Article
Immunology
Bandar Al-Saud, Nora AlRumayyan, Areej Alfattani, Sawsan Abu Awwad, Dema Al Saud, Reem Mohammed, Sultan Albuhairi, Sahar Elshorbagi, Sakra S. Balhareth, Hasan Al-Dhekri, Rand Arnaout, Edward B. De Vol, Hamoud Al-Mousa
Summary: This study aimed to determine the quality of life (QoL) of patients with primary immunodeficiency (PID) after switching to home-based subcutaneous immunoglobulin (SCIG). QoL was determined using a validated questionnaire at baseline and 3 and 6 months after switching to SCIG. The results showed a significant improvement in patients' global health and serum IgG level after switching to SCIG.
JOURNAL OF CLINICAL IMMUNOLOGY
(2023)
Article
Medicine, General & Internal
Ori Hassin, Yahya Abu Freih, Ran Hazan, Atar Lev, Keren S. Zrihen, Raz Somech, Arnon Broides, Amit Nahum
Summary: Immunoglobulin replacement therapy is a key treatment for patients with primary immunodeficiency (PID). This study compares specific antibody concentrations in PID patients with healthy controls, showing higher levels of certain antibodies in healthy individuals. XLA patients had significantly higher antibody concentrations compared to CID patients. Consideration should be given to higher target IgG levels in CID patients.
JOURNAL OF CLINICAL MEDICINE
(2021)
Review
Immunology
Yesim Yilmaz Demirdag, Sudhir Gupta
Summary: Bacterial respiratory tract infections are common in primary antibody deficiencies (PADs) but often overlooked in healthy individuals. This review focuses on infections in relatively common PADs, discussing diagnostic challenges and management strategies to prevent infections.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Immunology
Bas M. Smits, Ilona Kleine Budde, Esther de Vries, Ineke J. M. ten Berge, Robbert G. M. Bredius, Marcel van Deuren, Jaap T. van Dissel, Pauline M. Ellerbroek, Michiel van der Flier, P. Martin van Hagen, Chris Nieuwhof, Bram Rutgers, Lieke E. A. M. Sanders, Anna Simon, Taco W. Kuijpers, Joris M. van Montfrans
Summary: The study compared the efficacy of immunoglobulin replacement therapy (IRT) and prophylactic antibiotics (PA) in patients with IgSD +/- SPAD, finding similar overall efficacy between the two treatment methods, with a lower proportion of related adverse events for patients using PA. Patients with persistent infections during PA treatment had reduced infections after switching to IRT.
JOURNAL OF CLINICAL IMMUNOLOGY
(2021)
Review
Immunology
John T. Anderson, Juthaporn Cowan, Antonio Condino-Neto, Donald Levy, Subhransu Prusty
Summary: Accurate and timely diagnosis of PID is crucial for patient QoL and outcomes, with adults generally experiencing longer diagnostic delays. Treatment burden is not the biggest impact on QoL, and the cost of PID is high, with support for national registries playing a major role in delayed diagnosis.
CLINICAL IMMUNOLOGY
(2022)
Article
Medicine, Research & Experimental
Maria Fasshauer, Michael Borte, Michaela Bitzenhofer, Christine Pausch, David Pittrow, Michelle Park, Andre Gladiator, Peter Jandus
Summary: The CORE study provides real-world evidence of the flexibility, feasibility, safety, and tolerability of Ig20Gly infusions, at mostly weekly intervals, over 1 year in patients with PIDs.
ADVANCES IN THERAPY
(2023)
Article
Immunology
Andy Ka Chun Kan, Garret Man Kit Leung, Valerie Chiang, Elaine Yuen Ling Au, Chak Sing Lau, Philip Hei Li
Summary: This study revealed an increasing burden of adult antibody deficiency and immunoglobulin consumption in Hong Kong. Patients on SCIg replacement had better clinical outcomes and quality of life compared to those on IVIg.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Immunology
Maha N. Syed, Carleigh Kutac, Jennifer M. Miller, Rebecca Marsh, Kathleen E. Sullivan, Charlotte Cunningham-Rundles, Ramsay L. Fuleihan, Farrah Kheradmand, Joud Hajjar
Summary: This study investigated the risk factors of developing pneumonia in primary antibody deficiency (PAD) patients receiving immunoglobulin replacement therapy (IgRT), revealing that PAD patients with chronic and structural lung disease, splenomegaly, and allergies were associated with persistent pneumonia post-IgRT.
JOURNAL OF CLINICAL IMMUNOLOGY
(2022)
Article
Immunology
Ailsa Robbins, Mathilde Bahuaud, Maxime Hentzien, Quentin Maestraggi, Coralie Barbe, Delphine Giusti, Richard Le Naour, Frederic Batteux, Amelie Servettaz
Summary: This study evaluated the immunogenicity and protection persistence after a 13-valent pneumococcal conjugate vaccine in patients with primary humoral immunodeficiency. Results showed varying levels of immune response and protection over the course of one year, with factors such as IgG subclass deficiency and Ig replacement therapy being associated with long-term protection. Pneumococcal conjugate vaccine was found to improve immune protection and antibodies' functionality in a subset of patients, highlighting the importance of individualized vaccine strategies.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Allergy
Anoop Mayampurath, Aswathy Ajith, Colin Anderson-Smits, Shun-Chiao Chang, Emily Brouwer, Julie Johnson, Michael Baltasi, Samuel Volchenboum, Giovanna Devercelli, Christina E. Ciaccio
Summary: A machine learning model using historical data related to symptomatic care was developed to predict primary immunodeficiency diseases (PIDD). The model showed good discrimination and has the potential to shorten the time to diagnose PIDD and improve patient outcomes.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE
(2022)
Article
Immunology
Manuel Santamaria, Olaf Neth, Jo A. Douglass, Gergely Krivan, Robin Kobbe, Ewa Bernatowska, Sofia Grigoriadou, Claire Bethune, Anita Chandra, Gerd Horneff, Michael Borte, Anja Sonnenschein, Pavlina Kralickova, Silvia Sanchez Ramon, Daman Langguth, Luis Ignacio Gonzalez-Granado, Laia Alsina, Montse Querolt, Rhonda Griffin, Carrie Hames, Elsa Mondou, Jeffrey Price, Ana Sanz, Jiang Lin
Summary: IGSC 20% demonstrated efficacy and good safety and tolerability in patients with primary immunodeficiency.
JOURNAL OF CLINICAL IMMUNOLOGY
(2022)
Article
Immunology
Riccardo Scarpa, Alessandro Dell'Edera, Carla Felice, Roberta Buso, Francesco Muscianisi, Renato Finco Gambier, Sara Toffolo, Ugo Grossi, Mario Giobbia, Giuseppina Barberio, Nicholas Landini, Cesarina Facchini, Carlo Agostini, Marcello Rattazzi, Francesco Cinetto
Summary: This study investigated the impact of hypogammaglobulinemia on COVID-19 patients. The results showed that hypogammaglobulinemia was associated with a more severe disease course and more frequent sICU/ICU admission. Therefore, routine serum protein electrophoresis evaluation in hospitalized COVID-19 patients is important for clinicians in decision-making and patient care.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Medicine, General & Internal
Lourdes Mateu, Aina Teniente-Serra, Gemma Rocamora, Antoni Marin-Muniz, Noemi Parraga, Irma Casas, Esteban Reynaga, Nieves Sopena, Miguel Sabria, Maria Luisa Pedro-Botet
Summary: The study evaluated the association between an awareness campaign of PIDs among physicians and an increase in number of diagnoses, a reduction in diagnostic delay and diagnosis at earlier stages. The results showed a significant increase in incidence rate, a decrease in diagnosis delay, and a tendency towards lower disease burden at diagnosis after the implementation of the awareness campaign.
Article
Immunology
Miguel A. Park, Sarah M. Jenkins, Carin Y. Smith, Regan C. Pyle, Keith A. Sacco, Euijung Ryu, John B. Hagan, Avni Y. Joshi, Melissa R. Snyder, Roshini S. Abraham
Summary: The study investigates the antibody responses to Pneumovax(R) in healthy adults and finds that antibodies to 19 out of 23 serotypes are informative for distinguishing subjects who responded to vaccination, with a serotype threshold of 9 out of 19 serotypes characterizing the response. Further research in healthy children and adults vaccinated with S. pneumoniae protein-conjugate vaccines is needed for future studies.
Article
Immunology
Jesmeen Maimaris, Anjel O'Sullivan, Isabella Underhill, Ghiselle Green, Andrew Symes, David Lowe, Siobhan Burns, Mari Campbell, Reem Elfeky
Summary: This study investigated the impact of modifying immunoglobulin treatment regimen on infection and mental health burden during COVID-19 shielding restrictions. The findings suggest that changing treatment modality did not negatively affect infection rates or psychological wellbeing.
JOURNAL OF CLINICAL IMMUNOLOGY
(2023)
Letter
Allergy
Caitlin M. Burk, Kara E. Coffey, Emily M. Mace, Bret L. Bostwick, Ivan K. Chinn, Zeynep H. Coban-Akdemir, Shalini N. Jhangiani, James R. Lupski, Damara Ortiz, Jessie L. Barnum, Steven W. Allen, Leanna-Marie Robertson, Jordan S. Orange, Hey J. Chong
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE
(2020)
Article
Allergy
Alexander Vargas-Hernandez, Agnieszka Witalisz-Siepracka, Michaela Prchal-Murhpy, Klara Klein, Sanjana Mahapatra, Waleed Al-Herz, Emily M. Mace, Alexandre F. Carisey, Jordan S. Orange, Veronika Sexl, Lisa R. Forbes
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
(2020)
Article
Immunology
Joud Hajjar, An L. Nguyen, Gregory Constantine, Carleigh Kutac, Maha N. Syed, Jordan S. Orange, Kathleen E. Sullivan
JOURNAL OF CLINICAL IMMUNOLOGY
(2020)
Article
Immunology
Jordan S. Orange
JOURNAL OF CLINICAL IMMUNOLOGY
(2020)
Article
Biophysics
Nicholaus J. Trenton, R. Tyler McLaughlin, Satya K. Bellamkonda, David S. Tsao, Alexandra Rodzinski, Emily M. Mace, Jordan S. Orange, Volker Schweikhard, Michael R. Diehl
BIOPHYSICAL JOURNAL
(2020)
Article
Medicine, Research & Experimental
Juan C. Ravell, Mami Matsuda-Lennikov, Samuel D. Chauvin, Juan Zou, Matthew Biancalana, Sally J. Deeb, Susan Price, Helen C. Su, Giulia Notarangelo, Ping Jiang, Aaron Morawski, Chrysi Kanellopoulou, Kyle Binder, Ratnadeep Mukherjee, James T. Anibal, Brian Sellers, Lixin Zheng, Tingyan He, Alex B. George, Stefania Pittaluga, Astin Powers, David E. Kleiner, Devika Kapuria, Marc Ghany, Sally Hunsberger, Jeffrey I. Cohen, Gulbu Uzel, Jenna Bergerson, Lynne Wolfe, Camilo Toro, William Gahl, Les R. Folio, Helen Matthews, Pam Angelus, Ivan K. Chinn, Jordan S. Orange, Claudia M. Trujillo-Vargas, Jose Luis Franco, Julio Orrego-Arango, Sebastian Gutierrez-Hincapie, Niraj Chandrakant Patel, Kimiyo Raymond, Turkan Patiroglu, Ekrem Unal, Musa Karakukcu, Alexandre G. R. Day, Pankaj Mehta, Evan Masutani, Suk S. De Ravin, Harry L. Malech, Gregoire Altan-Bonnet, V. Koneti Rao, Matthias Mann, Michael J. Lenardo
JOURNAL OF CLINICAL INVESTIGATION
(2020)
Article
Immunology
Jessica Quinn, Vicki Modell, Jennifer Holle, Rebecca Truty, Swaroop Aradhya, Britt Johnson, Jordan Orange, Fred Modell
IMMUNOLOGIC RESEARCH
(2020)
Editorial Material
Immunology
Helen C. Su, Jordan S. Orange
JOURNAL OF CLINICAL IMMUNOLOGY
(2020)
Article
Pediatrics
Audrea M. Burns, Kate G. Ackerman, Satid Thammasitboon, Caroline E. Rassbach, Mark A. Ward, Rebecca L. Blankenburg, Catherine S. Forster, Heather A. McPhillips, Tara L. Wenger, Weston T. Powell, Melvin B. Heyman, Michael D. Hogarty, Debra Boyer, Margaret Hostetter, Pnina Weiss, Suong T. Nguyen, Donald Williams Parsons, Daniel J. Moore, Bobbi J. Byrne, Anthony R. French, Jordan S. Orange
PEDIATRIC RESEARCH
(2020)
Review
Immunology
Hassan Abolhassani, Gholamreza Azizi, Laleh Sharifi, Reza Yazdani, Monireh Mohsenzadegan, Samaneh Delavari, Mahsa Sohani, Paniz Shirmast, Zahra Chavoshzadeh, Seyed Alireza Mahdaviani, Arash Kalantari, Marzieh Tavakol, Farahzad Jabbari-Azad, Hamid Ahanchian, Tooba Momen, Roya Sherkat, Mahnaz Sadeghi-Shabestari, Soheila Aleyasin, Hossein Esmaeilzadeh, Waleed Al-Herz, Ahmed Aziz Bousfiha, Antonio Condino-Neto, Mikko Seppanen, Kathleen E. Sullivan, Lennart Hammarstrom, Vicki Modell, Fred Modell, Jessica Quinn, Jordan S. Orange, Asghar Aghamohammadi
EXPERT REVIEW OF CLINICAL IMMUNOLOGY
(2020)
Article
Immunology
Conor N. Gruber, Jorg J. A. Calis, Sofija Buta, Gilad Evrony, Jerome C. Martin, Skyler A. Uhl, Rachel Caron, Lauren Jarchin, David Dunkin, Robert Phelps, Bryn D. Webb, Jeffrey M. Saland, Miriam Merad, Jordan S. Orange, Emily M. Mace, Brad R. Rosenberg, Bruce D. Gelb, Dusan Bogunovic
Article
Immunology
Deepti R. Deshpande, Yesim Y. Demirdag, Rebecca A. Marsh, Kathleen E. Sullivan, Jordan S. Orange
Summary: The study found that low absolute CD3+ T cell counts below 50% of age-adjusted normal values in patients with DiGeorge syndrome are associated with higher odds of autoimmunity and/or asthma.
JOURNAL OF CLINICAL IMMUNOLOGY
(2021)
Editorial Material
Pediatrics
Steven H. Abman, Sarah Armstrong, Susan Baker, Clifford W. Bogue, Waldemar Carlo, Lina Chalak, Stephen R. Daniels, Stephanie Davis, Michael R. Debaun, Candice Fike, Lauren Frazer, Keisha Gibson, Michelle Gill, Hannah Glass, Catherine M. Gordon, Monika Goyal, Joel Hirschhorn, Lori Holtz, David Hunstad, Mary B. Leonard, Nathalie Maitre, Larry Markham, Linda McAllister-Lucas, Jordan Orange, Prachi Shah, Tamar Simon, Robin H. Steinhorn, Beth Tarini, Leslie R. Walker-Harding
PEDIATRIC RESEARCH
(2022)
Article
Biochemistry & Molecular Biology
Yu Li, Jordan S. Orange
Summary: This study demonstrates that NK cells are protected from perforin-mediated autolysis by densely packed and highly ordered presynaptic lipid membranes. The aggressive breast cancer cell line is perforin resistant due to a densely packed postsynaptic membrane, but disrupting membrane packing can make these cells susceptible to NK cell-mediated killing. Lipid membranes play an unexpected role in NK cell functionality, protecting them from autolysis and creating local ordered lipid shields against self-destruction during degranulation.
Article
Oncology
Yao-Yu Gong, Hongguang Shao, Yu Li, Patricia Brafford, Zachary E. Stine, Jing Sun, Dean W. Felsher, Jordan S. Orange, Steven M. Albelda, Chi Dang
Summary: This study demonstrates the feasibility of metabolic engineering immune effector cells to overcome inhibition in the tumor microenvironment, an approach that could enhance the efficacy of adoptive transfer immunotherapy.
CANCER RESEARCH COMMUNICATIONS
(2022)