氨苯砜治疗成人线状IgA大疱性皮病一例

患者,女,42岁.周身红斑水疱3个月.经病理和免疫病理确诊为线状IgA大疱性皮病.既往曾使用中等剂量糖皮质激素治疗,效果不佳.经加用氨苯砜后病情得以控制,之后激素逐渐减量之停用,目前单用氨苯砜治疗控制中.     

Facial transplantation: adding to the reconstructive options after severe facial injury and disease

The ability to reconstruct complex facial injuries is still a considerable challenge despite the development of microsurgical techniques. The reconstructive options for conditions such as panfacial burns are severely limited. The result after multiple surgical procedures in this group is often poor in terms of function and cosmesis. Facial transplantation provides a potential solution, but opinion is currently divided about the extent to which the potential benefits to the quality of life can be justified when weighed against the technical, psychological and immunological risks. This paper reviews the current status of the debate and argues that a rigorous research strategy is the only logical basis for countering the ethical objections to a procedure that offers considerable benefits over existing reconstructive options.     

Intraoperative lateral rectus electromyographic recordings optimized by deep intraorbital needle electrodes

ObjectiveWe demonstrate the advantages and safety of long, intraorbitally-placed needle electrodes, compared to standard-length subdermal electrodes, when recording lateral rectus electromyography (EMG) during intracranial surgeries.MethodsInsulated 25 mm and uninsulated 13 mm needle electrodes, aimed at the lateral rectus muscle, were placed in parallel during 10 intracranial surgeries, examining spontaneous and stimulation-induced EMG activities. Postoperative complications in these patients were reviewed, alongside additional patients who underwent long electrode placement in the lateral rectus.ResultsIn 40 stimulation-induced recordings from 10 patients, the 25 mm electrodes recorded 6- to 26-fold greater amplitude EMG waveforms than the 13 mm electrodes. The 13 mm electrodes detected greater unwanted volume conduction upon facial nerve stimulation, typically exceeding the amplitude of abducens nerve stimulation. Except for one case with lateral canthus ecchymosis, no clinical or radiographic complications occurred in 36 patients (41 lateral rectus muscles) following needle placement.ConclusionsIntramuscular recordings from long electrode in the lateral rectus offers more reliable EMG monitoring than 13 mm needles, with excellent discrimination between abducens and facial nerve stimulations, and without significant complications from needle placement.SignificanceLong intramuscular electrode within the orbit for lateral rectus EMG recording is practical and reliable for abducens nerve monitoring.     

关于中西医结合治疗面瘫患者的护理心得体会

观察面瘫患者中西结合治疗面瘫的临床疗效，配合针对性护理，并总结临床护理经验，促进患者康复。方法：就我院2015年10月---2016年10月72例面瘫患者，在中医整体观的指导下，通过辩证施护，同时结合西医辅以优质的护理，随机将72名患者分为温灸针结合西医治疗辅以优质护理观察组和温灸针组，分别用温灸针结合肌肉注射腺苷钴胺辅以优质的护理和温灸针治疗，每日一次，治疗时间1个月，对比观察两组面瘫临床治疗效果。结果采用中西医结合方法的观察组36例共痊愈30例，显效4例，有效2例，无效0，总有效率100%；温灸组36例，痊愈20例，显效3例，有效4例，无效9例，总有效率75%，通过两组比较，P〉0.05，差异无显著意义。结论在中医整体观指导下，通过辩证施护，同时结合西医治疗辅以优质的护理，均有效果。     

Anatomic approximation approach to correction of transverse facial clefts

Transverse clefts of the oral cavity have significant impacts on both appearance and function. Many methods of repair have been described, but there is no consensus on optimal approach. In addition, dissatisfaction with scars, distortion of appearance, and recurrent deformity have led to complex surgical designs that are difficult to understand and reproduce. We describe a simple approach to repair that is based upon anatomic approximation of lip components and accurate repair of the muscle.Twenty patients underwent repair by the senior author, who devised the approach, and the corresponding author, who adopted it. Eight (62%) patients had right-sided clefts, three (23%) patients had left-sided clefts, and two (15%) patients had bilateral clefts. One patient had an associated branchial cleft remnant, two patients had multiple branchial cleft remnants and tragus deformities, one patient had craniofacial microsomia with microtia, and one patient had a contralateral Tessier 1 cleft. Mean age of the patients at repair was 23 months. All patients achieved normal oral competence, have favorable scars and commissure appearance, and have had no recurrent deformity. None of the patients have required revision.The described surgical approach is reproducible, easy to understand, and can produce favorable outcomes.     

Fetal facial expressions in small-for-gestational-age and growth-restricted fetuses

Objective: To evaluate the frequencies of fetal facial expressions among appropriate-for-gestational-age (AGA), small-for-gestational-age (SGA), and growth-restricted (FGR) fetuses.

Methods: Four-dimensional (4D) ultrasound was used to examine the facial expressions of 50 AGA, 25 SGA, and six FGR fetuses between 28 and 35 weeks of gestation. The frequencies of seven facial expressions during 15-minute recordings were assessed. Comparison of facial expressions among the three groups was performed.

Results: Mouthing was the commonest facial expression at 28–35 weeks, and the frequency of mouthing was significantly higher than those of the other six facial expressions in AGA fetuses. Mouthing was the most frequent facial expression, but there was no significant difference in the frequency among mouthing, smiling and blinking in SGA fetuses. Moreover, mouthing displayed a significantly higher frequency than the other facial expressions, except for yawning, smiling, and blinking in FGR fetuses. However, there was no significant difference in the frequency of each facial expression among the three groups.

Conclusions: Our results suggest that the frequencies of fetal facial expressions are not decreased in either SGA or FGR pregnancies. The absence of a decrease in the frequency of each fetal expression in FGR fetuses may be due to increased brain blood flow because of the brain-sparing effect. Moreover, accelerated maturation and development of the brain function, especially the central dopamine system, might be suspected in SGA and FGR fetuses.     

“Doll #135 with vitiligo”: Are alopecia and vitiligo Barbie worth the hype?

Toy manufacturer Mattel released a new line of diverse Barbie dolls earlier this year, including dolls with alopecia and vitiligo. The new dolls have been widely celebrated, with both media and dermatologists proposing that the dolls could provide significant benefits for the low self-esteem and societal exclusion suffered by children with similar dermatoses. However, the reality may be very different. Here, we present existing research on the impact of diverse dolls on children’s play and psychology to argue that the dolls’ proposed benefits for children with alopecia and vitiligo are unlikely to materialize; rather, alopecia and vitiligo Barbie could prove more harmful than beneficial.     

Congenital infiltrating lipomatosis of the face with lingual mucosal neuromas associated with a PIK3CA mutation

We report the case of a 5-year-old girl with congenital right-sided facial hemihypertrophy and right hemi-macroglossia with lingual mucosal neuromas. The segmental presentation of findings suggested the diagnosis of congenital infiltrating lipomatosis of the face (CILF), which belongs within the PIK3CA-related overgrowth spectrum (PROS). This was confirmed by genetic analysis showing a mosaic mutation in PIK3CA H1047R. CILF/PROS should be considered in the differential diagnosis of mucosal neuromas.     

Effects of Oxytocin on Attention to Emotional Faces in Healthy Volunteers and Highly Socially Anxious Males

Background:

Evidence suggests that individuals with social anxiety demonstrate vigilance to social threat, whilst the peptide hormone oxytocin is widely accepted as supporting affiliative behaviour in humans.

Methods:

This study investigated whether oxytocin can affect attentional bias in social anxiety. In a double-blind, randomized, placebo-controlled, within-group study design, 26 healthy and 16 highly socially anxious (HSA) male volunteers (within the HSA group, 10 were diagnosed with generalized social anxiety disorder) were administered 24 IU of oxytocin or placebo to investigate attentional processing in social anxiety. Attentional bias was assessed using the dot-probe paradigm with angry, fearful, happy and neutral face stimuli.

Results:

In the baseline placebo condition, the HSA group showed greater attentional bias for emotional faces than healthy individuals. Oxytocin reduced the difference between HSA and non-socially anxious individuals in attentional bias for emotional faces. Moreover, it appeared to normalize attentional bias in HSA individuals to levels seen in the healthy population in the baseline condition. The biological mechanisms by which oxytocin may be exerting these effects are discussed.

Conclusions:

These results, coupled with previous research, could indicate a potential therapeutic use of this hormone in treatment for social anxiety.