Estimating health state utilities in Duchenne muscular dystrophy using the health utilities index and EQ-5D-5L.

BACKGROUND: The progression of Duchenne muscular dystrophy (DMD) is characterized by loss of ambulation, respiratory insufficiency, cardiomyopathy, and early mortality. DMD profoundly impacts health-related quality-of-life (HRQoL). However, few health state utility data exist; published utilities tend to be derived from small samples for a limited number of health states and are often based on caregiver-reported patient health status. This study estimated utility values for varied clinical and functional health states in DMD, based on patient-reported health status. METHODS: Individuals with DMD in the US aged 12-40 years completed the EQ-5D (5-level) and Health Utilities Index (HUI) preference-based instruments. Based on responses to a clinical questionnaire, participants self-classified into functional health states according to level of lower and upper limb function, use of respiratory support, and presence of cardiomyopathy. Mean [standard deviation (SD)] utility and EQ-5D visual analogue scale (VAS) scores were estimated according to health state; and median (interquartile range) attribute levels calculated to understand which domains of health are most severely affected in DMD. RESULTS: Of 63 males with DMD, mean (SD) age was 19.8 (6.1) years and 11 (17.5%) were ambulatory. Mean (SD) utility values were 0.92 (0.08; HUI2), 0.84 (0.20; HUI3), and 0.84 (0.13; EQ-5D) for ambulatory patients without cardiomyopathy (n = 10). For non-ambulatory patients with moderately impaired upper limb function, night and daytime ventilation without cardiomyopathy, mean (SD) utilities were 0.49 (0.07) for the HUI2, 0.16 (0.15) for the HUI3 and 025 (0.14) for the EQ-5D. Mean (SD) VAS scores for the same health states were 91 (9) and 83 (21), respectively. In addition to impairments in mobility/ambulation, and self-care, attributes like usual activities and pain also showed notable effects of DMD. CONCLUSIONS: In DMD, although a relationship between disease progression and HRQoL is observed, there is large variability in utility within functional health states, and across instruments. Utility values for less severe non-ambulatory health states described by level of upper limb function are novel. These utility values, derived based on direct patient feedback rather than from caregiver report, are relevant to individuals of varying functional statuses and augment scarce DMD-specific utility data.

Schley's inguinal hernia repair: a single unit's experience of a forgotten technique.

BACKGROUND: This study was done to assess the effectiveness of Schley's inguinal hernia repair. It is a retrospective study analysing the outcome of patients who received the said technique over a period of 1 year at our institution. The minimum and the maximum follow-up periods were 2 and 3 years. METHOD: The patients who underwent the above technique in 2011 were reviewed retrospectively in December 2013 by going through the hospital records and examining the patients in person. RESULTS: 105 patients had undergone surgery in 2011. The majority of them had right-sided hernias and were in 40-59 age group. Two cases of wound infection, one case of haematocele and one hernia recurrence were noted. Operating on the recurrent hernia was straightforward. CONCLUSION: Schley's inguinal hernia repair is an effective technique with favourable outcome.

Reaction mechanisms and rate constants of waste degradation in landfill bioreactor systems with enzymatic-enhancement.

Augmenting leachate before recirculation with peroxidase enzymes is a novel method to increase the available carbon, and therefore the food supply to microorganisms at the declining phase of the anaerobic landfill bioreactor operation. In order to optimize the enzyme-catalyzed leachate recirculation process, it is necessary to identify the reaction mechanisms and determine rate constants. This paper presents a kinetic model developed to ascertain the reaction mechanisms and determine the rate constants for enzyme catalyzed anaerobic waste degradation. The maximum rate of reaction (Vmax) for MnP enzyme-catalyzed reactors was 0.076 g(TOC)/g(DS).day. The catalytic turnover number (k(cat)) of the MnP enzyme-catalyzed was 506.7 per day while the rate constant (k) of the un-catalyzed reaction was 0.012 per day.

Waste degradation and gas production with enzymatic enhancement in anaerobic and aerobic landfill bioreactors.

The presence of lignin is the limiting factor at later stages of biodegradation of municipal solid waste under aerobic or anaerobic conditions. Supplying enzymes into the system could facilitate lignin degradation, thereby aiding anaerobic and aerobic waste degradation processes. A comprehensive set of laboratory experiments were conducted under both anaerobic and aerobic conditions to evaluate the feasibility of using enzymes in accelerating lignin-rich waste degradation. After 30 days of anaerobic operation, MnP and LiP enzyme treated reactors produced 36 and 23 times higher cumulative methane (CH4), respectively, compared to that of the control reactor devoid of enzyme treatments. The carbon dioxide (CO2) yield of MnP enhanced aerobic reactor showed more than two-fold increase.

Sri Lankan fetal/ birthweight charts: validation of global reference for fetal weight and birthweight percentiles.

INTRODUCTION: Small for gestational age (SGA) is defined as birthweight below the tenth centile at a particular gestational week. Birthweight centiles for different populations are varied. Generic reference for fetal weight and birthweight that could be adapted to local populations was recently described. The purpose of this study was to validate the reference for birthweights adapted to the local population. METHODS: This was a prospective validation study done between January 2012 and July 2012 in well dated pregnancies at General Hospital, Ampara. Observed frequencies of birthweights of 5th, 10th, 50th, 90th and 95th percentiles for Hadlock formula, World Health Organization (WHO) global survey data for Sri Lanka and India were calculated. The expected frequencies for each birthweight centile of our study were compared with observed frequencies. RESULTS: A total of 411 patients were recruited and 207 delivered at 40 weeks (40+0-40+6). The mean birth-weight (SD) at 40 weeks of gestation was 3140g (432g). Hadlock formula and WHO reference data for India overestimate and underestimate most of the birthweights respectively. WHO generic reference adapted to Sri Lanka fitted well with our data. The mean birthweight of our population is similar, and the adapted reference range would identify most of the small fetuses correctly. It would also identify almost all the babies with weight above the 90th centile. CCONCLUSIONS: The findings of the study show that the observed distribution of birthweight fitted well with the reference range derived from the WHO global reference range adapted to Sri Lankan population. WHO reference charts can be used effectively in Sri Lankan population.

Effect of enzyme additions on methane production and lignin degradation of landfilled sample of municipal solid waste.

Operation of waste cells as landfill bioreactors with leachate recirculation is known to accelerate waste degradation and landfill gas generation. However, waste degradation rates in landfill bioreactors decrease with time, with the accumulation of difficult to degrade materials, such as lignin-rich waste. Although, potential exists to modify the leachate quality to promote further degradation of such waste, very little information is available in literature. The objective of this study was to determine the viability of augmenting leachate with enzymes to increase the rate of degradation of lignin-rich waste materials. Among the enzymes evaluated MnP enzyme showed the best performance in terms of methane yield and substrate (lignin) utilization. Methane production of 200 mL CH(4)/g VS was observed for the MnP amended reactor as compared to 5.7 mL CH(4)/g VS for the control reactor. The lignin reduction in the MnP amended reactor and control reactor was 68.4% and 6.2%, respectively.