Journal ArticleJAMA internal medicine · September 2023
ImportanceFewer than 5% of patients labeled with a penicillin allergy are truly allergic. The standard of care to remove the penicillin allergy label in adults is specialized testing involving prick and intradermal skin testing followed by an oral ...
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Journal ArticleOtolaryngol Clin North Am · February 2023
Primary Ig deficiencies are a heterogeneous group of disorders with widespread implications for the unified airway. Manifestations can vary greatly, with some patients being asymptomatic, whereas others suffering from acute and chronic life-threatening pat ...
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Journal ArticleImmunotherapy · November 2022
Aim: A novel, Investigational Wearable Infusor (IWI) was evaluated in a randomized, controlled, crossover, open-label study to determine if its delivery of subcutaneous immunoglobulin (IgPro20) achieved a comparable area under the concentration-time curve ...
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Journal ArticleAllergy · March 2022
BACKGROUND: The aim was to evaluate long-term effectiveness and safety of lanadelumab in patients ≥12 y old with hereditary angioedema (HAE) 1/2 (NCT02741596). METHODS: Rollover patients completing the HELP Study and continuing into HELP OLE received one l ...
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Journal ArticleClin Exp Immunol · December 2021
Acquired angioedema due to C1-inhibitor (C1-INH) deficiency (AAE-C1-INH) is rare and is associated with underlying lymphoproliferative diseases. C1-INH deficiency may be due to neoplastic over-consumption of C1-INH and the generation of anti-C1-INH autoant ...
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Journal ArticleJAMA Netw Open · May 3, 2021
IMPORTANCE: Penicillin allergies are frequently mislabeled, which may contribute to use of less-preferred alternative antibiotics. OBJECTIVE: To evaluate a pharmacist-led allergy assessment program's association with antimicrobial use and clinical outcomes ...
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Journal ArticleAllergy · April 2021
BACKGROUND: An objective of the phase 3 HELP Study was to investigate the effect of lanadelumab on health-related quality of life (HRQoL) in patients with hereditary angioedema (HAE). METHODS: Patients with HAE-1/2 received either lanadelumab 150 mg every ...
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Journal ArticleJ Clin Immunol · January 2021
PURPOSE: To evaluate the safety and tolerability of IgPro20 manual push (also known as rapid push) infusions at flow rates of 0.5-2.0 mL/min. METHODS: Patients with primary immunodeficiency (PID) with previous experience administering IgPro20 (Hizentra®, C ...
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Journal ArticleAllergy · November 2020
BACKGROUND: Lanadelumab demonstrated efficacy in preventing hereditary angioedema (HAE) attacks in the phase 3 HELP Study. OBJECTIVE: To assess time to onset of effect and long-term efficacy of lanadelumab, based on exploratory findings from the HELP Study ...
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Journal ArticleJ Allergy Clin Immunol · January 2020
Genetic testing has become an integral component of the diagnostic evaluation of patients with suspected primary immunodeficiency diseases. Results of genetic testing can have a profound effect on clinical management decisions. Therefore clinical providers ...
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Journal ArticleJ Hosp Med · November 2019
Inspired by the ABIM Foundation's Choosing Wisely® campaign, the "Things We Do for No Reason™" (TWDFNR) series reviews practices that have become common parts of hospital care but may provide little value to our patients. Practices reviewed in the TWDFNR s ...
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Journal ArticleJ Clin Immunol · October 2019
PURPOSE: To evaluate mortality risk factors in pediatric-onset common variable immunodeficiency disorders (CVID), we evaluated the largest single-institution cohort of pediatric-onset CVID patients. Previous publications on CVID have provided valuable desc ...
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Journal ArticleJ Clin Immunol · April 2018
Although small prior studies have suggested that IgE can be low in common variable immunodeficiency (CVID), the workup for patients with recurrent infections and suspected hypogammaglobulinemia does not include the routine measurement of serum IgE. We soug ...
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Journal ArticleJ Allergy Clin Immunol · March 2018
BACKGROUND: We evaluated the overall and site-specific incidence of cancer in subjects with primary immunodeficiency diseases (PIDD) enrolled in the United States Immune Deficiency Network (USIDNET) registry compared with age-adjusted cancer incidence in t ...
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Journal ArticleJAMA internal medicine · September 2023
ImportanceFewer than 5% of patients labeled with a penicillin allergy are truly allergic. The standard of care to remove the penicillin allergy label in adults is specialized testing involving prick and intradermal skin testing followed by an oral ...
Full textOpen AccessCite
Journal ArticleOtolaryngol Clin North Am · February 2023
Primary Ig deficiencies are a heterogeneous group of disorders with widespread implications for the unified airway. Manifestations can vary greatly, with some patients being asymptomatic, whereas others suffering from acute and chronic life-threatening pat ...
Full textLink to itemCite
Journal ArticleImmunotherapy · November 2022
Aim: A novel, Investigational Wearable Infusor (IWI) was evaluated in a randomized, controlled, crossover, open-label study to determine if its delivery of subcutaneous immunoglobulin (IgPro20) achieved a comparable area under the concentration-time curve ...
Full textLink to itemCite
Journal ArticleAllergy · March 2022
BACKGROUND: The aim was to evaluate long-term effectiveness and safety of lanadelumab in patients ≥12 y old with hereditary angioedema (HAE) 1/2 (NCT02741596). METHODS: Rollover patients completing the HELP Study and continuing into HELP OLE received one l ...
Full textLink to itemCite
Journal ArticleClin Exp Immunol · December 2021
Acquired angioedema due to C1-inhibitor (C1-INH) deficiency (AAE-C1-INH) is rare and is associated with underlying lymphoproliferative diseases. C1-INH deficiency may be due to neoplastic over-consumption of C1-INH and the generation of anti-C1-INH autoant ...
Full textLink to itemCite
Journal ArticleJAMA Netw Open · May 3, 2021
IMPORTANCE: Penicillin allergies are frequently mislabeled, which may contribute to use of less-preferred alternative antibiotics. OBJECTIVE: To evaluate a pharmacist-led allergy assessment program's association with antimicrobial use and clinical outcomes ...
Full textLink to itemCite
Journal ArticleAllergy · April 2021
BACKGROUND: An objective of the phase 3 HELP Study was to investigate the effect of lanadelumab on health-related quality of life (HRQoL) in patients with hereditary angioedema (HAE). METHODS: Patients with HAE-1/2 received either lanadelumab 150 mg every ...
Full textOpen AccessLink to itemCite
Journal ArticleJ Clin Immunol · January 2021
PURPOSE: To evaluate the safety and tolerability of IgPro20 manual push (also known as rapid push) infusions at flow rates of 0.5-2.0 mL/min. METHODS: Patients with primary immunodeficiency (PID) with previous experience administering IgPro20 (Hizentra®, C ...
Full textLink to itemCite
Journal ArticleAllergy · November 2020
BACKGROUND: Lanadelumab demonstrated efficacy in preventing hereditary angioedema (HAE) attacks in the phase 3 HELP Study. OBJECTIVE: To assess time to onset of effect and long-term efficacy of lanadelumab, based on exploratory findings from the HELP Study ...
Full textLink to itemCite
Journal ArticleJ Allergy Clin Immunol · January 2020
Genetic testing has become an integral component of the diagnostic evaluation of patients with suspected primary immunodeficiency diseases. Results of genetic testing can have a profound effect on clinical management decisions. Therefore clinical providers ...
Full textLink to itemCite
Journal ArticleJ Hosp Med · November 2019
Inspired by the ABIM Foundation's Choosing Wisely® campaign, the "Things We Do for No Reason™" (TWDFNR) series reviews practices that have become common parts of hospital care but may provide little value to our patients. Practices reviewed in the TWDFNR s ...
Full textLink to itemCite
Journal ArticleJ Clin Immunol · October 2019
PURPOSE: To evaluate mortality risk factors in pediatric-onset common variable immunodeficiency disorders (CVID), we evaluated the largest single-institution cohort of pediatric-onset CVID patients. Previous publications on CVID have provided valuable desc ...
Full textLink to itemCite
Journal ArticleJ Clin Immunol · April 2018
Although small prior studies have suggested that IgE can be low in common variable immunodeficiency (CVID), the workup for patients with recurrent infections and suspected hypogammaglobulinemia does not include the routine measurement of serum IgE. We soug ...
Full textLink to itemCite
Journal ArticleJ Allergy Clin Immunol · March 2018
BACKGROUND: We evaluated the overall and site-specific incidence of cancer in subjects with primary immunodeficiency diseases (PIDD) enrolled in the United States Immune Deficiency Network (USIDNET) registry compared with age-adjusted cancer incidence in t ...
Full textLink to itemCite
Journal ArticleFrontiers in Immunology · January 9, 2018
Common variable immunodeficiency (CVID) is increasingly recognized for its association with autoimmune and inflammatory complications. Despite recent advances in immunophenotypic and genetic discovery, clinical care of CVID remains limited by our inability ...
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Journal ArticleJ Allergy Clin Immunol Pract · 2018
BACKGROUND: Autosomal dominant hyper-IgE syndrome (AD-HIES) is a rare condition. OBJECTIVE: Data from the USIDNET Registry provide a resource to examine the characteristics of patients with rare immune deficiency diseases. METHODS: A query was submitted to ...
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Journal ArticleJournal of clinical immunology · August 2017
PurposeCommon variable immunodeficiency (CVID) is a complex, heterogeneous immunodeficiency characterized by hypogammaglobulinemia, recurrent infections, and poor antibody response to vaccination. While antibiotics and immunoglobulin prophylaxis h ...
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Journal ArticleJ Exp Med · May 1, 2017
Enteropathy-associated T cell lymphoma (EATL) is a lethal, and the most common, neoplastic complication of celiac disease. Here, we defined the genetic landscape of EATL through whole-exome sequencing of 69 EATL tumors. SETD2 was the most frequently silenc ...
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Journal ArticleFront Immunol · 2014
PURPOSE: Use of the T cell-dependent neoantigen bacteriophage ΦX 174 has been described since the 1960s as a method to assess specific antibody response in patients with primary immunodeficiencies. We reviewed a cohort of patients at Duke University Medica ...
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Journal ArticleProc Natl Acad Sci U S A · January 22, 2013
Diffuse large B-cell lymphoma (DLBCL) is the most common form of lymphoma in adults. The disease exhibits a striking heterogeneity in gene expression profiles and clinical outcomes, but its genetic causes remain to be fully defined. Through whole genome an ...
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Journal ArticleNat Genet · December 2012
Burkitt lymphoma is characterized by deregulation of MYC, but the contribution of other genetic mutations to the disease is largely unknown. Here, we describe the first completely sequenced genome from a Burkitt lymphoma tumor and germline DNA from the sam ...
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Journal ArticlePLoS One · 2012
Systemic lupus erythematosus (SLE) is a generalized autoimmune disease characterized by abnormal B cell activation and the occurrence of increased frequencies of circulating plasma cells (PC). The molecular characteristics and nature of circulating PC and ...
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Journal ArticleBlood · December 2, 2010
A role for microRNA (miRNA) has been recognized in nearly every biologic system examined thus far. A complete delineation of their role must be preceded by the identification of all miRNAs present in any system. We elucidated the complete small RNA transcr ...
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Journal ArticleBlood · May 7, 2009
Mature B-cell differentiation provides an important mechanism for the acquisition of adaptive immunity. Malignancies derived from mature B cells constitute the majority of leukemias and lymphomas. These malignancies often maintain the characteristics of th ...
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Journal ArticleBlood · April 16, 2009
Subjects with X-linked hyper-IgM syndrome (X-HIgM) have a markedly reduced frequency of CD27(+) memory B cells, and their Ig genes have a low level of somatic hypermutation (SHM). To analyze the nature of SHM in X-HIgM, we sequenced 209 nonproductive and 9 ...
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Journal ArticleJ Exp Med · January 23, 2006
Interferon (IFN) consensus sequence-binding protein/IFN regulatory factor 8 (IRF8) is a transcription factor that regulates the differentiation and function of macrophages, granulocytes, and dendritic cells through activation or repression of target genes. ...
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