Background: The CDC and ACIP recommend COVID-19 vaccination for patients with inborn errors of im... more 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 estimate COVID-19 vaccination safety and examine effect on outcomes in patients with IEI. Methods: We built a secure registry database in conjunction with the United States Immunodeficiency Network to examine vaccination frequency and indicators of safety and effectiveness in IEI patients. The registry opened on January 1, 2022 and closed on August 19, 2022. Results: Physicians entered data on 1,245 patients from 24 countries. The most common diagnoses were antibody deficiencies (63.7%). At least 1 COVID-19 vaccine was administered to 806 patients (64.7%), and 216 patients received vaccination prior to the development of COVID-19. The most common vaccines administered were mRNA-based (84.0%). Seventeen patients were reported to seek outpatient clinic or e...
Chronic granulomatous disease (CGD) is an inborn error of immunity caused by inactivating genetic... more Chronic granulomatous disease (CGD) is an inborn error of immunity caused by inactivating genetic mutations in any one of the components of the phagocyte nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. Phagocytic cell reactive oxygen species generation is impaired in the absence of a functional NADPH oxidase complex. As a result, patients with CGD are at high risk of developing deep-seated infections with certain bacteria and fungi. Additionally, aberrant inflammation and granuloma formation may occur in multiple organs including the bowels, with inflammatory bowel disease seen as a common inflammatory complication of CGD. Traditionally, TNF-Ī± inhibitors are considered effective biological therapies for moderate-to-severe inflammatory bowel disease. While limited case series and reports of patients with CGD have shown improvement in fistula healing with use of TNF-Ī± inhibitors, several patients have developed severe, even fatal, infections with CGD-related patho...
Objectives Egg consumption may play an important role in early-life growth given their high-quali... more Objectives Egg consumption may play an important role in early-life growth given their high-quality protein, essential fatty acids, and micronutrients. The objective of this study was to examine the longitudinal associations of infant age at egg introduction with obesity outcomes in early childhood, middle childhood, and early adolescence. Methods We used existing data from 1,089 mother-child dyads from Project Viva to estimate age at egg introduction through a questionnaire completed by mothers at about 1 year postpartum (meanĀ Ā±Ā SD, 13.3Ā Ā±Ā 1.2Ā months). Outcome measures included height and weight, body composition including total fat mass, trunk fat mass, and lean mass, as well as plasma adiponectin and leptin in early childhood, mid-childhood, and early adolescence. We defined childhood obesity as sex- and age-specific body mass index (BMI) ā„95th percentile. We estimated the associations of infant age at egg introduction with risk of obesity using multivariable logistic regression ...
The Journal of Allergy and Clinical Immunology: In Practice, 2021
Recurrent and life-threatening respiratory infections are nearly universal in patients with prima... more Recurrent and life-threatening respiratory infections are nearly universal in patients with primary immunodeficiency diseases (PIDD). Early recognition, aggressive treatment, and prophylaxis with antimicrobials and immunoglobulin replacement have been the mainstays of management, and will be reviewed here with an emphasis on respiratory infections. Genetic discoveries have allowed direct translation of research to clinical practice - improving our understanding of clinical patterns of pathogen susceptibilities and guiding prophylaxis. The recent identification of inborn errors in type I interferon signaling as a basis for life threatening viral infections in otherwise healthy individuals suggests another targetable pathway for treatment and/or prophylaxis. The future of PIDD diagnosis will certainly involve early genetic identification by newborn screening before onset of infections, with early treatment offering the potential of preventing disease complications such as chronic lung changes. Gene editing approaches offer tremendous therapeutic potential, with rapidly emerging delivery systems. Anti-viral therapies are desperately needed and specific cellular therapies show promise in patients requiring hematopoietic stem cell transplantation. The introduction of approved therapies for clinical use in PIDD is limited by the difficulty of studying outcomes in rare patients/conditions with conventional clinical trials.
Background: The CDC and ACIP recommend COVID-19 vaccination for patients with inborn errors of im... more 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 estimate COVID-19 vaccination safety and examine effect on outcomes in patients with IEI. Methods: We built a secure registry database in conjunction with the United States Immunodeficiency Network to examine vaccination frequency and indicators of safety and effectiveness in IEI patients. The registry opened on January 1, 2022 and closed on August 19, 2022. Results: Physicians entered data on 1,245 patients from 24 countries. The most common diagnoses were antibody deficiencies (63.7%). At least 1 COVID-19 vaccine was administered to 806 patients (64.7%), and 216 patients received vaccination prior to the development of COVID-19. The most common vaccines administered were mRNA-based (84.0%). Seventeen patients were reported to seek outpatient clinic or e...
Chronic granulomatous disease (CGD) is an inborn error of immunity caused by inactivating genetic... more Chronic granulomatous disease (CGD) is an inborn error of immunity caused by inactivating genetic mutations in any one of the components of the phagocyte nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. Phagocytic cell reactive oxygen species generation is impaired in the absence of a functional NADPH oxidase complex. As a result, patients with CGD are at high risk of developing deep-seated infections with certain bacteria and fungi. Additionally, aberrant inflammation and granuloma formation may occur in multiple organs including the bowels, with inflammatory bowel disease seen as a common inflammatory complication of CGD. Traditionally, TNF-Ī± inhibitors are considered effective biological therapies for moderate-to-severe inflammatory bowel disease. While limited case series and reports of patients with CGD have shown improvement in fistula healing with use of TNF-Ī± inhibitors, several patients have developed severe, even fatal, infections with CGD-related patho...
Objectives Egg consumption may play an important role in early-life growth given their high-quali... more Objectives Egg consumption may play an important role in early-life growth given their high-quality protein, essential fatty acids, and micronutrients. The objective of this study was to examine the longitudinal associations of infant age at egg introduction with obesity outcomes in early childhood, middle childhood, and early adolescence. Methods We used existing data from 1,089 mother-child dyads from Project Viva to estimate age at egg introduction through a questionnaire completed by mothers at about 1 year postpartum (meanĀ Ā±Ā SD, 13.3Ā Ā±Ā 1.2Ā months). Outcome measures included height and weight, body composition including total fat mass, trunk fat mass, and lean mass, as well as plasma adiponectin and leptin in early childhood, mid-childhood, and early adolescence. We defined childhood obesity as sex- and age-specific body mass index (BMI) ā„95th percentile. We estimated the associations of infant age at egg introduction with risk of obesity using multivariable logistic regression ...
The Journal of Allergy and Clinical Immunology: In Practice, 2021
Recurrent and life-threatening respiratory infections are nearly universal in patients with prima... more Recurrent and life-threatening respiratory infections are nearly universal in patients with primary immunodeficiency diseases (PIDD). Early recognition, aggressive treatment, and prophylaxis with antimicrobials and immunoglobulin replacement have been the mainstays of management, and will be reviewed here with an emphasis on respiratory infections. Genetic discoveries have allowed direct translation of research to clinical practice - improving our understanding of clinical patterns of pathogen susceptibilities and guiding prophylaxis. The recent identification of inborn errors in type I interferon signaling as a basis for life threatening viral infections in otherwise healthy individuals suggests another targetable pathway for treatment and/or prophylaxis. The future of PIDD diagnosis will certainly involve early genetic identification by newborn screening before onset of infections, with early treatment offering the potential of preventing disease complications such as chronic lung changes. Gene editing approaches offer tremendous therapeutic potential, with rapidly emerging delivery systems. Anti-viral therapies are desperately needed and specific cellular therapies show promise in patients requiring hematopoietic stem cell transplantation. The introduction of approved therapies for clinical use in PIDD is limited by the difficulty of studying outcomes in rare patients/conditions with conventional clinical trials.
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Papers by Heather Lehman