Solitary fibrous tumor of parotid gland: a case report and short review of literature.

OBJECTIVE: This case report represents a solitary fibrous tumor, which is a very rare neoplasm in the parotid gland. CASE PRESENTATION: 80-year-old Caucasian female patient with palpable, immobile, painless, slow growing, semi-rigid-elastic neoplasm in the left parotid gland, that was existing for four months. There were no signs of inflammation and facial paralysis. The tumor was initially interpreted as a salivary gland neoplasm of uncertain origin. Fine needle aspiration was performed 2 times; however, the precise diagnosis was challenging. The tumor was excised, regional lymphadenectomy was performed. Morphological and immunophenotypical findings were consistent with solitary fibrous tumor of parotid gland. Currently, the patient is under regular follow-up period at 9 months with no evidence of metastasis or recurrence. CONCLUSIONS: Although very rare, solitary fibrous tumor (SFT) should be suspected in cases of slow growing, solid, well-defined nodules of the parotid gland. The SFT of the parotid gland are usually benign tumors, however there is a low risk of recurrency and malignant behavior with metastatic potential. Complete resection of the tumor should be performed since it is crucial for management without any recurrence.

FGF23-related hypophosphatemia in patients with low bone mineral density and fragility fractures: challenges in diagnosis and management.

PURPOSE: Hypophosphatemia (HP) can be observed in patients evaluated for skeletal fragility. We investigated prevalence of HP among outpatients referred for low bone density or fragility fractures, HP-associated clinical and biochemical features and outcomes of recommended diagnostic algorithm in our cohort. METHODS: Chronic HP (phosphate ≤ 2.7 mg/dL over 6 months or longer) was retrospectively investigated among 2319 patients. In renal wasting-related HP, intact FGF23 was assessed; non-suppressed FGF23 prompted the performance of 68Ga-DOTATOC PET/CT in the suspicion of tumor-induced steomalacia (TIO). RESULTS: Renal wasting-related HP (median 2.2, range 1.6-2.6 mg/dL) was observed in 19 patients (0.82%). FGF23 levels were suppressed in two patients diagnosed with renal tubular disease, increased in one and within normal range in most patients. X-linked hypophosphatemic rickets was diagnosed in one woman. In the remaining 16 patients, highly prevalent fragility fractures (50%) and severely reduced bone mineral density were detected, though diagnostic criteria for osteomalacia were not fulfilled. 68Ga-PET was performed in nine patients and was positive in four. While intact FGF23 levels alone failed to differentiate PET's outcomes (positive: FGF23 median 70.5 pg/mL; negative: 52 pg/mL, P = 0.462), the coexistence of multiple biochemical and radiologic alterations performed better in prediction of PET's positivity. CONCLUSION: Mild, apparently unexplained HP is observed in 0.82% of patients with low bone density or fragility fractures. In asymptomatic patients with isolated mild hypophosphatemia, the probability of finding an underlying tumor disease is very low, and utility of extensive and expensive diagnostic workup should be carefully considered in this setting.

T1 and T2* mapping of the human quadriceps and patellar tendons using ultra-short echo-time (UTE) imaging and bivariate relaxation parameter-based volumetric visualization.

Quantification of magnetic resonance (MR)-based relaxation parameters of tendons and ligaments is challenging due to their very short transverse relaxation times, requiring application of ultra-short echo-time (UTE) imaging sequences. We quantify both T1 and T2* in the quadriceps and patellar tendons of healthy volunteers at a field strength of 3 T and visualize the results based on 3D segmentation by using bivariate histogram analysis. We applied a 3D ultra-short echo-time imaging sequence with either variable repetition times (VTR) or variable flip angles (VFA) for T1 quantification in combination with multi-echo acquisition for extracting T2*. The values of both relaxation parameters were subsequently binned for bivariate histogram analysis and corresponding cluster identification, which were subsequently visualized. Based on manually-drawn regions of interest in the tendons on the relaxation parameter maps, T1 and T2* boundaries were selected in the bivariate histogram to segment the quadriceps and patellar tendons and visualize the relaxation times by 3D volumetric rendering. Segmentation of bone marrow, fat, muscle and tendons was successfully performed based on the bivariate histogram analysis. Based on the segmentation results mean T2* relaxation times, over the entire tendon volumes averaged over all subjects, were 1.8 ms ±â€¯0.1 ms and 1.4 ms ±â€¯0.2 ms for the patellar and quadriceps tendons, respectively. The mean T1 value of the patellar tendon, averaged over all subjects, was 527 ms ±â€¯42 ms and 476 ms ±â€¯40 ms for the VFA and VTR acquisitions, respectively. The quadriceps tendon had higher mean T1 values of 662 ms ±â€¯97 ms (VFA method) and 637 ms ±â€¯40 ms (VTR method) compared to the patellar tendon. 3D volumetric visualization of the relaxation times revealed that T1 values are not constant over the volume of both tendons, but vary locally. This work provided additional data to build upon the scarce literature available on relaxation times in the quadriceps and patellar tendons. We were able to segment both tendons and to visualize the relaxation parameter distributions over the entire tendon volumes.

Preliminary Experience With Hypothermic Oxygenated Machine Perfusion in an Italian Liver Transplant Center.

BACKGROUND: Machine perfusion is increasingly utilized in liver transplantation to face the detrimental consequences of the use of extended-criteria donors. Hypothermic oxygenated machine perfusion (HOPE) appears to be more protective relative to static cold storage. Conversely, normothermic machine perfusion (NMP) allows a better graft evaluation. We describe a pilot prospective study on machine perfusion in selected grafts. METHODS: HOPE was executed for all the grafts procured from donors after cardiac death (DCDs) and for livers from donors after brain death (DBDs) requiring prolonged preservation time. NMP was used when a more precise evaluation was needed. Both HOPE and NMP were performed through the portal vein and hepatic artery. RESULTS: From July 2016 to November 2017, we performed 7 HOPE procedures: 5 for DCD and 2 for DBD grafts. Two livers presented with macrovesicular steatosis >30% (1 DCD and 1 DBD). HOPE lasted 240 minutes (180-320 min) with a total ischemia time of 575 minutes (410-810 min). Six grafts were successfully transplanted. One DCD graft required additional evaluation using NMP. The graft was then discarded due to extensive hepatocellular necrosis. In the post-transplant course, acute and chronic renal failure were the main complications affecting 3 and 2 recipients, respectively. In our series, steatosis was the main risk factor for kidney injury. Patient and graft survival rate was 100% and no ischemic cholangiopathies were observed after 270 days (106-582 days). CONCLUSIONS: Our study confirms HOPE safety and efficacy for DCD and DBD grafts. These data are particularly significant for DCD management in the Italian setting where the mandatory 20-minute hands-off interval before death declaration further prolongs warm ischemia time.

Causal relationship of hepatic fat with liver damage and insulin resistance in nonalcoholic fatty liver.

BACKGROUND AND AIMS: Nonalcoholic fatty liver disease is epidemiologically associated with hepatic and metabolic disorders. The aim of this study was to examine whether hepatic fat accumulation has a causal role in determining liver damage and insulin resistance. METHODS: We performed a Mendelian randomization analysis using risk alleles in PNPLA3, TM6SF2, GCKR and MBOAT7, and a polygenic risk score for hepatic fat, as instruments. We evaluated complementary cohorts of at-risk individuals and individuals from the general population: 1515 from the liver biopsy cohort (LBC), 3329 from the Swedish Obese Subjects Study (SOS) and 4570 from the population-based Dallas Heart Study (DHS). RESULTS: Hepatic fat was epidemiologically associated with liver damage, insulin resistance, dyslipidemia and hypertension. The impact of genetic variants on liver damage was proportional to their effect on hepatic fat accumulation. Genetically determined hepatic fat was associated with aminotransferases, and with inflammation, ballooning and fibrosis in the LBC. Furthermore, in the LBC, the causal association between hepatic fat and fibrosis was independent of disease activity, suggesting that a causal effect of long-term liver fat accumulation on liver disease is independent of inflammation. Genetically determined hepatic steatosis was associated with insulin resistance in the LBC and SOS. However, this association was dependent on liver damage severity. Genetically determined hepatic steatosis was associated with liver fibrosis/cirrhosis and with a small increase in risk of type 2 diabetes in publicly available databases. CONCLUSION: These data suggest that long-term hepatic fat accumulation plays a causal role in the development of chronic liver disease.

Liver stiffness measurement reliability and main determinants of point shear-wave elastography in patients with chronic liver disease.

BACKGROUND: Liver stiffness (LS) measured by transient elastography (TE) accurately predicts the severity of chronic liver diseases (CLD). Point quantification shear-wave elastography (pSWE) is a new technique incorporated into a conventional ultrasound system for measuring LS. We evaluated pSWE feasibility, reproducibility and diagnostic accuracy in consecutively recruited CLD patients who concomitantly underwent TE and liver biopsy. AIM: To evaluate pSWE feasibility, reproducibility and diagnostic accuracy in consecutively recruited CLD patients who concomitantly underwent TE and liver biopsy. METHODS: Over 2 years 186 CLD patients (116 males, 132 viral hepatitis) consecutively underwent pSWE (10 valid measurements by ElastPQ) blindly performed by two raters. A further operator performed TE. Inter-observer agreement for pSWE was analysed by intraclass correlation coefficient (ICC) and correlated with histological liver fibrosis (METAVIR). Main determinants of pSWE were investigated by linear regression model. RESULTS: Three hundred and seventy-two (100%) reliable measurements were obtained by pSWE and 184 by TE (99%). LS was 8.1 ± 4.5 kPa for pSWE with the first rater and 8.0 ± 4.2 kPa with the second one vs. 8.8 ± 3.6 kPa for TE. pSWE ICC was 0.89 (95% CI 0.85-0.91), not influenced by age, sex, BMI, liver enzymes, liver aetiology. ICC increased over time with year 1 at 0.86 and 95% CI 0.81-0.90 vs. year 2 at 0.92 and 95% CI 0.87-0.95. Liver fibrosis was the only independent determinant of LS on pSWE. The AUROCs for diagnosing F ≥ 2, F ≥ 3 and F = 4 were 0.77, 0.85 and 0.88 for pSWE vs. 0.81, 0.88 and 0.94 for TE. After 1-year training they were 0.86, 0.94 and 0.91. CONCLUSION: Point quantification shear-wave elastography reliably and reproducibly evaluates liver stiffness, matching transient elastography for accuracy after a 1-year learning curve or 130 examinations.

Particulate matter phagocytosis induces tissue factor in differentiating macrophages.

Airborne exposure to particulate matter with diameter < 10 mcM (PM10) has been linked to an increased risk of thromboembolic events, but the mechanisms are not completely understood. The aim of this study was to evaluate the effect of PM10 phagocytosis on the release of procoagulant molecules in human differentiating macrophages, and that of PM10 inhalation in an experimental model in rats. Human monocytes were separated from the peripheral blood by the lymphoprep method, differentiated in vitro and treated with standard PM10 or vehicle. Sprague-Dawley rats were instilled intratracheally with PM10 or vehicle alone. The outcome was expression of proinflammatory genes and of tissue factor (TF). In human differentiating macrophages, PM10 exposure upregulated inflammatory genes, but most consistently induced TF mRNA and protein levels, but not TF protein inhibitor, resulting in increased TF membrane expression and a procoagulant phenotype. Differentiation towards the anti-inflammatory M2 phenotype inhibited PM10 -mediated TF expression. TF induction required phagocytosis of PM10 , whereas phagocytosis of inert particles was less effective. PM10 phagocytosis was associated with a gene expression profile consistent with intracellular retention of iron, inducing oxidative stress. Both PM10 and iron activated the stress kinases ERK1/2 pathway, involved in the induction of TF expression. In rats, alveolar exposure to PM10 was associated with pulmonary recruitment of inflammatory cells and resulted in local, but not systemic, induction of TF expression, which was sufficient to increase circulating TF levels. In conclusion, TF induction by differentiating lung macrophages, activated following phagocytosis, contributes to the increased risk of thromboembolic complications associated with PM10 exposure.

Anthropometric and physiologic profiles of female professional yoga practitioners and energy expenditure during asanas execution.

AIM: The present study aimed to: 1) define the anthropometric and physiological profiles of female professional yoga practitioner compared to that of other athletes; 2) evaluate the energy expenditure (EE) during a yoga session. METHODS: The percentage fat mass (FM%) and fat free mass (FFM%), the maximal aerobic power (VO2max), the maximal voluntary contraction (MVC) of knee extensor muscles and the maximal anaerobic alactacid power (Wmax) were assessed in a group of yoga practitioners (Yo), long distance runners (LDR), sprinters (Spr), karate practitioners (Ka) and sedentary control subjects (Con). EE was evaluated in Yo during a yoga session (execution of a sequence of six yoga postures, called asanas). RESULTS: FM% was significantly higher in Con (24.2±2.6%) than in other groups (18±1.9%, pooled data, P<0.05). FFM% did not differ among groups. VO2max was higher in LDR (55.6±1.8 mL min-1 kg-1) compared to other groups (41.7±3 mL min-1 kg-1, pooled data, P<0.05). MVC and Wmax were higher in Yo, Spr and Ka than in LDR and Con (P<0.05). In Yo, EE increased in comparison to baseline, during Sirasana execution only (+59%, P<0.05). CONCLUSION: These data suggest that chronic yoga practice is associated with 1) values of FM%, FFM%, MVC and Wmax similar to those induced by sports requiring high degree of force and power of lower limb muscles, with maximal aerobic performance similar to control subjects; 2) low EE during most asanas execution.

Relationship between anthropometric or functional characteristics and maximal serve velocity in professional tennis players.

AIM: This study aims at investigating the possible relationships between anthropometric and functional parameters and maximal serve speed in professional tennis players. METHODS: Eight professional male tennis players (age 23±4 [mean±SD] years; height 181±4 cm; body mass 80±4 kg; playing experience 14±4 years; weekly training practice 29±6 hours) were recruited. Anthropometric parameters (height, body mass, arm-racquet length, arm muscle area), jump performance (squat jump, counter movement jump; counter movement jump free), handgrip strength and first and second maximal serve speed were assessed. RESULTS: Pearson's correlation coefficient showed significant (P<0.05) positive relationships between height and ball speed in both the first (r=0.78; P=0.02) and second (r=0.80; P=0.017) serve, and a significant negative correlation between serve speed and arm muscle area in first serve only (r=-0.78; P=0.03). In addition, a trend towards a positive correlation was observed between string tensions and serves speed for both first and second serves (r=0.54; P=0.16 and r=0.60; P=0.11, respectively). No significant relationship was found between serve speed and the other variables considered, including jumping performance parameters. CONCLUSION: Height was confirmed to be the main anthropometric determinant of serves speed in professional tennis players.

Cardiovascular adaptation to mudpack therapy in hypertensive subjects treated with different antihypertensive drugs.

OBJECTIVE: In selected hypertensive subjects, cardiovascular adaptation to warm environments may be inadequate or even harmful: heating associated to mudpack therapy may cause unexpected hypotension. How different antihypertensive drugs may affect the cardiovascular response to mudpack therapy is poorly studied. AIMS: To evaluate the effects of ß-blockers and angiotensin II receptor antagonists/ACE inhibitors on the acute cardiovascular adaptation to mudpack treatment in SPA in elderly hypertensive patients. PATIENTS AND METHODS: Thirty-one elderly subjects were divided in normotensive subjects (N; n=10) and hypertensive patients treated with ACE-inhibitors/Angiotensin II receptor antagonists (HTA; n=12) or with selective ß1-blockers (HTB; n=9). Systolic (SBP) and diastolic (DBP) blood pressure were continuously recorded (10 min) in supine position, immediately before and during mudpack treatment (40°C). Heart rate (HR), stroke volume (SV), cardiac output (CO) and total peripheral resistance (TPR) were assessed. RESULTS: During mudpack treatment SBP did not significantly change in both HTA and N groups (132±11 and 112±13 mmHg, respectively), but significantly decreased in HTB (111±18 mmHg, p < 0.01 vs baseline) patients. HR increased in all groups (HTA: 72±10 bpm; HTB: 65±6 bpm; N: 70±10 bpm; p < 0.01 vs baseline). A significant reduction (p < 0.01 vs baseline) in SV and CO occurred in HTB, but not in HTA and N groups. TPR significantly increased in HTB (1335±464 dyn.s.cm-5, p < 0.01 vs baseline) but not in HTA and N subjects (1389±385 dyn.s.cm-5 and 1245±323 dyn.s.cm-5, respectively). CONCLUSIONS: Mud treatment did not cause relevant haemodynamic changes in normotensive and HTA-treated hypertensive subjects. Conversely, ß-blocking treatment apparently limited the cardiovascular adaptation to thermic stress, through a possible reduction in myocardial contractility, thereby, causing a significant decrease, although not dangerous, in systolic blood pressure.

Genetic evidence and new morphometric data as essential tools to identify the Patagonian seahorse Hippocampus patagonicus (Pisces, Syngnathidae).

A genetic study to support morphometric analyses was used to improve the description and validate the Patagonian seahorse Hippocampus patagonicus (Syngnathidae) on the basis of a large number of specimens collected in the type locality (San Antonio Bay, Patagonia, Argentina). DNA sequence data (from the cytochrome b region of the mitochondrial genome) were used to differentiate this species from its relatives cited for the west Atlantic Ocean. Both phylogenetic and genetic distance analyses supported the hypothesis that H. patagonicus is a species clearly differentiated from others, in agreement with morphometric studies. Hippocampus patagonicus can be distinguished from Hippocampus erectus by the combination of the following morphometric characteristics: (1) in both sexes and all sizes of H. patagonicus, the snout length is always less than the postorbital length, whereas the snout length of H. erectus is not shorter than the postorbital length in the largest specimens; (2) in both sexes of H. patagonicus, the trunk length:total length (LTr :LT ) is lower than in H. erectus (in female H. patagonicus: 0·27-0·39, H. erectus: 0·36-0·40 and in male H. patagonicus: 0·24-0·34, H. erectus: 0·33-0·43) and (3) in both sexes, tail length:total length (LTa :LT ) in H. patagonicus is larger than in H. erectus (0·61-0·78 v. 0·54-0·64).

Desenvolvimento de mudas de manjericão (Ocimum basilicum L. ) em função do recipiente e do tipo e densidade de substratos / Development of basil seedlings(Ocimum basilicum L. ) in different density and type of substrates and trays

Uma das etapas mais importantes na produção do manjericão é o desenvolvimento das mudas. Nesta etapa, o cuidado com o recipiente e o substrato é essencial, pois afetam diretamente o crescimento e a arquitetura do sistema radicular, bem como, o fornecimento de nutrientes. Assim, o objetivo deste trabalho foi avaliar o desenvolvimento de mudas de manjericão (Ocimum basilicum L.) influenciadas pelo tipo e densidade de diferentes substratos, cultivadas em bandejas de poliestireno com diferentes números de células. O experimento foi conduzido na área de Jardinocultura da Universidade Federal da Grande Dourados (UFGD) em Dourados - MS. O delineamento experimental utilizado foi em blocos casualizados com cinco repetições, sendo os tratamentos dispostos em esquema fatorial 3x3x5, sendo três tipos de bandejas de poliestireno expandido com 72, 128 e 200 células com volumes internos de 124,3; 44,8 e 17,7 mL, respectivamente. Três tipos de substratos comerciais (PlantMax Florestais®; Tropstrato Vida Verde® e PlantMax Hortaliças HA®), e cinco densidades de substratos (0,36; 0,42; 0,48; 0,54 e 0,60 kg dm-3). Decorridos 78 dias após a emergência das plantas avaliou-se alturas das plantas, massa fresca e seca da parte aérea e sistema radicular, e comprimento de raízes. A produção de mudas comerciais de manjericão (Ocimum basilicum L.) foi melhor com o uso do substrato Tropstrato Vida Verde® na bandeja de 72 células associado com a densidade de 0,47 kg dm-3.

One of the most important stages in the production of basil is the development of seedlings. In this step, care with the container and the substrate is essential, because it directly affects the root growth and architecture, as well as the supply of nutrients. Thus, the objective of this study was to evaluate the development of basil seedlings (Ocimum basilicum L.) influenced by the type and density of different substrates, grown in polystyrene trays with different cell numbers. The experiment was conducted at the Gardening area of the Federal University of Grande Dourados (UFGD) inDourados-MS, Brazil.The experimental design was a randomized block with fivereplications, with treatments arranged in a 3x3x5 factorial design, with three types of polystyrene trays with 72, 128 and 200 cells, with internal volumes of 124.3; 44.8 and 17.7 mL, respectively. We used three types of substrates (PlantMax Florestais®; Tropstrato Vida Verde®and PlantMax Hortaliças HA®) and five densities of substrate (0.36, 0.42, 0.48, 0.54 and 0.60 kg dm-3). After 78 days of plant emergence, we evaluatedthem regarding: plant height, fresh and dry shoot and root, and root length. The commercial production of basil seedlings was improved with the use of the substrate Tropstrato Vida Verde® in a 72 cell tray associated with the density of 0.47 kg dm-3.

Anthropometric characteristics of top-class Kenyan marathon runners.

AIM: This study aims to: 1) describe the current anthropometric profiles of Kenyan marathon runners and 2) establish a set of reference values useful for future investigations on athlete selection, talent identification, and training programme development. METHODS: The participants were 14 male top-class Kenyan marathon runners (mean [s] age 27.71 [3.75] yrs, height 171.21 [6.12] cm, body mass 57.71 [4.02] kg, marathon personal best 02h 07min 16s (01min 55s); training volume: 180-220 km·wk-1; high:low intensity training ratio: 1:2). The anthropometric profiles included the measurement of skinfolds, and segment lengths, breadths, and girths. To estimate body density (BD) multiple regression equations were calculated using the sum of 7-skinfolds method and then converted to percentage of body fat (%BF). The somatotype, somatotype dispersion mean (SDM), somatotype attitudinal mean (SAM), and height to weight ratio (HWR) as well as the skinfolds extremity to trunk ratio (E:T) were also calculated. RESULTS: The mean (s) of BD, %BF, SDM, SAM, HWR and E:T were 1.13 (0.02), 8.87 (0.07) %, 4.58 (3.62), 0.51 (0.09), 44.32 (1.06), and 0.36 (0.11), respectively. The mean (s) endomorphy, mesomorphy, and ectomorphy were 1.53 (0.32), 1.61 (1.81), and 3.86 (0.78), respectively. CONCLUSION: Top-class Kenyan marathon runners seem to have ectomorphy as dominant, with endomorphy and mesomorphy more than one-half unit lower. Despite population comparisons would be required to identify any connection between specific anthropometric dimensions, these reference data should be useful to practitioners and researchers, providing useful information for talent identification and development and for the assessment of training progression in marathon.

Polymorphisms of alpha-actinin-3 and ciliary neurotrophic factor in national-level Italian athletes.

AIM: The R577X polymorphism of the alpha-actinin-3 (ACTN3) gene and the IVS1-6G>A polymorphism of the ciliary neurotrophic factor (CNTF) gene have been associated with a favourable muscle phenotype (more muscle fibres with high glycolytic activity), reduced predisposition for congenital dystrophy and resistance to sarcopenia in old age. The aim of this study was to look for evidence of selective pressure towards genotypes favourable for strong muscle activity in a sample of national-level Italian athletes. METHODS: We analysed two stop codon polymorphisms in the DNA of 50 Italian athletes, specialised in power or endurance sports, and compared their genotypic distribution with those of a population of 50 controls. In a representative sub-group of athletes (N.=42) we then compared the genetic data with anaerobic threshold, assessed by an incremental exercise test up to exhaustion. RESULTS: The athlete group showed an allelic distribution of ACTN3 (R/R:64%, R/X:16%, X/X:20%) and CNTF (G/G:72%, G/A:26%, A/A:2%), significantly imbalanced towards alleles R/R and G/G, respectively, compared to controls (ACTN3=R/R:40% R/X:22% X/X:38% and CNTF=G/G:52%, G/A:24%, A/A:24%) (p=0.0024 and p=0.0001, respectively). Only the ACTN3 577X/X polymorphism showed a significant association with the anaerobic threshold of athletes (F-ratio= 4.037; p=0.025). Factorial ANOVA demonstrated a non significant interaction between favourable allelic patterns of ACTN3 and CNTF genes on aerobic performance in the athlete group. CONCLUSION: The relationship found between favourable muscle phenotype and this genetic profile may have interesting implications in sport performance and training, athlete selection and different clinical activities, such as physical rehabilitation and modifying phenotypes associated with neuromuscular diseases.

Heart adaptations to long-term aerobic training in paraplegic subjects: an echocardiographic study.

STUDY DESIGN: Case-control. OBJECTIVES: To execute an echocardiographic comparison between trained and untrained spinal cord injury (SCI) subjects, and to evaluate whether long-term heart adjustments to endurance training are comparable to those observed in able-bodied (ABL) subjects. SETTING: Italy. METHODS: We enrolled: (1) 17 male SCI patients (lesion level T1-L3, 34±8 years, body mass index (BMI) 23.0±2.8 kg m(-2)), 10 of whom were aerobically trained for >5 years (SCI(T)); (2) 18 age-, sex- and BMI-matched ABL subjects (35±6 years, BMI 23.6±2.8 kg m(-2)), 10 of whom were aerobically trained for >5 years (ABL(T)). Training frequency and volume were recorded by a dedicated questionnaire. All subjects underwent a trans-thoracic echocardiography; SCI subjects also performed an exhaustive incremental exercise test. Comparisons were made between ABL and SCI groups, between trained and untrained subjects within each group (analysis of variance). RESULTS: Effects of SCI-Compared with ABL subjects, SCI patients showed lower end-diastolic volume (76±21 vs. 113±23 ml, P<0.05) and ejection fraction (61±7% vs. 65±5%, P<0.05). Effects of training-Compared with untrained status, the intra-ventricular septum thickness (SCI, +18%; ABL, +4%), the posterior wall thickness (SCI, +17%; ABL, +2%) and the total normalized heart mass (SCI, +48%; ABL, +5%) were higher in both SCI(T) and in ABL(T). VO2peak was higher in the SCI(T) subgroup compared with the SCI(U) group. CONCLUSIONS: Heart seems to positively adapt to long-term endurance training in SCI patients. Regular exercise may therefore increase heart size, septum and posterior wall thickness, which likely contributes to improved VO2peak. These morphological and functional changes may reduce cardiovascular risk in SCI individuals.

Fulminant multiorgan failure due to varicella zoster virus and HHV6 in an immunocompetent adult patient, and anhepatia.

Varicella is a well-known contagious disease of childhood that can also affect both immunodepressed and immunocompetent adults. The present observations concern a previously healthy adult patient who presented with a fulminant hepatitis evolving in multiorgan failure (MOF), associated with an atypical papulo-ethemateous cutaneous rash without fever. An hepatic biopsy showed massive necrosis. Because of the persistent MOF and severe hemodynamic instability, total hepatectomy was performed as a bridge to urgent liver transplantation (OLT). Despite temporary improvement, the patients condition progressively deteriorated and he died 11 hours after the hepatectomy, i.e. 7 days after admission to the intensive care unit. High viral loads of varicella zoster virus (VZV) and human herpes virus 6 (HHV6) were demonstrated in the blood and in DNA at post mortem examination of the liver, kidneys, lung, and heart. We hypothesize that VZV infection may occasionally occur in immunocompetent patients due to extremely virulent strains that can be rapidly fatal. The clinical influence of simultaneous infection with HHV6 is not clear. Moreover, the role of a previous steroid treatment as a trigger for a temporary immunodepressed state must be considered. The diagnosis of liver disease from VZV should always be clinically suspected in the presence of concurrent atypical skin lesions and a temporarily immunocompromised state. Therapy with acyclovir was ineffective in our patient. Based on the wide spectrum of VZV infections, fulminant MOF in immunocompetent adults must raise the possibility of VZV with simultaneous HHV6 infection with early listing of the patient for a urgent OLT, possibly with a total hepatectomy as a bridge, due to the therapeutic uncertainty of medical treatments.

Genetic variants regulating insulin receptor signalling are associated with the severity of liver damage in patients with non-alcoholic fatty liver disease.

BACKGROUND/AIMS: The aim of this study was to assess the effect of functional ENPP1(ectoenzyme nucleotide pyrophosphate phosphodiesterase 1)/PC-1 (plasma cell antigen-1) and IRS-1 (insulin receptor substrate-1) polymorphisms influencing insulin receptor activity on liver damage in non-alcoholic fatty liver disease (NAFLD), the hepatic manifestation of the metabolic syndrome, whose progression is associated with the severity of insulin resistance. PATIENTS AND METHODS: 702 patients with biopsy-proven NAFLD from Italy and the UK, and 310 healthy controls. The Lys121Gln ENPP1/PC-1 and the Gly972Arg IRS-1 polymorphisms were evaluated by restriction analysis. Fibrosis was evaluated according to Kleiner. Insulin signalling activity was evaluated by measuring phosphoAKT levels by western blotting in a subset of obese non-diabetic patients. RESULTS: The ENPP1 121Gln and IRS-1 972Arg polymorphisms were detected in 28.7% and 18.1% of patients and associated with increased body weight/dyslipidaemia and diabetes risk, respectively. The ENPP1 121Gln allele was significantly associated with increased prevalence of fibrosis stage >1 and >2, which was higher in subjects also positive for the 972Arg IRS-1 polymorphism. At multivariate analysis, the presence of the ENPP1 121Gln and IRS-1 972Arg polymorphisms was independently associated with fibrosis >1 (OR 1.55, 95% CI 1.24 to 1.97; and OR 1.57, 95% CI 1.12 to 2.23, respectively). Both polymorphisms were associated with a marked reduction of approximately 70% of AKT activation status, reflecting insulin resistance and disease severity, in obese patients with NAFLD. CONCLUSIONS: The ENPP1 121Gln and IRS-1 972Arg polymorphisms affecting insulin receptor activity predispose to liver damage and decrease hepatic insulin signalling in patients with NAFLD. Defective insulin signalling may play a causal role in the progression of liver damage in NAFLD.