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Hypogammaglobulinemia

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https://www.readbyqxmd.com/read/28640123/recurrent-campylobacter-jejuni-bacteremia-in-a-patient-with-hypogammaglobulinemia-a-case-report
#1
Youie Kim, Ju Ae Shin, Seung Beom Han, Bin Cho, Dae Chul Jeong, Jin Han Kang
RATIONALE: Although some cases of recurrent bacteremia due to Campylobacter jejuni have been reported in immunocompromised patients, antibiotic treatment strategies to eradicate C. jejuni and prevent recurrent infections in immunocompromised patients have not been established. Authors' experience of such rare cases should be shared for improving patients' outcomes. PATIENT CONCERNS: An 18-year-old boy with hypogammaglobulinemia, who received intravenous immunoglobulin replacement therapy every 3 weeks, was admitted to hospital repeatedly due to recurrent diarrhea and cellulitis of the leg...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28639577/characteristics-of-good-s-syndrome-in-china-a-systematic-review
#2
REVIEW
Jin-Pei Dong, Wen Gao, Gui-Gen Teng, Yu Tian, Hua-Hong Wang
BACKGROUND: Good's syndrome (GS) is a rare disease characterized by thymoma, hypogammaglobulinemia, low or absent B-cells, decreased T-cells, an inverted CD4+/CD8+ T-cell ratio and reduced T-cell mitogen proliferative responses. GS is difficult to diagnose preoperatively due to its rarity and lack of typical symptoms, the characteristics of Chinese GS patients are still lacking. This study aimed to systematically review all the clinical, laboratory, and immunologic findings of reported cases of Chinese patients with GS...
July 5, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28603521/novel-combined-immune-deficiency-and-radiation-sensitivity-blended-phenotype-in-an-adult-with-biallelic-variations-in-zap70-and-rnf168
#3
Ivan K Chinn, Robert P Sanders, Asbjørg Stray-Pedersen, Zeynep H Coban-Akdemir, Vy Hong-Diep Kim, Harjit Dadi, Chaim M Roifman, Troy Quigg, James R Lupski, Jordan S Orange, I Celine Hanson
With the advent of high-throughput genomic sequencing techniques, novel genetic etiologies are being uncovered for previously unexplained Mendelian phenotypes, and the underlying genetic architecture of disease is being unraveled. Although most of these "mendelizing" disease traits represent phenotypes caused by single-gene defects, a percentage of patients have blended phenotypes caused by pathogenic variants in multiple genes. We describe an adult patient with susceptibility to bacterial, herpesviral, and fungal infections...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28575178/predominance-of-cd4-t-cells-in-t-cell-histiocyte-rich-large-b-cell-lymphoma-and-identification-of-a-subset-of-patients-with-peripheral-b-cell-lymphopenia
#4
Christian Kunder, Michael J Cascio, Anthony Bakke, Girish Venkataraman, Dennis P O'Malley, Robert S Ohgami
Objectives: T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) is a morphologic variant of large B-cell lymphoma whose flow cytometry findings are not well characterized. Methods: Nineteen cases with flow cytometric immunophenotyping were identified from the case records of four institutions between 2001 and 2016. Results: In most cases, neoplastic B cells were not detected by flow cytometry. Overall, cases showed a predominance of CD4+ T cells, which in some cases was marked...
June 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28571224/waldmann-s-disease-primary-intestinal-lymphangiectasia-with-atrial-septal-defect
#5
Shrikiran Aroor, Suneel Mundkur, Shravan Kanaparthi, Sandeep Kumar
Waldmann's disease or Primary Intestinal Lymphangiectasia (PIL) is a rare disorder of gastrointestinal tract characterized by dilated lymphatics and widened villi causing leakage of lymph into intestinal lumen. Loss of lymph leads to hypoalbuminemia, hyogammaglobulinemia and lymphopenia. Secondary lymphangiectasia occurs secondary to an elevated lymphatic pressure as in lymphoma, systemic lupus erythematosus, constrictive pericarditis, cardiac surgeries (Fontan's procedure), inflammatory bowel disease and malignancies...
April 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28543378/renal-transplantation-in-patients-with-pre-existing-hypogammaglobulinemia
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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...
July 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
#17
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
#18
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
#19
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
#20
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
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