HMB Supplementation with 1.5 g of HMB taken twice daily. Supplementation will be provided for 12 weeks

Mannitol 1.5 g twice daily. Supplementation will be provided for 12 weeks

Sarcopenia

Cirrhosis, Liver

1.5 g of HMB will be provided for 12 weeks twice daily

BETA-HYDROXY-BETA-METHYLBUTYRATE (HMB)

Mannitol

Gastroenterology Department, Sapienza University of Rome

Beta-Hydroxy-Beta-Methylbutyrate Supplementation and Physical Activity in Liver Cirrhosis: a Controlled Trial

Increase of Fat Free Mass evaluated by BIA

Recovery of muscle mass function evaluated by Recovery of muscle mass function evaluated by Hand grip Test (HG)

Recovery of muscle mass function evaluated by 6 minute walk test-6MWT

evaluation of minimal hepatic encephalopathy

episodes of decompensation during th study

Manuela Merli

Associate Professor

University of Roma La Sapienza

patients do not know if they are placebo or HMB group. Investigators who perform measurement and patients visit do not know the group of patients

enriched Branched Chain Amino Acid nutrition supplement 3.6 g twice a day for 5 weeks then withdrawal nutrition supplement for 12 weeks

Participants were purposively sampled to receive one sachet enriched BCAA twice a day(7.2g) for 5 weeks.

Branched Chain Amino Acid

Nutritional Intervention for Sarcopenia

Associated Risk Factors and Effect of Nutritional Intervention for Sarcopenia and Constipation in Middle-Aged and Old People

Skeletal muscle mass index was measured by bioelectrical impedance analysis (BC-418, Tanita)

Grip strength was measured by Grip-D; TKK 5401

Chimei Medical Center

The Taiwanese Osteoporosis Association.

amino acid tracer injection for metabolism analysis The dose needed to be injected from the tracer element in the beginning of the study is a bolus of 16ml Amino acid tracer (non-radioactive).
it is a single bolus, no other elements or drug will be administered.

amino acid tracer injection for metabolism analysis The dose needed to be injected from the tracer element in the beginning of the study is a bolus of 16ml Amino acid tracer (non-radioactive) it is a single bolus, no other elements or drug will be administered.

Metabolism and Nutrition Disorder

the intervention includes injecting stable amino acid tracers in order to evaluate differences in processing of amino acid in critically ill patients compared to a normal population.
after injection 5 ml of blood will be drawn at time 0, 2, 5, 10, 15, 20, 30, 40, 50, 60, 90, 120, 150, 180 minutes from the start of the study, a total of 14 blood samples (70 cc).

amino acid tracer injection for metabolism analysis

Rabin medical center

Amino Acid Metabolism in Fed Surgical Critically Ill Patients

Twenty Four Hours Amino Acid Metabolism in Fed Surgical Critically Ill Patients: a Stable Isotope Tracer Study.

In the study stable isotopes of amino acids will be injected, and afterward the amount of each amino acid will be checked in order to assess degradation and metabolism. Composite measure.

Pierre Singer

Professor

Rabin Medical Center

Texas A&M University

Cancer in Children.

Hearing Loss

study of mutations of metabolic enzymes and membrane transport genes, which will be performed by sequencing.

Genetic study

study of mutations of the mitochondrial gene MT-RN1, which will be performed by sequencing.

Genetic study MT-RN1

Institut Curie

Cancer and Hearing Loss Related in Children

Cancer and Deafness Associated With the Use of Platinum Derivatives in Children

Assistance Publique - Hôpitaux de Paris

Individuals randomized to this group received phenylbutyrate (500 mg/kg/day in patients weighing less than 20kg and 10 g/m2/day in larger patients in divided doses per day) for 14 days (week 1 and week 2) and then crossed over to receive placebo powder in identical doses for the next 14 days (Week 3 and week 4)

Phenylbutyrate First and Placebo Second

Individuals randomized to this group received first received placebo powder for 14 days (week 1 and week 2) and then were crossed over to receive phenylbutyrate (500 mg/kg/day in patients weighing less than 20kg and 10 g/m2/day in larger patients in 3 divided doses per day) for 14 days (week 3 and week 4) week 4).

Placebo First and Phenylbutyrate Second

Total

Sex: Female, Male

Race/Ethnicity, Customized

Region of Enrollment

Total Leucine exposure over 24 hours was calculated by serial blood draws at times 0, 2, 4, 8, 12, 16, 20, and 24 hours

Cumulative results when individuals were treated with phenylbutyrate

Phenylbutyrate

Cumulative results from when individuals were treated with placebo

Placebo First and Phenylbutyrate Second Group

0-24 Hour AUC Leucine (Samples Collected at 0, 2, 4, 8, 12, 16, 20, and 24 Hours)

Maximal leucine concentration in 0-24 hours

Cumulative results when individuals were treated with placebo

Placebo

Leucine CMax 0-24 Hours

Individuals randomized to this group received phenylbutyrate powder (500 mg/kg/day in patients weighing less than 20kg and 10 g/m2/day in larger patients in 3 divided doses per day) for 14 days (week 1 and week 2) and then crossed over to receive placebo powder in identical doses for the next 14 days (Week 3 and week 4)

Phenylbutyrate First and Placebo Second Group

Overall Study

Alyssa Tran, BS

Study Drug
Phenylbutyrate: Dosage of phenylbutyrate powder will be 500 mg/kg/day in patients weighing less than 20kg and 10 g/m2/day in larger patients in four divided doses per day, the standard UCD dose studied in our preliminary studies, for 14 days.

Placebo powder
Placebo powder: Dosage of inactive placebo powder will be 500 mg/kg/day in patients weighing less than 20kg and 10 g/m2/day in larger patients in four divided doses per day for 14 days. Subjects will receive the same amount of powder for each arm of the study.

Inactive Powder

Maple Syrup Urine Disease

Dosage of phenylbutyrate powder will be 500 mg/kg/day in patients weighing less than 20kg and 10 g/m2/day in larger patients in four divided doses per day, the standard UCD dose studied in our preliminary studies, for 14 days.

Dosage of inactive placebo powder will be 500 mg/kg/day in patients weighing less than 20kg and 10 g/m2/day in larger patients in four divided doses per day for 14 days. Subjects will receive the same amount of powder for each arm of the study.

isoleucine/atrofi

Beta-Hydroxy-Beta-Methylbutyrate Supplementation and Physical Activity in Liver Cirrhosis: a Controlled Trial

Nutritional Intervention for Sarcopenia

Amino Acid Metabolism in Fed Surgical Critically Ill Patients

Cancer and Hearing Loss Related in Children

Phenylbutyrate Therapy for Maple Syrup Urine Disease

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