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Journal of Clinical Immunology

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https://www.readbyqxmd.com/read/28432485/persistent-impairment-of-t-cell-regeneration-in-a-patient-with-activated-pi3k-%C3%AE-syndrome
#1
LETTER
Fumihiro Goto, Toru Uchiyama, Yumiko Nakazawa, Kohsuke Imai, Toshinao Kawai, Masafumi Onodera
No abstract text is available yet for this article.
April 21, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28429104/paecilomyces-formosus-infection-in-an-adult-patient-with-undiagnosed-chronic-granulomatous-disease
#2
LETTER
Jalal Heshmatnia, Majid Marjani, Seyed Alireza Mahdaviani, Parvaneh Adimi, Mihan Pourabdollah, Payam Tabarsi, Fereshte Mahdavi, Hamidreza Jamaati, Ian M Adcock, Johan Garssen, Aliakbar Velayati, Davood Mansouri, Esmaeil Mortaz
No abstract text is available yet for this article.
April 20, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28429103/resolution-of-primary-immune-defect-in-22q11-2-deletion-syndrome
#3
Yiwa Suksawat, Achara Sathienkijkanchai, Jittima Veskitkul, Orathai Jirapongsananuruk, Nualanong Visitsunthorn, Pakit Vichyanond, Punchama Pacharn
PURPOSE: Patients with 22q11.2 deletion syndrome have a variable decrease in immunological parameters, especially regarding T cell counts. The aim of this study was to investigate immunological change over time and factors associated with immunological recovery among patients with 22q11.2 deletion syndrome. METHODS: Patients with 22q11.2 deletion syndrome diagnosed by fluorescence in situ hybridization (FISH) were studied. Immunological parameters were evaluated every 6 months until patients returned to normal...
April 20, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28417298/disseminated-mycobacterium-malmoense-and-salmonella-infections-associated-with-a-novel-variant-in-nfkbia
#4
LETTER
Emily Staples, Beatriz Morillo-Gutierrez, Jessica Davies, Daniel Petersheim, Michel Massaad, Mary Slatter, Dimitra Dimou, Rainer Doffinger, Scott Hackett, Dinkantha Kumararatne, James Hadfield, Matthew D Eldridge, Raif S Geha, Mario Abinun, James E D Thaventhiran
No abstract text is available yet for this article.
April 17, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28386703/erratum-to-low-iga-and-igm-is-associated-with-a-higher-prevalence-of-bronchiectasis-in-primary-antibody-deficiency
#5
John P Hodkinson, Catherine Bangs, Andrea Wartenberg-Demand, Artur Bauhofer, Patrick Langohr, Matthew S Buckland, David Guzman, Patrick F K Yong, Sorena Kiani-Alikhan
No abstract text is available yet for this article.
April 6, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28386702/placental-transfer-of-canakinumab-in-a-patient-with-muckle-wells-syndrome
#6
LETTER
Makiko Egawa, Kohsuke Imai, Masaaki Mori, Naoyuki Miyasaka, Tetsuo Kubota
No abstract text is available yet for this article.
April 6, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28378256/first-case-of-x-linked-moesin-deficiency-identified-after-newborn-screening-for-scid
#7
LETTER
Ottavia M Delmonte, Catherine M Biggs, Anthony Hayward, Anne M Comeau, Hye Sun Kuehn, Sergio D Rosenzweig, Luigi D Notarangelo
No abstract text is available yet for this article.
April 4, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28353166/immunodeficiencies-associated-with-abnormal-newborn-screening-for-t-cell-and-b-cell-lymphopenia
#8
Soma Jyonouchi, Artemio M Jongco, Jennifer Puck, Kathleen E Sullivan
Newborn screening for SCID has revealed the association of low T cells with a number of unexpected syndromes associated with low T cells, some of which were not appreciated to have this feature. This review will discuss diagnostic approaches and the features of some of the syndromes likely to be encountered following newborn screening for immune deficiencies.
March 28, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28353165/poikiloderma-with-neutropenia-in-morocco-a-report-of-four-cases
#9
Ayoub Aglaguel, Houria Abdelghaffar, Fatima Ailal, Norddine Habti, Sebastian Hesse, Naschla Kohistani, Christoph Klein, Ahmed Aziz Bousfiha
PURPOSE: Poikiloderma with Neutropenia (PN) is inherited genodermatosis which results from a biallelic mutation in the USB1 gene (U Six Biogenesis 1). PN, first described in Navajo Native Americans, is characterized by early onset poikiloderma, pachyonychia, palmo-plantar hyperkeratosis, and permanent neutropenia. This condition results in frequent respiratory tract infections during infancy and childhood. From 2011 to 2013, four cases of PN were diagnosed in Morocco. In this paper, we report the first four cases of PN diagnosed in Morocco, out of three unrelated consanguinous families...
March 28, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28353164/preference-of-genetic-diagnosis-of-cxcr4-mutation-compared-with-clinical-diagnosis-of-whim-syndrome
#10
LETTER
Asghar Aghamohammadi, Hassan Abolhassani, Jacek Puchalka, Naschla Greif-Kohistani, Samaneh Zoghi, Christoph Klein, Nima Rezaei
No abstract text is available yet for this article.
March 28, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28342009/severe-aspergillus-pneumonia-and-pulmonary-artery-hypertension-in-a-child-with-autosomal-recessive-chronic-granulomatous-disease-and-selective-iga-deficiency
#11
LETTER
Ankur K Jindal, Amit Rawat, Deepti Suri, Madhubala Sharma, Jitendra K Shandilya, Shubham Goel, Kushaljit S Sodhi, Surjit Singh
No abstract text is available yet for this article.
March 24, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28337619/raised-serum-il-8-levels-are-associated-with-excessive-fatigue-in-female-carriers-of-x-linked-chronic-granulomatous-disease-in-the-uk
#12
LETTER
Alexandra C Battersby, Alexander J Martin, Jessica Tarn, Fai W Ng, Catherine M Cale, David Goldblatt, Andrew R Gennery
No abstract text is available yet for this article.
March 23, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28332028/infection-profile-in-chronic-granulomatous-disease-a-23-year-experience-from-a-tertiary-care-center-in-north-india
#13
Amit Rawat, Pandiarajan Vignesh, Avinash Sharma, Jitendra K Shandilya, Madhubala Sharma, Deepti Suri, Anju Gupta, Vikas Gautam, Pallab Ray, Shivaprakash M Rudramurthy, Arunaloke Chakrabarti, Kohsuke Imai, Shigeaki Nonoyama, Osamu Ohara, Yu L Lau, Surjit Singh
PURPOSE: Chronic granulomatous disease (CGD) is an inherited phagocytic disorder characterized by recurrent infections with usually catalase-positive organisms. Infections in CGD from developing countries are expected to be different from those in the Western countries. We report the profile of infections in children diagnosed with CGD from a tertiary care center in North India. METHODOLOGY: Case records of children diagnosed with CGD at Pediatric Immunodeficiency Clinic, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India, from August 1993 to April 2016 (23 years) were analyzed...
March 22, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28321612/serial-serum-immunoglobulin-g-igg-trough-levels-in-patients-with-x-linked-agammaglobulinemia-on-replacement-therapy-with-intravenous-immunoglobulin-its-correlation-with-infections-in-indian-children
#14
Deepti Suri, Sagar Bhattad, Avinash Sharma, Anju Gupta, Amit Rawat, Shobha Sehgal, Surjit Singh, Sudhir Gupta
Patients with primary antibody deficiency (PAD) are being increasingly diagnosed in the developing world. However, care of these children continues to remain suboptimal due to financial and social constraints. Immunoglobulin (Ig) trough level is an important predicting factor for infections in children on replacement immunoglobulin therapy. There are no data on this aspect from the developing world. Therefore, we studied serial immunoglobulin G (IgG) trough levels in 14 children with X-linked agammaglobulinemia (XLA) receiving replacement intravenous immunoglobulin (IVIG)...
March 21, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28316003/evaluation-of-the-safety-tolerability-and-pharmacokinetics-of-gammaplex-%C3%A2-10-versus-gammaplex-%C3%A2-5-in-subjects-with-primary-immunodeficiency
#15
Richard L Wasserman, Isaac R Melamed, Mark R Stein, Stephen Jolles, Miranda Norton, James N Moy
PURPOSE: This phase 3, multicenter, open-label, randomized, two-period, crossover bioequivalence trial evaluated the safety, tolerability, and pharmacokinetics of intravenous immunoglobulins (IVIGs) Gammaplex 5% and Gammaplex 10% in 33 adults and 15 children with primary immunodeficiency diseases (PIDs). METHODS: Eligible adults received five Gammaplex 5% infusions followed by five Gammaplex 10% infusions, or vice versa, stratified by a 21- or 28-day dosing regimen...
March 18, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28303442/different-immunological-pathways-underlie-the-immune-response-to-pneumococcal-polysaccharides
#16
LETTER
Leen Moens, Capucine Picard, Mohammad Shahrooei, Greet Wuyts, Adrian Liston, Alain Fischer, Xavier Bossuyt
No abstract text is available yet for this article.
March 16, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28299599/incidence-of-typically-severe-primary-immunodeficiency-diseases-in-consanguineous-and-non-consanguineous-populations
#17
Arnon Broides, Amit Nahum, Amarilla B Mandola, Lihi Rozner, Vered Pinsk, Galina Ling, Baruch Yerushalmi, Jacov Levy, Noga Givon-Lavi
PURPOSE: Primary immunodeficiency diseases are considered to be rare diseases; however, data on the exact birth incidences of these diseases are sparse. Southern Israel is inhabited by two major populations: a relatively non-consanguineous Jewish population and a highly consanguineous Muslim Bedouin population. We sought to calculate the incidences of typically severe primary immunodeficiency diseases and compare the incidences in these populations. METHODS: A retrospective analysis of all typically severe primary immunodeficiency diseases evaluated at a single center from January 1, 1996 to December 31, 2016...
March 16, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28293897/low-iga-and-igm-is-associated-with-a-higher-prevalence-of-bronchiectasis-in-primary-antibody-deficiency
#18
LETTER
John P Hodkinson, Catherine Bangs, Andrea Wartenberg-Demand, Artur Bauhofer, Patrick Langohr, Matthew S Buckland, David Guzman, Patrick F K Yong, Sorena Kiani-Alikhan
No abstract text is available yet for this article.
March 14, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28265964/rescue-of-cytokine-storm-due-to-hlh-by-hemoadsorption-in-a-ctla4-deficient-patient
#19
LETTER
Christine Greil, Fabian Roether, Paul La Rosée, Bodo Grimbacher, Daniel Duerschmied, Klaus Warnatz
No abstract text is available yet for this article.
March 6, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28224354/a-stable-mixed-chimera-after-sct-with-ric-in-an-infant-with-i%C3%AE%C2%BAb%C3%AE-hypermorphic-mutation
#20
LETTER
Masayuki Nagasawa, Teppei Ohkawa, Masatoshi Takagi, Kohsuke Imai, Tomohiro Morio
No abstract text is available yet for this article.
February 21, 2017: Journal of Clinical Immunology
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