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Journal of Clinical Immunology

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https://www.readbyqxmd.com/read/28714047/erratum-to-evaluation-of-the-safety-tolerability-and-pharmacokinetics-of-gammaplex%C3%A2-10-versus-gammaplex%C3%A2-5-in-subjects-with-primary-immunodeficiency
#1
Richard L Wasserman, Isaac R Melamed, Mark R Stein, Stephen Jolles, Miranda Norton, James N Moy
No abstract text is available yet for this article.
July 17, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28711959/a-multicentre-study-on-the-efficacy-safety-and-pharmacokinetics-of-iqymune%C3%A2-a-highly-purified-10-liquid-intravenous-immunoglobulin-in-patients-with-primary-immune-deficiency
#2
Gergely Krivan, Ludmila Chernyshova, Larysa Kostyuchenko, Andrzej Lange, Zoltan Nyul, Beata Derfalvi, Jacek Musial, Anne Bellon, Martin Kappler, Alain Sadoun, Ewa Bernatowska
This multicentre, open-label, prospective, single-arm study was designed to evaluate the efficacy, pharmacokinetics, and safety of IqYmune®, a highly purified 10% polyvalent immunoglobulin preparation for intravenous administration in patients with primary immunodeficiency. IqYmune® was administered to 62 patients (aged 2-61 years) with X-linked agammaglobulinemia or common variable immune deficiency at a dose from 0.22 to 0.97 g/kg every 3 to 4 weeks for 12 months with an infusion rate up to 8 mL/kg/h...
July 15, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28702714/immunodeficiency-in-two-female-patients-with-incontinentia-pigmenti-with-heterozygous-nemo-mutation-diagnosed-by-lps-unresponsiveness
#3
Hidenori Ohnishi, Yuka Kishimoto, Tomohide Taguchi, Norio Kawamoto, Mina Nakama, Tomoki Kawai, Manabu Nakayama, Osamu Ohara, Kenji Orii, Toshiyuki Fukao
PURPOSE: Anhidrotic ectodermal dysplasia with immunodeficiency (EDA-ID) is caused by mutations in the NF-κB essential modulator (NEMO) or NF-κB inhibitor, alpha (IKBA) genes. A heterozygous NEMO mutation causes incontinentia pigmenti (IP) in females, while a hemizygous hypomorphic mutation of NEMO causes EDA-ID in males. In general, immunodeficiency is not shown in IP patients. Here, we investigated two female patients with IP and immunodeficiency. METHODS: The patients were initially suspected to have IRAK4 deficiency and Mendelian susceptibility to mycobacterial disease, respectively, because of recurrent pneumonia with delayed umbilical cord detachment or disseminated mycobacterial infectious disease...
July 12, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28698914/multiple-brain-abscesses-caused-by-trichosporon-inkin-in-a-patient-with-x-linked-chronic-granulomatous-disease-cgd-successfully-treated-with-antifungal-therapy
#4
LETTER
Joud Hajjar, Alejandro Restrepo, Heta Javeri, Nathan P Wiederhold, Alexander M Papanastassiou, Thomas F Patterson
No abstract text is available yet for this article.
July 11, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28695366/assessment-of-local-adverse-reactions-to-subcutaneous-immunoglobulin-scig-in-clinical-trials
#5
LETTER
Mark Ballow, Richard L Wasserman, Stephen Jolles, Helen Chapel, Mel Berger, Siraj A Misbah
No abstract text is available yet for this article.
July 10, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28681255/delayed-puberty-and-gonadal-failure-in-patients-with-hax1-mutation
#6
Sukru Cekic, Halil Saglam, Orhan Gorukmez, Tahsin Yakut, Omer Tarim, Sara S Kilic
PURPOSE: Homozygous mutations in the HAX1 gene cause an autosomal recessive form of severe congenital neutropenia (SCN). There are limited data on cases of gonadal insufficiency that involve the HAX1 gene mutation. We aimed to evaluate the pubertal development and gonadal functions of our patients with a p.Trp44X mutation in the HAX1 gene. METHOD: Pubertal development, physical and laboratory findings of one male and seven female patients with HAX1 deficiency were evaluated...
July 5, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28639167/azathioprine-associated-complete-nk-cell-deficiency
#7
LETTER
Amir B Orandi, Tiphanie P Vogel, Molly P Keppel, Elizabeth C Utterson, Megan A Cooper
No abstract text is available yet for this article.
June 22, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28639166/ifn%C3%A9-block-treosulfan-conditioning-and-%C3%AE-%C3%AE-t-cell-deplete-pbsct-for-xiap-deficient-hlh
#8
LETTER
Ciara O'Rafferty, Mark Velangi, Sarah Lawson, Prashant Hiwarkar, Jayashree Motwani
No abstract text is available yet for this article.
June 21, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28631025/diverse-autoantibody-reactivity-in-cartilage-hair-hypoplasia
#9
LETTER
Catherine M Biggs, Svetlana Kostjukovits, Kerry Dobbs, Saila Laakso, Paula Klemetti, Helena Valta, Mervi Taskinen, Outi Mäkitie, Luigi D Notarangelo
No abstract text is available yet for this article.
June 19, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28624913/persistent-skin-pouches-following-subcutaneous-immunoglobulin-infusions-in-a-girl-with-immunodeficiency-bullous-skin-lesions-and-melanosis-oculi
#10
LETTER
Renate Krüger, Cornelia Feiterna-Sperling, Ulrike Blume-Peytavi, Birgit Lala, Horst von Bernuth, Volker Wahn
No abstract text is available yet for this article.
June 17, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28601916/thyroid-carcinoma-in-a-child-with-activated-phosphoinositide-3-kinase-%C3%AE-syndrome-somatic-effect-of-a-germline-mutation
#11
LETTER
Giorgia Bucciol, Lien Willems, Esther Hauben, Anne Uyttebroeck, Marijke Proesmans, Isabelle Meyts
No abstract text is available yet for this article.
June 11, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28597145/quality-of-life-treatment-beliefs-and-treatment-satisfaction-in-children-treated-for-primary-immunodeficiency-with-scig
#12
Serge Sultan, Émélie Rondeau, Marie-Claude Levasseur, Renée Dicaire, Hélène Decaluwe, Élie Haddad
Despite the development of subcutaneous treatment, children and adolescents with primary immunodeficiency (PID) are vulnerable to a lower quality of life (QoL) than non-clinical participants. Comparisons have been offered in rare reports with limited sample sizes. No description is available of treatment beliefs and treatment satisfaction with standard tools. The objective of this study was to describe a large sample of patients with pediatric PID on QoL, treatment beliefs and satisfaction, and identify perceived benefits and issues of treatment both in children and parents...
June 8, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28597144/multicolor-flow-cytometry-for-the-diagnosis-of-primary-immunodeficiency-diseases
#13
Takehiro Takashima, Miko Okamura, Tzu-Wen Yeh, Tsubasa Okano, Motoi Yamashita, Keisuke Tanaka, Akihiro Hoshino, Noriko Mitsuiki, Masatoshi Takagi, Eiichi Ishii, Kohsuke Imai, Hirokazu Kanegane, Tomohiro Morio
PURPOSE: Primary immunodeficiency diseases (PIDDs) are rare inherited diseases that impair the human immune system. We established a multicolor flow cytometric assay to comprehensively evaluate the immune status and immunological characteristics of patients with PIDDs. METHODS: Fifty-nine normal controls and 75 patients with PIDDs, including X-linked severe combined immunodeficiency (X-SCID), X-linked agammaglobulinemia (XLA), X-linked hyper IgM syndrome (X-HIGM), ataxia telangiectasia (AT), Wiskott-Aldrich syndrome (WAS), hyper IgE syndrome (HIES), and chronic mucocutaneous candidiasis disease (CMCD), were enrolled in this study...
June 8, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28589420/the-use-of-salmonella-typhim-vaccine-to-diagnose-antibody-deficiency
#14
Mary T Bausch-Jurken, James W Verbsky, Katherine A Gonzaga, Nancy P Elms, Mary K Hintermeyer, Stephen B Gauld, John M Routes
PURPOSE: The specific antibody response to the unconjugated 23-valent pneumococcal polysaccharide vaccine is one of the most common tests used to assess for possible humoral immunodeficiency. The results can be difficult to interpret because most people have been immunized with one or more of the pneumococcal vaccines and there is controversy regarding what constitutes a normal response. To circumvent this problem, we developed an ELISA to measure IgG-specific antibodies to the Salmonella Vi Typhim (S...
June 7, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28508932/enterovirus-related-immune-reconstitution-inflammatory-syndrome-iris-following-haploidentical-stem-cell-transplantation-in-an-mhc-class-ii-deficient-child
#15
LETTER
Ravi M Shah, Sheila Waugh, Khuen F Ng, Andrew R Gennery, Mary Slatter, Andrew J Cant
No abstract text is available yet for this article.
May 16, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28503715/deficiency-of-interleukin-1-receptor-antagonist-dira-report-of-the-first-indian-patient-and-a-novel-deletion-affecting-il1rn
#16
Leonardo O Mendonca, Louise Malle, Frank X Donovan, Settara C Chandrasekharappa, Gina A Montealegre Sanchez, Megha Garg, Ulf Tedgard, Mariana Castells, Shiv S Saini, Sourabh Dutta, Raphaela Goldbach-Mansky, Deepti Suri, Adriana A Jesus
PURPOSE: Deficiency of interleukin-1 receptor antagonist (DIRA) is a rare life-threatening autoinflammatory disease caused by autosomal recessive mutations in IL1RN. DIRA presents clinically with early onset generalized pustulosis, multifocal osteomyelitis, and elevation of acute phase reactants. We evaluated and treated an antibiotic-unresponsive patient with presumed DIRA with recombinant IL-1Ra (anakinra). The patient developed anaphylaxis to anakinra and was subsequently desensitized...
May 15, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28597146/human-i%C3%AE%C2%BAb%C3%AE-gain-of-function-a-severe-and-syndromic-immunodeficiency
#17
REVIEW
Bertrand Boisson, Anne Puel, Capucine Picard, Jean-Laurent Casanova
Germline heterozygous gain-of-function (GOF) mutations of NFKBIA, encoding IκBα, cause an autosomal dominant (AD) form of anhidrotic ectodermal dysplasia with immunodeficiency (EDA-ID). Fourteen unrelated patients have been reported since the identification of the first case in 2003. All mutations enhanced the inhibitory activity of IκBα, by preventing its phosphorylation on serine 32 or 36 and its subsequent degradation. The mutation certainly or probably occurred de novo in 13 patients, whereas it was inherited from a parent with somatic mosaicism in one patient...
July 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28540525/identification-of-22q11-2-deletion-syndrome-via-newborn-screening-for-severe-combined-immunodeficiency
#18
Jessica C Barry, Terrence Blaine Crowley, Soma Jyonouchi, Jennifer Heimall, Elaine H Zackai, Kathleen E Sullivan, Donna M McDonald-McGinn
PURPOSE: Chromosome 22q11.2 deletion syndrome (22q11.2DS), the most common cause of DiGeorge syndrome, is quite variable. Neonatal diagnosis traditionally relies on recognition of classic features and cytogenetic testing, but many patients come to attention only following identification of later onset conditions, such as hypernasal speech due to palatal insufficiency and developmental and behavioral differences including speech delay, autism, and learning disabilities that would benefit from early interventions...
July 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28536745/health-related-quality-of-life-in-adult-patients-with-common-variable-immunodeficiency-disorders-and-impact-of-treatment
#19
Nicholas L Rider, Carleigh Kutac, Joud Hajjar, Chris Scalchunes, Filiz O Seeborg, Marcia Boyle, Jordan S Orange
PURPOSE: Common variable immunodeficiency disorder (CVID) is a primary immunodeficiency disease (PIDD) often associated with severe and chronic infections. Patients commonly receive immunoglobulin (Ig) treatment to reduce the cycle of recurrent infection and improve physical functioning. However, how Ig treatment in CVID affects quality of life (QOL) has not been thoroughly evaluated. The purpose of a recent Immune Deficiency Foundation (IDF) mail survey was to assess the factors that are associated with QOL in patients with CVID receiving Ig treatment...
July 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28523402/advances-in-the-care-of-primary-immunodeficiencies-pids-from-birth-to-adulthood
#20
Nizar Mahlaoui, Klaus Warnatz, Alison Jones, Sarita Workman, Andrew Cant
Primary immunodeficiencies (PIDs) are a widely heterogeneous group of inherited defects of the immune system consisting of many clinical phenotypes with at least 300 underlying genetic deficits currently known. Patients with PIDs can present with, or develop during the course of their life, a susceptibility to recurrent and chronic infection along with autoimmune, allergic, inflammatory, and/or proliferative disorders, all potentially leading to end-organ damage. In recent years, a combination of basic and clinical research has greatly improved understanding of the underlying immunological and genetic defects in PIDs, leading to improved diagnosis, classification, and treatment approaches...
July 2017: Journal of Clinical Immunology
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