Craniosynostosis,ectopia lentis,and congenital heart defects: Further delineation of an autosomal dominant syndrome with incomplete penetrance

The association of craniosynostosis with ectopia lentis is extremely rare. This was recently reported in monozygotic twin sisters, supporting a genetic etiology for this syndromic association. We report on female first cousins once removed who were born with unilateral coronal synostosis. One cousin also had peripheral pulmonic branch stenosis at birth and was later found to have ectopia lentis and severe myopia. The other cousin had an atrial septal defect, mitral valve prolapse, and only mild myopia. Their intelligence is normal. The inheritance is likely autosomal dominant with variable expression and incomplete penetrance and further defines this syndrome to include congenital heart defects. These findings will have important implications for genetic counseling. © 2001 Wiley‐Liss, Inc.     

杏仁内侧核微量注射AVP和AVPMcAb对兔ET性发热效应的影响

在杏仁内侧核微量注射精氨酸加压素（ＡＶＰ）和精氨酸加压素单克隆抗体（ＡＶＰＭｃ－Ａｂ），观察其对兔内毒素（ＥＴ）性发热效应的影响。结果表明，杏仁内侧核微量注射ＡＶＰ能明显抑制兔ＥＴ性发热效应，注射ＡＶＰＭｃＡｂ则能明显提高兔ＥＴ性发热效应；而将ＡＶＰ和ＡＶＰＭＣＡｂ注射到杏仁外侧核则对兔ＥＴ性发热效应无明显影响。提示：杏仁内侧核也是ＡＶＰ抗热作用的一个重要作用部位。     

Localization and characterization of epidermal growth-factor receptors in the developing rat medial septal area in culture

The presence and binding properties of epidermal growth-factor receptors (EGF-Rs) in different cell types purified from the rat medial septal area in culture were investigated. We report that astrocytes, oligodendrocytes and neurons from this area possess EGF-Rs while microglia do not. EGF-binding sites are detectable on astrocytes derived from the medial septum of both embryonic and neonatal rats. Scatchard analysis of the data for astrocytes from the fetal rats show that EGF specifically binds to both high- (K_d = 7.21 × 10⁻¹⁰ M, B_max = 3602 receptors/cell) and low-affinity (K_d = 3.99 × 10⁻⁸ 10⁻⁸ M, B_max = 6,265 receptors/cell) receptors on these cells. On the other hand, astrocytes purified from neonatal tissue possess a greater number of high-affinity receptors (B_max = 10,938 receptors/cell) when compared with the embryonic astroglia. With time in culture, the number of both types of receptors on neonatal astrocytes decreases. Oligodendrocytes also possess high- and low-affinity EGF-Rs with dissociation constants of 3.25 × 10⁻¹⁰ M and 3.85 × 10⁻⁸ M, respectively. The number of receptors on oligodendrocytes is significantly lower than those on neonatal astrocytes (B_max = 1185 and 25,081 receptors/cell for high- and low-affinity binding sites, respectively). Finally, neurons from this area also exhibit two different EGF-R types with dissociation constants similar to those described for astrocytes. As the number of receptors/neuron (B_max = 136 and 1159 receptors/cell for high- and low-affinity binding sites, respectively) appears to be extremely low, it is possible that EGF specifically binds only to a subpopulation of neurons from this area. These studies demonstrate which cell types in the developing medial sepal area posses EGF-Rs and provide a detailed characterization of these binding sites. These EGF-R-bearing cells may be potential targets for this growth factor or for transforming growth factor α in this brain area.     

腓动脉皮支逆行岛状皮瓣的解剖与应用改进

目的探讨腓动脉皮支逆行岛状皮瓣的解剖学基础及临床应用效果。方法采用新鲜及防腐尸体下肢共25肢,对腓动脉皮支进行解剖学观测。于2003年9月~2005年6月收治足跖趾关节处软组织缺损患者10例,男8例,女2例。年龄21~55岁。其中背侧6例,跖侧4例,缺损范围10cm×6cm~15cm×10cm。应用带有外踝上11.0±1.7cm腓动脉固定皮支的腓动脉皮支逆行岛状皮瓣修复,皮瓣范围11.0cm×6.5cm~16.0cm×11.0cm。供区6例直接减张缝合,4例行自体游离皮片覆盖。结果解剖学观测外踝上11.0±1.7cm自腓动脉发出一固定皮支,起始点直径1.45±0.12mm,入深筋膜点距腓肠神经营养血管皮瓣轴线15.70±1.20mm。10例患者术后皮瓣均成活,血运良好,无静脉回流障碍;供区愈合良好。术后获随访6~12个月,皮瓣外形良好,质地、色泽与周边相似,足功能恢复良好,两点辨别觉11~18mm。结论腓动脉皮支逆行岛状皮瓣经改进后血运好,逆转距离长,修复范围大,操作简便,为临床修复足中远端软组织缺损提供了一种新方法。     

颊脂体的外科解剖及其临床意义

对30侧成人颊脂体的形态，位置和血液供应进行了观测。颊脂体血供为多源性，主要血管蒂为上颌动脉的颊动脉和面动脉的颊支。依据上述血管蒂的解剖学走向和分布，颊脂体转位可修复邻近的颌面部和口腔软组织缺损。     

Combined use of medial thigh skin flaps and gracilis musculocutaneous flaps for the reconstruction of scrotum

Summary A case of combined use of bilateral medial thigh skin flaps and gracilis musculocutaneous flaps for the reconstruction of a scrotal defect resulting from Fournier's gangrene is presented. The procedure provided a sac-like scrotum, and the donor sites were successfully reconstructed using bilateral gracilis musculocutaneous V-Y advancement flaps.     

Excitability properties of medial forebrain bundle axons of A9 and A10 dopamine cells

A9 and A10 units identified as dopaminergic were recorded with extracellular micropipettes. The units were antidromically activated by electrical stimulation at the level of the preoptic area. The absolute refractory periods ranged from 1.2 to 2.5 ms. During the 2–8 ms of the relative refractory period, conduction was slower than normal by up to 1.5 ms. The time constant, C, of the strength-duration curve ranged from 0.4 to 0.6 ms. The current (I)-distance (D) relationship, tested by moving the stimulating electrode past the axon, was approximately parabolic (I = K D exp 2), with the constant of the equation, K, ranging from 900 to 2000 μA/mm exp 2, for 0.5 ms pulses. This relationship allows calculation of the radius of the field of dopamine axon excitation at any current. These high K values show that axons of dopamine cells cannot be activated unless high current densities are derivered, even when electrodes are placed near the axons. These data allow determination of the extent to which dopamine axons can be the directly activated substrates for behaviors, such as self-stimulation and circling, which are evoked by electrical stimulation of the medial forebrain bundle or internal capsule.     

尺神经腕背支营养血管逆行皮瓣的临床应用

目的探讨应用尺神经腕背支营养血管逆行皮瓣修复小指皮肤缺损的临床效果及手术操作要点。方法以尺神经腕背支营养血管远端为蒂,选择小指尺侧分支为皮瓣轴心血管,将皮瓣向小指远端转移,修复小指掌背侧皮肤缺损8例。结果8例全部成活,其中1例因创面止血不彻底,皮瓣受压导致远端部分坏死,经换药后痊愈。结论尺神经腕背支走向较恒定,本组未发现变异。沿皮神经干有纵行的皮神经旁血管网及皮神经干内血管网,此皮瓣血供可靠的,皮瓣切取容易,对供区影响小,是修复小指皮肤缺损的理想方法。     

Vasopressin receptors in the mouse (Mus musculus) brain: sex-related expression in the medial preoptic area and hypothalamus

We have investigated the distribution of vasopressin binding sites in the brain of male and female adult mice using a radio-iodinated ligand and film autoradiography. Vasopressin receptors were uncovered in various regions of the brain including the basal nucleus of Meynert, the substantia innominata, the hypothalamic paraventricular nucleus, the substantia nigra pars compacta and the hypoglossal nucleus. A sex-related difference in the expression of vasopressin receptors was seen in the medial preoptic area/anterior hypothalamus corresponding to the rat sexually dimorphic nucleus in the rat and in the hypothalamic mammillary nuclei. In both structures the autoradiographic labeling is more intense in females than in males. These observations confirm that vasopressin binding sites are present in the hypothalamic preoptic area of most species examined so far and that sex-related expression of neuropeptide receptors could trigger sex-related behavioral differences.     

Patients with heart attacks are not valid models for medial temporal lobe amnesia. A neuropsychological and FDG-PET study with consequences for memory research

Equating the condition after cardiac arrest with that of medial temporal damage, and consequently medial temporal lobe amnesia, is questioned on the basis of results from a patient who was studied neuropsychologically as well as with static and dynamic imaging methods (MRI, PET) 6–9 months after a heart attack. The patient manifested severe and persistent anterograde and retrograde amnesia, as well as further cognitive deteriorations. While MRI only indicated non-specific cortical atrophy, PET revealed a severe bilateral affection of the thalamus and of both medial and lateral temporal cortices as well as occipito-parietal hypometabolism. The neuropsychological status indicates that patients with a diagnosis of cardiac arrest may suffer very severe and persistent cognitive deficits; the imaging analyses show that cardiac arrests may lead to quite severe and widespread brain damage which, however, may not be visible with current magnetic resonance imaging technology, but which is clearly apparent from positron emission tomography. These data suggest that patients with a condition after a heart attack may not be valid models for pure hippocampal—or even medial temporal lobe—pathology, as they may suffer much more widespread brain damage.