healthy children...
April 1, 2017: International Journal of Immunopathology and Pharmacology
https://www.readbyqxmd.com/read/28423227/a-national-survey-of-screening-and-management-of-hypogammaglobulinemia-in-canadian-transplantation-centers
#16
Samuel Bourassa-Blanchette, Greg Knoll, Jason Tay, Christopher Bredeson, Donald W Cameron, Juthaporn Cowan
BACKGROUND: Infection remains one of the most common transplant-related causes of death in patients undergoing transplantation. Secondary hypogammaglobulinemia (HGG) as a component of immune suppression and deficiency is associated with both solid organ transplantation (SOT) and hematopoietic cell transplantation (HCT). Available data and clinical experience for the supplementation of immunoglobulin (Ig) in these patients is conflicting, and differing clinical opinion accounts for non-uniform practice in the use of Ig treatment...
April 18, 2017: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/28414152/rituximab-for-immunologic-renal-disease-what-the-nephrologist-needs-to-know
#17
REVIEW
Andreas Kronbichler, Martin Windpessl, Herwig Pieringer, David R W Jayne
Rituximab (RTX), a chimeric, monoclonal anti-CD20 antibody, is increasingly used in immune-mediated renal diseases. While licensed in the induction treatment of ANCA-associated vasculitis, it represents one of the most commonly prescribed off-label drugs. Much of the information regarding its safety has been drawn from experience in hematology and rheumatology. Ample evidence illustrates the safety of RTX, however, rare but serious adverse events have emerged that include progressive multifocal leucoencephalopathy and hepatitis B reactivation...
April 13, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28394553/drug-induced-oral-ulcerations-case-report
#18
Vanja Vučičević Boras, Snježana Židovec-Lepej, Branka Marinović, Sven Seiwerth, Ivana Škrinjar, Dražen Pulanić, Danica Vidović Juras
A 70-year-old patient was admitted to the Department of Oral Medicine for multiple oral ulcerations on the left buccal mucosa, around 0.5 cm in diameter, as well as on the gingiva. Otherwise, the patient suffered from chronic lymphocytic leukemia, hypogammaglobulinemia, chronic renal insufficiency, with complete afunction of the right kidney, asthma, hypertension, gastritis and prostate hyperplasia. Differential diagnosis of oral ulcerations included drug induced oral ulcerations, paraneoplastic pemphigus, viral ulcerations (cytomegalovirus, herpes simplex viruses), fungal ulcerations (candidiasis, aspergillosis, histoplasmosis, cryptococcosis) and bacterial ulcerations, as well as neutropenic ulcers...
June 2016: Acta Clinica Croatica
https://www.readbyqxmd.com/read/28385786/host-virus-and-pneumococcus-specific-immune-responses-in-high-count-monoclonal-b-cell-lymphocytosis-and-chronic-lymphocytic-leukemia-implications-for-disease-progression
#19
Ignacio Criado, Santiago Muñoz-Criado, Arancha Rodríguez-Caballero, Wendy G Nieto, Alfonso Romero, Paulino Fernández-Navarro, Miguel Alcoceba, Teresa Contreras, Marcos González, Alberto Orfao, Julia Almeida
Patients diagnosed with chronic lymphocytic leukemia display a high incidence of infections due to an associated immunodeficiency that includes hypogammaglobulinemia. A higher risk of infections has also been recently reported for high-count monoclonal B-cell lymphocytosis, while no information is available in low-count monoclonal B-cell lymphocytosis. Here, we evaluated the status of the humoral immune system in patients with chronic lymphocytic leukemia (n=58), as well as in low- (n=71) and high-count (n=29) monoclonal B-cell lymphocytosis vs...
April 6, 2017: Haematologica
https://www.readbyqxmd.com/read/28382272/pure-red-cell-aplasia-associated-with-good-syndrome
#20
Masayuki Okui, Takashi Yamamichi, Ayaka Asakawa, Masahiko Harada, Hirotoshi Horio
Pure red cell aplasia (PRCA) and hypogammaglobulinemia are paraneoplastic syndromes that are rarer than myasthenia gravis in patients with thymoma. Good syndrome coexisting with PRCA is an extremely rare pathology. We report the case of a 50-year-old man with thymoma and PRCA associated with Good syndrome who achieved complete PRCA remission after thymectomy and postoperative immunosuppressive therapy, and provide a review of the pertinent literature.
April 2017: Korean Journal of Thoracic and Cardiovascular Surgery
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