Effect of branched-chain amino acid supplementation and exercise on quadriceps muscle quantity and quality in patients with cirrhosis as assessed by ultrasonography: A randomized controlled trial.

BACKGROUND/AIM: Sarcopenia and myosteatosis are common in patients with cirrhosis. The study aimed to evaluate efficacy of ultrasound to monitor muscle status during branched-chain amino acid (BCAA) supplementation and/or muscle exercise interventional approaches. PATIENTS AND METHODS: A randomized controlled study, included 220 liver cirrhosis patients with Child- Pugh B and C, randomized into a control group (55 patients) received only the standard care, and interventional groups (165 patients) equally distributed into three subgroups, in addition to standard care, they received BCAA, programmed exercise, or BCAA and programmed exercise. At baseline and after 28 days, all participants were subjected to ultrasound-measured quadriceps muscle thickness and echo-intensity, muscle strength using handgrip, performance using short physical performance battery (SPPB), Model for End-Stage Liver Disease (MELD) score and nutritional assessment using 7- point Subjective Global Assessment Score (SGA) and laboratory assessment. RESULTS: All interventional groups showed a significant improvement in the ultrasound detected quadriceps muscle thickness (p = 0.001) and echo intensity, in addition to muscle strength, muscle performance, and SGA. Hematological parameters (hemoglobin and platelet count), biochemical parameters (ALT, AST, bilirubin, creatinine, urea and INR) and MELD score were also improved in the interventional groups. In Child-Pugh B patients BCAA combined with exercise showed an add-on effect. CONCLUSION: BCAA supplements, programed muscle exercise and both are useful interventional methods in improving muscle quality and quantity in cirrhosis patients, which can be monitored by ultrasound. The best results can be achieved by combined intervention in Child-Pugh B, while in Child-Pugh C single intervention may lead to an acceptable improvement. The trial was registered retrospectively in the Clinical Trials Registry (registration number NCT06088550).

Ultrasonographic assessment of muscle layer thickness and its relation to patient outcome in a medical intensive care unit.

AIM: Low skeletal muscle mass in ICU patients is associated with poor clinical outcome. Ultrasonography is a noninvasive method that can measure muscle thickness at the bedside. We aimed at studying the relation of the ultrasonography measured muscle layer thickness (MLT) at time of ICU admission with the patients' outcome namely mortality, duration of mechanical ventilation (MV) and ICU length of stay (LOS). In addition to define the best cut-off values that can predict mortality in medical ICU patients. METHOD: this observational prospective study was conducted on 454 adult critically ill patients admitted to the medical ICU of a university hospital. At the time of admission, MLT of the anterior mid-arm and lower 1/3 thigh were assessed using ultrasonography with and without transducer compression. The clinical scores for assessment of disease severity; Acute Physiology and Chronic Health Evaluation score (APACHE-II) and Sequential Organ Failure Assessment score (SOFA) in addition to nutrition risk; modified Nutrition Risk in Critically ill score (mNUTRIC) were estimated for all patients. ICU LOS, duration on MV and mortality were reported. RESULTS: The mean age of our patients was 51 years ± 19. The ICU mortality rate was 36.56%. The baseline MLT was negatively associated with APACHE-II, SOFA and NUTRIC scores but not with duration of MV or ICU-LOS. The non-survivors had lower values of baseline MLT. A cut-off value of 0.895 cm (AUC: 0.649, 95% CI of 0.595-0.703) using the mid-arms as a reference point with maximum probe compression showed the highest sensitivity (90%) to predict mortality compared to other techniques however with low specificity (22%). CONCLUSION: the baseline ultrasonography measured mid-arm MLT is a sensitive risk assessment tool that can reflect disease severity and predict ICU mortality.

Eccentric Versus Concentric Exercises in Patients With Rheumatoid Arthritis and Rotator Cuff Tendinopathy: A Randomized Comparative Study.

OBJECTIVE: To determine the efficacy of concentric vs. eccentric exercise in improving shoulder function, pain, and tendon characteristics for patients with rheumatoid arthritis and rotator cuff tendinopathy. METHODS: Forty patients with rheumatoid arthritis and rotator cuff tendinopathy were divided into either concentric or eccentric exercise groups, with 20 patients in each group. Patients received 12 sessions at a pace of 3 sessions per week. Shoulder Pain and Disability Index (SPADI), the visual analogue scale (VAS), supraspinatus and subscapularis thickness, echo pixels, and the Disease Activity Score-28-erythrocyte sedimentation rate (DAS-28-ESR) were assessed at baseline and post-treatment. RESULTS: There was a significant difference between the concentric and eccentric groups regarding SPADI and VAS scores in favor of the eccentric group. However, there was no significant difference between the two groups regarding tendon thickness, echo pixels, or DAS-28-ESR. CONCLUSION: Eccentric exercises were more effective than concentric exercises in improving shoulder function and pain intensity. However, neither of the two types of exercises was superior in improving tendon characteristics or disease activity.

IgG4 related pericardium and lung disease in pediatric patient complicated with fatal massive hemoptysis: a case report and review of literature.

BACKGROUND: IgG4-related disease (IgG4-RD) is a progressive and sometimes fatal disease that rarely affects pediatric age group. It may affect the orbits, lacrimal and salivary glands, pancreas, kidneys, peritoneum and other organs. Lung and pleura are not commonly reported in IgG4-RD. We here present a rare case of pediatric IgG4-RD with rare involvement of pericardium, pleura and lungs. CASE PRESENTATION: A 13-year-old girl presented with intrathoracic IgG4-RD with pleuropericardial involvement. She showed initial improvement on prednisolone. Azathioprine and then mycophenolate failed to control relapses during steroid tapering. Her last relapse was treated by rituximab however, the patient developed acute fatal massive hemoptysis. CONCLUSIONS: Pediatric IgG4-RD is a rare entity with pericardio-pulmonary affection as the rare of the rare. Usual treatment of prednisolone and steroid sparing agents should be used, with rituximab used as a rescue therapy, but fatal complications may occur.

Measuring Serum Sclerostin in Egyptian Patients With Systemic Lupus Erythematosus and Evaluating Its Effect on Disease Activity: A Case-Control Study.

OBJECTIVE: Sclerostin is an osteocyte-secreted protein that downregulates bone formation by blocking the Wnt/ß-catenin signaling pathway. Sclerostin can be induced by inflammation, and high levels have been reported in patients with proteinuria and renal impairment. Studies evaluating the role of sclerostin in systemic lupus erythematosus (SLE) patients are scarce. This study aims to measure serum sclerostin in SLE patients and correlate its level with bone biomarkers and disease activity, particularly in lupus nephritis and arthritis. Finally, we evaluated factors that may predict sclerostin concentrations. METHODS: This cross-sectional, case-control study was conducted from May 2017 to April 2018. Serum sclerostin was measured by enzyme-linked immunosorbent assay in 100 SLE patients, including 50 patients with current lupus nephritis and 27 patients with current arthritis, as well as in 50 healthy controls. Correlation analysis of serum sclerostin with demography, bone biomarkers, and disease activity in SLE patients was carried out. RESULTS: Sclerostin levels were significantly elevated in SLE patients, particularly those with lupus nephritis, compared with healthy controls. Higher levels were identified in patients without arthritis compared with those with; however, the former group had more proteinuria and renal impairment. Significant correlations were observed between sclerostin levels and serum creatinine, proteinuria, consumed C3 and C4 complement, and corrected Ca. Using multiple linear regression, proteinuria was the only significant predictor for serum sclerostin in SLE patients. CONCLUSIONS: This study is the first to report that serum sclerostin is associated with proteinuria in SLE patients and could be used as a valuable biomarker for lupus nephritis.

Evaluating the role of serum sclerostin as an indicator of activity and damage in Egyptian patients with rheumatoid arthritis: university hospital experience.

OBJECTIVE: Sclerostin is an osteocyte-derived glycoprotein which inhibits the canonical Wnt pathway essential for osteoblastic activity decreasing bone formation. Its potential role in rheumatoid arthritis (RA) pathogenesis was highlighted by experimental studies. Here we measured the serum sclerostin in RA patients and evaluated its relationship with disease activity and damage. METHODS: One hundred RA patients and 80 age and sex-matched healthy controls were enrolled in the study. Bone biomarkers were evaluated for all participants including total calcium, phosphorus, alkaline phosphatase, 25-hydroxy vitamin D, and intact parathyroid hormone, in addition to fibroblast growth factor-23 (FGF23) and serum sclerostin. For RA patients, carotid intima-media thickness, brachial artery flow dilatation, and musculoskeletal ultrasonography using ultrasonography-7 joint score were done, and DAS28-ESR was calculated. RESULTS: Median serum sclerostin in our patients was 186.5 ± 22.7 pg/ml which was significantly higher than in controls 60.6 ± 7.1 pg/ml (p < 0.002). Serum sclerostin showed no correlation with disease activity, bone erosions, carotid intima-media thickness, brachial flow dilatation, and the examined bone biomarkers. However, it had a strong correlation with FGF23 (r coefficient 0.988, p < 0.000). CONCLUSION: Although serum sclerostin was elevated in RA patients, it could not be used as a prognostic marker for disease activity, bone erosions or atherosclerosis.Key Points• Serum sclerostin may not reflect changes in the joint microenvironment being not correlated with ultrasonography-detected synovitis or erosions.• Serum sclerostin was elevated in RA patients irrespective to their age or gender.• The positive correlation with FGF23 may provide evidence for sclerostin contribution in bone demineralization in RA patients.