Short Telomeres: Cause and Consequence in Liver Disease

Penrice, Daniel D.; Simonetto, Douglas A.

doi:10.1055/s-0040-1713007

Cited by 8 publications

(13 citation statements)

References 61 publications

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“…These rare, but increasingly recognized, short telomere syndromes often present with distinct liver disease phenotypes such as cryptogenic cirrhosis and nodular regenerative hyperplasia. (18)(19)(20) In this study, we sought to leverage cross-sectional data from the National Health and Nutrition Examination Survey (NHANES) to explore the relationship between telomeres and liver disease at the population level. These survey data have been used to examine associations between leukocyte TL, as a proxy for somatic TL, and a multitude of clinical states, including presumed NAFLD (21,22) ; however, broader expansion to include other liver diseases and to assess the contribution of TL to long-term survival in CLD remain unmet needs.…”

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confidence: 99%

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Inverse Association of Telomere Length With Liver Disease and Mortality in the US Population

Rattan¹,

Penrice²,

Ahn³

et al. 2021

Hepatol Commun

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147

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Physiologic aging leads to attrition of telomeres and replicative senescence. An acceleration of this process has been hypothesized in the progression of chronic liver disease. We sought to examine the association of telomere length (TL) with liver disease and its impact on mortality risk. A cohort of 7,072 adults with leukocyte TL measurements from the National Health and Nutrition Examination Survey 1999-2002 with mortality follow-up through 2015 was analyzed. Liver disease was defined by aminotransferase levels and classified into etiology-based and advanced fibrosis categories. Multivariable-adjusted linear regression models estimated effect sizes, with 95% confidence intervals (CIs), of the presence of liver disease on TL. Cox regression models evaluated associations between TL and all-cause mortality risk using adjusted hazard ratios (HRs). The cohort was representative of the US population with mean age 46.1 years and mean TL 5.79 kilobase pairs. No overall association between TL and liver disease was found; however, there was a significant negative association of TL and advanced liver fibrosis in individuals aged 65 and above. The liver disease cohort (HR 1.22, 95% CI 0.99-1.51) and those with metabolic syndrome (HR 1.26, 95% CI 0.96-1.67) had increased mortality risk with shorter TL. The relationship between TL and all-cause mortality was stronger in women (HR 1.51, 95% CI 1.02-2.23) and in non-Hispanic Whites (HR 1.37, 95% CI 1.02-1.84). Conclusion: Shortened leukocyte TL is independently associated with advanced liver disease at older ages, and with a higher risk of all-cause mortality in those with liver disease. These associations reaffirm the need to better understand the role of telomeres in the progression of liver disease. (Hepatology Communications 2021;0:1-12).

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confidence: 99%

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confidence: 99%

Inverse Association of Telomere Length With Liver Disease and Mortality in the US Population

Rattan¹,

Penrice²,

Ahn³

et al. 2021

Hepatol Commun

Self Cite

147

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“…Les manifestations hépatiques les plus fréquentes sont la cirrhose et la maladie vasculaire du foie, typiquement, l'hyperplasie nodulaire régénérative (HNR). 14 Ces atteintes s'associeront souvent à une hypertension portale, voire à un syndrome hépatopulmonaire (SHP). 18 Un STC doit donc être recherché chez tout patient présentant une cirrhose cryptogénique.…”

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Syndrome des télomères courts chez l’adulte : une entité rare qu’il faut savoir évoquer

Coukos

Daccord

Lazor

et al. 2022

Revue Médicale Suisse

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“…TBDs have been increasingly recognized as multisystem diseases of premature aging, 1 with manifestations ranging from the prototypical dyskeratosis congenita to varying mucocutaneous, pulmonary, and hepatic involvements. 2 – 6 …”

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confidence: 99%

“…TBDs have been increasingly recognized as multisystem diseases of premature aging, [1] with manifestations ranging from the prototypical dyskeratosis congenita to varying mucocutaneous, pulmonary, and hepatic involvements. [2][3][4][5][6] The exact prevalence and incidence of TBD remains unclear, and long-term data is lacking. This is likely in part due to under-diagnosis, given variable and often nonspecific clinical presentation.…”

Section: Introductionmentioning

confidence: 99%

Liver disease and transplantation in telomere biology disorders: An international multicenter cohort

Wang,

Kaj-Carbaidwala,

Lane

et al. 2024

Hepatology Communications

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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 these, 20 underwent LT (Group AT). Group M (“Mild”) included 43 patients not warranting LT evaluation, none of whom were felt to be medically unfit for liver transplantation. Supplemental oxygen requirement, pulmonary arteriovenous malformation, hepatopulmonary syndrome, and higher bilirubin and international normalized ratio values were associated with Group A. Other demographics, clinical manifestations, and laboratory findings were similar between groups. Six group A patients were declined for LT; 3 died on the waitlist. Median follow-up post-LT was 2.9 years (range 0.6–13.2 y). One-year survival post-LT was 73%. Median survival post-LT has not been reached. Group AT patients had improved survival by age compared to all nontransplant patients (log-rank test p = 0.02). Of 14 patients with pretransplant hypoxemia, 8 (57%) had improved oxygenation after transplant. Conclusions: LT recipients with TBD do not exhibit excessive posttransplant mortality, and LT improved respiratory status in 57%. A TBD diagnosis should not exclude LT consideration.

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Short Telomeres: Cause and Consequence in Liver Disease

Cited by 8 publications

References 61 publications

Inverse Association of Telomere Length With Liver Disease and Mortality in the US Population

Inverse Association of Telomere Length With Liver Disease and Mortality in the US Population

Syndrome des télomères courts chez l’adulte : une entité rare qu’il faut savoir évoquer

Liver disease and transplantation in telomere biology disorders: An international multicenter cohort

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