Inflammatory features of melasma lesions in Asian skin

Melasma is triggered by various factors including ultraviolet radiation and estrogen; however, its pathogenesis is unclear. To investigate the inflammatory features of melasma lesions as triggers for this disorder, 197 women with melasma who attended Asan Medical Center and Kangskin Clinic, Seoul, from June 2011 to October 2011 completed a questionnaire concerning triggering or aggravating factors. These cases were divided into “non‐inflammatory” and “inflammatory” groups. Skin biopsies and immunostaining for CD68, CD117, and leukocyte common antigen (LCA) were performed in the lesional and peri‐lesional skin of ten cases in the non‐inflammatory group and nine cases in the inflammatory group. Among the 197 subjects (mean age, 41.5 years; mean age of melasma onset, 33.8 years), 50 patients (25.4%) were categorized into the inflammatory group. This group comprised cases that had inflammatory symptoms and events that triggered the melasma lesions. The lesional dermis contained more CD68⁺ melanophages, CD117⁺ mast cells, and LCA⁺ leukocytes in the inflammatory group than in the non‐inflammatory group. Inflammatory clinical features and an increased number of inflammatory cells in the lesion may be involved in the development of melasma in Asian skin.     

A qualitative exploration of mothers' and fathers' experiences of having a child with Klinefelter syndrome and the process of reaching this diagnosis

Klinefelter syndrome (KS) is a common genetic condition that is currently under-diagnosed. The phenotype is broad, with physical, medical and psychosocial features ranging from mild to severe. When a child is diagnosed with KS, the parents may spend months to years searching for a diagnosis. This study used a qualitative methods approach to explore parents'' experiences of having a child with KS and receiving a diagnosis. Fifteen semistructured one-to-one in-depth interviews were conducted to explore their experiences and views. The interviews were then transcribed, coded and thematically analysed. The interviews revealed that parents had diverse experiences related to: the timing of the diagnosis of their child and reasons why their child was investigated for KS; the information that was provided at the time of diagnosis; the supports that were available and the concerns that parents held for the future of their child. The conclusions from this study were that parents'' experiences of having a child with KS and receiving a diagnosis were complex and multifaceted. This experience was shaped by the timing of when the diagnosis was received, who provided the diagnosis, what information was provided from health-care professionals and that which parents may have encountered on the internet. The long-term experiences for parents were also impacted by the level of support they received. These findings have implications for the process by which KS is recognised by the health-care community and supports available for families.     

九年制岐黄国医实验班中医诊断学教学特色

九年制岐黄国医实验班是北京中医药大学中医学专业首次推出以9年直博为特色的教学实验班,在全国尚属首例,也是我校学制最长、培养要求最高的班级。通过9年的学习,直接获得博士学位,最终目标为培养从事中医医疗、预防、保健等工作的中医药临床精英人才。为了实现这一目标,中医诊断学课程的教授面临着更大的挑战,无论从教学大纲的制定、教学时数的安排、教学方法的采用和教学内容的教授上,我们都进行了深入探索、改革和提高,具有不同于其他学制（如五年制、八年制）的教学特色,通过2届的教授和教学研究,均取得了良好的教学效果,达到了预期的中医诊断学教学目标和要求,为探索如何培养中医高精尖人才进行了有益尝试,积累了一定的经验。     

硬膜下积脓的临床特点及治疗分析

目的探讨硬膜下积脓(Subdural empyema,SDE)的临床特点,为临床治疗提供借鉴。方法回顾性分析本科保守治疗的硬膜下积脓病例1例,并对已有文献报道进行综述,总结其临床、影像学表现及治疗。结果文献报道共50例患者,48例外科手术治疗,2例内科保守治疗。SDE起病急,主要临床表现为大脑镰综合征、抽搐,意识障碍。头磁共振表现:大脑镰及小脑幕下积脓呈长T1,长T2信号,FLAIR呈高信号,DWI呈高信号,积脓周围脑膜强化。治疗上主要以外科手术为主,少数病例通过合理的抗菌药物治疗也可好转。结论镰幕硬膜下积脓的临床特点是大脑镰综合征和镰幕下积脓;以外科治疗为主,内科保守治疗也是一种可行的治疗方法。     

Clinical characteristics and medical management of Iranian patients with ankylosing spondylitis

Abstract

Objectives. Ankylosing spondylitis (AS) is a chronic inflammatory rheumatic disease with variable clinical expression. Ethnic, racial and geographical factors have been associated with disease occurrence and expression. We intended to describe the clinical characteristics and assess the disease severity and treatment status in Iranian AS patients.

Methods. A total of 320 AS patients were assessed for demographic variables, clinical manifestations, human leukocyte antigen (HLA) status, disease severity, functional capacities, quality of life and treatment status.

Results. A gender ratio of 3.8:1, an average age onset of 27 ± 7.3 and a mean diagnostic delay of 8 years were observed. Eleven percent had juvenile onset AS. Positive family history was higher than that observed in most other countries. Enthesitis was a very common finding involving more than two-thirds of our patients. Uveitis was the leading extra-articular manifestation. We found an HLA-B27 prevalence of 73% and four HLA-B27 subtypes. Disease activity was high and the functional status was poor as indicated by mean Bath AS Disease Activity, Functional and Metrology indices. Quality of life was considerably impaired in our patients. We found a low percentage of patients on biological medications and a relatively higher percentage on disease modifying anti-rheumatic drugs and corticosteroids.

Conclusions. Our results demonstrate a broad characterization of Iranian AS patients providing a better understanding of this disease. A national multicenter registry would enable larger- scale prospective studies to be carried out further evaluating the disease burden on patients and society.     

人结直肠锯齿状腺癌的研究现状

结直肠锯齿状腺癌（serrated adenocarcinoma，SAC）作为大肠癌的一种特殊临床类型，因其癌变途径的特殊性，检测以及较差的治疗反应和预后逐渐引起众多研究者的兴趣。其产生的途径主要为“锯齿状通路”途径，锯齿状通路途径以BRAF突变、微卫星体不稳定（microsatellite instability，MSI））和CpG岛的甲基化（CpG island methylator phenotype，CIMP）为特征。由于特殊的形态和内镜特征及特殊的发生部位，在结肠镜检查时很容易漏诊，甚至病理评估时也极易被忽视，临床确诊时往往已处于进展期，患者预后差，生存率低。本文针对锯齿状结直肠腺癌流行病学、危险因素、临床特征、病理学特征、分子特征等作一综述。     

X-连锁低血磷性佝偻病的治疗现状及展望

X-连锁低血磷性佝偻病(XLH)属于遗传性代谢性骨病,发病机制复杂,在临床上较为罕见,其主要临床表现为骨骼畸形及身材矮小,致残率高,因而针对XLH进行及早诊断及治疗对患者康复具有极为重要的意义。传统治疗以补充磷酸盐和维生素D类似物为主,随着学者对XLH病理机制研究逐渐深入,分子靶向治疗等新型治疗方式横空出世,通过多学科联合诊断及治疗的方法为患者带去了福音。本文就XLH的临床特征、发病遗传机制、治疗现状及治疗的未来发展趋势进行综述。     

Pancreatic acinar cell carcinoma: A comprehensive review

Acinar cell carcinoma (ACC) is a rare pancreatic malignancy with distinctive clinical, molecular, and morphological features. The long-term survival of ACC patients is substantially superior to that of pancreatic adenocarcinoma patients. As there are no significant patient series about ACCs, our understanding of this illness is mainly based on case reports and limited patient series. Surgical resection is the treatment of choice for patients with the disease restricted to one organ; however, with recent breakthroughs in precision medicine, medicines targeting the one-of-a-kind molecular profile of ACC are on the horizon. There are no standard treatment protocols available for people in which a total surgical resection to cure the condition is not possible. As a result of shared genetic alterations, ACCs are chemosensitive to agents with activity against pancreatic adenocarcinomas and colorectal carcinomas. The role of neoadjuvant or adjuvant chemoradiotherapy has not been established. This article aims to do a comprehensive literature study and present the most recent information on acinar cell cancer.