Biomedical subjects
Publications and source records attributed to C Wiles.
We report a simple and efficient technique for the covalent immobilisation of a tris(2,2'-bipyridyl)ruthenium(II) derivative suitable for both chemiluminescence and electrochemiluminescence detection.
We have demonstrated the use of silyl enol ethers in the aldol reaction within a micro reactor. Quantitative conversion of the silyl enol ether to a beta-hydroxyketone was observed in a 20 min period compared to traditional batch systems, where quantitative yields were only obtained when extended reaction times of 24 h were employed.
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We report the clinical features in 27 Australasian patients with Angelman syndrome (AS), all with a DNA deletion involving chromosome 15(q11-13), spanning markers from D15S9 to D15S12, about 3 center dot 5 Mb of DNA. There were nine males and 18 females. All cases were sporadic. The mean age at last review (end of 1994) was 11 center dot 2 years (range 3 to 34 years). All patients were ataxic, severely retarded, and lacking recognisable speech. In all patients, head circumference (HC) at birth was normal but skewed in distribution, with 62 center dot 5% at the 10th centile. At last review HC was around the 50th centile in three patients (12 center dot 5%) while 15 had poor postnatal head growth. Short stature was not invariable, 5/26 (19%) were on or above the 50th centile. Hypotonia at birth was recorded in 15/24 (63%) and neonatal feeding difficulties were recorded in 20/26 (77%). Epilepsy was present in 26/27 (96%) with onset by the third year of life in 20 patients (83%). Improvement in epilepsy was reported in 11/16 patients (69%) with age. An abnormal EEG was reported in 25/25 patients. Hypopigmentation was present in 19/26 (73%). One patient had oculocutaneous albinism. Five patients could not walk independently. Of the remaining 22 who could walk, age of onset of walking ranged from 2 to 8 years. Disrupted sleep patterns were present in 18/21 patients (86%), with improvement in 9/12 patients (75%) over 10 years of age. The clinical features in this group of deletional AS patients were similar to previous reports, but these have not separated patients into subgroups based on DNA studies. In our group of deletional cases, 100% showed severe mental retardation, ataxic movements, absent language, abnormal EEG, happy disposition (noted in infancy in 95%), normal birth weight and head circumference at birth, and a large, wide mouth. These features occurred with a higher frequency than in AS patients as a whole. Our study also provided information on the evolution of the phenotype. The data can act as a benchmark for comparisons of AS resulting from other genetic mechanisms.
UNLABELLED: Thirty-seven ventilator-dependent blunt trauma patients (ISS 36 +/- 15) were randomized at 24 hours after injury to receive parenteral (TPN) (n = 15), enteral (TEN) (n = 12), or parenteral plus enteral (PN/EN) (n = 10) nutrition. The TEN and PN/EN patients had endoscopically placed transpyloric feeding tubes. Patients who had nutritional complications were two TPN (13%), three TEN (25%), and five PN/EN (50%). Enteral complications were tube occlusion (two), failed duodenal intubation (one), patient extubation of feeding tube (one), gastric reflux (two), and abdominal distention (two). Mortality rates were not different between the groups, but were significantly related to the nutrition-associated complications (p = 0.01): four deaths in ten (40%) with complications and one death in 27 (3.7%) without complications. All four deaths associated with complications occurred in the four with gastric reflux or abdominal distention. No deaths occurred in the other 18 TEN or PN/EN patients (p = 0.0001). Of the four deaths, three were associated with ARDS and respiratory infection (75%). CONCLUSIONS: In mechanically ventilated blunt trauma patients, endoscopic transpyloric tube placement and feeding has a substantial failure rate (36%). Intolerance to duodenal feeding has a remarkably high mortality (100%) in patients in whom gut dysfunction may be a manifestation of injury severity or directly affect survival.
A blinded, prospective trial of the effects of branched-chain amino acid (BcAA)-enriched total parenteral nutrition (TPN) versus standard TPN was undertaken in nonseptic noncirrhotic abdominal surgery patients and patients with multiple traumatic injuries. The study reflected data from the immediate 7-day postoperative period. With isocaloric and isonitrogenous input, the BcAA-TPN patients achieved positive nitrogen balance on day 3. Although the urinary nitrogen output was decreased by day 3 in BcAA-TPN, the plasma BcAA levels did not increase until day 6. With no change in 3-methylhistidine urinary excretion, the early nitrogen retention with BcAA-TPN probably reflects a stimulation of protein synthesis. The ability to favorably modulate the metabolic stress response with alternate fuels has become a clinical reality.
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