BACKGROUND:Sarcopenia is associated with increased mortality in patients awaiting liver transplant (LT), but the impact of sarcopenia on cirrhosis progression remains under-evaluated. AIMS:We aimed to explore the relationship between sarcopenia and the risk of cirrhosis progression, mortality and hospitalisation in compensated and decompensated cirrhosis. METHODS:Sarcopenia was measured using the skeletal muscle index (SMI) obtained from abdominal CT imaging at the 3rd lumbar vertebra (L3). Progression of cirrhosis was defined by an increase in clinical stage from baseline using the D'Amico classification. Factors associated with progression or death, death alone and hospitalisation were evaluated with univariate and multivariate Cox regression models. RESULTS:One thousand six hundred and twenty-four adult patients with cirrhosis from five centres across Europe, North America and Asia were included. Mean age was 54 ± 9 years, 26% of patients were female, 31% had compensated disease and mean MELD-Na was 15 ± 7. With separate analyses for compensated and decompensated cirrhosis, sarcopenia was independently associated with the risk of progression or death (aHR 1.43, 95% CI 1.14-1.79, p = 0.002) and death alone (aHR 1.55, 95% CI 1.19-2.03, p = 0.001) only in decompensated cirrhosis. Sarcopenia was independently associated with unplanned hospitalisation in both compensated (aHR 1.70, 95% CI 1-15-2.51, p = 0.010) and decompensated cirrhosis (aHR 1.32, 95% CI 1.08-1.63, p = 0.007). CONCLUSIONS:Sarcopenia was independently associated with cirrhosis progression or death and death alone in decompensated cirrhosis. Future studies are needed to evaluate the possibility of slowing cirrhosis disease progression by reversing or preventing sarcopenia.

Background and Aims Poor body composition is associated with impaired outcomes in patients with chronic liver disease (CLD), and various assessment tools are used. The aim of this study is to gain insight into the clinical practice of nutritional assessment in patients with CLD. Methods A semi-structured online survey on nutritional assessment tools for measuring body composition in patients with CLD was conducted among hepatologists and dietitians, mainly from hospitals between April 2023 and May 2023. Results A total of 45 eligible surveys were included in this study (dieticians N=35, hepatologists N=10). All dieticians had at least one nutritional assessment tool available. Bio-electrical impedance and hand-grip strength were the most available and used. The most important reasons for assessment were diagnosis of nutritional status, evaluation of nutritional intervention and for assessing risk of mortality. A proportion of 49% of the dieticians and 60% of the hepatologists respectively were familiar with the ‘European society of parenteral and enteral nutrition Guideline Clinical Nutrition in Liver disease’. Only 13% of dieticians and 60% of the hepatologists knew the ‘European Association for the Study of the Liver Clinical Practice Guidelines on nutrition in chronic liver disease’ and only eight dieticians and one hepatologist used a protocol for nutritional assessment in patients with CLD. Conclusion The importance of measuring body composition in patients with CLD for diagnosing and monitoring nutritional status and for assessing risk of mortality is well-known by dieticians and most hepatologists. However, implementation of the current guidelines is substandard and should be improved.

Neuromuscular disorders (NMD) require complex multidisciplinary care. There is an urgent need to understand the natural history, factors that influence its variability, and to develop biomarkers. Standardizing assessments in outpatient clinics provides an opportunity to combine care and research while minimizing patients’ efforts and optimizing the use of data. We present the organization of the Leiden University Medical Center NMD pediatric and adult clinics in which yearly functional assessments, cardiological, pulmonological and bone health evaluations according to the standards of care are combined with the possibility to conduct quantitative muscle MRI for specific natural history studies, and the collection of biomaterials in the morning. This is followed by a multidisciplinary team meeting and individual consultations with medical specialists in the afternoon. Data are stored in a standardized form in the electronic medical records, and transcribed into a clinical letter containing treatment recommendations that is sent to the family and local healthcare professionals. With consent from patients and families, the same data are used for Dutch Dystrophinopathy Database, a national disease registry open to all Dutch patients and female carriers combining clinician and patient reported data, and the Duchenne and Becker national biobank, a collaboration between the Dutch University Medical Centers. To facilitate interoperability the Framework for Information Specification, Modelling, and Architecture (FISMA) governs the design of our electronic health record system and these databases. The integration of clinical care and research minimizes redundant data entry and enhances data accessibility for both clinical and research purposes. As a result, relevant information can also be seamlessly incorporated into patient communications, while at the same time allowing long term natural history assessment and biomarker development.

BackgroundLiver transplantation is the only curative therapy for end-stage liver disease (ESLD). Sarcopenia is often defined as the loss of muscle quantity (skeletal muscle index [SMI]), but muscle attenuation (MA), a surrogate marker of muscle quality, is also decreased in ESLD. We assessed pre-liver transplant SMI and MA and their association with post-transplant mortality, complications, and length of intensive care unit (ICU) and hospital stay. MethodsIn 169 consecutive patients with ESLD who underwent a liver transplantation between 2007 and 2014, SMI and MA were measured on computed tomography scans at time of placement on the waiting list for liver transplantation. The primary outcome of interest was 1-year post-transplant mortality. Secondary posttransplantation outcomes of interest were complications within 30 days and length of stay in the ICU > 3 days and in the hospital >3 weeks. Logistic and Cox regression analyses were performed. ResultsMA was associated with 1-year post-transplant mortality rate (hazard ratio=0.656, 95% CI=0.464-0.921, P = 0.015). The highest quartile of SMI had a lower odds for the total length of stay in the hospital lasting >3 weeks (odds ratio=0.211, 95% CI=0.061-0.733, P = 0.014). MA was associated with a prolonged ICU stay; this was, however, not statistically significant after adjustment for age, sex, and Model for ESLD score. ConclusionLower MA is associated with a longer length of ICU stay and 1-year mortality after liver transplantation, whereas low SMI was associated with a total length of hospital stay.

Background: Physical fitness is an important modifiable factor related to quality of life. Sarcopenia and myosteatosis are associated with morbidity and mortality in patients with end-stage liver disease (ESLD). However, their relationship with physical fitness has not been established yet. Therefore, the main purpose of this study was to investigate the association between both low skeletal muscle index (SMI) and myosteatosis with physical fitness in patients with ESLD.Methods: In this retrospective cross-sectional cohort study, a cohort of patients with ESLD who were evaluated for liver transplantation (LT) was included. Physical fitness was reflected by cardiorespiratory fitness (CRF) and skeletal muscle strength, as measured by the 6-min walking distance (6MWD) and handgrip strength (HGS), respectively. Both were included in routine LT evaluation. Skeletal Muscle Index (SMI) and Muscle Radiation Attenuation (MRA) were evaluated based on the routine abdominal computed tomography. Linear and logistic regression analyses were performed.Results: Out of the 130 patients 94 (72%) were male, mean age was 56 +/- 11 years. Myosteatosis was significantly associated with low 6MWD as percentage of predicted (b =-12.815 (CI-24.608 to-1.022, p-value 0.034)) as well as with low absolute 6MWD (<250 m) (OR 3.405 (CI 1.134-10.220, p-value 0.029)). No association was found between SMI and/or myosteatosis with HGS, or between SMI and 6MWD.Conclusion: In contrast to SMI, myosteatosis is associated with low CRF. Neither low SMI nor myo-steatosis was associated with skeletal muscle strength. Therefore physical exercise training might be especially beneficial for LT candidates with myosteatosis.(c) 2023 The Author(s). Published by Elsevier Ltd on behalf of European Society for Clinical Nutrition and Metabolism. This is an open access article under the CC BY license (http://creativecommons.org/licenses/ by/4.0/).

We aimed to investigate BMI-z course in patients with Duchenne muscular dystrophy (DMD) during transition to loss of ambulation, and to explore the contribution of caloric intake and corticosteroid use. A retrospective multicenter longitudinal study was conducted. First, analyses of characteristics at first visit were carried out. Second, discontinuous change models were fitted to explore associations between BMI-z, loss of ambulation, caloric intake and corticosteroid use. 790 visits of 159 patients were collected. Cross sectional first visit analyses showed the presence of overweight and obesity was 44% in the ambulant group and 51% in the non-ambulant group. In the non-ambulatory group, exceeding the recommended caloric intake was associated with higher BMI-z scores (r 0.36, p = 0.04). Patients who were using corticosteroids had significantly higher BMI-z scores compared with patients not using corticosteroids (1.06 and 0.51 respectively, p = 0.02). Longitudinal analyses on patients ambulant at first visit showed an increase in BMI-z score during transition to the non-ambulatory phase. Caloric intake and corticosteroid use were not associated with BMI-z. Transition to the non-ambulatory phase may be crucial in the development of excessive weight gain. Early measures - starting before this time frame - may contribute to reduce development of obesity. (c) 2022 The Author(s). Published by Elsevier B.V.

BACKGROUND:Overweight is a common problem in Duchenne muscular dystrophy (DMD) and is associated with reduced mobility and quality of life. The influence of nutritional intake on (over)weight is unclear.OBJECTIVE:To investigate weight and energy and macronutrients intake compared to age-specific requirements in DMD patients (4-18 years).METHODS:We assessed weight and body mass index (BMI) and the amount of energy (kcal/day) and macronutrients based on self-reported nutrition diaries. Nutritional intake was compared to requirements for 3 age-groups according to the Dutch Healthy Diet Guideline (4-8/9-13/14-18 years) using a student's t-test, and relations with age and BMI were investigated by means of Pearson's correlations.RESULTS:Forty-eight patients participated, 22 ambulatory, median age 10.8 years. The majority used corticosteroids (N = 41). Overweight (BMI z-score > 2.07) was present in 19 patients; 6% (4-8 years), 73% (9-13 years) and 47% (14-18 years). Overweight was more common in non-ambulatory (61.6%) than ambulatory patients (13.6%). Patients aged 4-8 received 290 kcal/day more than required (p < 0.001). Patients aged 9-13 received 349 kcal/day (p = 0.005) less than required. Overall, intake of fibre, nuts, meat/fish/eggs/legumes and dairy was lower than recommended (p < 0.05). The difference between energy intake versus requirement correlated moderately to age (r = -0.549, p < 0.001) and BMI (r = -0.562, p < 0.001).CONCLUSIONS:Overweight was found especially in patients aged 9-18 even though they received less energy than required. Younger patients (4-8) had good weight but consumed more energy than required. All patients did not consume enough fibre, nuts, meat/fish/eggs/legumes and dairy. Limiting energy and increasing fibre/protein intake at an early age may prevent overweight at a later age.

Background and aims: Malnutrition is highly prevalent in patients with end-stage liver disease (ESLD) and associated with impaired clinical outcome. Previous studies focused on one component of body composition and not in combination with nutritional intake, while both are components of the nutritional status. We aimed to evaluate the most important risk factors regarding body composition (muscle mass, muscle quality and fat mass) and nutritional intake (energy and protein intake) for waiting list mortality in patients with ESLD awaiting liver transplantation (LTx). Methods: Consecutive patients with ESLD listed for LTx between 2007 and 2014 were investigated. Muscle mass quantity (Skeletal Muscle Mass Index, SMI), and muscle quality (Muscle Attenuation, MA), and various body fat compartments were measured on computed tomography using SliceOmatic. Nutritional intake (e.g. energy and protein intake) was assessed. Multivariable stepwise forward Cox regression analysis was used for statistical analysis. Results: 261 Patients (mean age 54 years, 74.7% male) were included. Low SMI and MA were found to be statistically significant predictors of an increased risk for waiting list mortality in patients with ESLD, with a HR of 2.580 (95%CI 1.055-6.308) and HR of 9.124 (95%CI 2.871-28.970), respectively. No association between percentage adipose tissue, and protein and energy intake with waiting list mortality was found in this study. Conclusion: Both low muscle quantity and quality, and not nutritional intake, were independent risk factors for mortality in patients with ESLD. (C) 2021 The Author(s). Published by Elsevier Ltd on behalf of European Society for Clinical Nutrition and Metabolism.

Hepatoma Research is an open access journal and focuses on all topics related to hepatoma. The following articles are especially welcome: pathogenesis, clinical examination and early diagnosis of hepatoma, complications of hepatoma, and their preventions and treatments, etc.

BACKGROUND & AIMS:Frail patients with low model for end-stage liver disease (MELD) scores may be under-prioritised. Low skeletal muscle mass, namely sarcopenia, has been identified as a risk factor for waiting list mortality. A recent study proposed incorporating sarcopenia in the MELD score (MELD-Sarcopenia score). We aimed to investigate the association between sarcopenia and waiting list mortality, and to validate the MELD-Sarcopenia score (i.e. MELD + 10.35 * Sarcopenia). METHODS:We identified consecutive patients with cirrhosis listed for liver transplantation in the Eurotransplant registry between 2007-2014 and measured skeletal muscle mass on computed tomography. A competing risk analysis was used to compare survival of patients with and without sarcopenia, and concordance (c) indices were calculated to assess performance of the MELD and MELD-Sarcopenia score. We created a nomogram of the best predictive model. RESULTS:We included 585 patients with a median MELD score of 14 (interquartile range 9-19), of which 254 (43.4%) were identified as having sarcopenia. Median waiting list survival was shorter in patients with sarcopenia than those without (p <0.001). This effect was even more pronounced in patients with MELD ≤15. The discriminative performance of the MELD-Sarcopenia score (c-index 0.820) for three-month mortality was lower than MELD score alone (c-index 0.839). Apart from sarcopenia and MELD score, other predictive variables were occurrence of hepatic encephalopathy before listing and recipient age. A model including all these variables yielded a c-index of 0.851. CONCLUSIONS:Sarcopenia was associated with waiting list mortality in liver transplant candidates with cirrhosis, particularly in patients with lower MELD scores. The MELD-Sarcopenia score was successfully validated in this cohort. However, incorporating sarcopenia in the MELD score had limited added value in predicting waiting list mortality. LAY SUMMARY:In this study among patients with liver cirrhosis listed for liver transplantation, low skeletal muscle mass was associated with mortality on the waiting list, particularly in patients who were listed with low priority based on a low MELD score. However, adding these measurements to the currently used system for donor and organ allocation showed no added value.