Smith-Magenis syndrome (SMS) is a syndrome of multiple malformations caused by an interstitial deletion of chromosome 17p11.2. We report the case of an 8-year-old boy with SMS. Down syndrome was ...initially suspected in infancy based on the findings of generalized hypotonia, flat midface, and upslanting palpebral fissures. His sleep had been disturbed since infancy, and self-injurious behavior developed at 2 years. When he was 8 years old, these unusual neurobehavioral features led to suspicion of SMS, and chromosome analysis showed the 17p deletion, which was confirmed by fluorescence in situ hybridization of the SMS region. Bilateral vesicoureteral reflux, grade IV, was found at the same time, and he underwent bilateral ureteroplasty. The postoperative course was smooth and he was discharged with antibiotic prophylaxis. His sleep disturbance improved after treatment with melatonin. A high index of suspicion is needed for the timely diagnosis of SMS. Patients should be thoroughly evaluated for associated complications both at the time of diagnosis and at regular follow-up.
Background: This nationwide study was performed to evaluate the evolution of distributions of patients with COPD according to the 2011 and 2017 Global Initiative for Chronic Obstructive Pulmonary ...Disease (GOLD) guidelines and to assess the concordance between the prescribed medications and the pharmacological management recommended by the two distinct classification systems in Taiwan. Subjects and methods: Data were retrospectively retrieved from stable COPD patients in 11 participating hospitals across Taiwan. Patients were grouped according to GOLD 2011 and 2017 guidelines respectively. Definitions of undertreatment and overtreatment were based on the pharmacological recommendations in the individual guidelines. Results: A total of 1,053 COPD patients were included. The percentages of patients in GOLD 2011 groups A, B, C and D were 18.4%, 40.6%, 6.7% and 34.2%, respectively. When reclassified according to the GOLD 2017, the percentages of group A and B increased to 23.3% and 63.2%, and groups C and D decreased to 1.9% and 11.6%, respectively. Up to 67% of patients in GOLD 2011 groups C and D were reclassified to GOLD 2017 groups A and B. The pharmacological concordance rate was 60.9% for GOLD 2011 and decreased to 44.9% for GOLD 2017. Overtreatment was found in 29.5% of patients according to GOLD 2011 and the rate increased to 46.1% when classified by the GOLD 2017. The major cause of overtreatment was unnecessary inhaled corticosteroids and the main cause of undertreatment was a lack of maintenance long-acting bronchodilators. Conclusion: The distribution of COPD patients in Taiwan was more uneven with the GOLD 2017 than with the GOLD 2011. A pharmacological discordance to the guidelines was identified. Updated guidelines with reclassification of COPD patients resulted in more discordance between prescribed medications and the guidelines. Physicians should make proper adjustments of the prescriptions according to the updated guidelines to ensure the mostly appropriate treatment for COPD patients. Keywords: chronic obstructive pulmonary disease, GOLD guidelines, inhaled corticosteroids, long-acting bronchodilators
Background: This nationwide study was performed to evaluate the evolution of distributions of patients with COPD according to the 2011 and 2017 Global Initiative for Chronic Obstructive Pulmonary ...Disease (GOLD) guidelines and to assess the concordance between the prescribed medications and the pharmacological management recommended by the two distinct classification systems in Taiwan. Subjects and methods: Data were retrospectively retrieved from stable COPD patients in 11 participating hospitals across Taiwan. Patients were grouped according to GOLD 2011 and 2017 guidelines respectively. Definitions of undertreatment and overtreatment were based on the pharmacological recommendations in the individual guidelines. Results: A total of 1,053 COPD patients were included. The percentages of patients in GOLD 2011 groups A, B, C and D were 18.4%, 40.6%, 6.7% and 34.2%, respectively. When reclassified according to the GOLD 2017, the percentages of group A and B increased to 23.3% and 63.2%, and groups C and D decreased to 1.9% and 11.6%, respectively. Up to 67% of patients in GOLD 2011 groups C and D were reclassified to GOLD 2017 groups A and B. The pharmacological concordance rate was 60.9% for GOLD 2011 and decreased to 44.9% for GOLD 2017. Overtreatment was found in 29.5% of patients according to GOLD 2011 and the rate increased to 46.1% when classified by the GOLD 2017. The major cause of overtreatment was unnecessary inhaled corticosteroids and the main cause of undertreatment was a lack of maintenance long-acting bronchodilators. Conclusion: The distribution of COPD patients in Taiwan was more uneven with the GOLD 2017 than with the GOLD 2011. A pharmacological discordance to the guidelines was identified. Updated guidelines with reclassification of COPD patients resulted in more discordance between prescribed medications and the guidelines. Physicians should make proper adjustments of the prescriptions according to the updated guidelines to ensure the mostly appropriate treatment for COPD patients. Keywords: chronic obstructive pulmonary disease, GOLD guidelines, inhaled corticosteroids, long-acting bronchodilators
We report on a highly compact, one diode-one resistor (1D-1R) nanopillar device architecture for SiO sub(x)-based ReRAM fabricated using nanosphere lithography (NSL). The intrinsic SiO sub(x)-based ...resistive switching element and Si diode are self-aligned on an epitaxial silicon wafer using NSL and a deep-Si-etch process without conventional photolithography. AC-pulse response in 50 ns regime, multibit operation, and good reliability are demonstrated. The NSL process provides a fast and economical approach to large-scale patterning of high-density 1D-1R ReRAM with good potential for use in future applications.
In vivo microdialysis experiments were performed on the nucleus accumbens (NAc) during observation of sexual behavior (including motivation and copulation) to determine if there were any changes in ...NAc dopamine (DA) transmission in prenatally stressed (PS) adult male rats. Approximate 37% of control males and 83% of PS males did not exhibit copulation during the sexual behavior tests and no significant changes in NAc DA release were seen during exposure to estrous females. In contrast, both control and PS males that displayed copulatory behavior showed a marked increase in NAc DA release when presented with a sexually receptive female behind a screen and this increased further during actual copulation. The increase in DA release in copulatory PS males was not significantly different from that in sexually active control males. In addition, a similar extent in DA release induced by high potassium perfusate was observed in all rats. These results suggest that prenatal stress may result in a deficit in DA neurotransmission in the NAc and this deficit may possibly cause impaired male sexual behavior in rats.
1 Cardiology Section, Department of Internal Medicine,
National Taiwan University College of Medicine, National Taiwan
University Hospital, 600 Taipei; 2 College of Medicine, National
Cheng Kung ...University, Tainan; and 3 College of Public Health,
National Taiwan University and 4 Department of Surgery,
National Taiwan University Hospital, 600 Taipei, Taiwan
Distension of the
urinary bladder causes an increase in efferent sympathetic activity,
which can precipitate myocardial ischemia. Smoking has been shown to
modulate activities of afferent nerves from the distended urinary
bladder and to impair endothelial function in response to sympathetic
activation. To assess the effect of bladder distension on coronary
dynamics in smokers, we measured epicardial and microvascular responses
in 24 patients with early atherosclerosis (< 50% diameter stenosis).
Patients were classified into habitual smokers ( group 1 ,
n = 14) and nonsmokers ( group 2 ,
n = 10). Habitual smokers were randomized into two
subgroups on the basis of the use of doxazosin, as follows:
subgroup 1A ( n = 7), without administration
of doxazosin before catheterization; subgroup 1B
( n = 7), with dosing doxazosin. In response to bladder distension (mean intravesical pressure 21.5 mmHg), bladder distension significantly decreased coronary diameter at the stenotic segments, coronary blood flow, and increased coronary resistance compared with
baseline values, in subgroup 1A patients. In subgroup
1B patients during bladder distension, coronary diameter, coronary blood flow, and coronary resistance did not show significant changes compared with baseline values. There were significant differences of
coronary diameter at the stenotic segments, coronary blood flow, and of
changes of coronary vascular resistance between subgroup 1A
and group 2 during bladder distension, despite similar
changes in rate-pressure product. The present study showed that urinary bladder distension caused an abnormal vasomotor response of epicardial vasoconstriction and a concomitant increased coronary resistance, which
leads to reduction in coronary blood flow in patients with early
atherosclerosis. Smoking may further impair the response, implying that
smoking has exaggerated response to sympathetic stimulation of conduit
and resistance vessels. The abnormal response was abolished by
pretreated administration of doxazosin, suggesting that the involved
mechanisms are related to 1 -adrenoceptors.
intracoronary Doppler flow; urinary distension
We demonstrate the competition mechanism between the coherent population trapping and two-photon Raman absorption in a ladder-type system of cesium cell. When the Rabi frequency of the probe becomes ...comparable with the effective decay rate, an electromagnetically induced absorption (EIA) dip emerges at the center of the power broadened EIT peak. While the Rabi frequency of the probe exceeds the effective decay rate, a decoherence created by the strong probe field takes place and two-photon Raman absorption dominates the interaction process.
Doubly dressed states in a ladder-type two-photon, three-level coupling system are observed in our system. The quantum nature of the EIT system significantly suppresses Doppler broadening. The ...experiments were performed on a room-temperature atomic cesium cell .The cesium atoms are excited by two linearly polarized fields with parallel polarization. The spacing of the EIT doublet is strongly consistent with the theoretical description of the Mollow spectrum, and the numerical simulation demonstrates that Doppler broadening can be greatly reduced using this detection technique.
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