Ductal paucity and Warkany syndrome in a patient with congenital extrahepatic portocaval shunt

An eleven-year-old clinically dysmorphic and devel-opmentally retarded male child presenting with com-plaints of 5 episodes of recurrent cholestatic jaundice since 3 years of age was evaluated. Imaging revealed features consistent with congenital extrahepatic porto-caval shunt(Abernethy type 1b), multiple regenerative liver nodules and intrahepatic biliary radical dilatation. The presence of ductal paucity and trisomy 8 were con-firmed on liver biopsy and karyotyping. The explanation for unusual and previously unreported features in the present case has been proposed.     

淋巴细胞和Ⅲ型前胶原氨基端肽与肺气肿合并肺纤维化的关系

目的:观察肺气肿合并肺纤维化(CPFE)淋巴细胞亚型的分布和III型前胶原氨基端肽(PIIINP)水平及纤维化程度,探讨其炎症反应、免疫状态与肺纤维化(PF)的关系。方法:对2005-03/2007-03西安交通大学第一附属医院收治的21例CPFE、25例特发性肺纤维化(IPF)19例对照进行如下检测:光镜下观察肺活检标本的病理改变;对支气管肺泡灌洗液(BALF)进行炎细胞计数和分类;流式细胞术(FCM)检测BALF中T淋巴细胞亚型;放射免疫法检测BALF和血清PⅢNP水平。结果:活检病理结果发现,IPF组纤维化率高于CPFE组(P0.01),但两组纤维化率与是否吸烟均不相关(P0.05);BALF炎细胞分类结果显示CPFE组细胞总数和淋巴细胞比例均高于IPF组和对照组(P0.05,P0.01);FCM检测BALF发现CPFE组CD8+T淋巴细胞百分比高于IPF组和对照组(P0.05);CPFE组和IPF组CD4+/CD8+比值均明显低于对照组(P0.01);三组之间CD4+的百分率无统计学差异;CPFE组与IPF组血清PIIINP检测值均高于对照组(P0.01),其中CPFE组血清PIIINP检测值显著低于IPF组(P0.01)。BALF与血清PIIINP水平呈正相关(γ=0.82)。结论:CPFE的PF病变具有自身特点和发展规律,香烟烟雾刺激可能并非其直接或主要致纤维化因素;CPFE肺组织局部具有较IPF和对照明显且以CD8+T淋巴细胞增高为主的炎性反应,抗淋巴细胞炎症和免疫调节治疗可能有助于改善病情;血清PⅢNP可用作早期发现CPFE和监测PF治疗的指标。     

Dual depolarization responses generated within the same lateral septal neurons by TRPC4-containing channels

In the central nervous system, canonical transient receptor potential (TRPC) channels have been implicated in mediating neuronal excitation induced by stimulating metabotropic receptors, including group 1 metabotropic glutamate receptors (mGluRs). Lateral septal (LS) neurons express high levels of TRPC4 and group I mGluRs. However, to what extent native TRPC4-containing channels (TRPC4-cc) are activated as well as the impact of different levels of TRPC4-cc activation on neuronal excitability remain elusive. Here, we report that stimulating LS neurons with group I mGluR agonist, (S)-3,5-DHPG, causes either an immediate increase in firing rate or an initial burst followed by a pause of firing, which can be correlated with below-threshold-depolarization (BTD) or above-threshold-plateau-depolarization (ATPD), respectively, in whole-cell recordings. The early phase of BTD and the entire ATPD are completely absent in neurons from TRPC4^?/? mice. Moreover, in the same LS neurons, BTD can be converted to ATPD at more depolarized potentials or with a brief current injection, suggesting that BTD and ATPD may represent partial and full activations of TRPC4-cc, respectively. We show that coincident mGluR stimulation and depolarization is required to evoke strong TRPC4-cc current, and Na⁺ and Ca²⁺ influx, together with dynamic changes of intracellular Ca²⁺, are essential for ATPD induction. Our results suggest that TRPC4-cc integrates metabotropic receptor stimulation with intracellular Ca²⁺ signals to generate two interconvertible depolarization responses to affect excitability of LS neurons in distinct fashions.     

Behavioral effects of the novel cannabinoid full agonist AM 411

AM 411 ((-)-1-adamantyl-Delta8-tetrahydrocannabinol) is a novel full agonist at cannabinoid CB1 receptors. The present studies were conducted to provide behavioral characterization of this compound in rats. It was hypothesized that AM 411 should produce behavioral effects similar to known cannabinoid agonists, and that these effects should be inhibited by co-treatment with a CB1 antagonist. In Experiments 1 and 2, AM 411 dose-dependently produced behaviors consistent with CB1 agonism, including analgesia, hypothermia, catalepsy and reductions in locomotion, which were blocked by a CB1-selective antagonist. In Experiment 3, AM 411 produced a dose-dependent suppression of lever-pressing on a fixed-ratio 5 (FR5) schedule, a task known to be sensitive to administration of CB1 agonists. Detailed analysis of the temporal patterns of operant responding showed that AM 411 altered the distribution of interresponse times. Experiment 4 showed that AM 411 decreased relative interior activity in the open field, which is suggestive of an anxiogenic effect. It is concluded that AM 411 produces CB1 agonist-like behavior with potency between that of WIN 55,212-2 and AM 356. AM 411 could be a useful tool for understanding the behavioral and neural effects of CB1 receptor stimulation.     

Exposure time reduction of secondary radiographs used in digital subtraction radiography in detecting intrabony change

Objectives

Digital subtraction radiography (DSR) is a suitable technique for detecting incipient bone changes. However, in DSR, one or more follow-up radiographs must be taken. The aim of this study was to assess the possibility of reducing the exposure time for the radiographs that follow the initial one.

Methods

Maxillary premolar and molar radiographic images of a dry skull were taken with a digital radiography system. The initial radiographs, without bone chips, were taken at 0.32 and 0.16 s. Then, five bone chips (weight range 7–15 mg) were placed on the maxillary molar buccal side of the dry skull. Secondary radiographs were taken at 0.32-, 0.16-, 0.08-, 0.04-, and 0.02-s exposure times. For each bone chip, radiographs were taken three times. The secondary and initial images were subtracted to yield subtraction images. Four observers were asked to evaluate bone change visibility in the subtraction images. The Friedman test was used for statistical analysis.

Results

Significant differences were seen at each of the settings for the 0.32-s group (p = 1.24e?030) and 0.16-s group (p = 7.52e?009). By comparing the different groups, observer evaluations indicated that visibility changed when the secondary radiograph was taken at 1/8 of the exposure time of the initial radiograph. In both groups, the visibility of the 0.02-s subtraction image was significantly lower than that of the other subtraction images.

Conclusion

In DSR, the exposure time of the secondary radiograph can be reduced to 1/4 of the exposure time of the initial radiograph.     

Rehabilitation of a missing ear with an implant retained auricular prosthesis

Burns can leave a patient with a severely debilitating disability even after treatment. The objectives of burn rehabilitation are to minimize the adverse effects caused by the injury while rehabilitating the patient''s physical and psychological well-being, maximizing social integration. Long-term success of maxillofacial prostheses mainly depends on the retention. Extra oral implant retained prostheses have proved to be a predictable treatment option for maxillofacial rehabilitation. Replacement of a severely deformed external ear with burned tissues may be satisfactorily accomplished by a cosmetic prosthesis anchored by implants integrated in the skull. The use of such implants is now a well-recognized method for creating a stable result in maxillofacial rehabilitation. This case report describes a safe, simple and economical method for the rehabilitation of a patient with missing right auricle using an implant supported silicone prosthesis. The implant was placed in the mastoid region of the temporal bone. Reconstruction of the ear was done with auricular silicone prosthesis, retained using magnets incorporated in an autopolymerizing resin shim to decrease the weight of the prosthesis on a single implant. This method eliminates the need of tedious laboratory procedures and exact casting and fitting requirements of a metal substructure while minimizing the overall weight and cost of the prosthesis while maintaining adequate support, esthetics and retention of the prosthesis.