Digital Commons Network^™

Comparing Goldin And The Philippines: Analyzing The Research Efforts In Women And Labor Economics Using Basic Text Mining Techniques, Arianna Liza Ortillan

Angelo King Institute for Economic and Business Studies (AKI)

No abstract provided.

Abc Efflux Transporters And Solute Carriers In The Early Developing Brain Of A Marsupial Monodelphis Domestica (South American Gray Short-Tailed Opossum), Yifan Huang, Katarzyna M. Dziegielewska, Mark D. Habgood, Fiona Qiu, Ana C. Leandro, Paul D. Callaghan, Joanne E. Curran, John L. Vandeberg, Norman R. Saunders

School of Medicine Publications

This study used a marsupial Monodelphis domestica, which is born very immature and most of its development is postnatal without placental protection. RNA-sequencing (RNA-Seq) was used to identify the expression of influx and efflux transporters (ATP-binding cassettes [ABCs] and solute carriers [SLCs]) and metabolizing enzymes in brains of newborn to juvenile Monodelphis. Results were compared to published data in the developing eutherian rat. To test the functionality of these transporters at similar ages, the entry of paracetamol (acetaminophen) into the brain and cerebrospinal fluid (CSF) was measured using liquid scintillation counting following a single administration of the drug along with …

Genetic Correlations Between Liver Fat Content, Metabolic Health, And Adiposity Distribution In The Fels Longitudinal Study, Ariana L. Garza, Miryoung Lee, John Blangero, Cici X. Bauer, Stefan A. Czerwinski, Audrey C. Choh

School of Medicine Publications

Highlights

• Steatosis and all other related phenotypes examined were significantly heritable.

• Liver fat showed significant genetic correlation with various phenotypes.

• The strongest genetic correlation of liver fat was with insulin resistance.

• Glucose homeostasis phenotypes were also genetically correlated to adiposity traits.

Abstract

Background and aims

Hepatic steatosis is known to be heritable, but its genetic basis is mostly uncharacterized. Steatosis is associated with metabolic and adiposity features; recent studies hypothesize that shared genetic effects between these traits could account for some of the unexplained heritability. This study aimed to quantify these genetic associations in a family-based sample of non-Hispanic white …

2024 Deerfield Fair 4-H Building Premium Book, Extension

UNH Cooperative Extension

No abstract provided.

2024 Deerfield Fair 4-H Animal Shows Premium Book, Extension

UNH Cooperative Extension

No abstract provided.

Medical Practice As Rhetorical Art Functional Medicine’S Therapeutic Partnership, Cristina Elena De León-Menjivar

English Theses & Dissertations

The current rates of provider burnout are at an all-time high, and our healthcare system is currently seeing numerous providers leave the system. The U.S. Surgeon General has deemed burnout rates at crisis levels, creating an exigency for research and work to help ameliorate this issue. One main issue at the heart of provider burnout is the idea of meaning and purpose in one’s professional life, and Functional Medicine methodology argues that it provides the means by which it can mitigate burnout while improving professional fulfillment and joy through deeper connections with patients. Their methodology is rooted in a concept …

Examining The Physical Activity Experiences Of Blind And Visually Impaired Women, Lindsay E. Ball

Human Movement Studies & Special Education Theses & Dissertations

Substantial research has indicated that blind and visually impaired adults often do not meet physical activity guidelines, and therefore, are not experiencing the numerous affordances of physical activity participation. However, those who report meeting physical activity guidelines express higher rates of health-related quality of life. Largely, the research examining the physical activity behaviors and perspectives of blind and visually impaired adults have been survey studies leaving a rich description of their physical activity experiences unexplored. The overall aim of this project was to engage with blind or visually impaired women to understand their experiences accessing and participating in physical activity, …

Effects Of Ordered Eating On Various Postprandial Measures, Brian K. Ferguson

Human Movement Studies & Special Education Theses & Dissertations

Postprandial glucose (PPG) is an indicator of acute and chronic overall health, and aberrations in glucose, insulin, and other postprandial (PP) markers are common in obesity and cardiometabolic disorders. Chronically elevated glucose can lead to a number of health problems such as type 2 diabetes mellitus. Additionally, PPG responses can impact substrate utilization responses to exercise, which may have implications for both healthy and diseased populations. One potentially simple lifestyle modification to alter PPG and related markers is to change the order in which foods are consumed within meals. The purpose of this dissertation was to further explore the impact …

A Landscape Evaluation Of Caffeine Citrate Availability And Use In Newborn Care Across Five Low- And Middle-Income Countries, Osayame Ekhaguere, Olufunke Bolaji, Helen Nabwera, Andrew Storey, Nicholas Embleton, Stephen Allen, Zelalem Demeke, Olufunke Fasawe, Betty Wariari, Mansharan Seth

Centre of Excellence in Women and Child Health

Apnoea of prematurity (AOP) is a common complication among preterm infants (< 37 weeks gestation), globally. However, access to caffeine citrate (CC) that is a proven safe and effective treatment in high-income countries is largely unavailable in low- and-middle income countries, where most preterm infants are born. Therefore, the overall aim of this study was to describe the demand, policies, and supply factors affecting the availability and clinical use of CC in LMICs. A mixed methods approach was used to collect data from diverse settings in LMICs including Ethiopia, Kenya, Nigeria, South Africa, and India. Qualitative semi-structured interviews and focus group discussions were conducted with 107 different health care providers, and 21 policymakers and other stakeholders from industry. Additional data was collected using standard questionnaires. A thematic framework approach was used to analyze the qualitative data and descriptive statistics were used to summarize the quantitative data. The findings indicate that there is variation in in-country policies on the use of CC in the prevention and treatment of AOP and its availability across the LMICs. As a result, the knowledge and experience of using CC also varied with clinicians in Ethiopia having no experience of using it while those in India have greater knowledge and experience of using it. This, in turn, influenced the demand, and our findings show that only 29% of eligible preterm infants are receiving CC in these countries. There is an urgent need to address the multilevel barriers to accessing CC for managing AOP in Africa. These include cost, lack of national policies, and, therefore, lack of demand stemming from its clinical equivalency with aminophylline. Practical ways to reduce the cost of CC in LMICs could potentially increase its availability and use.

Establishing Marine Protected Areas In The Southern Ocean, Lessons For The Bbnj Agreement, Nengye Liu

Research Collection Yong Pung How School Of Law

This paper first gives a brief overview of the Commission for the Conservation of Antarctic Marine Living Resources (CCAMLR)’s struggle to establish Antarctic Marine Protected Areas (MPAs). The paper then addresses the question: what lessons can countries learn from CCAMLR's experience in establishing high seas MPAs under the Biodiversity in Areas Beyond National Jurisdiction (BBNJ) Agreement? The paper finds that the final text of the BBNJ Agreement did largely reflect CCAMLR's experience when it comes to MPAs. This is particularly so with respect to 1) clarification of the relationship between conservation and use; 2) the interpretation and application of a …

Application Of Clavien-Dindo Classification System In The Early Complications Of Radical Cystectomy In Al-Yarmook Teaching Hospital, Ahmed Abbass Al-Sadie, Saad Al-Sammarraie, Taha Kadhum

Iraqi Postgraduate Medical Journal

BACKGROUND: The most effective treatment for muscle-invasive bladder cancer and non-muscle invasive bladder cancer with highest risk of progression, is Radical cystectomy and urinary diversion. This operation carries                  a high risk of complications of multiple types that can affect not only the urinary system but also other systems. OBJECTIVE: This study analyzes the early complications (within 30 days) of Radical Cystectomy and urinary diversion, using a standardized classification system i.e. Clavien-Dindo classification system. PATIENTS AND METHODS: This is a combined retrospective and prospective study analyzing the early complications of RC.                    We review the early (30 days) post-operative period retrospectively for …

Liver Disease And Transplantation In Telomere Biology Disorders: An International Multicenter Cohort, Yunzu Michele Wang, Steven K Brennan, Sakil Kulkarni, Et Al.

2020-Current year OA Pubs

BACKGROUND: Patients with telomere biology disorders (TBD) develop hepatic disease, including hepatitis, cirrhosis, and hepatopulmonary syndrome. No specific treatment exists for TBD-related liver disease, and the role of liver transplantation (LT) remains controversial. Our study objectives were to describe the clinical characteristics, management, and outcomes in patients with TBD-related liver disease, and their LT outcomes.

METHODS: Data from 83 patients with TBD-associated liver disease were obtained from 17 participating centers in the Clinical Care Consortium of Telomere-Associated Ailments and by self-report for our retrospective, multicenter, international cohort study.

RESULTS: Group A ("Advanced") included 40 patients with advanced liver disease. Of …

The Shared Experiences Of Novice, Full-Time Allied Dental Community College Educators, Misty L. Mesimer

Educational Leadership & Workforce Development Theses & Dissertations

A well-prepared oral healthcare workforce is essential to the overall health of the general population. A knowledgeable and qualified workforce would be non-existent without strong academic programs and faculty to prepare oral health professionals. For over 20 years, the dental and allied dental educator workforce has been a concern for the oral health professions. Since most allied dental education programs are based in community and technical colleges, it is crucial to thoroughly understand the transition experiences of faculty teaching at these institutions. It is only through application and use of this knowledge that these faculty can be recruited, supported, and …

Spatial And Temporal Variability In Nutritional Physiology Of Pyropia Perforata, Matthew S. Elliott

Capstone Projects and Master's Theses

The red alga, Pyropia perforata, commonly referred to as nori, is ubiquitous in intertidal communities throughout coastal California and offers high nutritional value to marine herbivores. However, California rocky shores are dynamic ecosystems, with dramatic abiotic and biotic shifts resulting in environmental heterogeneity. Research has shown that seaweed nutrient physiology is impacted by environmental heterogeneity, yet there is sparse data on how internal nitrogen and total protein content change as a result of environmental heterogeneity. Here, I studied the impacts of seasonality, geography (spanning 4° of latitude from Northern to Southern California), and the onset of environmental heterogeneity on internal …

Exploring The Effects Of An Alfalfa Leaf-Derived Adsorbent On Microbial Community, Ileal Morphology, Barrier Function, And Immunity In Turkey Poults During Chronic Aflatoxin B1 Exposure, Maria De Jesus Nava-Ramirez, Jing Liu, Juan Omar Hernandez-Ramirez, Xochitl Hernandez-Velasco, Juan D. Latorre, Alma Vazquez-Duran, Guolong Zhang, Roberto Señas-Cuesta, Sergio Gomez-Rosales, Andressa Stein, Billy M. Hargis, Guillermo Tellez-Isaias, Abraham Mendez-Albores, Jesus A. Maguey-Gonzalez

Poultry Science Faculty Publications and Presentations

This article follows-up on our recently published work, which evaluated the impact of the addition of an alfalfa leaf-derived adsorbent in the aflatoxin B₁ (AFB₁)-contaminated diet in regard to the production parameters, blood cell count, serum biochemistry, liver enzymes, and liver histology of turkey poults. This paper presents complementary results on microbial community, ileal morphology, barrier function, and immunity. For this purpose, 350 1-day-old female turkey poults were randomly distributed into five groups: (1) Control, AFB₁-free diet; (2) AF, AFB₁-contaminated diet at 250 ng/g; (3) alfalfa, AFB₁-free diet + 0.5% ( …

Pediatric Oral Health Literacy Among Caregivers Of Young Children, Melissa L. Sullivan

Health Services Research Dissertations

Early childhood caries (ECC) is a common dental condition that affects the primary teeth of infants and young children. ECC can have a significant negative impact on a child’s oral health and overall well-being. Caregivers have an important role in the prevention of early childhood caries through knowledge, understanding, and awareness of good oral hygiene practices. Caregivers who possess adequate oral health literacy (OHL) may be more likely to recognize the risks associated with ECC, implement proper oral hygiene practices, and make informed oral health choices for themselves and their child.

The overarching purpose of this dissertation is to explore …

Transcriptome Reveals The Key Genes Related To The Metabolism Of Volatile Sulfur-Containing Compounds In Lentinula Edodes Mycelium, Zheng Li, Fei Pan, Wen Huang, Shuangshuang Gao, Xi Feng, Meijie Chang, Lianfu Chen, Yinbing Bian, Wenli Tian, Ying Liu

Faculty Research, Scholarly, and Creative Activity

Lentinula edodes (L. edodes) is a globally popular edible mushroom because of its characteristic sulfur-containing flavor compounds. However, the formation of the volatile sulfur-containing compounds in the mycelium of L. edodes has not been studied. We found that there were also sulfur-containing aroma compounds in the mycelium of L. edodes, and the content and composition varied at different stages of mycelial growth and development. The γ-glutamyl-transpeptidase (GGT) and cysteine sulfoxide lyase (C-S lyase) related to the generation of sulfur compounds showed the highest activities in the 15-day sample. Candidate genes for the metabolism of volatile sulfur compounds in mycelium were …

The Effect Of Angiogenesis On Thermal Properties Of In-Vivo Human Tissue, Shriya Musuku

Theses

Bioheat transfer is the study of heat transfer in biology. There are several sources of heat in biology including external ones such as surgical applications of heat, or internal ones like cancer tissue. Blood flow networks affect both types of processes and are often generated during disease phenomenon through a process called angiogenesis. This affects the properties of the tissue. Several attempts were made to model flow and heat transfer within various tissues through physical measurements or computer simulations. Both types of models used bioheat transfer models do not consider the geometry of vasculature at its most fundamental level. The …

How Can Courts–Practically For Free–Help Parties Prepare For Mediation Sessions?, John Lande

Journal of Dispute Resolution

Consider two hypothetical scenarios of mediations of a personal injury lawsuit. In one scenario, Kenji, the plaintiff, arrived at the mediation session feeling anxious because his attorney hadn’t told him much about the process and he didn’t know what to expect. He didn’t understand the factual and legal issues, how the mediation would unfold, or how he might participate in the process. He felt demoralized because he didn’t know enough to feel confident and assertive about making decisions in his case. In the mediation session, he spent a lot of time alternately hearing the mediator explain why he couldn’t get …

Ombuds Programs: How Alternative Dispute Resolution Improves The Lives Of Native Long-Term Care Residents, Brett Newberry

Journal of Dispute Resolution

Many Americans will spend at least some time in a long-term care facility. During their stay, every resident should receive competent care and maintain the highest possible quality of life. Long-term care ombuds programs exist to help achieve these goals. Long-term care ombuds (“LTCOs”) advocate for individuals living in long-term care facilities and for improvements to the long-term care system. Tribal long-term care ombuds are a subset of LTCOs who connect with and advocate for Native residents living in long-term care facilities. LTCOs help facilitate dispute resolution between residents, facilities, and family members, and work to improve resident care. The …

Language Discrimination Using Bilingual Picture Exchange For Culturally And Linguistically Diverse Children With Autism Spectrum Disorder, River Waits

LSU Doctoral Dissertations

Recent research suggests that one-fifth of United States households use non-English languages in the home setting. Ethically and effectively supporting culturally and linguistically diverse (CLD) clients (e.g., autistic individuals) presents a growing challenge to practitioners of Applied Behavior Analysis (ABA). Though efforts to address the linguistic needs of the population have grown in recent years, current literature focuses on language selection for monolingual services and underemphasizes the need for bilingual service provision. Studies were conducted to (I) evaluate the efficacy of bilingual picture exchange (i.e., mand response) across items, speakers, and languages (i.e., English and Spanish) as well as (II) …

The Effect Of Season On Neuroendocrine Interactions In The Mare, Victoria N. Bailey

LSU Doctoral Dissertations

Reproductive seasonality in the mare involves various neuroendocrine modulations, and a series of experiments were conducted to investigate the anatomical and physiological roles of some of these seasonal modulators. The first experiment incorporated kisspeptin-10 (Kp10) into a treatment with estradiol benzoate (EB) and sulpiride, aiming to induce early cyclicity in seasonally anovulatory mares. Incorporation of Kp10 resulted in more mares ovulating earlier compared to mares receiving EB-sulpiride. Although plasma prolactin increased in both groups, concentrations were notably higher in Kp10-treated mares. Therefore, the subsequent experiment explored the anatomical interaction of kisspeptin and prolactin, aiming to determine whether kisspeptin may regulate …

Reliability Of The Cvms Method To Assess The Craniofacial Growth Spurt, Holly Ingersoll

Master's Theses (2009 -)

Objective: Cervical vertebral maturation staging (CVMS) is a popular method to determine the growth potential of a child utilizing the changing shapes of C2, C3, and C4. Staging is separated into six stages where the first two stages are before the growth spurt, the second two stages are during the growth spurt, and the last two stages are after the growth spurt. This method is highly practical because it is read on a lateral cephalogram, a radiograph taken by the orthodontist for diagnostic records. However, many clinicians question its reliability and validity as a test. The objective of this study …

Consensus Guidelines For The Monitoring And Management Of Metachromatic Leukodystrophy In The United States, Laura A Adang, Joshua L Bonkowsky, Jaap Jan Boelens, Eric Mallack, Rebecca Ahrens-Nicklas, John A Bernat, Annette Bley, Barbara Burton, Alejandra Darling, Florian Eichler, Erik Eklund, Lisa Emrick, Maria Escolar, Ali Fatemi, Jamie L Fraser, Amy Gaviglio, Stephanie Keller, Marc C Patterson, Paul Orchard, Jennifer Orthmann-Murphy, Jonathan D Santoro, Ludger Schöls, Caroline Sevin, Isha N Srivastava, Deepa Rajan, Jennifer P Rubin, Keith Van Haren, Melissa Wasserstein, Ayelet Zerem, Francesca Fumagalli, Lucia Laugwitz, Adeline Vanderver

Faculty, Staff and Students Publications

Metachromatic leukodystrophy (MLD) is a fatal, progressive neurodegenerative disorder caused by biallelic pathogenic mutations in the ARSA (Arylsulfatase A) gene. With the advent of presymptomatic diagnosis and the availability of therapies with a narrow window for intervention, it is critical to define a standardized approach to diagnosis, presymptomatic monitoring, and clinical care. To meet the needs of the MLD community, a panel of MLD experts was established to develop disease-specific guidelines based on healthcare resources in the United States. This group developed a consensus opinion for best-practice recommendations, as follows: (i) Diagnosis should include both genetic and biochemical testing; (ii) …

Artificial Intelligence To Classify Acquired Intestinal Injury In Preterm Neonates-A New Perspective, Alain Cuna, Muralidhar H Premkumar, Venkatesh Sampath

Faculty, Staff and Students Publications

No abstract provided.

Executive Summary: The Pediatric Extracorporeal Membrane Oxygenation Anticoagulation Collaborative (Peace) Consensus Conference, Peta M A Alexander, Melania M Bembea, Katherine Cashen, Ira M Cheifetz, Heidi J Dalton, Adam S Himebauch, Oliver Karam, Katie M Moynihan, Marianne E Nellis, Caroline Ozment, Lakshmi Raman, Natalie E Rintoul, Ahmed S Said, Arun Saini, Marie E Steiner, Ravi R Thiagarajan, Kevin Watt, Ariane Willems, Nicole D Zantek, Ryan P Barbaro, Katherine Steffen, Adam M Vogel, Christopher Almond, Marc M Anders, Gail M Annich, Leonardo R Brandão, Wayne Chandler, Megan Delaney, Robert Digeronimo, Sitaram Emani, Samir K Gadepalli, Alejandro V Garcia, Bereketeab Haileselassie, Robert Hyslop, Martin C J Kneyber, Lisa Baumann Kreuziger, Jennifer Le, Laura Loftis, Ali B V Mcmichael, D Michael Mcmullan, Paul Monagle, Kathleen Nicol, Matthew L Paden, Jason Patregnani, John Priest, Leslie Raffini, Lindsay M Ryerson, Steven R Sloan, Jun Teruya, Andrew R Yates, Alison Gehred, Elizabeth Lyman, Jennifer A Muszynski

Faculty, Staff and Students Publications

OBJECTIVES: To present recommendations and consensus statements with supporting literature for the clinical management of neonates and children supported with extracorporeal membrane oxygenation (ECMO) from the Pediatric ECMO Anticoagulation CollaborativE (PEACE) consensus conference.

DATA SOURCES: Systematic review was performed using PubMed, Embase, and Cochrane Library (CENTRAL) databases from January 1988 to May 2021, followed by serial meetings of international, interprofessional experts in the management ECMO for critically ill children.

STUDY SELECTION: The management of ECMO anticoagulation for critically ill children.

DATA EXTRACTION: Within each of eight subgroup, two authors reviewed all citations independently, with a third independent reviewer resolving any …

The Role Of Transoral Incisionless Fundoplication (Tif) In The Management Of Gastroesophageal Reflux Disease (Gerd) Following Peroral Endoscopic Myotomy (Poem): A Pilot, Prospective, Patient-Driven Study, Fares Ayoub, Tara Keihanian, Noor Zabad, Salmaan Jawaid, Kalpesh Patel, Mohamed O Othman

Faculty, Staff and Students Publications

BACKGROUND: Gastroesophageal reflux (GERD) is a concern after peroral endoscopic myotomy (POEM). Transoral incisionless fundoplication (TIF) has been recently described as a possible therapy for post-POEM GERD in case series.

METHODS: We prospectively enrolled patients undergoing POEM who agreed to participate in objective post-procedure GERD evaluation. Patients with objective evidence of GERD and suitable anatomy were offered TIF vs. proton pump inhibitor (PPI) only. Patients who underwent TIF were compared to those on PPI-only therapy after follow-up.

RESULTS: Of 21 enrolled POEM patients with objective testing, GERD was found in 11 (52%). Of those eligible for TIF, 4 (40%) opted …

Homozygous Missense Variants In Ykt6 Result In Loss Of Function And Are Associated With Developmental Delay, With Or Without Severe Infantile Liver Disease And Risk For Hepatocellular Carcinoma, Mengqi Ma, Mythily Ganapathi, Yiming Zheng, Kai-Li Tan, Oguz Kanca, Kevin E Bove, Norma Quintanilla, Sebnem O Sag, Sehime G Temel, Charles A Leduc, Amanda J Mcpartland, Elaine M Pereira, Yufeng Shen, Jacob Hagen, Christie P Thomas, Nhu Thao Nguyen Galván, Xueyang Pan, Shenzhao Lu, Jill A Rosenfeld, Daniel G Calame, Michael F Wangler, James R Lupski, Davut Pehlivan, Paula M Hertel, Wendy K Chung, Hugo J Bellen

Faculty, Staff and Students Publications

PURPOSE: YKT6 plays important roles in multiple intracellular vesicle trafficking events but has not been associated with Mendelian diseases.

METHODS: We report 3 unrelated individuals with rare homozygous missense variants in YKT6 who exhibited neurological disease with or without a progressive infantile liver disease. We modeled the variants in Drosophila. We generated wild-type and variant genomic rescue constructs of the fly ortholog dYkt6 and compared their ability in rescuing the loss-of-function phenotypes in mutant flies. We also generated a dYkt6

RESULTS: Two individuals are homozygous for YKT6 [NM_006555.3:c.554A>G p.(Tyr185Cys)] and exhibited normal prenatal course followed by failure to thrive, …

Progressive Thrombocytopenia, Splenomegaly, And Abnormal Tone In An Infant With Growth Faltering, Gal Barak, Gail Demmler-Harrison, Linda Rossetti, Venée N Tubman, Ameya S Walimbe, Rathi Asaithambi

Faculty, Staff and Students Publications

A 4-month-old full-term female presented with growth faltering associated with progressive feeding difficulty, rash, abdominal distension, and developmental delays. She was found to have disconjugate gaze, abnormal visual tracking, mixed tone, bruising, and splenomegaly on examination. Initial workup was notable for thrombocytopenia and positive cytomegalovirus (CMV) immunoglobulin G and immunoglobulin M antibodies. She initially presented to the infectious diseases CMV clinic, where she was noted to have severe malnutrition, prompting referral to the emergency department for hospital admission to optimize nutrition with nasogastric tube feeding and facilitate additional evaluation. An active CMV infection with viruria and viremia was confirmed, but …

Liver Disease And Transplantation In Telomere Biology Disorders: An International Multicenter Cohort, Yunzu Michele Wang, Batul Kaj-Carbaidwala, Adam Lane, Suneet Agarwal, Fabian Beier, Alison Bertuch, Kristin A Borovsky, Steven K Brennan, Rodrigo T Calado, Luiz Fernando B Catto, Carlo Dufour, Christen L Ebens, Francesca Fioredda, Neelam Giri, Nicholas Gloude, Frederick Goldman, Paula M Hertel, Ryan Himes, Sioban B Keel, Divya T Koura, Christian P Kratz, Sakil Kulkarni, Iris Liou, Taizo A Nakano, Silvia Nastasio, Marena R Niewisch, Daniel D Penrice, Ghadir S Sasa, Sharon A Savage, Douglas A Simonetto, David S Ziegler, Alexander G Miethke, Kasiani C Myers

Faculty, Staff and Students Publications

Background: Patients with telomere biology disorders (TBD) develop hepatic disease, including hepatitis, cirrhosis, and hepatopulmonary syndrome. No specific treatment exists for TBD-related liver disease, and the role of liver transplantation (LT) remains controversial. Our study objectives were to describe the clinical characteristics, management, and outcomes in patients with TBD-related liver disease, and their LT outcomes.

Methods: Data from 83 patients with TBD-associated liver disease were obtained from 17 participating centers in the Clinical Care Consortium of Telomere-Associated Ailments and by self-report for our retrospective, multicenter, international cohort study.

Results: Group A ("Advanced") included 40 patients with advanced liver disease. Of …