Diagnosis of Major Organic Acidurias in Children: Two Years Experience at a Tertiary Care Centre

Organic acid disorders are inherited metabolic disorders in which organic acids accumulate in tissues and biological fluids of affected individuals. Classical organic acidurias include methylmalonic aciduria, propionic aciduria, isovaleric aciduria and maple syrup urine disease (MSUD). They are considered the most frequent metabolic disorders among severely ill children. Patients frequently present with acute symptoms early in life. 420 cases clinically suspected to have organic aciduria, with upper age limit of 12 years for a 2-year period (January 2007–December 2008) were enrolled into this study. Metabolic acidosis and neurological symptoms were the most common signs. Screening tests and thin layer chromatography were done for detection of organic acidurias. Identification and quantitation of organic acids in urine and quantification of amino acids in blood were done by high performance liquid chromatography. Out of 420 patients, 45 patients (10.7%) were found to have organic acidurias. 15 cases of methylmalonic aciduria, 16 cases of propionic aciduria, 13 cases of MSUD, and one case of isovaleric aciduria were diagnosed. Results demonstrate the importance of testing for organic acidurias. Since organic aciduria may cause irreversible brain damage if not treated, we recommend selective screening amongst severely ill children despite implied extra costs.     

The Hungarica Program of the National Széchényi Library (Budapest,Hungary)

The author discusses the history and work of the program at the National Széchényi Library to find, document, and collect Hungarica—defined as published in Hungary, written in the Hungarian language, written by a Hungarian author, or written about Hungary or Hungarians—published in Hungary or outside of it. Today both domestically-published and foreign Hungarica are documented in the library's online catalog and in a database of humanities research in Hungarian studies.     

Relationship between laboratory-measured variables and heart rate during an ultra-endurance triathlon

The aim of the present study was to examine the relationship between the performance heart rate during an ultra-endurance triathlon and the heart rate corresponding to several demarcation points measured during laboratory-based progressive cycle ergometry and treadmill running. Less than one month before an ultra-endurance triathlon, 21 well-trained ultra-endurance triathletes (mean +/- s: age 35 +/- 6 years, height 1.77 +/- 0.05 m, mass 74.0 +/- 6.9 kg, = 4.75 +/- 0.42 l x min(-1)) performed progressive exercise tests of cycle ergometry and treadmill running for the determination of peak oxygen uptake (VO2peak), heart rate corresponding to the first and second ventilatory thresholds, as well as the heart rate deflection point. Portable telemetry units recorded heart rate at 60 s increments throughout the ultra-endurance triathlon. Heart rate during the cycle and run phases of the ultra-endurance triathlon (148 +/- 9 and 143 +/- 13 beats x min(-1) respectively) were significantly (P < 0.05) less than the second ventilatory thresholds (160 +/- 13 and 165 +/- 14 beats x min(-1) respectively) and heart rate deflection points (170 +/- 13 and 179 +/- 9 beats x min(-1) respectively). However, mean heart rate during the cycle and run phases of the ultra-endurance triathlon were significantly related to (r = 0.76 and 0.66; P < 0.01), and not significantly different from, the first ventilatory thresholds (146 +/- 12 and 148 +/- 15 beats x min(-1) respectively). Furthermore, the difference between heart rate during the cycle phase of the ultra-endurance triathlon and heart rate at the first ventilatory threshold was related to marathon run time (r = 0.61; P < 0.01) and overall ultra-endurance triathlon time (r = 0.45; P < 0.05). The results suggest that triathletes perform the cycle and run phases of the ultra-endurance triathlon at an exercise intensity near their first ventilatory threshold.     

Does student self‐assessment empower or discipline students?

Student self‐assessment is a popular practice for enhancing student empowerment in the assessment process. However, in recent times various writers have questioned whether the practice of student self‐assessment automatically enhances student autonomy. Some writers have even warned that students' participation in the assessment process may discipline, rather than empower, students. How can student self‐assessment be practised in a way that empowers its students instead of disciplining or controlling them? It is argued that student empowerment can only be realized if the ways that power is exercised over students in self‐assessment practices are first understood. This paper examines the issues of power that underlie student self‐assessment practices and analyses how different notions of power enhance or undermine student empowerment. The notion of the teacher's unilateral power as the basis for student self‐assessment is critically examined against three contrasting notions of power in student self‐assessment: sovereign power, epistemological power and disciplinary power.     

Effect of Caffeine Ingestion on Cardiorespiratory Endurance in Men and Women

Abstract

The effect of caffeine ingestion on submaximal endurance performance of 15 females and 13 males was investigated. After completing a [Vdot]O ₂ max test, each subject performed two submaximal cycling tests at approximately 75% of [Vdot]O ₂ max to exhaustion. For the caffeine (C) trials, 300 mg of caffeine was added to 250 ml of decaffeinated coffee and ingested one hour prior to the exercise. The decaffeinated (D) trial involved consuming 250 ml of decaffeinated coffee an hour prior to the test. The C and D trials were administered randomly using a standard double blind design. Physiological parameters were monitored each 9, 10, and 11 minute intervals throughout each trial and averaged. As expected the [Vdot]O ₂ (L · min ^?1 ), [Vdot]E and work outputs (kgm) were significantly (p < 0.001) higher for the males than the females. All other variables, time to exhaustion, [Vdot]O ₂ (ml · kg · min ^?1 ), R, HR, and rating of perceived exertion (RPE) were not significantly (p > 0.05) different between the sexes for either the C or D trials. Time to exhaustion was 14.4 and 3.1% longer for the C trials for the females and males, respectively, however these increases were not significant (p > 0.05). Furthermore, there were no significant differences (p > 0.05) for any of the measured variables during successive 10 minute work intervals between the C and D trials for either sex. These results do not support the general use of caffeine in moderate amounts as an ergogenic aid for either males or females, but from a practical point it appears that caffeine may have an ergogenic effect on specific individuals.     

Pennsylvania's transition to enterprise computing as a study in strategic alignment

We theorize about the strategic alignment of computing with organizational mission, using the Commonwealth of Pennsylvania's efforts to pursue digital government initiatives as evidence. To do this we draw on a decade (1995–2004) of changes in Pennsylvania to characterize how a state government shifts from an organizational to an enterprise perspective regarding computing. We document and analyze the strategic and operational aspects of a series of information and communications technology (ICT) planning efforts to highlight the nature and roles of strategic alignment used to achieve this transition. Findings from this analysis mark the importance of combining several approaches to strategic planning regarding the adoption and application of ICT.     

An analysis of selected kinematic variables in national and elite male and female 100-m and 200-m breaststroke swimmers

The kinematic analysis of competition breaststroke swimming has tended to focus on the mean values of swimming speed, stroke rate and stroke length; values in individual lengths, as well as the start, turns and finish, have largely been ignored. This study includes all such variables and aims to improve the coach's holistic understanding of breaststroke racing by determining the relationships and differences between and within these selected kinematic variables. We also compare 100-m events with 200-m events to determine if there are characteristic differences between them. Competitive breaststroke swimming performances in 100-m events (males: n = 159, finishing time = 65.05 +/- 2.62 s; females: n = 158, finishing time = 74.04 +/- 3.66 s) and 200-m events (males: n = 159, finishing time = 141.47 +/- 6.15 s; females: n = 158, finishing time = 158.66 +/- 7.87 s) were collected and analysed from 12 world, international and national championships. The better 100-m and 200-m breaststroke swimmers were found to demonstrate greater competency in the kinematic variables measured, except stroke kinematics, which were unique to each individual. These findings suggest that coaches should place emphasis on all of the kinematic components in training and that they should attempt to identify the stroke rate to stroke length ratio most appropriate for the individual. Finally, characteristic differences do exist between the 100-m and 200-m events, which has implications for how swimmers might train for each event.