Growth, bone maturation and pubertal development in children with the EMG-syndrome

In 7 patients (5 girls, 2 boys) with the EMG or Wiedemann-Beckwith syndrome, statural growth, bone age (BA), weight and pubertal development were studied longitudinally. Height was above the 90th percentile (%) for chronological age (CA) after age 2 years, reaching an average of 2.5 SD above the mean at or after puberty. Adult or attained height also exceeded significantly (P<0.015) parental (genetic) target height by 13.2 cm on the average. In one girl, adult height prognosis (190 cm) could be reduced to an adult height of 183 cm by high-dose estrogen treatment. In most children, growth velocity remained above the 90th % up to 4–6 years of age and normalized thereafter. In all patients studied, bone age was markedly advanced and particularly so during the first 4 years after birth. Weight was above the 90th–97th % during infancy and early childhood and remained there, appropriate or slightly subnormal for height, until adulthood, except for 3 girls who reached and maintained the 50th % during or after puberty. Spontaneous pubertal development occurred within normal limits for CA and around the 50th % for BA. Except for the marked bone age acceleration, the reason for the increased statural growth and adult height in patients with the EMG syndrome is still unknown.     

Conditional estimation of sensitivity and specificity from a phase 2 biomarker study allowing early termination for futility

Development of a disease screening biomarker involves several phases. In phase 2 its sensitivity and specificity is compared with established thresholds for minimally acceptable performance. Since we anticipate that most candidate markers will not prove to be useful and availability of specimens and funding is limited, early termination of a study is appropriate, if accumulating data indicate that the marker is inadequate. Yet, for markers that complete phase 2, we seek estimates of sensitivity and specificity to proceed with the design of subsequent phase 3 studies. We suggest early stopping criteria and estimation procedures that adjust for bias caused by the early termination option. An important aspect of our approach is to focus on properties of estimates conditional on reaching full study enrollment. We propose the conditional‐UMVUE and contrast it with other estimates, including naïve estimators, the well‐studied unconditional‐UMVUE and the mean and median Whitehead‐adjusted estimators. The conditional‐UMVUE appears to be a very good choice. Copyright © 2008 John Wiley & Sons, Ltd.     

实验性隐睾术后大鼠附睾的反应性变化

本研究描述了实验性隐睾术后大鼠附睾的形态学变化。结果表明,隐睾术后由于腹温作用和附皋管管腔内容物的改变,附睾管上皮细胞的形态和功能明显改变。术后早期上皮细胞出现无丝分裂相,上皮细胞的分泌和吞噬功能加强,胞质内含PAS阳性颗粒的细胞增多。随术后时间的延长,附睾上皮细胞变化逐渐趋于稳定。[     

A New Acceleration Driven Pacemaker: Rate Modulation Versus Normal Sinus Rhythm—Comparison During Treadmill Exercise

The pocing rate response of a new acceleration driven pulse generator (SWING 100, SORIN BIOMEDICA) was compared with simultaneous normal sinus rhythm (NSR) during two different treadmill exercises. This pacemaker has a gravitational acceleration sensor able to discriminate between physical activities and vibrations. Six healthy volunteers (three male, three female; aged 21.7 ± 4,3 years), with the pacemaker strapped to their right infraclavicular area, performed each test three times with different rise response curve (RRC) each time: fast, normal, and slow. The fall response curve used was the same as the rising one during each test. Pacing rates were recorded using the VEGA analyzer (SORIN BIOMEDICA) and compared with simultaneous NSR recorded by a 7-channel ECG recorder (MINGOGRAF 7, SIEMENS), During all tests immediate (within seconds) rapid increase in pacemaker rate was seen up to about 60 seconds, then a slower increase followed thereafter. The mean correlation between pacing rates and NSR during the Bruce tests were 0.7941 ± 0.10, 0.8562 ± 0.14, and 0.8292 ± 0.07; during the discontinous tests 0.7292 ± 0.16, 0.7233 ± 0.10, and 0.7480 ± 0.11 for fast, normal, and slow RRC, respectively. Each 30 seconds, nonsignificant differences were present between pacing rate and NSR during all the discontinuous tests; similar responses were observed only during the first two stages of Bruce tests after which NSR was significantly higher than pacemaker rates. The speed of rise to upper rate was the main difference between the different programs (fast, normal, and slow). The discontinuous tests showed that the pacemaker responds more to speed than to grade. In conclusion, the Swing pacemaker is easy to use and program, fast, reliable, and is able to mimic the normal sinus behavior especially during discontinuous activities.     

Remarkable correlation between increased HLA-DQ antigen positive monocytes and prognosis of renal transplantation

Since cyclosporin A (CsA), a widely used immunosuppressive drug, strongly suppresses interleukin-2 (IL-2) secretion, it is frequently difficult to estimate T lymphocyte activation in early acute rejection. We found that, when evaluated based on HLA-DQ antigen expression, monocyte activation in the peripheral blood of renal transplantation patients was a very sharp parameter in diagosing acute rejection. All of 16 episodes of early acute rejection, which were relatively easily suppressed by steroid pulse therapy, showed a sharp increase in the proportion of HLA-DQ antigen-positive monocytes (DQ⁺ mono) and a quick return of DQ⁺ mono to previous values, along with a fall in serum creatinine levels. Since, however, HLA-DR antigen-positive T lymphocytes (DR ⁺T) were markedly increased over a long period in episodes of therapy-resistant and chronic rejection, their prolonged high value was regarded as a parameter indicative of poor prognosis.     

无创面罩通气BiPAP治疗COPD呼吸衰竭32例临床分析

目的 评价双水平气道正压通气（BiPAP）治疗COPD呼吸衰竭患者的疗效，可行性及相关影响因素。方法 回顾性临床分析随机选取32例COPD呼吸衰竭患者给予BiPAP治疗的临床资料，并与84例常规治疗作对照分析。结果 治疗组及对照组均有效，但治疗组住院时间明显缩短p〈0．05；BiPAP治疗后lh有效，一般情况下，3d后各项指标趋于正常。结论 应用BiPAP治疗有显著的临床疗效，可起到事半功倍的效率，减少住院天数。     

Effects of PEEP on extra vascular lung water and central blood volume in the dog

Twenty-four mongrel dogs were anaesthetized and ventilated mechanically in the supine position. Extravascular lung water (EVLW) and central blood volume (CBV) were measured with a double indicator (dye/cold) dilution technique. Both indicators were detected intravascularly in the aortic root with a fibreoptic thermistor catheter. Seven dogs ventilated with a positive end-expiratory pressure (PEEP) of 1.0 kPa (10 cmH2O) for a short period of time (less than 20 min) displayed no significant change in EVLW as measured with the indicator dilution technique (= EVLWi), while reductions were seen in both CBV (15%, P less than 0.01) and cardiac output (CO-thermodilution technique) (10%, P less than 0.05). Another seven dogs ventilated with a PEEP of 1.0 kPa for 8 h showed a gradual increase in EVLWi. After 8 h, a mean increase of 34% (P less than 0.01) was recorded, and the increase was also verified by post-mortem gravimetric determination of EVLW (= EVLWg), displaying an increase of 61% (P less than 0.01). In five dogs ventilated with zero end-expiratory pressure (ZEEP) for 8 h, no changes in EVLWi, CO, and CBV were observed, and EVLWg was mean 4.39 g/kg body weight (BW). Five additional dogs were sacrificed after 15 min of anaesthesia without catheterization and EVLWg was found to be 4.24 g/kg BW. It is concluded that EVLWi does not change measurably during ZEEP or short periods of PEEP. However, long periods (8 h) of PEEP result in elevated EVLWi values. Gravimetry supports these conclusions.(ABSTRACT TRUNCATED AT 250 WORDS)     

Comparison of the haemodynamic effects of intermittent positive pressure ventilation with high frequency jet ventilation

The cardiorespiratory effects of intermittent positive pressure ventilation and high frequency jet ventilation with and without positive end expiratory pressure were compared in patients following valvular heart surgery (mitral and/or aortic). Twenty patients received intermittent positive pressure ventilation and high frequency jet ventilation with 0, 0.5 and 1.0 kPa positive end expiratory pressure. High frequency jet ventilation was well tolerated. The addition of 1.0 kPa positive end expiratory pressure was associated with preservation of the arterial oxygen tension without any increase in shunt or significant adverse haemodynamic effect. The results are discussed and compared with a previous study of high frequency jet ventilation following aortocoronary bypass graft surgery.     

Pressure stability of nasal CPAP and bilevel devices

Summary Question of the study   Nasal continuous positive airway pressure (CPAP) prevents collapse of the upper airway during sleep in patients with obstructive sleep apnea provided that a positive transmural pressure can be maintained during inspiration. We examined pressure-flow characteristics in seven CPAP and bilevel devices during spontaneous breathing.
Methods   The CPAP devices were set to a pressure level of 9.8  hPa (10  cm H₂O) and adapted to a pneumotachograph using a standard CPAP hose and an outlet valve. We continuously measured flow, volume and pressure during resting ventilation and increasing voluntary hyperventilation and analysed the dependence of the variables on a breath-to-breath basis.
Results   Mean CPAP pressures differed between the devices (9.9 – 10.6  hPa) despite the same settings. In all machines pressure fell during inspiration to 8.4 – 9.8  hPa and increased during expiration to 11.1 – 11.7  hPa. This effect increased with higher flow rates. Maximum expiratory pressures rose to 12 – 19  hPa at peak flow rates of 2 l/s, mean expiratory pressures to 9.5 – 16  hPa. Inspiratory pressures dropped to 8.5 – 4.5  hPa (minimum) and 10.5 – 6.0 (mean). Bilevel devices showed a higher stability than CPAP devices. Pressure swings during the respiratory cycle increased the additional work of breathing.
Conclusions   Due to differences in mean and effective CPAP levels CPAP devices are not simply exchangeable but should be individually adapted. Patients with higher minute ventilation might benefit from more stable CPAP machines. The impact on patients' compliance remains to be evaluated.     

米利酮对豚鼠心乳头肌及主动脉条的作用

米利酮是非洋地黄，非儿茶酚胺类强心药，具有明显的正性肌力和扩血管作用。国外用于治疗难治性心衰及洋地黄中毒的心衰病人。本文用豚鼠乳头肌及主动脉条进行实验，结果表明合成与进口米利酮的正性肌力作用效价相同，对抗去甲肾上腺素缩血管的作用也等同。