Abnormal Body Composition Research Articles

Sarcopenia as a Prognostic Index of Nutritional Status in Concurrent Cirrhosis and Hepatocellular Carcinoma

Abnormal body composition such as severe skeletal muscle depletion or sarcopenia has emerged as an independent predictor of clinical outcomes in a variety of clinical conditions. This study is the first study to report the frequency and prognostic significance of sarcopenia as a marker of nutritional status in patients with hepatocellular carcinoma (HCC). We analyzed 116 patients with HCC who were consecutively evaluated for liver transplant. Skeletal muscle cross-sectional area was measured by CT. Sarcopenia was defined using previously established cutpoints. Ninety-eight patients were males (85%), and the mean age was 58±6 years. Sarcopenia was present in 35 patients (30%). By univariate Cox analysis, male sex (HR, 3.84; P=0.02), lumbar skeletal muscle index (HR, 0.97; P=0.04), INR (HR, 8.18; P<0.001), MELD score (HR, 1.19; P<0.001), Child-Pugh (HR, 3.95; P<0.001), serum sodium (HR, 0.84; P<0.001), TNM stage (HR, 2.59; P<0.001), treatment type (HR, 0.53; P<0.001), and sarcopenia (HR, 2.27; P=0.004) were associated with increased risks of mortality. By multivariate Cox regression analysis, only MELD score (HR, 1.08; P=0.04), Child-Pugh (HR, 2.14; P=0.005), sodium (HR, 0.89; P=0.01), TNM stage (HR, 1.92; P<0.001), and sarcopenia (HR, 2.04; P=0.02) were independently associated with mortality. Median survival for sarcopenic patients was 16±6 versus 28±3 months in nonsarcopenic (P=0.003). Sarcopenia is present in almost one third of patients with HCC, and constitutes a strong and independent risk factor for mortality. Our results highlight the importance of body composition assessment in clinical practice.

Cardiometabolic risk in overweight subjects with or without relative fat-free mass deficiency: The Strong Heart Study

Background and aimSarcopenia is a condition mainly due to loss of fat-free mass (FFM) in elderly individuals. RFFMD, however, is also frequent in obese subjects due to abnormal body composition. Objective of this study was to evaluate the impact of relative fat-free mass deficiency (RFFMD) on cardiometabolic (CM) risk in obese normoglycemic individuals. Methods and resultsOverweight/obese American Indians from the Strong Heart Study population, without diabetes and with FBG ≤110 mg/dL and with GFR >60 mg/mL/1.73 m2 were selected for this analysis (n = 742). RFFMD was defined on the basis of a multivariable equation previously reported. Fasting glucose and 2 h-OGTT were measured together with urine albumin/creatinine excretion, laboratory and anthropometric parameters. In addition to lower FFM and greater adipose mass, participants with RFFMD had higher body mass index, waist circumference, C-reactive protein, fibrinogen, insulin resistance and urinary albumin/creatinine than participants with normal FFM (all p < 0.001); they also had a greater prevalence of hypertension, impaired glucose tolerance (IGT) or OGTT-diabetes than participants with normal FFM (all p < 0.003) and a near 2-fold greater probability of significant proteinuria (p < 0.01). RFFMD was more frequent in women than in men: significant sex-RFFMD interactions were found for BMI and waist circumference (both p < 0.0001). ConclusionsRFFMD in overweight/obese normoglycemic individuals is associated with greater probability of hypertension, abnormalities of glucose tolerance and proteinuria. Assessment of RFFRMD might, therefore, help stratifying cardiometabolic risk among normoglycemic individuals with overweight/obesity.

Metabolic Rather Than Body Composition Measurements Are Associated With Lower Serum Natriuretic Peptide Concentrations in Normal Weight and Obese Men

Several studies have shown that obese persons have lower circulating natriuretic peptide (NP) concentrations. The cause of the relative NP deficiency seen in obese persons is poorly understood, although variation in body composition and metabolic abnormalities has been suggested to play a role. Thus, the aim of this study was to assess whether variation in circulating NP concentrations would be associated with differences in metabolic disturbances rather than with differences in body composition. In 27 normal weight men (body mass index (BMI) = 20.0-24.9kg/m(2)) and 103 obese men (BMI ≥ 30kg/m(2)), we determined body composition (total, android, and gynoid fat mass) by dual energy x-ray absorptiometry scanning, and we measured fasting serum concentrations of midregional proatrial NP (MR-proANP) and insulin, as well as fasting plasma glucose concentrations. Mean weight ± SD was 74.9±6.7kg in the normal weight men and 106.1±10.8kg in obese men. Applying multiple regressions, adjusting for age and weight status (normal weight vs. obese), serum MR-proANP concentrations were significantly inversely associated with serum insulin concentrations (β = -0.39; P < 0.0001) and plasma glucose concentrations (β = -0.21; P = 0.02) but not with total (β = 0.00), android (β = -0.01), or gynoid (β = 0.03) fat mass percentage (P > 0.76). No significant interaction effects between metabolic measurements or body composition measurements and weight status on MR-proANP concentrations were found (P > 0.08). In normal weight and obese men, lower circulating NP concentrations are associated with higher insulin and glucose concentrations and not with the proportion of total fat mass or the distribution of fat mass.

Assessment of nutritional status in children with chronic kidney disease and on dialysis

Protein-energy wasting (PEW) is defined as a state of decreased body protein mass and fuel reserves (body protein and fat mass) and is a common complication of chronic kidney disease (CKD). It is multifactorial: the main causative factors are hormonal imbalances and a low nutrient intake, but low residual renal function, inadequate dialysis dose, chronic inflammation and metabolic acidosis are other important contributory factors. Adult PEW has been defined, but there is no accepted definition of pediatric PEW and consequently no precise diagnostic criteria. Assessing nutritional status in children is also complicated by the absence of a gold standard, specific abnormalities in body composition, and the slowly progressive course of the disease. The evaluation of PEW should take into account all of its pathogenetic aspects, which include dietary assessment, clinical and anthropometric assessment (based on weight, height, and body mass index), a panel of biochemical parameters, and a normalized protein catabolic rate (in the case of adolescents on hemodialysis). Bioimpedance indices can be used in individual patients on a regular basis in centers with expertise. The longitudinal follow-up data relating to the above parameters are valuable for comparing patient and normative data. Given the complex nature of PEW, only a multidisciplinary approach can provide an accurate assessment of nutritional status and its derangements in children with CKD and on dialysis.

Relationships Between Right Ventricular Function, Body Composition, and Prognosis in Advanced Heart Failure

This study sought to examine the relationships between right ventricular (RV) function, body composition, and prognosis in patients with advanced heart failure (HF). Previous studies investigating HF-related cachexia have not examined the impact of RV function on body composition. We hypothesized that RV dysfunction is linked to weight loss, abnormal body composition, and worsened prognosis in advanced HF. Subjects with advanced HF (n= 408) underwent prospective assessment of body composition (skinfold thickness, dual-energy X-ray absorptiometry), comprehensive echocardiography, and blood testing. Subjects were followed up for adverse events (defined as death, transplantation, or circulatory assist device). Subjects with RV dysfunction (51%) had lower body mass index, lower fat mass index, and were more likely to display cachexia (19%). The extent of RV dysfunction correlated with greater antecedent weight loss and a lower fat/lean body mass ratio. Over a median follow-up of 541 days, there were 150 events (37%). Risk of event was greater in subjects with RV dysfunction (hazard ratio: 3.09 [95% confidence interval (CI): 2.18 to 4.45]) and cachexia (hazard ratio: 2.90 [95% CI: 2.00 to 4.12]) in univariate and multivariate analyses. Increased body mass index was associated with a lower event rate (HR per kg/m(2): 0.92 [95% CI: 0.88 to 0.96]), and this protection was mediated by a higher fat mass (0.91 [95% CI: 0.87 to 0.96]) but not a fat-free mass index (0.97 [95% CI: 0.92 to 1.03]). RV dysfunction and cardiac cachexia often coexist, have additive adverse impact, and might be mechanistically interrelated. Wasting of fat but not of lean mass was predictive of adverse outcome, suggesting that fat loss is either a surrogate of enhanced catabolism or adipose tissue is cardioprotective in the context of HF.

Clinical characteristics and effects of GH replacement therapy in adults with childhood-onset craniopharyngioma compared with those in adults with other causes of childhood-onset hypothalamic–pituitary dysfunction

Adults with childhood-onset (CO) craniopharyngioma (COCP) have poor quality of life (QoL) and clinical outcomes, but few studies have compared these patients with adults with other causes of CO hypothalamic-pituitary dysfunction. In this study, we compared baseline clinical characteristics and patient-reported outcomes before starting GH replacement therapy in adults with GH deficiency (GHD) due to COCP with those of adults either with CO idiopathic/congenital hypopituitarism (COH) or with CO extrasellar (COE) tumours, and evaluated the 1- and 5-year effects of GH replacement therapy. Retrospective analysis of the data recorded in KIMS (Pfizer International Metabolic Database) was carried out. Patients with COCP, COH and COE tumours were evaluated at baseline, and after 1 and 5 years of therapy. Compared with COH and COE patients, more COCP patients underwent surgery, had greater abnormalities of body composition and higher prevalence of pituitary hormone deficits (all P<0.001), but comparable fasting glucose, HbA1c, total cholesterol and LDL-cholesterol levels, marital status, parenthood, living arrangements, education, employment and annual sick-leave days. After 1 and 5 years of GH replacement therapy, similar changes were evident with regard to body composition, fasting glucose and HbA1c levels, QoL, and the level of and satisfaction with physical activity across the three groups. Adults with untreated COCP with GHD at baseline demonstrated more co-morbidities including greater abnormalities of body composition, pituitary hormone deficits and visual field defects. Overall, adults with COCP, COH and COE tumours responded comparably to short- and long-term GH replacement therapy, suggesting that patients with GHD due to COCP benefited from GH replacement therapy to a similar degree as those with other causes of CO hypothalamic-pituitary dysfunction did.

AB0211 Sarcopenia in patients with inflammatory arthritis

BackgroundSarcopenia is the progressive generalised loss of skeletal muscle and strength related to age and disease activity and is defined by appendicular skeletal mass/height squared (ASM/h2). Sarcopenia is common in...

Use of Fat Mass and Fat Free Mass Standard Deviation Scores Obtained Using Simple Measurement Methods in Healthy Children and Patients: Comparison with the Reference 4-Component Model

BackgroundClinical application of body composition (BC) measurements for individual children has been limited by lack of appropriate reference data.Objectives(1) To compare fat mass (FM) and fat free mass (FFM) standard deviation scores (SDS) generated using new body composition reference data and obtained using simple measurement methods in healthy children and patients with those obtained using the reference 4-component (4-C) model; (2) To determine the extent to which scores from simple methods agree with those from the 4-C model in identification of abnormal body composition.DesignFM SDS were calculated for 4-C model, dual-energy X-ray absorptiometry (DXA; GE Lunar Prodigy), BMI and skinfold thicknesses (SFT); and FFM SDS for 4CM, DXA and bioelectrical impedance analysis (BIA; height2/Z)) in 927 subjects aged 3.8–22.0 y (211 healthy, 716 patients).ResultsDXA was the most accurate method for both FM and FFM SDS in healthy subjects and patients (mean bias (limits of agreement) FM SDS 0.03 (±0.62); FFM SDS −0.04 (±0.72)), and provided best agreement with the 4-C model in identifying abnormal BC (SDS ≤−2 or ≥2). BMI and SFTs were reasonable predictors of abnormal FM SDS, but poor in providing an absolute value. BIA was comparable to DXA for FFM SDS and in identifying abnormal subjects.ConclusionsDXA may be used both for research and clinically to determine FM and FFM SDS. BIA may be used to assess FFM SDS in place of DXA. BMI and SFTs can be used to measure adiposity for groups but not individuals. The performance of simpler techniques in monitoring longitudinal BC changes requires investigation. Ultimately, the most appropriate method should be determined by its predictive value for clinical outcome.

Risk factors for body composition abnormalities in systemic sclerosis

The aim of this study was to evaluate the body composition (BC), bone mineral density (BMD), and the food intake in women with systemic sclerosis (SSc) compared to a control group, in order to identify main risk factors for BC abnormalities in SSc. Sixty-one SSc women and 67 age- and gender-matched controls were included. Spine, femur, and total body BMD measurements were performed using dual-energy X-ray absorptiometry. BC measurements included total lean (LM), fat mass (FM), and relative skeletal muscle mass index (RSMI) assessment. The food intake was calculated from 3-day food records and transformed into energy and nutrients. The 61 SSc patients [30 with diffuse cutaneous disease (dcSSc) and 31 with limited cutaneous SSc (lcSSc)] had significantly lower body mass index (BMI), LM, and FM, as well as lower BMD values compared to controls. Besides, the group with dcSSc, but not those with lcSSc, showed significantly lower BC and BMD measurements than controls. There was a significant inverse correlation between disease duration and BMI, LM, and RSMI. The total energy, macronutrients, and essential amino acids intakes were similar between patients and controls. After multivariate analysis, longer disease duration was the only risk factor associated with sarcopenia (RSMI below 5.45 kg/m(2); OR = 1.36, 95% CI 1.07-1.7). The present study showed an abnormal BC and a lower BMD, especially in dcSSc women, regardless of current food intake. Longer disease duration was associated with a higher risk of sarcopenia in SSc patients.

Prader-Willi syndrome and growth hormone therapy: effect on sleep

Because of reports that growth hormone therapy causes increased severity of obstructive sleep apnea in Prader-Willi syndrome, a retrospective review was performed of polysomnograms performed in children with Prader-Willi syndrome before onset of therapy and 6 weeks, 6 months, 1 year, and 2 years after starting growth hormone therapy. The results from Al-Saleh et al are published in this issue of The Journal, which show that 13 of 15 children did well with no adverse sleep outcomes. However, 2 of the 15 had growth hormone therapy discontinued because of the development of severe obstructive sleep apnea after 6 weeks. The authors recommend that growth hormone therapy appears to be a safe treatment after an initial period of increased risk with regards to sleep-related breathing, but they recommend that children selected for treatment should be evaluated very carefully. This issue of growth hormone related-obstructive sleep apnea in Prader-Willi syndrome is discussed in the accompanying Editorial by Whitman and Myers. They propose recommendations for how to manage children with Prader-Willi syndrome with regards to starting growth hormone therapy. Article page 263▶ Editorial page 224▶ Longitudinal Evaluation of Sleep-Disordered Breathing in Children with Prader-Willi Syndrome during 2 Years of Growth Hormone TherapyThe Journal of PediatricsVol. 162Issue 2PreviewTo review longitudinal polysomnography data to assess sleep-related disordered breathing (SRDB) before and up to 2 years after initiation of growth hormone (GH) therapy in children with Prader-Willi syndrome (PWS). Full-Text PDF Prader-Willi Syndrome and Growth Hormone Therapy: Take a Deep Breath and Weigh the DataThe Journal of PediatricsVol. 162Issue 2PreviewPrader-Willi syndrome (PWS), a complex neurogenetic disorder originally described in 1956,1 was relatively unknown outside genetics and developmental clinics until the late 1990s when growth hormone replacement therapy (GHRT) emerged as an effective intervention. The cardinal feature is the early onset, unrelenting, treatment-refractory hyperphagia leading to morbid obesity and attendant comorbidities. In addition, however, PWS is characterized by infantile hypotonia and failure to thrive, followed by short stature, hypogonadism, abnormal body composition, sleep and respiratory abnormalities (including impaired ventilatory control, central and obstructive apnea, and excessive daytime sleepiness), and behavioral difficulties. Full-Text PDF

The study of relationship spinal abnormalities with flexibility and body composition in down syndrome girls

Introduction: Down syndrome is one of the most common genetic causes of mild-to-moderate mental retardation. The main signs and symptoms of the disorder are the loss of mental functions and physical abnormalities that affect motor activities. The purpose of this research was to determine the possible relationship between flexibility, body composition and skeletal abnormalities in student with Down syndrome. Materials and Methods: 50 mentally-retarded female students with Down syndrome (age: 13.96 ± 1.77 yr, height: 135.40 ± 8.19 cm, weight: 42.86 ± 9.21 kg, IQ: 59.12 ± 3.73), were randomly selected and enrolled in the study. Demographic and health-related information including age, height, weight, IQ and medical histories were obtained from the students’ medical records. Flexibility and body composition were respectively assessed through asking subjects to complete “sit and reach” test and calculating their body mass index (BMI). Continued spinal abnormalities (kyphosis, lordosis, thoracic and lumbar scoliosis) were measured through Spinal mouse. Results: The results showed a significant correlation between BMI and lordosis (P ≤ 0.05) but there was no significant correlation between flexibility and skeletal abnormalities in the sample studies. Conclusion: Considering the interaction between postural, physical fitness and Special needs among patients with Down syndrome, it would be reasonable to provide affected students with postural corrective exercises, physical fitness and weight control programs in order to ensure that they perform their daily activities optimally. Keywords: Down syndrome, Spinal abnormalities, Flexibility, Body composition

Effects of growth hormone deficiency on body composition and biomarkers of cardiovascular risk after definitive therapy for acromegaly

Both growth hormone (GH) excess and GH deficiency are associated with abnormalities in body composition and biomarkers of cardiovascular risk in patients with pituitary disorders. However, the effects of developing GH deficiency after definitive treatment of acromegaly are largely unknown. To determine whether development of GH deficiency after definitive therapy for acromegaly is associated with increased visceral adiposity and biomarkers of cardiovascular risk compared with GH sufficiency after definitive therapy for acromegaly. Cross-sectional. We studied three groups of subjects, all with a history of acromegaly (n=76): subjects with subsequent GH deficiency (GHD; n=31), subjects with subsequent GH sufficiency (GHS; n=25) and subjects with active acromegaly (AA; n=20). No study subjects were receiving somatostatin analogues, dopamine agonists or hGH. Body composition (by DXA), abdominal adipose tissue depots (by cross-sectional CT), total body water (by bioimpedance analysis) and carotid intima-media thickness (IMT) were measured. Fasting morning serum was collected for high-sensitivity C-reactive protein (hsCRP), lipids and lipoprotein levels. An oral glucose tolerance test was performed, and homoeostasis model of assessment-insulin resistance (HOMA-IR) was calculated. Abdominal visceral adipose tissue, total adipose tissue and total body fat were higher in subjects with GHD than GHS or AA (P<0·05). Subcutaneous abdominal fat was higher, and fibrinogen and IMT were lower in GHD (but not GHS) than AA (P<0·05). Patients with GHD had the highest hsCRP, followed by GHS, and hsCRP was lowest in AA (P<0·05). Fasting glucose, 120-min glucose, fasting insulin, HOMA-IR and per cent total body water were lower in GHD and GHS than AA (P<0·05). Triglycerides were higher in GHS than AA (P<0·05). Lean body mass, mean arterial pressure, total cholesterol, HDL and LDL were comparable among groups. Development of GHD after definitive treatment of acromegaly may adversely affect body composition and inflammatory biomarkers of cardiovascular risk but does not appear to adversely affect glucose homoeostasis, lipids and lipoproteins, or other cardiovascular risk markers.

Fetal programming and the risk of noncommunicable disease.

The "developmental origins of health and disease" (DOHaD) hypothesis proposes that environmental conditions during fetal and early post-natal development influence lifelong health and capacity through permanent effects on growth, structure and metabolism. This has been called 'programming'. The hypothesis is supported by epidemiological evidence in humans linking newborn size, and infant growth and nutrition, to adult health outcomes, and by experiments in animals showing that maternal under- and over-nutrition and other interventions (e.g., glucocorticoid exposure) during pregnancy lead to abnormal metabolism and body composition in the adult offspring. Early life programming is now thought to be important in the etiology of obesity, type 2 diabetes, and cardiovascular disease, opening up the possibility that these common diseases could be prevented by achieving optimal fetal and infant development. This is likely to have additional benefits for infant survival and human capital (e.g., improved cognitive performance and physical work capacity). Fetal nutrition is influenced by the mother's diet and body size and composition, but hard evidence that the nutrition of the human mother programmes chronic disease risk in her offspring is currently limited. Recent findings from follow-up of children born after randomised nutritional interventions in pregnancy are mixed, but show some evidence of beneficial effects on vascular function, lipid concentrations, glucose tolerance and insulin resistance. Work in experimental animals suggests that epigenetic phenomena, whereby gene expression is modified by DNA methylation, and which are sensitive to the nutritional environment in early life, may be one mechanism underlying programming.

Body Composition and Cardiovascular Risk Markers after Remission of Cushing's Disease: A Prospective Study Using Whole-Body MRI

Cushing's Disease (CD) alters fat distribution, muscle mass, adipokine profile, and cardiovascular risk factors. It is not known whether remission entirely reverses these changes. Our objective was to determine whether the adverse body composition and cardiovascular risk profile in CD change after remission. Fourteen CD patients were studied prospectively: before surgery (active disease) and again postoperatively 6 months after discontinuing oral glucocorticoids (remission). Whole-body magnetic resonance imaging was used to examine lean and fat tissue distributions. Body composition (skeletal muscle and fat in the visceral, bone marrow, sc, and inter-muscular compartments) and cardiovascular risk factors (serum insulin, glucose, leptin, high-molecular-weight adiponectin, C-reactive protein, and lipid profile) were measured in active CD and remission (mean 20 months after surgery). Remission decreased visceral, pelvic bone marrow, sc (including trunk and limb sc), and total fat; waist circumference; and weight (P < 0.05). Remission altered fat distribution, resulting in decreased visceral/total fat (P = 0.04) and visceral fat/skeletal muscle ratios (P = 0.006). Remission decreased the absolute muscle mass (P = 0.015). Cardiovascular risk factors changed: insulin resistance, leptin, and total cholesterol decreased (P < 0.05), but adiponectin, C-reactive protein, and other lipid measures did not change. CD remission reduced nearly all fat depots and reverted fat to a distribution more consistent with favorable cardiovascular risk but decreased skeletal muscle. Remission improved some but not all cardiovascular risk markers. Remission from CD dramatically improves body composition abnormalities but may still be associated with persistent cardiovascular risk.

Weight gain in long-term survivors of kidney or liver transplantation—Another paradigm of sarcopenic obesity?

ObjectiveObesity in transplant recipients is a frequent phenomenon but data from body composition analyses in long-term survivors are limited. Body composition and energy metabolism were studied in patients after liver (LTX) and kidney (KTX) transplantation and patients with liver cirrhosis (LCI) or on chronic hemodialysis (HD) and compared to healthy controls. MethodsIn 42 patients 50.0 mo (median; range 17.1–100.6) after LTX and 30 patients 93.0 mo (31.2–180.1) after KTX as wells as in LCI (n = 39) or HD (n = 10) patients mid-arm muscle and fat area, body cell mass, and phase angle (bioimpedance analysis), and resting energy expenditure (indirect calorimetry, REECALO) were measured. ResultsObesity was more prevalent in LTX (17%) than LCI (3%) and in KTX (27%) than in HD (10%). In LTX and KTX, phase angle was higher than in end-stage disease (LTX 5.6° [4.1–7.2] versus LCI 4.4° [2.9–7.3], P < 0.001; KTX 5.9° [4.4–8.7] versus HD 4.3° [2.9–6.8]) but was lower in all patient groups than in controls (7.1°; 4.6–8.9; P < 0.001). In LCI and HD REECALO was higher than predicted, while in LTX and KTX REECALO was not different from predicted REE. ConclusionsDespite excellent graft function, many long-term LTX or KTX survivors exhibit a phenotype of sarcopenic obesity with increased fat but low muscle mass. This abnormal body composition is observed despite normalization of the hypermetabolism found in chronic disease and cannot be explained by overeating. The role of appropriate nutrition and physiotherapy after transplantation merits further investigation.

General anesthetic management of Prader-Willi syndrome patient undergoing middle cerebral artery-superficial temporal artery anastomosis

We experienced a case of a 17-year-old patient with genetically proven Prader-Willi syndrome who had left middle cerebral artery-left superficial temporal artery anastomosis surgery under general anesthesia with a non-depolarizing muscle relaxant. Prader-Willi syndrome is a genetic disorder associated with hypotonia. Using general anesthesia with a non-depolarizing muscle relaxant in these patients is difficult due to hypotonia. We, therefore, monitored the status of muscle relaxation with a train of four (TOF, TOF-Watch®, Organon, Ireland) during general anesthesia. Unlike infants, adolescents can have the muscle relaxation monitor on their body surface. Because lengthy, complex microsurgery was required for this vascular surgery, we had to use a muscle relaxant. However, when using a muscle relaxant, predicting the extent of the effect and the recovery time are difficult. The TOF ratio was 0.85 in the preanesthetic state. Anesthetic induction was done using the total intravenous anesthesia of the target concentration infusion method. Propofol target concentration at 4 µg/ml was initially injected. Remifentanil was injected at 0.15 µg/kg/min. Five minutes after an atracurium 15 mg injection, the TOF watch count was still at 2. Therefore, an additional 15 mg of atracurium was injected for intubation. When the TOF watch count was over 4, during the operation, an additional 5 mg of atracurium was injected. For the entire surgery, 65 mg of atracurium was used. The operation lasted for 2 hours and 50 minutes. After the operation, when the TOF ratio was 0.85, glycopyrrolate 0.4 mg and pyridostigmine 15 mg were injected. The endotracheal tube was removed once the patient regained spontaneous respiration and was fully awake. After extubation, the patient was arousable using painful stimulation. In the post anesthetic care unit, the patient had no respiratory problem and his TOF ratio was 0.85. The patient was then transferred to the general ward. Prader-Willi syndrome is a genetic disorder classically characterized by hypotonia, hypomentia, and hypogonadism that is associated with obesity [1]. The cause of hypotonia, muscle weakness, and motor problems in Prader-Willi patients are not clear. The contributing factors may be the abnormal body composition found in Prader-Willi patients, an increase in fat mass and a decrease in muscle mass, and, possibly, some degree of neuromuscular abnormality [2]. It has been reported that the motor problems are still present in childhood and adulthood, which causes the decrease in physical activity in Prader-Willi patients [3]. Thus, the hypotonia makes the choice of muscle relaxant difficult. Anesthetic management using atracurium and rocuronium have been reported [4,5]. However, few reports are available on using the intraoperative monitoring of the neuromuscular blockade for Prader-Willi patients. In this case, though the patient had hypotonia, anesthesia was successfully used with a dose adjusted muscle relaxant using the TOF watch.

Growth hormone treatment in adults with Prader-Willi syndrome: the Scandinavian study

Prader-Willi syndrome (PWS) is characterized by short stature, muscular hypotonia, cognitive dysfunction, and hyperphagia usually leading to severe obesity. Patients with PWS share similarities with growth hormone deficiency (GHD). Few studies have dealt with growth hormone (GH) treatment in PWS adults. The purpose of the Scandinavian study was to evaluate the effects of GH on body composition, lipid and glucose metabolism, physical performance and safety parameters in adults with PWS. Twenty-five women and 21 men with PWS were randomized to treatment with GH or placebo during 1year followed by 2years of open labeled GH treatment. At baseline 1/3 had normal BMI, six patients severe GHD, ten impaired glucose tolerance and seven diabetes. At 1year insulin-like growth factor I (IGF-I) SDS had increased by 1.51 (P<0.001) and body composition improved in the GH treated group. Visceral fat decreased by 22.9ml (P=0.004), abdominal subcutaneous fat by 70.9ml (P=0.003) and thigh fat by 21.3ml (P=0.013), whereas thigh muscle increased 6.0ml (P=0.005). Lean body mass increased 2.25kg (P=0.005), and total fat mass decreased 4.20kg (P<0.001). The positive effects on body composition were maintained after 2years of GH treatment. Peak expiratory flow increased by 12% (P<0.001) at 2years of GH treatment. Lipid and glucose metabolism were unchanged, however, three patients developed diabetes at 2years of GH treatment. In conclusion GH treatment had beneficial effects on the abnormal body composition without serious adverse events making it a logic treatment option in adults with PWS.

Abnormal body composition and early biomarkers of metabolic complications

Introduction A disturbed balance between energy metabolism and dietary intake is at the basis of obesity-related chronic diseases that rapidly increase in prevalence and pose a high burden on society. In advanced stages, chronic disease progression is characterized by metabolic aberrations and wasting that significantly contributes to complications and physical disability with high medical and nonmedical costs. From a body compositional point of view, the focus in chronic metabolic and cardiovascular disease (CVD) prevention is predominantly on adipose tissue, whereas in the course of many chronic diseases, skeletal muscle tissue becomes an important therapeutic target to compress morbidity and enhance survival. Recent experimental and clinical research has shed new light on early biomarkers and metabolic processes within fat and skeletal muscle causing body compositional shifts that may lead to reconsiderations in disease risk perception and modification strategies.

Body Composition Abnormalities in Long-Term Survivors of Pediatric Hematopoietic Stem Cell Transplantation

To quantify lean mass (LM) and fat mass (FM) in survivors of childhood allogeneic hematopoietic stem-cell transplantation (alloHSCT) compared with healthy reference participants and identify risk factors for body composition abnormalities. Whole body LM and FM were measured with dual energy x-ray absorptiometry in 54 survivors (ages 5-25 years) and 894 healthy reference participants in a cross-sectional study. Multivariate regression models were used to compare sex- and race-specific Z-scores for LM for height (LM-Ht) and FM for height (FM-Ht) in survivors and reference participants and to identify correlates of LM-Ht and FM-Ht Z-scores in alloHSCT. Height Z-scores were significantly lower in alloHSCT survivors (P < .001) compared with reference participants; body mass index Z-scores did not differ (P = .13). Survivors had significantly lower mean LM-Ht Z-scores (-0.72; 95% CI, -1.02--0.42; P < .001) and greater FM-Ht Z-scores (1.10; 95% CI, 0.84-1.39; P < .001) compared with reference participants. LM-Ht Z-score deficits in alloHSCT survivors were larger (-1.26; 95% CI, -1.53--0.99; P < .001) after adjustment for FM-Ht Z-scores. Endocrinopathies and alloHSCT characteristics were not associated with LM-Ht or FM-Ht Z-scores. Survivors of childhood alloHSCT have significant LM deficits and FM excess. Future studies should identify the mechanism and consequences of these abnormalities.

Abnormalities in body composition and nutritional status in HIV-infected children and adolescents on antiretroviral therapy

This cross-sectional study aimed to compare growth, nutritional status and body composition outcomes between a group of 94 HIV-infected children and adolescents on antiretroviral therapy (ART) and 364 healthy controls, and to evaluate their association with clinical and lifestyle variables within the HIV-infected group. When compared with the control group, HIV patients had higher risk of stunting (odds ratio [OR] 5.33, 95% confidence interval [CI]: 2.83-10.04) and thinness (OR 4.7, 95% CI: 2.44-9.06), higher waist-to-hip ratios (medians 0.89 versus 0.82 for boys and 0.90 versus 0.77 for girls, P < 0.001), and lower prevalence of overweight or obesity (OR 0.33, 95% CI: 0.14-0.78). Protease inhibitor usage was associated with thinness (OR 3.51, 95% CI 1.07-11.44) and lipoatrophy (OR 3.5, 95% CI 1.37-8.95). HIV-infected children on ART showed significant nutritional status and body composition abnormalities, consistent with the severity of vertical HIV infection and the consequences of prolonged ART.