As a result, the electro-optical properties of the PDLC films are

As a result, the electro-optical properties of the PDLC films are significantly improved as the level of HEMA addition is increased. Overall, the results show that a PDLC comprising 40 wt % E7 liquid crystals, 33.51 mol % TeGDA and 66.49 mol % HEMA has a high contrast ratio (13 : 1) and a low driving voltage (10 V) and is therefore an ideal candidate for a wide variety of intelligent

photoelectric applications. (C) 2010 Wiley Periodicals, Inc. J Appl Polym Sci 118: 1349-1355, 2010″
“The effect of negatively charged liposome on in vitro synthesis of a reporter protein, green fluorescent protein (UP), was investigated using a cell-free translation system. GFP was expressed with and without negatively charged liposome prepared with 1-palmitoyl-2-oleoyl-sn-glycero-3-phosphatidylchorine (POPC) and 1-palmitoyl-2-oleoyl-sn-glycero-3-phosphoglycerol (POPG), resulting in the GFP fluorescence

being reduced BEZ235 to approximately 60% in the presence of POPC/POPG at more than 30% POPG, depending oil its concentration. However, the amount of synthesized GFP products, as analyzed by SDS-PAGE, did not change with and without the POPC/POPG liposome. The results of the ultrafiltration operation indicate that the liposome interacts not only with synthesized polypeptide GFP but also with folded synthesized GFP (mature GFP). Liposome also inhibited refolding of unfolded GFP to its native state. The above results show that the POPC/POPG LY2835219 could inhibit

the folding of GFP in the post-translational process of the gene expression product. (C) 2009 Elsevier B.V. All rights reserved.”
“This paper demonstrates an electric-field tuning of the ferromagnetic resonance (FMR) responses at millimeter wave frequencies for a monolithic magneto-electric Blebbistatin solubility dmso heterostructure. The layered stack is comprised of c-axis oriented and low loss barium hexaferrite (BaM) and (111) oriented ferroelectric barium strontium titanate (BSTO) layers along with embedded platinum electrode layers, all fabricated by pulsed laser deposition technique. A tunability of the FMR frequency as large as 3.5 MHz/V has been observed at 60 GHz due to application of bias voltages in the range of several volts. The realization of such a large tunability relies on the quasi-lattice-to-lattice contact between the BaM and BSTO layers as well as the high quality of those layers. (C) 2010 American Institute of Physics. [doi: 10.1063/1.3467777]“
“The dynamic rheological and mechanical properties of the binary blends of two conventional high-density polyethylenes [HDPEs; low molecular weight (LMW) and high molecular weight (HMW)] with distinct different weight-average molecular weights were studied. The rheological results show that the rheological behavior of the blends departed from classical linear viscoelastic theory because of the polydispersity of the HDPEs that we used.

In this review we summarize the current knowledge on mechanisms o

In this review we summarize the current knowledge on mechanisms of liver injury in post-transplant hepatitis C, discuss the pros and cons of immunosuppressive agents in this specific setting and describe potential novel approaches to prevent HCV reinfection.”
“Intersubband cavity polaritons in a quantum well waveguide structure are optically generated within less than one cycle of light by a 12-femtosecond near-infrared pulse. Mid-infrared probe transients trace the nonadiabatic switch-on of ultrastrong light-matter coupling and the conversion of bare photons HSP990 cell line into cavity polaritons

directly in the time domain. Future perspectives of room-temperature subcycle control of ultrastrong electron-photon interaction are discussed. (C) 2011 American Institute PHA-848125 mouse of Physics. [doi :10.1063/1.3578341]“
“Polyomavirus-associated nephropathy (PyVAN) is rare in nonrenal solid organ transplantation and only limited information is available from single cases. We describe a 67-year-old female presenting

with hypertension and progressive kidney failure due to PyVAN 60 months after lung transplantation. Plasma BK virus (BKV) loads were 4.85 log(10) copies/mL at diagnosis and cleared slowly over 14 months after switching from tacrolimus, mycophenolate and prednisone to low-dose tacrolimus, sirolimus and leflunomide, the latter being discontinued for anemia and diarrhea. BKV- and JC virus-specific immunoglobulins were detectable prior to transplantation. Only BKV-specific IgG and IgM increased during follow-up. BKV-specific T cells were detectable Mocetinostat research buy in blood following in vitro expansion, but cleared with reincreased sirolimus, yet BKV viremia remained undetectable. We identified eight other cases of PyVAN in nonrenal solid organ transplantation including lung (n = 1), heart (n = 6) and pancreas (n = 1). Overall, diagnosis was later than commonly seen in kidney transplants (median 18 months, interquartile range 10-29). Seven patients were male, five received

triple immunosuppression consisting of tacrolimus, mycophenolate, prednisone. Immunosuppression was reduced in four cases and cidofovir and/or leflunomide administered in five and two cases, respectively. Renal function deteriorated in five requiring hemodialysis in four. We discuss mTOR inhibitors versus cidofovir and leflunomide as potential PyVAN rescue therapy.”
“. Experts from seven countries convened as a Specialist Panel for the Middle East to share information on practical issues relating to the epidemiology, diagnosis and management of chronic hepatitis B (CHB) infection. The Middle East is regarded as a region of high-to-intermediate epidemicity; however, infant vaccination programmes have successfully lowered the prevalence of hepatitis B infection in most countries to that of low-to-intermediate endemicity.

This review aims to put in perspective, the knowledge acquired in

This review aims to put in perspective, the knowledge acquired in the last decade or so on leflunomide for CMV. Evidence suggests that it might have activity against human CMV with good oral bioavailability and, more importantly in the resource-poor setting, is economical. Although the data presented here are not from randomized trials, several relevant observations have been made that could influence future, more structured assessments of the drug. An immune suppressive compound with antiviral features and experimental activity in chronic rejection is an attractive combination

for organ transplantation, and it appears that leflunomide may just fit that niche.”
“Renal dysfunction is reported to be associated with poor outcomes in patients KU-57788 research buy with chronic heart failure (CHF). A recent study showed that learn more acidic urine is related to chronic kidney disease, which is a risk factor for the development of CHF. However, it remains to be determined whether acidic urine is associated with poor outcomes in patients with CHF. We measured urine pH using dipsticks in 537 patients with CHF. Acidic urine was defined as urine

pH a parts per thousand currency sign5.5. Patients were prospectively followed during a median follow-up period of 556 days. There were 145 cardiac events. Prevalence of acidic urine was increased with advancing stage of chronic kidney disease. Patients with acidic urine had a more severe New York Heart Association functional class compared with those with normal urine. In the multivariate Cox proportional hazard analysis, acidic urine was independently associated with poor outcomes Selleck OICR-9429 in patients with

CHF after adjustment of confounding factors. A Kaplan-Meier analysis demonstrated that the rate of cardiac events was higher in patients with acidic urine than in those with normal urine. The presence of acidic urine can reliably identify patients at high risk of future cardiac events in patients with CHF.”
“Objective: To identify the indications for which treatments were promoted, the segments of population targeted, and the type and extent of advertising appeal used for over-the-counter (OTC) products in a Nigerian urban setting.

Methods: Using a cross-sectional design, the content of advertisements for OTC products on radio, television, and billboards in a city in southwestern Nigeria were assessed during a 3-month period. Two coders independently assessed 1,492 advertisements for 49 brands of OTC products (interrater reliability [Cohen's.] = 0.83 [95% CI 0.80-0.90]).

Results: The most frequent indications for OTC products were aches and pain (42.9%), anemia/malnutrition (34.8%), and malaria (22.2%). Of advertisements, 92% were targeted at the primary end user.

In order to review and meta-analyse prospective cohort studies in

In order to review and meta-analyse prospective cohort studies investigating adiponectin concentration and the risk for incident coronary heart disease (CHD) or stroke, a systematic search of MEDLINE, EMBASE GSK1904529A and Cochrane databases was performed. Two independent reviewers selected prospective cohort studies investigating the relationship between adiponectin level and incident CHD or stroke using adiponectin’ and cardiovascular disease’ or stroke’ and their synonyms, excluding patients with clinically manifest vascular disease. Random-effects models were used to calculate pooled relative risks (RRs) and 95% confidence intervals (95% CI).

Generalized least squares regression was used to assess dose-response relationships for adiponectin concentrations from studies that provided RRs solely based upon categorical data regression. In total, 16 prospective cohort studies, comprising 23,919 patients and 6,870 CHD or stroke outcome events, were included SCH 900776 in the meta-analyses. An increase of 1 standard deviation in log-transformed adiponectin did not lower the risk for CHD (RR 0.97; 95% CI 0.86-1.09). A 10gmL-1 increase in adiponectin conferred a RR of 0.91 (95% CI 0.80-1.03) for CHD and a RR 1.01 (95% CI 0.97-1.06) for stroke. In conclusion, plasma adiponectin is not

related to the risk for incident CHD or stroke.”
“The makeup of a new surgical bariatric team may Lonafarnib be associated with a higher number of postoperative complications due to the learning curve. The aim of this study was to evaluate the outcomes during the learning curve of laparoscopic gastric bypass (LGBP) depending on surgeons’ training. A systematic approach was used to review studies from the Pubmed, Embase (Ovid), Cancer Lit, Biomes Central via Scirus, Current Contens (ISI), and Web of Science (SCI) databases. Two reviewers independently screened all titles/abstracts and included/excluded studies based on full copies of manuscripts. The outcomes included were: specific training of the surgeon, postoperative complications (leaks, occlusion, hemorrhage, pneumonia, etc.), mortality, and surgical technique. One reviewer

put data onto an Excel spreadsheet. Statistical analysis was performed with weighted linear regression. We identified 448 citations, of which 120 abstract and 50 full-text publications were reviewed. Fourteen papers were selected. Data from 1,848 patients were included. Eighteen different surgeons were analyzed during their learning curve (including the first author of this study). Surgeons were divided into two groups: (1) without formal laparoscopic bariatric training (13 surgeons) and (2) with formal laparoscopic bariatric training (five surgeons). Postoperative complications were more frequent in group 1: 18.1% (+/- 7.6) vs. 7.7% (+/- 1.96, p = 0.046); also, mortality was more frequent in group 1: 0.57% (+/- 0.87) vs. 0% (p = 0.05).

In addition to high levels of antibiotic resistance, the ability

In addition to high levels of antibiotic resistance, the ability to form biofilms on abiotic surfaces and on in-dwelling devices within the host complicates treatment strategies Selleck AZD2014 and successful outcomes of antibiotic therapy. Despite rapid advances made in recent years in understanding the genomics and virulence of this organism, much remains to be learned regarding the host response to enterococcal infections. In this

study, we investigated the interaction of RAW264.7 macrophages and JAWS II dendritic cells with biofilm and planktonic E. faecalis, in vitro. Specifically, we compared phagocytosis, intracellular survival, secretion of proinflammatory cytokines, and the activation

and maturation of phagocytes. Our results revealed that both macrophages and dendritic cells phagocytize biofilm mode cells at levels equal to or better than their planktonic counterparts. Internalized biofilm bacteria showed relatively greater survival at 24 h in macrophages than in dendritic cells and led to slightly higher expression of phagocyte activation markers. Macrophages infected with biofilm cells also secreted lower levels of proinflammatory cytokines studied. Overall, these results suggest that biofilm E. faecalis may be better adapted to overcome host defenses in vivo.”
“Green vanilla beans were subjected to the traditional curing process in Mexico. buy CB-839 Odor-active potential from beans including 23 compounds was monitored through the analysis of both free and glucosidically bound volatiles. 8 of them were aliphatic aldehydes, acids, alcohol and ketone. 15 molecules were shikimate derivatives in which 13 were detected in glucosylated form. Some glucosides were efficiently hydrolyzed while some others partly or not at all after 90 days of curing. Moreover kinetics of hydrolysis of glucosides were not the same. A major part of glucovanillin was hydrolyzed at the first stages of curing while some other glucosides at the advanced stages. Data support

hypothesis that hydrolysis of glucosides during curing is rather enzymatic origin than chemical one. Free shikimate derivatives or those liberated from glucosides Evofosfamide nmr are prone to chemical or enzymatic interconversions leading to a significant change in the aroma profile of cured vanilla. (C) 2011 Elsevier Ltd. All rights reserved.”
“Ear acupuncture can be a useful mean for controlling migraine pain. It has been shown that a technique called the Needle Contact Test (NCT) can identify the most efficacious ear acupoints for reducing current migraine pain through just a few seconds of needle contact. The majority of the points were located on the antero-internal part of the antitragus (area M) on the same side of pain.

PLA and SP were blended at 175 C in a microcompounder followed by

PLA and SP were blended at 175 C in a microcompounder followed by extrusion of composite fibers and removal of SP from composite fibers by using hydrodispersion to form micropores in the composite fibers. Differential scanning calorimetric studies on unhydrolysed composite fibers showed that SP was partially miscible M PLA. Fourier transform infrared mapping of composite fiber cross sections revealed that SP was randomly dispersed throughout the cross section where the degree of dispersion depended on the SP content. As revealed by the scanning electron

micrographs, the size of the micropores was dependent on the SP content. Micropores on fiber cross sections were observed in fibers above find more 3% SP indicating that at least 3% SP content is needed to produce droplet morphology of SP in these fibers. These results show that SP can be successfully used in a blend with PLA to produce microporous fibers to fabricate three-dimensional nonwoven scaffolds for tissue engineering applications. (C) 2010 Wiley Periodicals, Inc. J click here Appl Polym Sci 117: 3350-3361, 2010″
“Background: Many researchers use observed questionnaire scores to evaluate score reliability

and to make conclusions and inferences regarding quality-of-life outcomes. The amount of false alarms from medical diagnoses that would be avoided if observed scores were substituted with expected scores is interesting, and understanding these differences is important for the care of cancer patients. Using expected scores to estimate the reliability of 95% confidence intervals (CIs) is rarely reported in published papers. We investigated the reliability of patient responses

to a quality-of-life questionnaire and made recommendations for future studies of the quality of life of patients.

Methods: A total of 115 patients completed the EORTC core questionnaire QLQ-C30 (version 3) after radiotherapy. The observed response scores, assumed to be one-dimensional, were summed and transformed into expected scores using the Rasch rating scale model with WINSTEPS software. selleck products A series of simulations was performed using a unified bootstrap procedure after manipulating scenarios with different questionnaire lengths and patient numbers to estimate the reliability at 95% confidence intervals. Skewness analyses of the 95% CIs were compared to detect different effects between groups according to the two data sets of observed and expected response scores.

Results: We found that (1) it is necessary to report CIs for reliability and skewness coefficients in papers; (2) data derived from expected response scores are preferable to making inferences; and (3) visual representations displaying the 95% CIs of skewness values applied to item-by-item analyses can provide a useful interpretation of quality-of-life outcomes.


“Twenty-one mixed-parity (average 2.4 +/- 0.49) crossbred


“Twenty-one mixed-parity (average 2.4 +/- 0.49) crossbred sows and their offspring were used to determine whether sow milk leptin at farrowing was related to neonatal serum leptin and pig growth to weaning. During farrowing, pigs were randomly allotted to suckling (n = 99) or delayed suckling (n = 89) groups, with delayed suckling pigs placed in a buy AZD1480 group pen apart from the dam before suckling. Both groups had access to heat lamps. Colostrum samples were collected no more than

2 h after farrowing the first pig. Blood samples were collected from all pigs approximately 2 h after farrowing was complete; pigs were then ear notched and returned to their dams. Pig BW was recorded at 1.2 +/- 0.04 d of age and again at weaning. Milk and blood serum were collected after centrifugation; leptin concentrations were estimated using RIA. Leptin was detected in colostral milk, as expected, and averaged 46.0 +/- 1.1 ng/mL. Pig serum leptin (P < 0.02) was greater in suckling

pigs than in delayed suckling pigs, averaging 0.69 +/- 0.08 and 0.54 +/- 0.07 ng/mL, respectively. Although male pigs were heavier (P < 0.01) at birth than female pigs (1,507 +/- 52 vs. 1,381 +/- 43 g), ADG to weaning and weaning weights were similar for both sexes, averaging 229 +/- 14 g and 5,829 +/- 323 g, respectively, for all pigs; serum leptin concentrations were not affected by sex of the pig. Milk serum leptin was not associated with litter size, parity, pig birth ACY-241 weight, ADG to weaning, or weaning weight. Suckling status did not influence ADG to weaning or weaning

weight of pigs; neonatal pig serum leptin was not related to birth weight, weaning weight, or ADG to weaning. Poziotinib supplier These results indicate that leptin is not directly related to early neonatal growth in the pig; however, more indepth studies are needed to determine possible indirect or long-term effects of early leptin exposure.”
“Novel information processing techniques are being actively explored to overcome fundamental limitations associated with CMOS scaling. A new paradigm of adaptive electronic devices is emerging that may reshape the frontiers of electronics and enable new modalities. Creating systems that can learn and adapt to various inputs has generally been a complex algorithm problem in information science, albeit with wide-ranging and powerful applications from medical diagnosis to control systems. Recent work in oxide electronics suggests that it may be plausible to implement such systems at the device level, thereby drastically increasing computational density and power efficiency and expanding the potential for electronics beyond Boolean computation.

CONCLUSIONS: NSAP persistence may be associated with a decreased

CONCLUSIONS: NSAP persistence may be associated with a decreased quality of life. Emphasis should be put on providing early counselling and support, with the aim of minimising the long term detrimental side effects of NSAP.”
“Safe and efficient exposure of Descemet membrane is the key to success in deep anterior lamellar keratoplasty. MAPK inhibitor Although widely used, the big-bubble technique has the drawback of difficulty in maintaining appropriate needle insertion depth in the corneal stroma, resulting in injected air sometimes escaping to the peripheral cornea without separation of Descemet membrane. We describe a variation of the big-bubble technique in which air

is injected into the anterior chamber before it is injected into the

stroma. By observing the reflection created on the surface of the air, a needle can be inserted deep into the stroma without puncturing Descemet membrane. This allows safe and efficient separation of Descemet membrane. Moreover, air in the anterior https://www.selleckchem.com/products/citarinostat-acy-241.html chamber can be used as an indicator of successful Descemet membrane separation as air is shifted to the periphery with creation of the big bubble.”
“Irradiation effect in C-60 films induced by 170 keV B ion was investigated by means of Fourier transform infrared (FTIR) and Raman spectroscopies. The damage cross section sigma and the effective damage radius R are deduced from the experimental data of all four IR active modes and evident four Raman active modes of C-60 molecule. The differences on irradiation sensitivity and structural stability of the different active modes of C-60 molecule are compared. The results indicate that T-1u (4) of infrared active mode and A(g) (1) of Raman active mode are most sensitive for B ion irradiation. On the other hand T-1u (2) of infrared active mode and H-g (3) of Raman active mode are comparatively stable under B ion irradiation. (C) 2010 American Institute of Physics. [doi:10.1063/1.3512968]“
“QUESTION UNDER STUDY: To investigate the aetiology and long-term clinical outcomes of patients diagnosed with digital

ischemia.

METHOD: Data of 36 consecutive patients Akt inhibitor presenting with digital ischemia were collected in July 2000 to June 2001 from a vascular referral centre. Demographic data, aetiology, medication and treatment were abstracted from the medical records. Clinical outcomes were assessed at 5 year follow-up including ulcer healing, digital amputation and mortality.

RESULTS: Of the 36 patients, 69.4% were male and the mean age was 55 +/- 14 years. In 15 patients (41.7%) a systemic disease was present and of those 53.3% was due to connective tissue disease. Twelve patients (33.3%) had hypothenar hammer syndrome and in 8 patients (22.2%) no apparent cause was found. Whereas 13 patients (36.1%) presented with rest pain or trophic lesions at baseline, no patients presented with these symptoms at follow-up. At follow-up, 18 (62.

1; 95% confidence interval [CI] 0.8-1.6; P = .7) or NINDS rt-PAtr

1; 95% confidence interval [CI] 0.8-1.6; P = .7) or NINDS rt-PAtrial patients (OR 0.8; 95% CI 0.6-1.3; P=.4), respectively. In subgroup analysis, aspirin treatment failure was not found to be associated with recurrent stroke or with the combined endpoint of stroke and death among categories defined by etio-logic subtype, including those with large artery atherosclerosis. Conclusions: In a post-hoc analysis of 2 randomized ischemic stroke trials, aspirin treatment failure was not found to be associated with an increased risk of recurrent stroke or death.”
“Adenocarcinomas of the ampulla of Vater

and duodenum are more rare than pancreatic cancer and have a better prognosis. However, studies conducted on the management of these cancers, such as adjuvant chemotherapy AZD2014 manufacturer and radiation therapy, are limited by small sample sizes and series that are retrospective. This review evaluates ampullary and duodenal adenocarcinomas with regard to incidence, anatomy, prognostic features, patterns of failure, and the available literature studying adjuvant therapy. (c) 2014 Elsevier Inc. All rights reserved.”
“Laparoscopic sleeve gastrectomy (LSG) has been rapidly accepted as a valuable bariatric procedure before

its effectiveness on weight loss in the long-term is clearly Immunology & Inflammation inhibitor demonstrated. We report a feasibility study including 13 patients undergoing a redo LSG for either progressive weight regain after initial weight loss of insufficient weight loss.

From October 2005 to April 2010, 13 patients

underwent a re-sleeve gastrectomy procedure for progressive weight regain or insufficient weight loss (< 50% of excess weight (EW)) associated with the persistence of the gastric fundus on upper gastrointestinal series.

Mean initial body mass index (BMI) and EW were 44.6 (37-52.9) kg/m(2) and 61.8 (38.2-93.9) kg, respectively. There were ten comorbid conditions in five out of the 13 patients. The revision resulted in a mean BMI, percent of excess weight loss (%EWL), and percentage of excess BMI loss (%EBL) of 32.3 kg/m(2), 50.3%, and 57% at 1 month; 32 kg/m(2), 47.9%, and 54.5 at 6 months; and 27.5 kg/m(2), 71.4%, and 82.8% at 12 months, respectively. There was no morbidity.

Laparoscopic revision of LSG is safe and effective in the short PFTα term to obtain substantial loss of weight and improvement in comorbidities.”
“The aim of the present study is to examine all cases of intraosseous benign ameloblastomas treated between 1970 and 2010 in a single institution and to look for a possible correlation between the histopathological aspects and the demographical and clinical parameters, as well as the treatment outcome. The data of a total number of 44 patients were retrieved from the records. Nine patients were excluded because of doubt about the correct diagnosis (8 patients) or because of an extra-osseous presentation (1 patient).

(PACE 2009; 32:248-260).”
“This systematic review and meta-a

(PACE 2009; 32:248-260).”
“This systematic review and meta-analysis

investigated the use of routine hysteroscopy prior to starting the first IVF cycle on treatment outcome in asymptomatic women. Searches were conducted on MEDLINE, EMBASE, Cochrane Library, National Research Register and ISI Conference Proceedings. The main outcome measures were clinical pregnancy and live birth rates achieved in the index IVF cycle. One randomized and five non-randomized controlled studies including a total of 3179 participants were included comparing BMS-777607 research buy hysteroscopy with no intervention in the cycle preceding the first IVF cycle. There was a significantly higher clinical pregnancy rate (relative risk, RR, 1.44, 95% CI 1.08-1.92, P = 0.01) and LBR (RR 1.30, 95% CI 1.00-1.67, P = 0.05) in the subsequent IVF cycle in the hysteroscopy group. The number needed to treat after hysteroscopy to achieve one additional clinical pregnancy was 10 (95% CI 7-14) and live birth was 11 (95% CI 7-16). Hysteroscopy in asymptomatic woman prior to their first IVF cycle could improve treatment outcome when performed just before commencing the IVF cycle. Robust and high-quality randomized AICAR cost trials to confirm this finding are warranted. (C) 2013, Reproductive Healthcare Ltd. Published by Elsevier Ltd.

All rights reserved.”
“Muscular dystrophy (MD) comprises CYT387 inhibitor a group of diseases characterized by progressive muscle weakness that induces functional deterioration. Clinical management requires the use of a well-designed scale to measure patients’ functional status. This study aimed to investigate the quality of the functional scales used to assess patients with different types of MD. The Brooke scale and the Vignos scale were used to grade arm and leg function, respectively. The Barthel Index was used to evaluate the function of daily living activity. We performed tests to assess the acceptability of these scales. The characteristics of the different types

of MD are discussed. This was a multicenter study and included patients diagnosed with Duchenne muscular dystrophy (DMD) (classified as severely progressive MD), Becker muscular dystrophy (BMD), limb girdle muscular dystrophy (LGMD) and facioscapulohumeral muscular dystrophy (FSHD). BMD, LGMD, and FSHD were classified as slowly progressive MD. The results demonstrated that the Brooke scale was acceptable for grading arm function in DMD, but was unable to discriminate between differing levels of severity in slowly progressive MD. The floor effect was large for all types of slowly progressive MD (range, 20.0-61.9), and was especially high for BMD. The floor effect was also large for BMD (23.8%) and FSHD (50.0%,) using the Vignos scale.