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Biomedical subjects

T Webb

Publications and source records attributed to T Webb.

At least 19 recordsLinked to original sources

Prevalence of, and risk factors for, physical ill-health in people with Prader-Willi syndrome: a population-based study.

The medical findings from a population-based study of Prader-Willi syndrome (PWS) are discussed (in which birth incidence of PWS was estimated at 1:22,000 and death rate at over 3% per annum). In this study the prevalence of specific medical disorders that might account for a shortened life expectancy were investigated. Of all people with a possible diagnosis of PWS, only those meeting clinical criteria and/or with a confirmed genetic diagnosis were included in the study. Sixty-six individuals, 40 males and 26 females with a mean age of 19 years (range of 0 to 46 years) agreed to participate in the population-based study group. A prevalence rate of 25% for non-insulin dependent diabetes mellitus (NIDDM) was found in adults. Mean age at onset was 20 years. Those with NIDDM had a higher past maximum body weight and a greater likelihood of positive family history. Nearly 50% across the age groups reported a history of recurrent respiratory infections. High rates of fractures (29%), leg ulceration (22% in adults), sleep disorders (20%), and severe scoliosis (15% in childhood) were also reported. It is postulated that hypotonia is a possible contributory factor to the risk of strabismus, scoliosis, and respiratory infections. Other causes of morbidity, in particular the high rates of NIDDM, may be due to a failure to manage over-eating resulting in severe obesity. Early diagnosis and clear guidance to families about these risks and how they might be prevented is recommended. It is hypothesized that the high pain threshold may result in the presence of some illness not being apparent.

Adolescent↗

Prader-Willi syndrome, compulsive and ritualistic behaviours: the first population-based survey.

BACKGROUND: Obsessive-compulsive disorder has been reported in association with Prader-Willi syndrome. AIMS: To report the nature and prevalence of compulsive and similar symptoms associated with Prader-Willi syndrome in a population ascertained as completely as possible. METHOD: Attempted complete ascertainment of people with Prader-Willi syndrome in eight English counties. Administration of standardised rating scales and a structured interview. Comparison with people with learning disability and high body mass indices. RESULTS: Prader-Willi syndrome was associated with high rates of ritualistic behaviours, such as the need to ask or to tell something, insistence on routines, hoarding and ordering objects and repetitive actions and speech, compared with the control group, and was negatively correlated with IQ and socialisation age. Typical obsessive-compulsive symptoms, such as checking, counting and cleaning compulsions or obsessional thoughts, were not found. CONCLUSIONS: Ritualistic and compulsive behaviours occur more frequently in association with Prader-Willi syndrome than among people with intellectual disability and significant obesity.

Adolescent↗

Guidelines for reporting clinical features in cases with MECP2 mutations.

An international group recommends that papers relating phenotypes to genotypes involving mutations in the X chromosome gene MECP2 should provide a minimum data set reporting the range of disturbances frequently encountered in Rett Syndrome. A simple scoring system is suggested which will facilitate comparison among the various clinical profiles. Features are described which should prompt screening for MECP2 mutations.

Chromosomal Proteins, Non-Histone↗

The effect of timing of dolasetron administration on its efficacy as a prophylactic antiemetic in the ambulatory setting.

UNLABELLED: Dolasetron (12.5 mg IV) is effective in both preventing and treating postoperative nausea and vomiting (PONV) after ambulatory surgery. However, the optimal timing of dolasetron administration and its effect on the patient's quality of life after discharge have not been established. One-hundred-five healthy, consenting women undergoing gynecologic laparoscopic procedures with a standardized general anesthetic technique were enrolled in this randomized, double-blinded study. Group 1 received dolasetron 12.5 mg IV 10-15 min before the induction of anesthesia; Group 2 received dolasetron 12.5 mg IV at the end of the laparoscopy (79 +/- 48 min later than Group 1); and Group 3 received dolasetron 12.5 mg IV at the end of anesthesia (93 +/- 52 min later than Group 1). The incidence of PONV, complete responses (defined as no emetic episodes and no rescue medication within the 24-h period after anesthesia), recovery profiles, and patient satisfaction were recorded. In the postanesthesia care unit and during the 24-h follow-up period, the incidence of nausea and vomiting, as well as the need for rescue antiemetics, did not differ significantly among the three groups. The percentages of patients with complete responses to the study drug within the first postoperative 24 h were also similar in all three groups (55%, 59%, and 52% for Groups 1, 2, and 3, respectively). The early and intermediate recovery profiles, including resumption of a normal diet and patient satisfaction with the control of PONV, were not different among the three study groups. Dolasetron 12.5 mg IV administered before the induction of anesthesia is as effective as dolasetron given at the end of laparoscopy or at the end of anesthesia in preventing PONV after outpatient laparoscopy. IMPLICATIONS: The timing of dolasetron administration appears to have little effect on its efficacy when administered as a prophylactic antiemetic in the ambulatory setting.

Adult↗

The recovery of cognitive function after general anesthesia in elderly patients: a comparison of desflurane and sevoflurane.

UNLABELLED: We evaluated the cognitive recovery profiles in elderly patients after general anesthesia with desflurane or sevoflurane. After IRB approval, 70 ASA physical status I-III consenting elderly patients (> or =65 yr old) undergoing total knee or hip replacement procedures were randomly assigned to one of two general anesthetic groups. Propofol and fentanyl were administered for induction of anesthesia, followed by either desflurane 2%-4% or sevoflurane 1%-1.5% with nitrous oxide 65% in oxygen. The desflurane (2.5 +/- 0.6 MAC. h) and sevoflurane (2.7 +/- 0.5 MAC. h) concentrations were adjusted to maintain comparable depths of hypnosis using the electroencephalogram bispectral index monitor. The Mini-Mental State (MMS) test was used to assess cognitive function preoperatively and postoperatively at 1, 3, 6, and 24-h intervals. The use of desflurane was associated with a more rapid emergence from anesthesia (6.3 +/- 2.4 min versus 8.0 +/- 2.8 min) and a shorter length of stay in the postanesthesia care unit (213 +/- 66 min versus 241 +/- 87 min). However, there were no significant differences between the Desflurane and the Sevoflurane groups when the MMS scores were compared preoperatively, and postoperatively at 1, 3, 6, and 24 h. Compared with the preoperative (baseline) MMS scores, the values were significantly decreased at 1 h postoperatively (27.8 +/- 1.7 versus 29.5 +/- 0.5 in the Desflurane group, and 27.4 +/- 1.7 versus 29.2 +/- 1.0 in the Sevoflurane group, respectively). However, the MMS scores returned to preoperative baseline levels within 6 h after surgery. At 1 h and 3 h after surgery, 51% and 11% (versus 57% and 9%) of patients in the Desflurane (versus Sevoflurane) Group experienced cognitive impairment. In conclusion, desflurane is associated with a faster early recovery than sevoflurane after general anesthesia in elderly patients. However, recovery of cognitive function was similar after desflurane and sevoflurane-based anesthesia. IMPLICATIONS: Desflurane was associated with a faster early recovery than sevoflurane after general anesthesia in elderly patients. However, recovery of cognitive function was similar with both volatile anesthetics.

Aged↗

Ovarian hormones modulate cocaine-induced locomotor and stereotypic activity.

Interactions between ovarian hormones and cocaine may underlie gender and estrous cycle differences in cocaine-induced behavioral and neurochemical alterations. The aim of this study was to further understand how ovarian hormones modulate cocaine-induced behavioral alterations. Ovariectomized rats received acute or chronic saline or cocaine (15 mg/kg i.p.) administration and were further subdivided into one of four hormone-treatment conditions: cholesterol (vehicle-control), estrogen, progesterone, or estrogen + progesterone. Overall, acute and chronic cocaine administration increased all locomotor measurements (total locomotor, ambulatory, and rearing counts). Estrogen administration augmented cocaine-induced increases in ambulatory and rearing activity. After chronic cocaine administration, rats in the vehicle-control group developed behavioral tolerance (exhibited by a decrease in activity) in rearing and ambulatory activity. Estrogen replacement not only prevented the development of tolerance in ambulatory and rearing activities, but also enhanced total locomotor activity (sensitization) in response to chronic cocaine. Progesterone treatment did not alter the behavioral responses to acute or chronic cocaine administration. Estrogen + progesterone-treated animals had higher counts of locomotor activity in response to chronic cocaine than did vehicle-control or progesterone-treated rats. In stereotypic behaviors, the different hormonal treatments did not affect activity in cocaine- or saline-treated rats after acute or chronic drug administration. Plasma levels of cocaine did not change after different hormonal treatments. Interestingly, animals' coadministered chronic cocaine and estrogen had higher levels of corticosterone than did nonestrogen cocaine-treated rats. Thus, it is likely that alterations in HPA activation may underlie the observed behavioral differences. In summary, these data suggest that there are interactions between ovarian hormones and cocaine-induced behavioral alterations in female rats, and they extend previous results by showing that estrogen and progesterone affect the development of sensitization.

Animals↗

Feeling the effects of PPS. One year later.

The introduction of the PPS system has brought with it many unanticipated challenges and obstacles. It demands a constant reassessment and creative modification of the present and future services, as well as the modes of delivery, without any reduction in the quality of patient care. One agency shares its experience with PPS--one year later.

Aged↗

Familial Prader-Willi syndrome: case report and a literature review.

Prader-Willi syndrome (PWS) is a neurobehavioural disorder arising through a number of different genetic mechanisms. All involve loss of paternal gene expression from chromosome 15q11q13. Although the majority of cases of PWS are sporadic, precise elucidation of the causative genetic mechanism is essential for accurate genetic counselling as the recurrence risk varies according to the mechanism involved. A pair of siblings affected by PWS is described. Neither demonstrates a microscopically visible deletion in 15q11q13 or maternal disomy. Methylation studies at D15S63 and at the SNRPN locus confirm the diagnosis of PWS. Molecular studies reveal biparental inheritance in both siblings with the exception of D15S128 and D15S63 where no paternal contribution is present indicating a deletion of the imprinting centre. Family studies indicate that the father of the siblings carries the deletion which, he has inherited from his mother. The recurrence risk for PWS in his offspring is 50%.

Alleles↗

Reversal of rapacuronium block during propofol versus sevoflurane anesthesia.

UNLABELLED: We studied the antagonism of rapacuronium with edrophonium-atropine during propofol- or sevoflurane- based anesthesia in 60 healthy outpatients. After the induction of anesthesia with standardized doses of propofol and fentanyl, rapacuronium 1.5 mg/kg was administered to facilitate tracheal intubation. Patients were randomized to receive either a propofol infusion (100 microg. kg(-1). min(-1)) or sevoflurane (1.0%, end-tidal) in combination with nitrous oxide 66% for maintenance of anesthesia. Neuromuscular block was monitored by using electromyography at the wrist and reversed with edrophonium 1.0 mg/kg and atropine 0.015 mg/kg when the first twitch (T(1)) response of the train-of-four (TOF) stimulation recovered to 25% of the baseline value. The clinical duration of action (i.e., time to 25% T(1) recovery) was similar during both propofol (13.1 +/- 3.6 min) and sevoflu-rane (13.7 +/- 4.4 min) anesthesia. The time from 25% T(1) recovery to TOF ratio of 0.8 was also similar with propofol (3.4 +/- 2.1 min) and sevoflurane (5.9 +/- 8.7 min) (P > 0.05). Although none of the patients in the propofol group required more than 9 min to achieve a TOF ratio of 0. 8, two patients receiving sevoflurane required 31 min and 37 min. Adequate antagonism of rapacuronium block with edrophonium can be achieved within 10 min during propofol anesthesia. However, more prolonged recovery may occur in the presence of sevoflurane. IMPLICATIONS: We studied the reversal of rapacuronium-induced block with edrophonium and found that the residual rapacuronium block can be readily antagonized during propofol-based anesthesia. However, reversal of rapacuronium appears to be less predictable during sevoflurane-based anesthesia.

Adolescent↗

Spontaneous recovery profile of rapacuronium during desflurane, sevoflurane, or propofol anesthesia for outpatient laparoscopy.

UNLABELLED: We evaluated the spontaneous recovery characteristics of rapacuronium during desflurane-, sevoflurane-, or propofol-based anesthesia in 51 consenting women undergoing laparoscopic tubal ligation procedures. After the induction of the anesthesia with standardized doses of propofol and fentanyl, 1.5 mg/kg IV rapacuronium was administered to facilitate tracheal intubation. Patients were randomized to receive either 1 minimum alveolar anesthetic concentration of desflurane, 1 minimum alveolar concentration of sevoflurane, or 100 microg. kg(-1). min(-1) propofol infusion in combination with 66% nitrous oxide in oxygen for maintenance of anesthesia. Neuromuscular blockade was monitored at the wrist by using electromyography. The degree of maximum blockade and the times for first twitch recovery (T(1)) to 5%, 25%, 50%, 75%, and 90%, as well as the recovery index, were similar in all three anesthetic groups. However, recovery times for the train-of-four ratio to achieve 0.7 and 0.8 were significantly longer with desflurane (44.4 +/- 18.9 and 53.5 +/- 22.4 min) and sevoflurane (44.8 +/- 15.1 and 53.2 +/- 15.8 min) compared with propofol (31.8 +/- 5.3 and 36.5 +/- 6.5 min). Eight patients (16%) required a maintenance dose of 0.5 mg/kg rapacuronium and reversal of rapacuronium residual block occurred in three (6%) patients. We conclude that spontaneous recovery after an intubating dose of 1.5 mg/kg rapacuronium was significantly prolonged by both desflurane and sevoflurane compared with propofol-based anesthesia. Routine monitoring of neuromuscular activity is recommended even when a single bolus dose of rapacuronium is administered during ambulatory anesthesia. IMPLICATIONS: When administered for laparoscopic surgery, the duration of action of an intubating dose of rapacuronium was prolonged 40%-50% by desflurane and sevoflurane, respectively, (versus propofol). Monitoring recovery of neuromuscular blockade produced by rapacuronium is particularly important when desflurane or sevoflurane is administered to ensure that an adequate recovery (train-of-four > or = 0.8) is achieved by the end of anesthesia.

Adult↗

Violence and its injury consequences in American movies: a public health perspective.

OBJECTIVES: To evaluate the seriousness and frequency of violence and the degree of associated injury depicted in the 100 top-grossing American films of 1994. METHODS: Each scene in each film was examined for the presentation of violent actions on persons and coded by a systematic context-sensitive analytic scheme. Specific degrees of violence and indices of injury severity were abstracted. Only actually depicted, not implied, actions were coded, although both explicit and implied consequences were examined. RESULTS: The median number of violent actions per film was 16 (range, 0-110). Intentional violence outnumbered unintentional violence by a factor of 10. Almost 90% of violent actions showed no consequences to the recipient's body, although more than 80% of the violent actions were executed with lethal or moderate force. Fewer than 1% of violent actions were accompanied by injuries that were then medically attended. CONCLUSIONS: Violent force in American films of 1994 was overwhelmingly intentional and in 4 of 5 cases was executed at levels likely to cause significant bodily injury. Not only action films but movies of all genres contained scenes in which the intensity of the action was not matched by correspondingly severe injury consequences. Many American films, regardless of genre, tend to minimize the consequences of violence to human beings.

Humans↗