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Journal of Clinical Immunology

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https://www.readbyqxmd.com/read/30415311/long-term-efficacy-and-safety-of-hizentra%C3%A2-in-patients-with-primary-immunodeficiency-in-japan-europe-and-the-united-states-a-review-of-7-phase-3-trials
#1
Stephen Jolles, Mikhail A Rojavin, John-Philip Lawo, Robert Nelson, Richard L Wasserman, Michael Borte, Michael A Tortorici, Kohsuke Imai, Hirokazu Kanegane
Many patients with primary immunodeficiency (PID) require immunoglobulin G (IgG) replacement therapy, delivered as intravenous IgG (IVIG) or subcutaneous IgG (SCIG). We aim to identify trends in efficacy and safety that would not be evident in individual studies of small patient numbers. Seven open-label, Phase 3, prospective, multicenter studies of the efficacy and safety of Hizentra® (a SCIG), conducted in Japan, Europe, and the US were summarized. Overall, 125 unique patients received 15,013 weekly infusions during a total observation period of 250...
November 10, 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/30386947/warts-and-dada2-a-mere-coincidence
#2
LETTER
Katrijn Arts, Jenna R E Bergerson, Amanda K Ombrello, Morgan Similuk, Andrew J Oler, Anahita Agharahimi, Emily M Mace, Mike Hershfield, Carine Wouters, Lien De Somer, Marie-Anne Morren, Rebeca Perez-de Diego, Leen Moens, Alexandra F Freeman, Isabelle Meyts
No abstract text is available yet for this article.
November 1, 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/30353301/clinical-and-immunological-characterization-of-icf-syndrome-in-japan
#3
Chikako Kamae, Kohsuke Imai, Tamaki Kato, Tsubasa Okano, Kenichi Honma, Noriko Nakagawa, Tzu-Wen Yeh, Emiko Noguchi, Akira Ohara, Tomonari Shigemura, Hiroshi Takahashi, Shunichi Takakura, Masatoshi Hayashi, Aoi Honma, Seiichi Watanabe, Tomoko Shigemori, Osamu Ohara, Hiroyuki Sasaki, Takeo Kubota, Tomohiro Morio, Hirokazu Kanegane, Shigeaki Nonoyama
OBJECTIVE: Immunodeficiency, centromeric instability, and facial anomalies (ICF) syndrome is a rare autosomal recessive primary immunodeficiency. Hypogammaglobulinemia is a major manifestation of ICF syndrome, but immunoglobulin replacement therapy does not seem to be effective for some ICF patients. Therefore, we aimed to reassess the immunological characteristics of this syndrome. METHODS: Eleven Japanese patients with ICF syndrome were enrolled. We performed whole-exome sequencing in four cases and homozygosity mapping using SNP analysis in two...
October 23, 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/30306363/correction-to-allogeneic-hematopoietic-cell-transplantation-in-patients-with-primary-immunodeficiencies-in-korea-eleven-year-experience-in-a-single-center
#4
Eun Sang Yi, Young Bae Choi, Na Hee Lee, Ji Won Lee, Ki Woong Sung, Hong Hoe Koo, Eun-Suk Kang, Yae-Jean Kim, Keon Hee Yoo
The original version of this article unfortunately contained a mistake in the 7th author's given name. The correct version is presented above.
October 10, 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/30215144/correction-to-diabetes-renal-and-cardiovascular-disease-in-p47-phox-chronic-granulomatous-disease
#5
LETTER
Gouri P Hule
No abstract text is available yet for this article.
September 13, 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/30317461/novel-stat1-gain-of-function-mutation-presenting-as-combined-immunodeficiency
#6
LETTER
Stella P Hartono, Alexander Vargas-Hernández, Mark J Ponsford, Ivan K Chinn, Stephen Jolles, Keith Wilson, Lisa R Forbes
No abstract text is available yet for this article.
October 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/30311057/chronic-aichi-virus-infection-in-a-patient-with-x-linked-agammaglobulinemia
#7
LETTER
Giorgia Bucciol, Leen Moens, Kathryn Payne, Elke Wollants, Djalila Mekahli, Elena Levtchenko, François Vermeulen, Thomas Tousseyn, Paul Gray, Cindy S Ma, Stuart G Tangye, Marc Van Ranst, Julianne R Brown, Judy Breuer, Isabelle Meyts
No abstract text is available yet for this article.
October 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/30302727/increased-stat1-amounts-correlate-with-the-phospho-stat1-level-in-stat1-gain-of-function-defects
#8
LETTER
Andrea R Bernasconi, Judith Yancoski, Mariana Villa, Matías M Oleastro, Miguel Galicchio, Jorge G Rossi
No abstract text is available yet for this article.
October 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/30302726/fourth-update-on-the-iranian-national-registry-of-primary-immunodeficiencies-integration-of-molecular-diagnosis
#9
Hassan Abolhassani, Fatemeh Kiaee, Marzieh Tavakol, Zahra Chavoshzadeh, Seyed Alireza Mahdaviani, Tooba Momen, Reza Yazdani, Gholamreza Azizi, Sima Habibi, Mohammad Gharagozlou, Masoud Movahedi, Amir Ali Hamidieh, Nasrin Behniafard, Mohammamd Nabavi, Mohammad Hassan Bemanian, Saba Arshi, Rasol Molatefi, Roya Sherkat, Afshin Shirkani, Reza Amin, Soheila Aleyasin, Reza Faridhosseini, Farahzad Jabbari-Azad, Iraj Mohammadzadeh, Javad Ghaffari, Alireza Shafiei, Arash Kalantari, Mahboubeh Mansouri, Mehrnaz Mesdaghi, Delara Babaie, Hamid Ahanchian, Maryam Khoshkhui, Habib Soheili, Mohammad Hossein Eslamian, Taher Cheraghi, Abbas Dabbaghzadeh, Mahmoud Tavassoli, Rasoul Nasiri Kalmarzi, Seyed Hamidreza Mortazavi, Sara Kashef, Hossein Esmaeilzadeh, Javad Tafaroji, Abbas Khalili, Fariborz Zandieh, Mahnaz Sadeghi-Shabestari, Sepideh Darougar, Fatemeh Behmanesh, Hedayat Akbari, Mohammadreza Zandkarimi, Farhad Abolnezhadian, Abbas Fayezi, Mojgan Moghtaderi, Akefeh Ahmadiafshar, Behzad Shakerian, Vahid Sajedi, Behrang Taghvaei, Mojgan Safari, Marzieh Heidarzadeh, Babak Ghalebaghi, Seyed Mohammad Fathi, Behzad Darabi, Saeed Bazregari, Nasrin Bazargan, Morteza Fallahpour, Alireza Khayatzadeh, Naser Javahertrash, Bahram Bashardoust, Mohammadali Zamani, Azam Mohsenzadeh, Sarehsadat Ebrahimi, Samin Sharafian, Ahmad Vosughimotlagh, Mitra Tafakoridelbari, Maziar Rahimi, Parisa Ashournia, Anahita Razaghian, Arezou Rezaei, Setareh Mamishi, Nima Parvaneh, Nima Rezaei, Lennart Hammarström, Asghar Aghamohammadi
BACKGROUND: The number of inherited diseases and the spectrum of clinical manifestations of primary immunodeficiency disorders (PIDs) are ever-expanding. Molecular diagnosis using genomic approaches should be performed for all PID patients since it provides a resource to improve the management and to estimate the prognosis of patients with these rare immune disorders. METHOD: The current update of Iranian PID registry (IPIDR) contains the clinical phenotype of newly registered patients during last 5 years (2013-2018) and the result of molecular diagnosis in patients enrolled for targeted and next-generation sequencing...
October 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/30288645/newborn-screening-for-ikbkb-deficiency-in-manitoba-using-genetic-mutation-analysis
#10
LETTER
Tamar S Rubin, Cheryl Rockman-Greenberg, Paul Van Caeseele, Geoffrey D E Cuvelier, Luvinia Kwan, Marlis L Schroeder
No abstract text is available yet for this article.
October 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/30280306/pseudo-sarcoidosis-revealing-monomac-syndrome
#11
LETTER
Louise Damian, Gaëtan Sauvêtre, Florent Marguet, Mikael Verdalle-Cazes, Maxime Battistella, David Boutboul
No abstract text is available yet for this article.
October 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/30280305/treatment-of-hereditary-angioedema-attacks-with-icatibant-and-recombinant-c1-inhibitor-during-pregnancy
#12
Roman Hakl, Pavel Kuklínek, Irena Krčmová, Pavlína Králíčková, Tomáš Freiberger, Petr Janků, Marcela Vlková, Jiří Litzman
PURPOSE: Hereditary angioedema (HAE) is a rare disease caused by a C1 inhibitor (C1-INH) deficit. Clinically, HAE is manifested by repeated episodes of localized subcutaneous or submucosal oedema attacks. Managing HAE patients in pregnancy is challenging, since there are only limited data on the safety and efficacy of various therapeutic approaches. METHODS: We present our clinical experience treating acute HAE attacks during pregnancy in six consecutive patients...
October 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/30267241/high-rates-of-community-and-hospital-acquired-infections-in-patients-with-cellular-immunodeficiencies
#13
Benjamin R Hanisch, Blachy J Davila Saldana, Michael D Keller, Xiaoyan Song
PURPOSE: Patients with primary immunodeficiency diseases (PID) are perceived to be at high risk for acquiring as well as developing complications from infections. There is little data describing the infection type and frequency these patients may acquire in the community or during hospital admissions. Data is critically needed in order to inform best practices on how to protect these vulnerable patients. METHODS: This is a retrospective study which included PID patients who were discharged from Children's National Health System (CNHS) from January 1, 2011, through August 31, 2017, and were assigned a discharge diagnosis code indicating PID...
October 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/30264381/early-onset-invasive-infection-due-to-corynespora-cassiicola-associated-with-compound-heterozygous-card9-mutations-in-a-colombian-patient
#14
Carlos A Arango-Franco, Marcela Moncada-Vélez, Claudia Patricia Beltrán, Indira Berrío, Cristian Mogollón, Andrea Restrepo, Mónica Trujillo, Sara Daniela Osorio, Lorena Castro, Lina Vanessa Gómez, Ana María Muñoz, Verónica Molina, Delsy Yurledy Del Río Cobaleda, Ana Cristina Ruiz, Carlos Garcés, Juan Fernando Alzate, Felipe Cabarcas, Julio Cesar Orrego, Jean-Laurent Casanova, Jacinta Bustamante, Anne Puel, Andrés Augusto Arias, José Luis Franco
PURPOSE: CARD9 deficiency is an inborn error of immunity that predisposes otherwise healthy humans to mucocutaneous and invasive fungal infections, mostly caused by Candida, but also by dermatophytes, Aspergillus, and other fungi. Phaeohyphomycosis are an emerging group of fungal infections caused by dematiaceous fungi (phaeohyphomycetes) and are being increasingly identified in patients with CARD9 deficiency. The Corynespora genus belongs to phaeohyphomycetes and only one adult patient with CARD9 deficiency has been reported to suffer from invasive disease caused by C...
October 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/30255293/impaired-il-12-and-il-23-mediated-immunity-due-to-il-12r%C3%AE-1-deficiency-in-iranian-patients-with-mendelian-susceptibility-to-mycobacterial-disease
#15
Nioosha Nekooie-Marnany, Caroline Deswarte, Vajiheh Ostadi, Bahram Bagherpour, Elaheh Taleby, Mazdak Ganjalikhani-Hakemi, Tom Le Voyer, Hamid Rahimi, Jérémie Rosain, Zahra Pourmoghadas, Saba Sheikhbahaei, Razieh Khoshnevisan, Daniel Petersheim, Daniel Kotlarz, Christoph Klein, Stéphanie Boisson-Dupuis, Jean-Laurent Casanova, Jacinta Bustamante, Roya Sherkat
PURPOSE: Inborn errors of IFN-γ-mediated immunity underlie Mendelian Susceptibility to Mycobacterial Disease (MSMD), which is characterized by an increased susceptibility to severe and recurrent infections caused by weakly virulent mycobacteria, such as Bacillus Calmette-Guérin (BCG) vaccines and environmental, nontuberculous mycobacteria (NTM). METHODS: In this study, we investigated four patients from four unrelated consanguineous families from Isfahan, Iran, with disseminated BCG disease...
October 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/30251145/newborn-screening-for-severe-combined-immunodeficiency-analytic-and-clinical-performance-of-the-t-cell-receptor-excision-circle-assay-in-france-depistrec-study
#16
Marie A P Audrain, Alexandra J C Léger, Caroline A F Hémont, Sophie M Mirallié, David Cheillan, Marie G M Rimbert, Aurélie M-P Le Thuaut, Véronique A Sébille-Rivain, Aurore Prat, Enora M Q Pinel, Eléonore Divry, Cécile G L Dert, Maxime A G Fournier, Caroline J C Thomas
Severe combined immunodeficiency (SCID) is characterized by a major T cell deficiency. Infants with SCID are asymptomatic at birth but die from infections in the first year of life if not treated. Survival rates are better for early treatment. SCID therefore meets criteria for newborn screening (NBS). T cell receptor excision circle (TREC) quantification is a reliable marker of T cell deficiency and can be performed using Guthrie cards. The DEPISTREC project was designed to study the feasibility, clinical utility, and cost-effectiveness of generalized SCID screening in France...
October 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/30219982/gastric-cancer-screening-in-common-variable-immunodeficiency
#17
David K van der Poorten, Duncan McLeod, Golo Ahlenstiel, Scott Read, Avelyn Kwok, Cositha Santhakumar, Milan Bassan, Suzanne Culican, David Campbell, Sue W J Wong, Louise Evans, Bilel Jideh, Alisa Kane, Constance H Katelaris, Karuna Keat, Yanna Ko, Jessie A Lee, Sandhya Limaye, Ming Wei Lin, Ari Murad, Martina Rafferty, Dan Suan, Sanjay Swaminathan, Sean D Riminton, Catherine Toong, Lucinda J Berglund
Individuals with common variable immunodeficiency (CVID) have an increased risk of gastric cancer, and gastrointestinal lymphoma, yet screening for premalignant gastric lesions is rarely offered routinely to these patients. Proposed screening protocols are not widely accepted and are based on gastric cancer risk factors that are not applicable to all CVID patients. Fifty-two CVID patients were recruited for screening gastroscopy irrespective of symptoms or blood results and were compared to 40 controls presenting for gastroscopy for other clinical indications...
October 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/30167939/palatal-ulcer-in-leukocyte-adhesion-deficiency-an-unusual-occurrence
#18
LETTER
Avinash Sharma, Ankur K Jindal, Rakesh K Pilania, Piyush Gautam, Subhash Daroch
No abstract text is available yet for this article.
October 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/30151618/allogeneic-hematopoietic-cell-transplantation-in-patients-with-primary-immunodeficiencies-in-korea-eleven-year-experience-in-a-single-center
#19
Eun Sang Yi, Young Bae Choi, Na Hee Lee, Ji Won Lee, Ki Woong Sung, Hong Hoe Koo, Eun-Sook Kang, Yae-Jean Kim, Keon Hee Yoo
PURPOSE: We aimed to report our single-center experience of allogeneic hematopoietic cell transplantation (HCT), which has been the only curative option for certain patients with lethal primary immunodeficiencies (PIDs). METHODS: We summarized the results of HCT performed for patients with PIDs for 11 consecutive years from 2006 to 2016 at Samsung Medical Center, Seoul, Korea. Twenty-six patients with PIDs received HCT. Most had chronic granulomatous disease (42...
October 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/30146668/xl-eda-id-presenting-with-congenital-duodenal-atresia-and-perforations
#20
LETTER
Yusuke Mitani, Taizo Wada, Yusuke Matsuda, Seisho Sakai, Akihiro Yachie
No abstract text is available yet for this article.
October 2018: Journal of Clinical Immunology
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