Loss-of-function mutations in caspase recruitment domain-containing protein 14 (CARD14) are associated with a severe variant of atopic dermatitis

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肱骨髁上骨折合并肱动脉损伤的诊治

目的：为探讨肱骨髁上骨折合并不同类型肱动脉损伤的诊断与治疗。方法：采用交区克氏针固定骨折，应用机械扩张、血管探查、修补、吻合等措施修复肱动脉。结果：16例于术中探查肱动脉，有断裂伤、刺破伤及顽固性痉挛，均采用手术治疗，使前臂血运得到恢复，骨折得到固定。本组除1例因伤后24小时入院出现前臂缺血性肌挛维、1例因严重粉碎性骨折术后肘关节活动中度受限外，14例均恢复良好，结论：肱骨髁上骨折台井脏动脉损伤的后果严重，但通过及时诊断和有效手术治疗、预后良好。     

脑瘫婴儿的早期临床特征及综合治疗预后分析

目的为探讨脑瘫在婴儿期的临床特征、早期发现、早期干预，以减少其后遗症的发生。方法将2005．06～2007．06在我院新生儿科住院的新生儿，NBNA评定总分≤35分者，分为干预组及对照组，干预组进行早期干预，观察两组高危因素、0—3个月出现早期症状、体征及后遗症方面有无明显差异，并随机收集无高危因素组为健康组，观察三组运动发育、姿势反射建立时间的差别。结果高危因素发生情况、0-3个月出现早期症状、体征方面，干预组与对照组无显著性差异，后遗症方面干预组1岁时无一例发生脑瘫，对照组1岁时有3例诊断为脑瘫，癫痫1例，脑积水1例，白内障1例，双侧神经性耳聋1例。结论早期干预，可降低脑瘫发生率，减轻脑瘫程度。对高危儿进行早期干预开始时间越早越好。     

The neural basis of the psychomotor vigilance task

STUDY OBJECTIVE: To identify brain regions underlying the fastest and slowest reaction times on the Psychomotor Vigilance task (PVT) under well-rested conditions, as well as brain regions related to particularly poor performance after sleep deprivation. DESIGN: Subjects took the PVT twice while undergoing functional magnetic resonance imaging: once 12 hours after waking from a normal night of sleep and once after 36 hours of total sleep deprivation (TSD). Session order was counterbalanced. SETTING: UCSD J. Christian Gillin Laboratory for Sleep and Chronobiology (the sleep core of the General Clinical Research Center) and UCSD Magnetic Resonance Institute. PATIENTS OR PARTICIPANTS: Twenty right-handed healthy adults (8 women; age = 27.4 +/- 6.7 years; education = 15.6 +/- 1.5 years). MEASUREMENTS AND RESULTS: After a normal night of sleep, optimal performance was related to greater cerebral responses within a cortical sustained attention network and the cortical and subcortical motor systems. Slow reaction times, particularly after TSD, were associated with greater activity in the "default mode network" consisting of frontal and posterior midline regions. CONCLUSIONS: Optimal performance on the PVT appears to rely on activation both within the sustained attention system and within the motor system. Poor performance following TSD may result from a disengagement from the task and related inattention, and brain regions responsible for this localize within midline structures shown to be involved in the brain's "default mode." Finally, particularly poor performance after TSD may elicit a subsequent attentional recovery that manifests as greater activation within the same regions normally responsible for fast reaction times.     

Voltage clamp of a small muscle membrane area by means of a circular sucrose gap arrangement

Summary A new method is described which permits the measurement of membrane currents of thick muscle fibres (diameter 300 m or more) ofAstacus fluviatilis orBalanus balanus under voltage clamp conditions.The potential difference across a small patch of membrane (60–100 m in diameter) is controlled by connecting a voltage source across it with two external electrodes. One of them is connected to the fluid bathing the muscle fibre. The other, tubular one is in touch with the test area. The current flowing through the electrodes represents the sum of the membrane current flowing across the test area and the leak current flowing in the external fluid between the electrodes. In the first version of the method the leak current is limited by a circular sucrose gap around the test area. In the second, more elaborate method, the leak current is eliminated by a system of two concentric sucrose rings with a guard ring electrode between them. This method permits in addition the measurement of full sized action potentials in the test area.This work has been briefly reported in Cs. fysiol.17, 48 (1968).     

Zur Anwendung des Rektaltemperatur-Todeszeit-Nomogramms am leichenfundort

Summary The application of the rectal temperature time of death nomogram at the scene of death by 11 authors from 6 lego-medical institutes resulted in a standard deviation of the differences between nomographic and real death time of ± 1.3 h in 46 cases (group I) with met requirements and clearly defined points of contact, nevertheless, including 9 cases with a more progressive cooling (0.5 > Q 0.2). In cases with a real death time of more than 4 h (N = 26) the standard deviation was ± 1.0 hours corresponding to permissible variation of 95% of ± 2.0 hours. Consequently, the permissible variation of 95% was much smaller than that suggested by the nomogram. The nomographic death time interval did not agree with the real one in 5 cases out of additional 30 cases with recognizably unsure points of contact (group II).     

颅咽管瘤切除术后常见并发症的处理

目的：研究颅咽管瘤术后的常见并发症及其相应的防治措施。方法：分析110例颅咽管瘤手术后所出现的各种并发症,探讨其产生的原因并总结临床处理过程中的体会。结果：颅咽管瘤切除术后并发症的发生与肿瘤切除程度和术中垂体柄、下丘脑等的受损状况有关。尿崩、高热、水电解质平衡失调和癫痫是颅咽管瘤术后最常见的并发症,其发生率分别为79.1%、47.3%、88.2%和17.3%。结论;耐心细致的术中显微外科操作和严格规范化的术后管理对降低颅咽管瘤术后病残率,提高其术后生存率具有十分重要的临床意义。     

Preoperative evaluation of living renal donors with gadolinium-enhanced magnetic resonance angiography

BACKGROUND: Preoperative evaluation of living renal donors includes an intra-arterial digital subtraction angiography (DSA). Inasmuch as this technique is invasive, uses radiation and an iodine-containing contrast medium, an alternative technique would be preferable. The purpose of this study was to determine the accuracy of gadolinium-enhanced magnetic resonance (MR) angiography in the visualization of renovascular anatomy for the preoperative evaluation of renal donors. METHODS: Twenty-four consecutive potential renal donors underwent gadolinium-enhanced MR angiography before the standard of reference, intra-arterial DSA. Both modalities were evaluated in a blinded manner. The results were correlated with the surgical findings. RESULTS: Three MR angiograms were technically unacceptable because of inadequate breath-hold. The remaining 21 donors had 47 renal arteries, including 5 accessory renal arteries, which were all visualized by MR angiography. MR angiography failed to visualize one case of subtle fibromuscular dysplasia in the distal part of a renal artery. In one donor, a small accessory renal artery, which had not been visualized on DSA, was encountered during nephrectomy. CONCLUSION: Gadolinium-enhanced MR angiography is an accurate minimally invasive method for the detection of accessory renal arteries in the preoperative evaluation of potential renal donors. The accuracy for excluding stenosis in general is high; however, the depiction of stenosis that are located far distally, or in the branch vessels, is less accurate. Advantages of gadolinium-enhanced MR angiography over the currently used method, intra-arterial DSA, are the minimal invasive nature, lower costs, and superiority in detecting venous anomalies, renal cysts, and tumors.