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Journal of Clinical Immunology

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https://www.readbyqxmd.com/read/28879453/fecal-calprotectin-rise-in-chronic-granulomatous-disease-associated-colitis
#1
LETTER
Yumiko Nakazawa, Toshinao Kawai, Katsuhiro Arai, Eiichiro Tamura, Toru Uchiyama, Masafumi Onodera
No abstract text is available yet for this article.
September 6, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28875256/response-to-the-letter-to-the-editor-regarding-assessment-of-local-adverse-reactions-to-subcutaneous-immunoglobulin-scig-in-clinical-trials
#2
LETTER
Daniel Suez, Mark Stein, Sudhir Gupta, Iftikhar Hussain, Isaac Melamed, Kenneth Paris, Amy Darter, Christelle Bourgeois, Sandor Fritsch, Heinz Leibl, Barbara McCoy, David Gelmont, Leman Yel
No abstract text is available yet for this article.
September 5, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28871523/a-personalized-diagnostic-and-treatment-approach-for-macrophage-activation-syndrome-and-secondary-hemophagocytic-lymphohistiocytosis-in-adults
#3
Bharat Kumar, Sohaib Aleem, Hana Saleh, Jennifer Petts, Zuhair K Ballas
OBJECTIVE: We assessed the clinical features and outcomes based on therapeutic options adopted during hospital stay for adult patients with macrophage activation syndrome and secondary hemophagocytic lymphohistiocytosis (MAS/sHLH). METHODS: We conducted a retrospective chart review of all adult patients (age ≥ 18 years) diagnosed with MAS/sHLH at our center between 2010 and 2015. Inclusion criteria for patients were diagnosis of MAS/sHLH during admission and patients meeting at least 5 out of 8 of Henter's criteria or at least 4 out of 6 of the criteria that were tested...
September 4, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28865061/severe-enteropathy-and-hypogammaglobulinemia-complicating-refractory-mycobacterium-tuberculosis-complex-disseminated-disease-in-a-child-with-il-12r%C3%AE-1-deficiency
#4
Andrés Augusto Arias, Carlos M Perez-Velez, Julio César Orrego, Marcela Moncada-Velez, Jessica Lineth Rojas, Alejandra Wilches, Andrea Restrepo, Mónica Trujillo, Carlos Garcés, Catalina Arango-Ferreira, Natalia González, Carmen Oleaga-Quintas, Diana Fernández, Johana Marcela Isaza-Correa, Diego Eduardo Gongóra, Daniel Gonzalez-Loaiza, Juan Esteban Sierra, Jean Laurent Casanova, Jacinta Bustamante, José Luis Franco
PURPOSE: Mendelian susceptibility to mycobacterial disease is a rare clinical condition characterized by a predisposition to infectious diseases caused by poorly virulent mycobacteria. Other infections such as salmonellosis and candidiasis are also reported. The purpose of this article is to describe a young boy affected with various infectious diseases caused by Mycobacterium tuberculosis complex, Salmonella sp, Klebsiella pneumonie, Citrobacter sp., and Candida sp, complicated with severe enteropathy and transient hypogammaglobulinemia...
September 1, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28861675/kinetics-of-radiological-response-of-thoracic-invasive-fungal-disease-in-chronic-granulomatous-disease
#5
LETTER
Romain Guery, Benjamin Subran, Arnaud Lefevre, Fanny Lanternier
No abstract text is available yet for this article.
September 1, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28856582/helicobacter-bilis-associated-suppurative-cholangitis-in-a-patient-with-x-linked-agammaglobulinemia
#6
Nicolas Degand, Justine Dautremer, Benoît Pilmis, Agnès Ferroni, Fanny Lanternier, Julie Bruneau, Olivier Hermine, Stéphane Blanche, Xavier Nassif, Olivier Lortholary, Marc Lecuit
ᅟ: Helicobacter bilis is a commensal bacterium causing chronic hepatitis and colitis in mice. In humans, enterohepatic Helicobacter spp. are associated with chronic hepatobiliary diseases. PURPOSE: We aimed at understanding the microbial etiology in a patient with X-linked agammaglobulinemia presenting with suppurative cholangitis. METHODS: 16S rDNA PCR directly performed on a liver biopsy retrieved DNA of H. bilis. RESULTS: Clinical outcome resulted in the normalization of clinical and biological parameters under antibiotic treatment by a combination of ceftriaxone, metronidazole, and doxycyclin followed by a 2-week treatment with moxifloxacin and a 2-month treatment with azithromycin...
August 31, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28842786/neutropenia-in-patients-with-common-variable-immunodeficiency-a-rare-event-associated-with-severe-outcome
#7
Aurélien Guffroy, Rachel Mourot-Cottet, Laurence Gérard, Vincent Gies, Chantal Lagresle, Aurore Pouliet, Patrick Nitschké, Sylvain Hanein, Boris Bienvenu, Valérie Chanet, Jean Donadieu, Martine Gardembas, Marina Karmochkine, Raphaele Nove-Josserand, Thierry Martin, Vincent Poindron, Pauline Soulas-Sprauel, Fréderic Rieux-Laucat, Claire Fieschi, Eric Oksenhendler, Isabelle André-Schmutz, Anne-Sophie Korganow
BACKGROUND: Common variable immunodeficiency (CVID) is characterized by infections and hypogammaglobulinemia. Neutropenia is rare during CVID. METHODS: The French DEFI study enrolled patients with primary hypogammaglobulinemia. Patients with CVID and neutropenia were retrospectively analyzed. RESULTS: Among 473 patients with CVID, 16 patients displayed neutropenia (lowest count [0-1400]*10(6)/L). Sex ratio (M/F) was 10/6. Five patients died during the follow-up (11 years) with an increased percentage of deaths compared to the whole DEFI group (31...
August 26, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28842866/adenosine-deaminase-ada-deficient-severe-combined-immune-deficiency-scid-molecular-pathogenesis-and-clinical-manifestations
#8
REVIEW
Kathryn L Bradford, Federico A Moretti, Denise A Carbonaro-Sarracino, Hubert B Gaspar, Donald B Kohn
Deficiency of adenosine deaminase (ADA, EC3.5.4.4), a housekeeping enzyme of purine metabolism encoded by the Ada gene, is a cause of human severe combined immune deficiency (SCID). Numerous deleterious mutations occurring in the ADA gene have been found in patients with profound lymphopenia (T(-) B(-) NK(-)), thus underscoring the importance of functional purine metabolism for the development of the immune defense. While untreated ADA SCID is a fatal disorder, there are multiple life-saving therapeutic modalities to restore ADA activity and reconstitute protective immunity, including enzyme replacement therapy (ERT), allogeneic hematopoietic stem cell transplantation (HSCT) and gene therapy (GT) with autologous gene-corrected hematopoietic stem cells (HSC)...
August 25, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28825155/detection-of-sp110-by-flow-cytometry-and-application-to-screening-patients-for-veno-occlusive-disease-with-immunodeficiency
#9
Florian A Marquardsen, Fabian Baldin, Florian Wunderer, Waleed Al-Herz, Raymond Mikhael, Gérard Lefranc, Zeina Baz, Fariba Rezaee, Rabi Hanna, Shlomit Kfir-Erenfeld, Polina Stepensky, Benedikt Meyer, Annaise Jauch, Marc B Bigler, Anne-Valérie Burgener, Rebecca Higgins, Alexander A Navarini, Joeseph A Church, Janet Chou, Raif Geha, Luigi D Notarangelo, Christoph Hess, Christoph T Berger, Donald B Bloch, Mike Recher
Mutations in Sp110 are the underlying cause of veno-occlusive disease with immunodeficiency (VODI), a combined immunodeficiency that is difficult to treat and often fatal. Because early treatment is critically important for patients with VODI, broadly usable diagnostic tools are needed to detect Sp110 protein deficiency. Several factors make establishing the diagnosis of VODI challenging: (1) Current screening strategies to identify severe combined immunodeficiency are based on measuring T cell receptor excision circles (TREC)...
August 21, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28819871/health-related-quality-of-life-and-patients-empowerment-in-the-health-care-of-primary-immune-deficiencies
#10
EDITORIAL
Isabella Quinti, Federica Pulvirenti
No abstract text is available yet for this article.
August 17, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28815344/long-term-survival-after-hematopoietic-stem-cell-transplantation-for-complete-stat1-deficiency
#11
Samuele Naviglio, Elena Soncini, Donatella Vairo, Arnalda Lanfranchi, Raffaele Badolato, Fulvio Porta
PURPOSE: Complete signal transducer and activator of transcription 1 (STAT1) deficiency is a rare autosomal recessive condition characterized by impairment of intracellular signaling from both type I and type II interferons (IFN). Affected patients are prone to early severe mycobacterial and viral infections, which usually result in death before 18 months of age. We previously reported a patient affected by complete STAT1 deficiency who underwent hematopoietic stem cell transplantation (HSCT)...
August 16, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28808844/bialellic-mutations-in-tetratricopeptide-repeat-domain-7a-ttc7a-cause-common-variable-immunodeficiency-like-phenotype-with-enteropathy
#12
LETTER
Dylan Lawless, Anoop Mistry, Philip M Wood, Jens Stahlschmidt, Gururaj Arumugakani, Mark Hull, David Parry, Rashida Anwar, Clive Carter, Sinisa Savic
No abstract text is available yet for this article.
August 14, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28803389/gastrointestinal-manifestations-of-stat3-deficient-hyper-ige-syndrome
#13
Manish Arora, Preet Bagi, Anna Strongin, Jennifer Heimall, Xiongce Zhao, Monica G Lawrence, Apurva Trivedi, Carolyn Henderson, Amy Hsu, Martha Quezado, David E Kleiner, Aradhana M Venkatesan, Steven M Holland, Alexandra F Freeman, Theo Heller
OBJECTIVE: STAT 3 deficiency (autosomal dominant hyper immunoglobulin E syndrome (AD-HIES)) is a primary immunodeficiency disorder with multi-organ involvement caused by dominant negative signal transducer and activator of transcription gene 3 (STAT3) mutations. We sought to describe the gastrointestinal (GI) manifestations of this disease. METHODS: Seventy subjects aged five to 60 years with a molecular diagnosis of AD-HIES were evaluated at the National Institutes of Health (NIH)...
August 13, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28786026/emerging-infections-and-pertinent-infections-related-to-travel-for-patients-with-primary-immunodeficiencies
#14
Kathleen E Sullivan, Hamid Bassiri, Ahmed A Bousfiha, Beatriz T Costa-Carvalho, Alexandra F Freeman, David Hagin, Yu L Lau, Michail S Lionakis, Ileana Moreira, Jorge A Pinto, M Isabel de Moraes-Pinto, Amit Rawat, Shereen M Reda, Saul Oswaldo Lugo Reyes, Mikko Seppänen, Mimi L K Tang
In today's global economy and affordable vacation travel, it is increasingly important that visitors to another country and their physician be familiar with emerging infections, infections unique to a specific geographic region, and risks related to the process of travel. This is never more important than for patients with primary immunodeficiency disorders (PIDD). A recent review addressing common causes of fever in travelers provides important information for the general population Thwaites and Day (N Engl J Med 376:548-560, 2017)...
August 7, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28779413/rituximab-restores-ifn-%C3%AE-stat1-function-and-ameliorates-disseminated-mycobacterium-avium-infection-in-a-patient-with-anti-interferon-%C3%AE-autoantibody
#15
Yusuke Koizumi, Takuro Sakagami, Naoya Nishiyama, Jun Hirai, Yuta Hayashi, Nobuhiro Asai, Yuka Yamagishi, Hideo Kato, Mao Hagihara, Daisuke Sakanashi, Hiroyuki Suematsu, Kenji Ogawa, Hiroshige Mikamo
A 67-year-old Japanese female with back pain and severe cachexia visited our hospital. The diagnosis was disseminated Mycobacterium avium complex infection (dMAC) with multiple bone involvement. Anti-mycobacterial chemotherapy was started, but fever persisted and dislocation of cervical vertebrae has made her bedridden. Because anti-interferon (IFN)-γ autoantibody was positive, four doses of rituximab 375 mg/m(2), every 7 day, were administered. Soon after treatment, progression of osteolytic lesions and wasting has stopped...
August 5, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28755067/efficacy-and-safety-of-human-intravenous-immunoglobulin-10-panzyga%C3%A2-in-patients-with-primary-immunodeficiency-diseases-a-two-stage-multicenter-prospective-open-label-study
#16
Michael Borte, Isaac R Melamed, Grazyna Pulka, Barbara Pyringer, Alan P Knutsen, Hans D Ochs, Roger H Kobayashi, Ai Lan Kobayashi, Sudhir Gupta, Magdalena Strach, William Smits, Anna Pituch-Noworolska, James N Moy
PURPOSE: To assess the efficacy and safety of panzyga® (intravenous immunoglobulin 10%) in preventing serious bacterial infections (SBIs) in patients with primary immunodeficiency diseases (PIDs), a prospective, open-label, multicenter, phase 3 study and an open-label extension study were undertaken. METHODS: Initially, the study drug (infusion rate ≤0.08 mL/kg/min) was administered at intervals of 3 or 4 weeks for 12 months, followed by 3 months of panzyga® at infusion rates increasing from 0...
July 29, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28755066/two-sides-of-the-same-coin-pediatric-onset-and-adult-onset-common-variable-immune-deficiency
#17
Lauren A Sanchez, Solrun Melkorka Maggadottir, Matthew S Pantell, Patricia Lugar, Charlotte Cunningham Rundles, Kathleen E Sullivan
PURPOSE: Common variable immunodeficiency (CVID) is a complex, heterogeneous immunodeficiency characterized by hypogammaglobulinemia, recurrent infections, and poor antibody response to vaccination. While antibiotics and immunoglobulin prophylaxis have significantly reduced infectious complications, non-infectious complications of autoimmunity, inflammatory lung disease, enteropathy, and malignancy remain of great concern. Previous studies have suggested that CVID patients diagnosed in childhood are more severely affected by these complications than adults diagnosed later in life...
July 28, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28752258/allogeneic-reduced-intensity-hematopoietic-stem-cell-transplantation-for-chronic-granulomatous-disease-a-single-center-prospective-trial
#18
Mark Parta, Corin Kelly, Nana Kwatemaa, Narda Theobald, Diane Hilligoss, Jing Qin, Douglas B Kuhns, Christa Zerbe, Steven M Holland, Harry Malech, Elizabeth M Kang
PURPOSE: The purpose of this study was to evaluate engraftment and adverse events with a conditioning and prophylactic regimen intended to achieve high rates of engraftment with minimal graft-versus-host disease (GVHD) in allogeneic transplantation for chronic granulomatous disease in a single center. METHODS: Forty patients, 37 male, with chronic granulomatous disease were transplanted. Transplant products were matched sibling peripheral blood stem cells (PBSCs) in four and matched unrelated donor (MUD) bone marrow in three, and one patient received mismatched unrelated PBSCs...
July 28, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28748310/long-term-outcome-of-adenosine-deaminase-deficient-patients-a-single-center-experience
#19
Ori Scott, Vy Hong-Diep Kim, Brenda Reid, Anne Pham-Huy, Adelle R Atkinson, Alessandro Aiuti, Eyal Grunebaum
PURPOSE: Inherited defects in the adenosine deaminase (ADA) enzyme can cause severe combined immune deficiency (SCID) and systemic abnormalities. Management options for ADA-deficient patients include enzyme replacement therapy (ERT), hematopoietic stem cell transplantation (HSCT), and gene therapy (GT). Here, we describe the long-term benefits of these treatments. METHODS: Survival, infections, systemic sequelae, and laboratory assessments were recorded for all ADA-deficient SCID patients, managed at a single center since 1985, who survived 5 or more years following treatment...
July 26, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28741180/loss-of-nhej1-protein-due-to-a-novel-splice-site-mutation-in-a-family-presenting-with-combined-immunodeficiency-microcephaly-and-growth-retardation-and-literature-review
#20
Farrukh Sheikh, Abbas Hawwari, Safa Alhissi, Sulaiman Al Gazlan, Hasan Al Dhekri, Agha M Rehan Khaliq, Esteban Borrero, Lina El-Baik, Rand Arnaout, Hamoud Al-Mousa, Anas M Alazami
INTRODUCTION: Non-homologous end joining gene 1 (NHEJ1) defect is a rare form of primary immune deficiency. Very few cases have been described from around the world. PURPOSE: We are reporting the first family from the Arabian Gulf with three siblings presenting with combined immunodeficiency (CID), microcephaly, and growth retardation due to a novel NHEJ1 splice site mutation, in addition to a review of the previously published literature on this subject. METHODS: Patients' clinical, immunological, and laboratory features were examined...
July 24, 2017: Journal of Clinical Immunology
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