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View article: New cases expand the genotype, phenotype and therapeutic landscape of H syndrome
New cases expand the genotype, phenotype and therapeutic landscape of H syndrome Open
View article: Rasamsonia argillacea brain abscess in a lung transplant recipient: an unexpected infection by an unusual fungal pathogen
Rasamsonia argillacea brain abscess in a lung transplant recipient: an unexpected infection by an unusual fungal pathogen Open
View article: Real-world Experience with Advanced Long-term Prophylaxis Agents to Treat Hereditary Angioedema in Canada
Real-world Experience with Advanced Long-term Prophylaxis Agents to Treat Hereditary Angioedema in Canada Open
Hereditary angioedema (HAE) is a rare genetic disorder caused by a deficiency or dysfunction in the serine protease inhibitor of C1, which results in an accumulation of the vasodilator bradykinin. Angioedema is a type of temporary swelling…
View article: Humoral and cell-mediated immune responses to COVID-19 vaccines up to 6 months post three-dose primary series in adults with inborn errors of immunity and their breakthrough infections
Humoral and cell-mediated immune responses to COVID-19 vaccines up to 6 months post three-dose primary series in adults with inborn errors of immunity and their breakthrough infections Open
Purpose Many individuals with inborn errors of immunity (IEIs) have poor humoral immune (HI) vaccine responses. Only a few studies have examined specific cell-mediated immune (CMI) responses to coronavirus disease 2019 (COVID-19) vaccines …
View article: The impact of institutional measures on optimal use of intravenous immunoglobulin
The impact of institutional measures on optimal use of intravenous immunoglobulin Open
Background Intravenous immunoglobulin (IVIG) shortage represents an emerging issue in transfusion medicine. Limited data are available to determine effective strategies for optimal use. The objective of this retrospective observational stu…
View article: COVID-19 Vaccination in Patients with Inborn Errors of Immunity Reduces Hospitalization and Critical Care Needs Related to COVID-19: a USIDNET Report
COVID-19 Vaccination in Patients with Inborn Errors of Immunity Reduces Hospitalization and Critical Care Needs Related to COVID-19: a USIDNET Report Open
View article: Revaccination outcomes among adolescents and adults with suspected hypersensitivity reactions following COVID-19 vaccination: A Canadian immunization research network study
Revaccination outcomes among adolescents and adults with suspected hypersensitivity reactions following COVID-19 vaccination: A Canadian immunization research network study Open
Most individuals in this national cohort who experienced a hypersensitivity event following COVID-19 vaccination and were referred for specialist review were revaccinated without AEFI recurrence, suggesting that specialist evaluation can f…
View article: Severe Relapsing Autoimmune Encephalitis with GABA<sub>A</sub> Receptor, Titin, and AchR Antibodies in a Patient with Thymoma: A Case Report
Severe Relapsing Autoimmune Encephalitis with GABA<sub>A</sub> Receptor, Titin, and AchR Antibodies in a Patient with Thymoma: A Case Report Open
Introduction: We report a challenging case of autoimmune encephalitis in a patient with a thymoma harboring titin and acetylcholine receptor antibodies, who experienced multiple relapses despite thymectomy and aggressive firs…
View article: Refractory wound healing and cytopenias treated with a sodium-glucose cotransporter-2 inhibitor in a patient with glucose-6-phosphatase catalytic subunit 3 deficiency
Refractory wound healing and cytopenias treated with a sodium-glucose cotransporter-2 inhibitor in a patient with glucose-6-phosphatase catalytic subunit 3 deficiency Open
View article: COVID-19 Vaccination in Patients with Inborn Errors of Immunity Reduces Hospitalization and Critical Care Needs Related to COVID-19: a USIDNET Report
COVID-19 Vaccination in Patients with Inborn Errors of Immunity Reduces Hospitalization and Critical Care Needs Related to COVID-19: a USIDNET Report Open
Background The CDC and ACIP recommend COVID-19 vaccination for patients with inborn errors of immunity (IEI). Not much is known about vaccine safety in IEI, and whether vaccination attenuates infection severity in IEI. Objective To estimat…
View article: Efficacy And Safety Of Bradykinin B2 Receptor Antagonism With Oral Deucrictibant In Prophylaxis Of Hereditary Angioedema Attacks: Results Of CHAPTER-1 Phase 2 Trial
Efficacy And Safety Of Bradykinin B2 Receptor Antagonism With Oral Deucrictibant In Prophylaxis Of Hereditary Angioedema Attacks: Results Of CHAPTER-1 Phase 2 Trial Open
View article: Clinical and functional spectrum of RAC2-related immunodeficiency
Clinical and functional spectrum of RAC2-related immunodeficiency Open
Mutations in the small Rho-family guanosine triphosphate hydrolase RAC2, critical for actin cytoskeleton remodeling and intracellular signal transduction, are associated with neonatal severe combined immunodeficiency (SCID), infantile neut…
View article: The Disease Burden of Hereditary Angioedema: Insights from a Survey in French‐Canadians from Quebec
The Disease Burden of Hereditary Angioedema: Insights from a Survey in French‐Canadians from Quebec Open
Background . Limited data are available on the clinical profile and disease burden of hereditary angioedema (HAE) in Canadians. Objective . This study aimed to assess HAE disease characteristics and the burden of disease in Canadians with …
View article: LONG-TERM QUALITY-OF-LIFE AND PATIENT-REPORTED IMPROVEMENTS WITH GARADACIMAB FOR HEREDITARY ANGIOEDEMA: PHASE 3 OPEN-LABEL EXTENSION STUDY
LONG-TERM QUALITY-OF-LIFE AND PATIENT-REPORTED IMPROVEMENTS WITH GARADACIMAB FOR HEREDITARY ANGIOEDEMA: PHASE 3 OPEN-LABEL EXTENSION STUDY Open
View article: Transitioning from pediatric to adult healthcare with an inborn error of immunity: a qualitative study of the lived experience of youths and their families
Transitioning from pediatric to adult healthcare with an inborn error of immunity: a qualitative study of the lived experience of youths and their families Open
Introduction Transition from pediatric to adult healthcare is a multifaceted and consequential process with important health implications for youth. Although research on transition has grown significantly, research on transition for patien…
View article: RETRACTED AND REPLACED: Real-World Effectiveness of Nirmatrelvir/Ritonavir on Coronavirus Disease 2019–Associated Hospitalization Prevention: A Population-based Cohort Study in the Province of Quebec, Canada
RETRACTED AND REPLACED: Real-World Effectiveness of Nirmatrelvir/Ritonavir on Coronavirus Disease 2019–Associated Hospitalization Prevention: A Population-based Cohort Study in the Province of Quebec, Canada Open
This article has been retracted and replaced. Please see https://doi.org/10.1093/cid/ciaf144 for details. The replacement article can be found at https://doi.org/10.1093/cid/ciaf145.
View article: REAL-WORLD EFFECTIVENESS OF NIRMATRELVIR/RITONAVIR ON COVID-19-ASSOCIATED HOSPITALIZATION PREVENTION: A POPULATION-BASED COHORT STUDY IN THE PROVINCE OF QUÉBEC, CANADA
REAL-WORLD EFFECTIVENESS OF NIRMATRELVIR/RITONAVIR ON COVID-19-ASSOCIATED HOSPITALIZATION PREVENTION: A POPULATION-BASED COHORT STUDY IN THE PROVINCE OF QUÉBEC, CANADA Open
Introduction The nirmatrelvir/ritonavir (PAXLOVID™) is an antiviral blocking the replication of SARS-CoV-2. Early treatment with this antiviral has showed to reduce COVID-19 hospitalization and death in unvaccinated outpatients with mild-t…
View article: A unique presentation of NLRP3-associated autoinflammatory disease: case report
A unique presentation of NLRP3-associated autoinflammatory disease: case report Open
View article: Chronic granulomatous disease presenting at age 52 with fulminant mulch pneumonitis
Chronic granulomatous disease presenting at age 52 with fulminant mulch pneumonitis Open
Chronic granulomatous disease should be considered in adults of any age in the presence of refractory and/or atypical or fulminant pulmonary infections. This case of new large deletions in NCF1 was presented with mulch pneumonitis w…
View article: Bradykinin-induced angioedema in the emergency department
Bradykinin-induced angioedema in the emergency department Open
View article: Therapeutic options for CTLA-4 insufficiency
Therapeutic options for CTLA-4 insufficiency Open
View article: Sarilumab in patients admitted to hospital with severe or critical COVID-19: a randomised, double-blind, placebo-controlled, phase 3 trial
Sarilumab in patients admitted to hospital with severe or critical COVID-19: a randomised, double-blind, placebo-controlled, phase 3 trial Open
View article: Error in Abstract
Error in Abstract Open
Megan Lam, BSc; Jie Wei Zhu, BHSc; Talha Maqbool, MD; Gaelen Adam, MLIS, MPH; Mina Tadrous, PharmD, PhD; Paula Rochon, MD, MPH; Aaron M. Drucker, MD, ScM
View article: Error in Byline
Error in Byline Open
Caren Droesch, MD; Mytrang Hoang Do, BS; Maria DeSancho, MD, MSc; Eun-Ju Lee, MD; Cynthia Magro, MD; Joanna Harp, MD
View article: Partial Purine Nucleoside Phosphorylase Deficiency Helps Determine Minimal Activity Required for Immune and Neurological Development
Partial Purine Nucleoside Phosphorylase Deficiency Helps Determine Minimal Activity Required for Immune and Neurological Development Open
Introduction: Complete or near complete absence of the purine nucleoside phosphorylase (PNP) enzyme causes a profound T cell immunodeficiency and neurological abnormalities that are often lethal in infancy and early childhood. We hy…
View article: Correction to: The International/Canadian Hereditary Angioedema Guideline
Correction to: The International/Canadian Hereditary Angioedema Guideline Open
View article: Pharmacokinetic Analysis of Weekly Versus Biweekly IgPro20 Dosing in Patients With Primary Immunodeficiency
Pharmacokinetic Analysis of Weekly Versus Biweekly IgPro20 Dosing in Patients With Primary Immunodeficiency Open
Flexible dosing of IgPro20 (Hizentra®, CSL Behring, King of Prussia, Pennsylvania) maintains normal serum immunoglobulin G (IgG) levels in patients with primary immunodeficiencies (PID). Until now, clinical trials testing the pharmacokinet…
View article: Population pharmacokinetic analysis of weekly and biweekly IgPro20 (Hizentra®) dosing in patients with primary immunodeficiency
Population pharmacokinetic analysis of weekly and biweekly IgPro20 (Hizentra®) dosing in patients with primary immunodeficiency Open
The present analysis validated the ability of the previously published popPK model to predict serum IgG concentration time profiles after biweekly subcutaneous IgPro20 administration.
View article: The International/Canadian Hereditary Angioedema Guideline
The International/Canadian Hereditary Angioedema Guideline Open
This is an update to the 2014 Canadian Hereditary Angioedema Guideline with an expanded scope to include the management of hereditary angioedema (HAE) patients worldwide. It is a collaboration of Canadian and international HAE experts and …
View article: Hiding in plain sight: A case of fever, rash, and jaundice
Hiding in plain sight: A case of fever, rash, and jaundice Open
A 25-year-old man presented to the emergency department with a 3-day history of fever, anorexia, jaundice, and a generalized skin eruption. His liver function tests showed marked cholestatic and cytolytic abnormalities without liver insuff…