Rhinophyma: review and update

Learning Objectives: After studying this article, the participant should be able to discuss: 1. Clinical features and anatomy of rhinophyma. 2. The etiology and epidemiology of rhinophyma. 3. Associated diagnosis that can complicate rhinophyma. 4. Common nonsurgical and surgical therapies for rhinophyma. 5. A safe and integrated treatment plan for the patient with rhinophyma.     

Eating disorders. A review and update.

Anorexia nervosa and bulimia nervosa are prevalent illnesses affecting between 1% and 10% of adolescent and college age women. Developmental, family dynamic, and biologic factors are all important in the cause of this disorder. Anorexia nervosa is diagnosed when a person refuses to maintain his or her body weight over a minimal normal weight for age and height, such as 15% below that expected, has an intense fear of gaining weight, has a disturbed body image, and, in women, has primary or secondary amenorrhea. A diagnosis of bulimia nervosa is made when a person has recurrent episodes of binge eating, a feeling of lack of control over behavior during binges, regular use of self-induced vomiting, laxatives, diuretics, strict dieting, or vigorous exercise to prevent weight gain, a minimum of 2 binge episodes a week for at least 3 months, and persistent overconcern with body shape and weight. Patients with eating disorders are usually secretive and often come to the attention of physicians only at the insistence of others. Practitioners also should be alert for medical complications including hypothermia, edema, hypotension, bradycardia, infertility, and osteoporosis in patients with anorexia nervosa and fluid or electrolyte imbalance, hyperamylasemia, gastritis, esophagitis, gastric dilation, edema, dental erosion, swollen parotid glands, and gingivitis in patients with bulimia nervosa. Treatment involves combining individual, behavioral, group, and family therapy with, possibly, psychopharmaceuticals. Primary care professionals are frequently the first to evaluate these patients, and their encouragement and support may help patients accept treatment. The treatment proceeds most smoothly if the primary care physician and psychiatrist work collaboratively with clear and frequent communication.     

髓源性防御素的研究进展

防御素是一组含有6个半胱氨酸的阳离子抗微生物多肽。在中性粒细胞内,防御素含量丰富,具有广谱、高效的抗微生物活性,是机体天然免疫机制的重要组成部分。近年来的临床观察表明,测定血浆及各种体液中髓源性防御素的改变有助于某些炎症性疾患的诊断。此外,提纯或重组防御素作为“超级抗生素”则已成为药学界的研究热点。本文着重对髓源性防御素的生化特性、生物学活性及其与疾病的关系作一概述。     

Ultrasound angioplasty: an update review

The use of therapeutic ultrasound to treat atherosclerosis and thrombosis has been appreciated for decades. However, it was only the explosive growth of angioplasty in the 1980s that brought real momentum to the development of therapeutic catheter ultrasound. The idea behind this technique was that ultrasound, by its bioselectivity, might provide a solution to some of the shortcomings of balloon angioplasty. In the late 1980s, two groups, headed by Rosenschein and Siegel, began serious work to address the technical challenge of developing a catheter that would provide efficient external ultrasound energy to the lesion. Current catheters from both groups consist of a solid metal probe which is connected to a piezoelectric transducer. In the distal segment, the wire is specially designed to increase energy delivery. Initial in vitro studies concentrated on understanding the mechanisms of ablation and the effects of mechanical vibration, thermal phenomena and cavitation. Clinical studies of ultrasound ablation were initially performed in peripheral vessels. Later, after safety had been assured, clinical studies involving the coronary arteries began to take place. In this article we aim to update the reader about the experimental and limited clinical experience in this novel technique for treating different kinds of arterial obstruction.     

Left posterior fascicular block,state-of-the-art review: A 2018 update

We conducted a review of the literature regarding epidemiology, clinical, electrocardiographic and vectorcardiographic aspects, classification, and differential diagnosis of left posterior fascicular block.Isolated left posterior fascicular block (LPFB) is an extremely rare finding both in the general population and in specific patient groups. In isolated LPFB 20% of the vectorcardiographic (VCG) QRS loop is located in the right inferior quadrant and when associated with right bundle branch block (RBBB) ≥40%.The diagnosis of LPFB should always consider the clinical aspects, because a definite diagnosis cannot be made in the presence of right ventricular hypertrophy (RVH) (chronic obstructive pulmonary disease (COPD)/emphysema), extensive lateral myocardial infarction (MI) or extremely vertical heart.Intermittent LPFBs are never complete blocks (transient or second degree LPFB) and even in the permanent ones, one cannot be sure that they are complete. When LPFB is associated with RBBB and acute inferior MI, PR interval prolongation is very frequent.     

Mitochondrial ROS-induced ROS release: an update and review

Unstable mitochondrial membrane potential and redox transitions can occur following insults including ischemia/reperfusion injury and toxin exposure, with negative consequences for mitochondrial integrity and cellular survival. These transitions can involve mechanisms such as the recently described process, "Reactive Oxygen Species (ROS)-induced ROS-release" (RIRR), and be generated by circuits where the mitochondrial permeability transition (MPT) pore and the inner membrane anion channel (IMAC) are involved. The exposure to excessive oxidative stress results in an increase in ROS reaching a threshold level that triggers the opening of one of the requisite mitochondrial channels. In turn, this leads to the simultaneous collapse of the mitochondrial membrane potential and a transient increased ROS generation by the electron transfer chain. Generated ROS can be released into cytosol and trigger RIRR in neighboring mitochondria. This mitochondrion-to-mitochondrion ROS-signaling constitutes a positive feedback mechanism for enhanced ROS production leading to potentially significant mitochondrial and cellular injury. This review and update considers a variety of RIRR mechanisms (involving MPT, IMAC and episodes of mitochondrial transient hyperpolarization). RIRR could be a general cell biology phenomenon relevant to the processes of programmed mitochondrial destruction and cell death, and may contribute to other mechanisms of post-ischemic pathologies, including arrhythmias.     

细菌中钙信号的作用

摘要：越来越多的实验证明二价钙离子（Ca2+）在细菌中有重要调控作用。本文从Ca2+ 信号对细菌生理的影响、细胞内Ca2+ 浓度及测定方法、细菌中Ca2+ 的运输和Ca2+ 结合蛋白四个方面综述了目前细菌中钙信号的研究进展。     

Acute lymphoblastic leukemia: a comprehensive review and 2017 update

Remission rates remain low among adult patients with relapsed Philadelphia chromosome-negative acute lymphoblastic leukemia (Ph⁻ ALL). The standard 4-week reduction chemotherapy regimen cannot be administered due to serious hematopoietic toxicity and infection during induction therapy, which can decrease the elimination of blast cells. In this study, a modified shortened P-CIOD chemotherapy protocol (comprising pegaspargase, cyclophosphamide, idarubicin, vindesine, and dexamethasone) was used as a reinduction therapy for relapsed Ph⁻ ALL. Remarkably, 80% (4 out of 5) of the patients achieved complete remission (CR) with minimal residual disease (MRD) negativity and without treatment-related mortality. Two patients were lost to follow-up after consolidation treatment, while the remaining three patients survived. The longest disease-free survival (DFS) recorded was 27 months. The results suggest that the novel protocol shows a favorable second complete remission (CR2) with low toxicity for adult patients with relapsed Ph⁻ ALL.     

Secreted frizzled related protein 4 (sFRP4) update: A brief review

Secreted frizzled-related proteins control a multitude of biological phenomena throughout development and adult life in humans. In parallel, aberrant gene expression and abnormal secreted protein levels accompany a wide range of pathologies in humans. In this review, we provide a brief introduction to sFRP4, an update of the pathways it's involved, its various physiological actions that are reported to contribute to diseases, outlining the importance of its wider research and specific modulation by pharmacologic interventions. First recognized as a novel molecule that co-purified with a disparate protein, its identity was based on its sequence homology to the frizzled receptors. Once multiple members of the family were cloned, their genetic loci, tissue and subcellular distributions were located. Nucleotide and amino acid sequences were characterized and homology to different organisms was found to be present that helped elucidate their actions. Following subsequent experimental studies, they were found to be secreted proteins with an affinity to bind to the Wnt ligands, participating in different developmental and adult homeostatic pathways by the virtue of their regulatory function to the Wnt signal transduction system. Secreted frizzled related protein 4 has garnered considerable attention in the recent years following breakthrough discoveries implicating them in the pathogenesis of various diseases. Studies investigating them can provide information not only regarding their association with a disease but can also help use them as potential biomarkers and therapeutic targets.     

Treatment engagement of individuals experiencing mental illness: review and update

Individuals living with serious mental illness are often difficult to engage in ongoing treatment, with high dropout rates. Poor engagement may lead to worse clinical outcomes, with symptom relapse and rehospitalization. Numerous variables may affect level of treatment engagement, including therapeutic alliance, accessibility of care, and a client's trust that the treatment will address his/her own unique goals. As such, we have found that the concept of recovery‐oriented care, which prioritizes autonomy, empowerment and respect for the person receiving services, is a helpful framework in which to view tools and techniques to enhance treatment engagement. Specifically, person‐centered care, including shared decision making, is a treatment approach that focuses on an individual's unique goals and life circumstances. Use of person‐centered care in mental health treatment models has promising outcomes for engagement. Particular populations of people have historically been difficult to engage, such as young adults experiencing a first episode of psychosis, individuals with coexisting psychotic and substance use disorders, and those who are homeless. We review these populations and outline how various evidence‐based, recovery‐oriented treatment techniques have been shown to enhance engagement. Our review then turns to emerging treatment strategies that may improve engagement. We focus on use of electronics and Internet, involvement of peer providers in mental health treatment, and incorporation of the Cultural Formulation Interview to provide culturally competent, person‐centered care. Treatment engagement is complex and multifaceted, but optimizing recovery‐oriented skills and attitudes is essential in delivery of services to those with serious mental illness.     

Prenatal diagnosis of vascular anomalies: update and review of the literature

Optimal care for a subgroup of infants with complicated vascular anomalies requires prenatal diagnosis. Fetal vascular lesions are either vascular tumors or vascular malformations, both of which are often detected on routine ultrasound. Imaging, such as ultrasound and fetal MRI, can be used to examine lesions and provide the data for a differential diagnosis, which may impact the course of care both in utero and postnatally. Prenatal diagnosis provides the opportunity for antenatal intervention, parental counseling, and planning of the mode and location of delivery to optimize postnatal care. Prenatal diagnosis of vascular lesions also serves to alert the physician to the potential for associated syndromes and complications. Any indication of a vascular anomaly should be referred for further examination by an experienced multidisciplinary team of physicians to ensure the window in which evaluation, planning, and treatment can take place is not missed.     

混合性化生性乳腺癌2例临床病理分析并文献复习

目的探讨混合性化生性乳腺癌的临床特征、病理学特征、免疫表型、鉴别诊断、治疗及预后。方法应用光学显微镜及免疫组织化学方法分析2例混合性化生性乳腺癌的临床病理特点及免疫表型,并总结相关文献。结果患者均为老年女性,年龄分别为54岁和50岁。肿瘤大小分别为12cm×11cm×6cm、3.5cm×2.5cm×2cm。一例切面为灰白胶冻样,局部区域呈鱼肉状,另一例质中及灰黄伴局灶坏死。混合性化生性癌的构成成分分别为梭形细胞癌+骨肉瘤、鳞状细胞癌+非特殊型浸润性癌+软骨肉瘤。免疫表型:2例中,癌的成分均表达CK、EMA,其中鳞状细胞癌和梭形细胞癌成分均表达P63;肉瘤成分均表达vimentin、SMA、CK5/6,均不表达ER、PR、Her-2,Ki67增殖指数均较高,分别为40%、60%。结论混合性化生性乳腺癌的报道较少,预后较差,应广泛取材。当组成成分为3种及3种以上时,预后往往更差,应在报告中加以体现,免疫组织化学有助于不同组成成份的鉴别。     

Telemental health: A status update

A rather large body of literature now exists on the use of telemental health services in the diagnosis and management of various psychiatric conditions. This review aims to provide an up‐to‐date assessment of telemental health, focusing on four main areas: computerized CBT (cCBT), Internet‐based CBT (iCBT), virtual reality exposure therapy (VRET), and mobile therapy (mTherapy). Four scientific databases were searched and, where possible, larger, better‐designed meta‐analyses and controlled trials were highlighted. Taken together, published studies support an expanded role for telepsychiatry tools, with advantages that include increased care access, enhanced efficiency, reduced stigma associated with visiting mental health clinics, and the ability to bypass diagnosis‐specific obstacles to treatment, such as when social anxiety prevents a patient from leaving the house. Of technology‐mediated therapies, cCBT and iCBT possess the most efficacy evidence, with VRET and mTherapy representing promising but less researched options that have grown in parallel with virtual reality and mobile technology advances. Nonetheless, telepsychiatry remains challenging because of the need for specific computer skills, the difficulty in providing patients with a deep understanding or support, concerns about the “therapeutic alliance”, privacy fears, and the well documented problem of patient attrition. Future studies should further test the efficacy, advantages and limitations of technology‐enabled CBT, as well as explore the online delivery of other psychotherapeutic and psychopharmacological modalities.     

Teratogen update: vitamin A congeners

Despite animal vitamin A congener teratogenicity in animal studies since 1954, striking human findings only arose in 1983 following isotretinoin (ITR) marketing for oral treatment of severe acne. By November 1985, 44 outcomes with central nervous system (CNS), cardioaortic (CV), microtia, facial palsy, micrognathia, cleft palate, and/or thymic aplasia defects, and 33 spontaneous abortions have been reported. The critical period for exposure appears to be two to five weeks postconception, although this is clinically inexact. ITR half life is less than a day, although a teratogenic metabolite, 4-oxo-isoretinoin, has a half life of several days. Seven defect outcomes and one stillbirth have been reported with another congener, etretinate (ETR), used for psoriasis. Three of these had meningomyeloceles. Half life of several months makes levels cumulative. Only one additional defect, which may have occurred by chance, is reported with use stopped before conception (4 months). Other discontinuations 1 to 6 months before conception had 11 normal outcomes and two spontaneous abortions. ITR and ETR dose ranged from 0.5 to 1.5 mg/kg. Normal outcomes are reported both with ITR and ETR, but some of these appear not to have been exposed during the critical period. Less striking defects, abortions, and normal outcomes are less well reported. Because vitamin A analogs are therapeutically important and unplanned outcome not always avoidable, further animal research is needed for better risk/benefits. Megadose vitamin A (retinol) use is widespread, but experience poorly observed. Eighteen suspicious birth defect outcomes have been reported from pregnancies with high dose exposure. Twelve had findings similar to those seen in animals and in human retinoid syndromes, e.g., CNS, CV, microtia, and clefts. Epidemiological controls are lacking to establish human teratogenicity, but based on animal studies and experience with ITR and ETR, avoiding long term megadose Vitamin A use in fertile women is warranted.     

Calcium regulation in crustaceans during the molt cycle: a review and update

Epithelial cells of the gut, gills, antennal glands and integument regulate calcium concentrations in crustaceans during the molt cycle. A cellular calcium transport model has been proposed suggesting the presence of calcium pumps, cation antiporters and calcium channels in transporting epithelial membranes that regulate the movements of this cation across the cell layer. Basolateral calcium transport during postmolt appears mainly regulated by the low affinity NCX antiporter, while calcium regulating 'housekeeping' activities of these cells in intermolt are controlled by the high affinity calcium ATPase (PMCA). A model is proposed for the involvement of the epithelial ER in the massive transepithelial calcium fluxes that occur during premolt and postmolt. This model involves the endoplasmic reticulum SERCA and RyR proteins and proposed cytoplasmic unstirred layers adjacent to apical and basolateral plasma membranes where calcium activities may largely exceed those in the bulk cytoplasmic phase. A result of the proposed transepithelial calcium transport model is that large quantities of calcium can be moved through these cells by these processes without affecting the low, and carefully controlled, bulk cytoplasmic calcium activities.     

Juvenile neuronal ceroid-lipofuscinosis (Batten disease): a brief review and update

Juvenile neuronal ceroid-lipofuscinosis (JNCL, Batten disease, Spielmeyer-Vogt-Sjogren disease, CLN3) is the most common inherited, autosomal recessive, neurodegenerative disorder in man. Like the other neuronal ceroid-lipofuscinoses, it is characterized by progressive loss of vision, seizures, and loss of cognitive and motor functions, leading to premature demise. JNCL is caused by mutations of CLN3, a gene that encodes a hydrophobic transmembrane protein, which localizes to membrane lipid rafts in lysosomes, endosomes, synaptosomes, and cell membrane. While the primary function of the CLN3 protein (CLN3P) may be debated, its absence affects numerous cellular functions including pH regulation, arginine transport, membrane trafficking, and apoptosis. We have recently suggested that the unifying primary function of CLN3P may be in a novel palmitoyl-protein Delta-9 desaturase (PPD) activity that in our opinion could explain all of the various functional abnormalities seen in the JNCL cells. Another group of researchers has recently shown a correlation between the CLN3P expression and the synthesis of bis(monoacylglycerol)phosphate (BMP) and suggested that CLN3P may play a role in the biosynthesis of BMP. In this review, following an introduction to the neuronal ceroid-lipofuscinoses, we provide a brief overview and an update of the most recent research in JNCL, specifically that related to the function of CLN3P.