William T. Shearer
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View article: Infectivity and Immunogenicity of Live-Attenuated Respiratory Syncytial Virus Vaccines in Human Immunodeficiency Virus–Exposed Uninfected Children
Infectivity and Immunogenicity of Live-Attenuated Respiratory Syncytial Virus Vaccines in Human Immunodeficiency Virus–Exposed Uninfected Children Open
Background Respiratory syncytial virus (RSV) is the leading cause of acute lower respiratory illness among young children. Human immunodeficiency virus (HIV)–exposed, uninfected (HEU) children experience a higher burden of RSV disease and …
View article: Case Report: Post-Partum Complications of NFκB1 Deficiency Underscore a Need to Better Understand Primary Immunodeficiency Management During Pregnancy
Case Report: Post-Partum Complications of NFκB1 Deficiency Underscore a Need to Better Understand Primary Immunodeficiency Management During Pregnancy Open
Nuclear factor κappa-B (NFκB) is a family of transcription factors involved in regulating inflammation and immunity. Mutations in the NFκB1 pathway are associated with primary immune defects and underlie the most common monogenic etiology …
View article: Need for an External Proficiency Testing Program for Cytokines, Chemokines, and Plasma Markers of Immune Activation
Need for an External Proficiency Testing Program for Cytokines, Chemokines, and Plasma Markers of Immune Activation Open
An external evaluation program for measuring the performance of laboratories testing for cytokines and immune activation markers in biological fluids was developed. Cytokines, chemokines, soluble cytokine receptors, and other soluble marke…
View article: Left ventricular diastolic dysfunction in HIV-uninfected infants exposed in utero to antiretroviral therapy
Left ventricular diastolic dysfunction in HIV-uninfected infants exposed in utero to antiretroviral therapy Open
Objectives: To longitudinally measure LV diastolic function in HIV-exposed but uninfected (HEU) children perinatally exposed to ART. Design: HEU children who were perinatally exposed to antiretroviral therapy (ART) may be at risk for adver…
View article: Rates of Hospitalization and Infection-Related Hospitalization Among Human Immunodeficiency Virus (HIV)–Exposed Uninfected Children Compared to HIV-Unexposed Uninfected Children in the United States, 2007–2016
Rates of Hospitalization and Infection-Related Hospitalization Among Human Immunodeficiency Virus (HIV)–Exposed Uninfected Children Compared to HIV-Unexposed Uninfected Children in the United States, 2007–2016 Open
Background Studies from multiple countries have suggested impaired immunity in perinatally human immunodeficiency virus (HIV)–exposed uninfected children (HEU), with elevated rates of all-cause hospitalization and infections. We estimated …
View article: Healthcare Transition Outcomes Among Young Adults With Perinatally Acquired Human Immunodeficiency Virus Infection in the United States
Healthcare Transition Outcomes Among Young Adults With Perinatally Acquired Human Immunodeficiency Virus Infection in the United States Open
Background Young adults with perinatally acquired HIV (YPHIVs) living in the United States are transitioning to adult clinical care, yet there is little information on factors that affect transition outcomes. Methods YPHIVs aged ≥18 years …
View article: Human Papillomavirus Antibody Levels and Quadrivalent Vaccine Clinical Effectiveness in Perinatally Human Immunodeficiency Virus–infected and Exposed, Uninfected Youth
Human Papillomavirus Antibody Levels and Quadrivalent Vaccine Clinical Effectiveness in Perinatally Human Immunodeficiency Virus–infected and Exposed, Uninfected Youth Open
Background Persons who are infected with human immunodeficiency virus (HIV) are at high risk of human papillomavirus (HPV)-associated cancers. The objectives are to compare antibody titers to HPV 6, 11, 16, and 18 and rate of abnormal cyto…
View article: SCID genotype and 6-month posttransplant CD4 count predict survival and immune recovery
SCID genotype and 6-month posttransplant CD4 count predict survival and immune recovery Open
Key Points The genetic cause of SCID impacts on survival and immune reconstitution and should be considered in tailoring HCT for individual patients. Total and naive CD4+ cell counts in SCID patients 6 and 12 months post-HCT predict long-t…
View article: Cardiac status of perinatally HIV-infected children
Cardiac status of perinatally HIV-infected children Open
Despite cardioprotective effects of combination regimens in PHIV youth, individual antiretroviral medications were associated with altered cardiac structure, which could progress to symptomatic cardiomyopathy in adulthood.
View article: Adverse Pregnancy Outcomes Among Women Who Conceive on Antiretroviral Therapy
Adverse Pregnancy Outcomes Among Women Who Conceive on Antiretroviral Therapy Open
Women randomized to continue ART who subsequently conceived were more likely to have spontaneous abortion or stillbirth, compared with women randomized to stop ART; however, the findings did not remain significant in the as-treated analysi…
View article: Cardiac and inflammatory biomarkers in perinatally HIV-infected and HIV-exposed uninfected children
Cardiac and inflammatory biomarkers in perinatally HIV-infected and HIV-exposed uninfected children Open
PHEU and PHIV have modest, significant differences in serum levels of specific inflammatory and active myocardial injury biomarkers. Higher biomarker levels were associated with lower LV mass and shifts in LV structure. Further study is wa…
View article: Outcomes of Umbilical Cord Transplant (UCBT) Conditioned Without Serotherapy for Pediatric Malignant and Non-Malignant Diseases: Texas Children's Hospital Experience
Outcomes of Umbilical Cord Transplant (UCBT) Conditioned Without Serotherapy for Pediatric Malignant and Non-Malignant Diseases: Texas Children's Hospital Experience Open
UCBT is increasingly used as an alternative donor stem cell source for the treatment of malignant and non-malignant pediatric disorders but is associated with increased incidence of viral infection-related mortality in part due to delayed …
View article: Resolution of CGD Related Colitis after Allogeneic Hematopoietic Stem Cell Transplantation in Patients with Chronic Granulomatous Disease—Early Results From the 6903 Study of the Primary Immune Deficiency Treatment Consortium (PIDTC)
Resolution of CGD Related Colitis after Allogeneic Hematopoietic Stem Cell Transplantation in Patients with Chronic Granulomatous Disease—Early Results From the 6903 Study of the Primary Immune Deficiency Treatment Consortium (PIDTC) Open
Background: Phagocytes of patients with CGD have a deficient NADPH oxidase and respiratory burst, which limit production of reactive oxygen species. Allogeneic hematopoietic stem cell transplant (HSCT) offers a potential cure with resoluti…
View article: Long-term follow-up of IPEX syndrome patients after different therapeutic strategies: An international multicenter retrospective study
Long-term follow-up of IPEX syndrome patients after different therapeutic strategies: An international multicenter retrospective study Open
Patients receiving chronic IS were hampered by disease recurrence or complications, impacting long-term disease-free survival. When performed in patients with a low OI score, HSCT resulted in disease resolution with better quality of life,…
View article: Cord Blood Banking for Potential Future Transplantation
Cord Blood Banking for Potential Future Transplantation Open
This policy statement is intended to provide information to guide pediatricians, obstetricians, and other medical specialists and health care providers in responding to parents’ questions about cord blood donation and banking as well as th…
View article: Immune reconstitution and survival of 100 SCID patients post–hematopoietic cell transplant: a PIDTC natural history study
Immune reconstitution and survival of 100 SCID patients post–hematopoietic cell transplant: a PIDTC natural history study Open
Key Points Active infection pretransplant adversely impacts survival (81% in patients with active infection vs 95% in infection-free patients; P = .009). Preparative chemotherapy improved 1-year post-HCT median CD4 counts (P = .02) and fre…
View article: High-resolution phenotyping identifies NK cell subsets that distinguish healthy children from adults
High-resolution phenotyping identifies NK cell subsets that distinguish healthy children from adults Open
Natural killer (NK) cells are critical in immune defense against infected, stressed or transformed cells. Their function is regulated by the heterogeneous expression of a wide array of surface receptors that shape its phenotypic diversity.…
View article: Birth Weight and Preterm Delivery Outcomes of Perinatally vs Nonperinatally Human Immunodeficiency Virus-Infected Pregnant Women in the United States: Results From the PHACS SMARTT Study and IMPAACT P1025 Protocol
Birth Weight and Preterm Delivery Outcomes of Perinatally vs Nonperinatally Human Immunodeficiency Virus-Infected Pregnant Women in the United States: Results From the PHACS SMARTT Study and IMPAACT P1025 Protocol Open
IMPAACT 1025, NCT00028145.
View article: Human Immunodeficiency Virus Type 1 DNA Decay Dynamics With Early, Long-term Virologic Control of Perinatal Infection
Human Immunodeficiency Virus Type 1 DNA Decay Dynamics With Early, Long-term Virologic Control of Perinatal Infection Open
Early effective, long-term ART initiated from infancy leads to decay of HIV-1-infected cells to exceedingly low concentrations desired for HIV-1 remission strategies.
View article: Genotype, Phenotype and T Cell Counts at One Year Predict Survival and Long Term Immune Reconstitution after Transplantation in Severe Combined Immune Deficiency (SCID)—The Primary Immune Deficiency Treatment Consortium (PIDTC)
Genotype, Phenotype and T Cell Counts at One Year Predict Survival and Long Term Immune Reconstitution after Transplantation in Severe Combined Immune Deficiency (SCID)—The Primary Immune Deficiency Treatment Consortium (PIDTC) Open
Patients: We retrospectively studied 581 patients with typical SCID who received a hematopoietic cell transplant (HCT) at 32 PIDTC centers from 1982-2012. Known mutations (n = 355) included 178 gC, 35 JAK3, 40 IL7R, 6 CD3, 1 CD45, 27 DCLRE…
View article: Umbilical Cord Transplant (UCBT) Without Serotherapy for Malignant and Non Malignant Diseases Provides a Curative Alternative with Improved Immune Reconstitution
Umbilical Cord Transplant (UCBT) Without Serotherapy for Malignant and Non Malignant Diseases Provides a Curative Alternative with Improved Immune Reconstitution Open
UCBT is widely accepted as an alternative donor source in recent years but is associated with increased incidence of viral infection related mortality. In part this increased risk is due to delayed viral specific T cell function. In this p…