Publications by authors named "CL Perry"

von Willebrand disease (VWD) is an inherited bleeding disorder caused by quantitative or qualitative defects in the von Willebrand factor (VWF) protein. Type 3 VWD has a severe bleeding phenotype caused by the absence of VWF, in which treatment usually involves replacement therapy with VWF-containing products. The immune system can react to the VWF product and form anti-VWF antibodies to neutralize or clear the VWF, which can compromise efficacy of treatment or lead to anaphylaxis.

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A small number of cancer patients respond exceptionally well to therapies and survive significantly longer than patients with similar diagnoses. Profiling the germline genetic backgrounds of exceptional responder (ER) patients, with extreme survival times, can yield insights into the germline polymorphisms that influence response to therapy. As ERs showed a high incidence in autoimmune diseases, we hypothesized the differences in autoimmune disease risk could reflect the immune background of ERs and contribute to better cancer treatment responses.

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Article Synopsis
  • Genetic analysis for von Willebrand disease (VWD) often misses copy-number variants (CNVs), prompting a study to further characterize these variants in patients without known VWF mutations.* -
  • The research included 204 VWF mutation-negative patients and used a specialized genomic hybridization array, identifying 24 CNVs across 7 unique variations, with one being novel.* -
  • Findings revealed a specific in-frame deletion linked to type 1C VWD associated with altered levels of VWF activity, suggesting CNVs may significantly impact the disease's characteristics compared to single base mutations.*
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Introduction: VWD diagnosis is challenging requiring multiple VWF activity tests using many individual assays. We have developed an ELISA-based VWF Multiplex Activity Assay (VWF-MAA) to address this concern; however, the ability of the VWF-MAA to discriminate between type 1 VWD, variant VWD, and normal subjects has not been evaluated.

Aim: To evaluate the VWF-MAA and its ability to differentiate between type 1 VWD, variant VWD and normal subjects in individuals undergoing an initial laboratory evaluation for bleeding.

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Background: Rural residence has been associated with a lower incidence of inflammatory bowel disease (IBD) but higher health care utilization and worse outcomes. Socioeconomic status is intrinsically tied to both IBD incidence and outcomes. Inflammatory bowel disease outcomes have not been investigated in Appalachia: a rural, economically distressed region rife with risk factors for both increased incidence and unfavorable outcomes.

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Introduction: Tobacco-related content is prevalent on social media, yet many methods of measuring exposure are inadequate due to the personalized nature of online marketing. The purpose of this paper is to examine the association between exposure to pro-tobacco messages (both industry-sponsored and user-generated) and the use of tobacco products, as reported via ecological momentary assessment (EMA).

Methods: Young adults (n = 175) were instructed to record all sightings of marketing (both in-person and online) related to tobacco for 28 days.

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Introduction: Communities with more people of color and economically disadvantaged residents are disproportionately exposed to tobacco marketing from tobacco companies. This study examined if banning tobacco retail outlets (TROs) within 1000 ft of schools would reduce these marketing disparities through a greater reduction in the amount of tobacco advertising around schools in these communities.

Methods: Data from objectively audited advertisement data from 106 convenience stores and gas stations around 42 middle and high schools located in the four major metropolitan areas of Texas were linked with schools' enrollment data.

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Significance: Young adults, especially those who identify as racial/ethnic minorities, are legal targets of the tobacco industry. Cigarillo initiation is a risk among these vulnerable groups. Estimating the age of initiation of cigarillo use among young adults may inform the timing of prevention interventions.

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Background: Type 3 von Willebrand Disease (VWD) is a rare and severe form of VWD characterized by the absence of von Willebrand factor (VWF).

Objectives: As part of the Zimmerman Program, we sought to explore the molecular pathogenesis, correlate bleeding phenotype and severity, and determine the inheritance pattern found in type 3 VWD families.

Patients/methods: 62 index cases with a pre-existing diagnosis of type 3 VWD were analyzed.

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Type 2N von Willebrand disease is caused by mutations in the factor VIII (FVIII) binding site of von Willebrand factor (VWF), resulting in dysfunctional VWF with defective binding capacity for FVIII. We developed a novel type 2N mouse model using CRISPR/Cas9 technology. In homozygous VWF2N/2N mice, plasma VWF levels were normal (1167 ± 257 mU/mL), but the VWF was completely incapable of binding FVIII, resulting in 53 ± 23 mU/mL of plasma FVIII levels that were similar to those in VWF-deficient (VWF-/-) mice.

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Background: The use of new and emerging tobacco products (NETPs) and conventional tobacco products (CTPs) has been linked to several alarming medical conditions among young adults (YAs). Considering that 96% of YAs own mobile phones, SMS text messaging may be an effective strategy for tobacco risk communication.

Objective: Project Debunk is a community-based randomized trial aiming to identify specific types of messages that effectively improve perceived NETP and CTP risk among YAs in community colleges.

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Aside from prevalence estimates and comparisons to heterosexual and presumed cisgender (i.e., not transgender) samples, little is known about transgender and gender diverse (TGD) young adult tobacco use.

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Aims: To examine the longitudinal trajectory of young Texan (US) adults' electronic nicotine delivery systems (ENDS) use from 2014 to 2019, and to determine if there are changes in the trajectory among younger and older young adults post-2017, when vape pods surged in popularity in the United States.

Design: Nine-wave longitudinal study, with 6 months between each of the first eight waves and 1 year between the last two waves. Discontinuous, or piecewise, growth curve models were used to test the hypotheses that (a) the overall current/past 30-day ENDS use trajectory would decline from 2014 to spring 2017 but then increase from fall 2017 to 2019, and (b) the increasing trajectory from 2017 to 2019 would occur only for younger participants, but not older participants.

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E-cigarette use harms adolescent health, yet it continues to escalate rapidly among teens nationwide. This longitudinal study sought to identify and differentiate between developmental trajectories of past 30-day e-cigarette use with and without marijuana (i.e.

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Objectives: To prospectively estimate the age of cigarette initiation among young adults (18-24 years old) who were never cigarette users at their first wave of adult study participation overall, by sex, and by race/ethnicity given recent increases in cigarette initiation occurring in young adulthood.

Methods: Secondary analyses were conducted using the PATH restricted-use adult datasets among young adult never users of cigarettes in waves 1-3 (2013-2016) with outcomes followed-up in waves 2-4 (2014-2017). Interval censoring survival methods were used to estimate the age of initiation of (i) ever, (ii) past 30-day, and (iii) fairly regular cigarette use.

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Introduction: Approximately 25% of youth in the United States speak a language other than English at home. These youth may have less exposure to English-speaking media, including public smoking prevention initiatives such as the FDA's "The Real Cost" campaign. Research is needed to explore potential gaps in the reach of "The Real Cost" campaign among bilingual youth.

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Importance: Cigarettes are still a commonly used tobacco product among youth despite recent declines in cigarette use.

Objective: The aim of this study was to prospectively estimate the age of cigarette use initiation among youth (aged 12-17 years) overall, by sex, and by race/ethnicity.

Design, Setting, And Participants: This cohort study used data from waves 1 through 4 of the nationally representative Population Assessment of Tobacco and Health (PATH) study, conducted from September 12, 2013, to January 3, 2018.

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Objectives: This study examines the relationships between recall of exposure to digital marketing of smokeless tobacco, via the internet and social media, and subsequent initiation of smokeless tobacco use at one-year follow-up, among young adult never users of smokeless tobacco in Texas.

Methods: Data were from waves 6 (Spring 2017) and 7 (Spring 2018) of the Marketing and Promotions Across Colleges in Texas Study (Project M-PACT); a longitudinal study of two- and four-year Texas college students. Participants were 2731 young adult never smokeless tobacco users (ages 20-32) with complete data at both assessment periods.

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Objective: This study reports the prospectively estimated age of initiation of susceptibility to, ever, past 30-day, and fairly regular hookah use.

Design: Secondary data analyses of the first four waves (2013-2017) of the PATH study, a nationally representative longitudinal cohort study of US youth (ages 12-17).

Methods: Youth who were never hookah users at their first wave of participation were identified (n = 16,678; N = 31,136,834).

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Background: Chronic opioid use is associated with poorer clinical outcomes in inflammatory bowel disease.

Aims: To investigate an association between chronic opioid use and persistence with biologic agents in management of inflammatory bowel disease.

Methods: A total of 16 624 patients diagnosed with inflammatory bowel disease and receiving a first-time biologic prescription from 2011 to 2016 were identified retrospectively from the Truven MarketScan Database.

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Introduction: Few studies examine the impact of objective exposure to point-of-sale (POS) marketing for cigars including little cigars and cigarillos (LCCs) on tobacco use. The present study aimed to examine the relationship between exposure to LCC marketing at the POS and current and future use of LCCs and cigarettes among young adult college students.

Method: Data on LCC and cigarette use from 4201 young adult students (mean age = 22.

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