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11.
The suspicion of a diagnosis of Rett syndrome (RTT) is based on clinical criteria that are often not present in the first two stages of the disease, as many of its symptoms will appear at a later age. This sometimes postpones the genetic diagnosis and an early clinical intervention. We present the case of 19-months-old girl who came to the consultation because of an arrest of psychomotor development noticed 5 months earlier without change in sleep pattern, behavior, or social communication. In the observation of 1 hour videotape, she presented subtle stereotypic movements of the face and hands as well as repetitive dystonic posturing of her limbs. A genetic test confirmed the diagnosis of RTT, showing a truncating mutation in the MECP2 gene (R270X). This case confirms that stereotypic movement anomalies, albeit infrequent and subtle, are already present before the regression stage and while maintaining prehension and that, in addition, repetitive dystonic postures may occur. Recognition of these early movement disorders will improve clinicians' ability to perform an earlier diagnosis of RTT. 相似文献
12.
Sally J Wellard RN BA Renal Cert MN Colleen Rolls RN RM Child Hlth Cert BAppSc Grad Dip Child Development MEdSt de Sales Ferguson RN BA BEd MN 《Journal of advanced nursing》1995,21(4):737-742
An Australian school of nursing's attempt to introduce an evaluation process required by management for clinical educators involved in undergraduate preregistration education is discussed Reliance on student feedback and clinical agencies' perceptions of the quality of teaching were seen as inadequate mechanisms for evaluation The evaluation process adopted incorporated observation of the educators together with self-reflection on their performance We conclude that post-briefings were poorly used and generally not reflective Educators relied heavily on their clinical skills and used a limited range of teaching strategies Whilst evaluation of clinical educators was time consuming and therefore costly, it is argued that it is essential to the integrity of the course The evaluation process has provided direction for support and development of clinical educators within the context of the curriculum 相似文献
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Susan Kirk MSc BNurs RGN RM RHV DNCert Caroline Carlisle MSc BA RGN RM DNCert DipCouns RNT Karen A. Luker PhD BNurs RGN RHV DNCert 《Journal of advanced nursing》1997,26(5):1036-1044
A national study was conducted between 1991 and 1994 to explore and describe the changing role of the nurse teacher following the introduction of Project 2000 pre-registration nursing courses. Multiple methods were used to collect data from a wide variety of respondents (nurse teachers, midwife teachers, clinical nurses, health service managers and higher education lecturers). This paper presents the findings relating to the impact of Project 2000 and the move into higher education on the continuing educational needs of nurse teachers. Views on college strategies for staff development, the changing nature of teachers'academic and professional development needs and the problems of the conflicting demands experienced are reported. The research highlights the need for clinical credibility to be clearly defined in relation to nurse teachers and for educational institutions to place more emphasis on teachers'clinical development if the rhetoric of policy is to become a reality. 相似文献
15.
MIA SON JEONG‐OK KONG SANG‐BAEK KOH JAEYOUNG KIM MIKKO HÄRMÄ 《Journal of sleep research》2008,17(4):385-394
We investigated the effects of 12‐hour shift work for five to seven consecutive days and overtime on the prevalence of severe sleepiness in the automobile industry in Korea. [Correction added after online publication 28 Nov: Opening sentence of the summary has been rephrased for better clarity.] A total of 288 randomly selected male workers from two automobile factories were selected and investigated using questionnaires and sleep‐wake diaries in South Korea. The prevalence of severe sleepiness at work [i.e. Karolinska Sleepiness Scale (KSS) score of 7 or higher] was modeled using marginal logistic regression and included theoretical risk factors related to working hours and potential confounding factors related to socio‐economic status, work demands, and health behaviors. Factors related to working hours increased the risk for severe sleepiness at the end of the shift in the following order: the night shift [odds ratio (OR): 4.7; 95% confidence interval (CI): 3.6–6.0)], daily overtime (OR: 2.2; 95% CI: 1.7–2.9), weekly overtime (OR: 1.6; 95% CI: 1.0–2.6), and night overtime (OR: 1.6; 95% CI: 0.8–3.0). Long working hours and shift work had a significant interactive effect for severe sleepiness at work. Night shift workers who worked for 12 h or more a day were exposed to a risk of severe sleepiness that was 7.5 times greater than day shift workers who worked less than 11 h. Night shifts and long working hours were the main risk factors for severe sleepiness among automobile factory workers in Korea. Night shifts and long working hours have a high degree of interactive effects resulting in severe sleepiness at work, which highlight the need for immediate measures to address these characteristics among South Korean labor force patterns. 相似文献
16.
Adenosine and active hyperemia in dog skeletal muscle 总被引:5,自引:0,他引:5
17.
M Avellanet RM Mirapeix D Escudero C Riera JM Domenech-Mateu 《Surgical and radiologic anatomy : SRA》1996,18(4):271-273
Summary We present a case with a characteristic magnetic resonance image (MRI) of bilateral open-lipped schizencephaly and atypical clinical presentation. The patient is still alive and in good health in her forties, she has never presented seizures, and although the motor dysfunction is well correlated with cerebral lobe involvement, neurobehavioral dysfunction is not proportional to the MR image of the cerebral malformation.
Un cas inhabituel de schizencéphalie bilatérale
Résumé Nous présentons un cas de schizencéphalie bilatérale ouverte caractérisé par une présentation clinique atypique et une imagerie par résonance magnétique nucléaire caractéristique. La patiente est encore vivante, en bonne santé, à plus de 40 ans, elle n'a jamais présenté de crise comitiale et, bien que les troubles moteurs soient bien corrélés aux altérations cérébrales, les troubles neuro-comportementaux ne sont pas proportionnels aux images IRM de cette malformation cérébrale.相似文献
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Zelinski-Wooten MB; Slayden OD; Chwalisz K; Hess DL; Brenner RM; Stouffer RL 《Human reproduction (Oxford, England)》1998,13(2):259-267
Large doses of antiprogestin typically disrupt menstrual cyclicity. A
chronic low-dose regimen of the potent new antiprogestin ZK 137 316, which
permits continued menstrual cyclicity but alters gonadal- reproductive
tract activity, was established. Rhesus monkeys received vehicle (n = 6) or
0.01 (n = 8), 0.03 (n = 8) or 0.1 (n = 5) mg ZK 137 316/kg body weight
daily for five menstrual cycles (C-1 to C-5). Oestradiol, progesterone and
gonadotrophin profiles were normal during cycles involving vehicle and 0.01
and 0.03 mg ZK 137 316/kg body weight. In the 0.1 mg/kg group, mid-cycle
oestradiol and gonadotrophin surges, and subsequent progesterone
production, were absent in C-3 and C-5. Ovarian cyclicity was accompanied
by timely menstruation in the vehicle and 0.01 mg/kg groups. By C-3, half
the animals in the 0.03 mg/kg group and all animals in the 0.1 mg/kg group
were amenorrhoeic. A corpus luteum was noted during the mid-luteal phase of
C-5 in the vehicle, 0.01 mg/kg and 0.03 mg/kg groups. Large antral and
cystic follicles were evident in the 0.1 mg/kg group. Thus, a daily
treatment with 0.01 mg/kg ZK 136317 permitted normal menstrual cyclicity in
macaques. While the daily administration of 0.03 mg/kg ZK 136 317 allowed
ovarian cyclicity, menstruation was disrupted in some animals. Increasing
the dose to 0.1 mg/kg antagonized pituitary function and resulted in
anovulation and amenorrhoea. A chronic low-dose regimen of the
antiprogestin ZK 137 316, which permits normal ovarian/menstrual cyclicity,
has potential as a contraceptive in women.
相似文献