Primary localized gastric amyloidosis: A scoping review of the literature from clinical presentations to prognosis

Localized gastric amyloidosis (LGA) is a rare disease characterized by abnormal extracellular deposition of amyloid protein restricted to the stomach and it is confirmed by positive results of Congo red staining. Over decades, only a few cases have been reported and studies or research focusing on it are few. Although LGA has a low incidence, patients may suffer a lot from it and require proper diagnosis and management. However, the pathology of LGA remains unknown and no overall review of LGA from its presentations to its prognosis has been published. Patients with LGA are often asymptomatic or manifest atypical symptoms, making it difficult to differentiate from other gastrointestinal diseases. Here, we report the case of a 70-year-old woman with LGA and provide an overview of case reports of LGA available to us. Based on that, we conclude current concepts of clinical manifestations, diagnosis, treatment, and prognosis of LGA, aiming at providing a detailed diagnostic procedure for clinicians and promoting the guidelines of LGA. In addition, a few advanced technologies applied in amyloidosis are also discussed in this review, aiming at providing clinicians with a reference of diagnostic process. With this review, we hope to raise awareness of LGA among the public and clinicians.     

Primary aldosteronism due to bilateral micronodular hyperplasia and concomitant subclinical Cushing’s syndrome: A case report

BACKGROUNDAdrenal incidentaloma (AI) has been frequently encountered in the clinical setting. It has been shown that primary aldosteronism (PA) or subclinical Cushing’s syndrome (SCS) are the representative causative diseases of AI. However, the coexistence of PA and SCS has been reportedly observed. Recently, we encountered a case of AI, in which PA and SCS coexisted, confirmed by histopathological examinations after a laparoscopic adrenalectomy. We believe that there were some clinical implications in the diagnosis of the present case.CASE SUMMARYA 58-year-old man presented with lower right abdominal pain with a blood pressure of 170/100 mmHg. A subsequent computed tomography scan revealed right ureterolithiasis, which was the cause of right abdominal pain, and right AI measuring 22 mm × 25 mm. After the disappearance of right abdominal pain, subsequent endocrinological examinations were performed. Aldosterone-related evaluations, including adrenal venous sampling, revealed the presence of bilateral PA. In addition, several cortisol-related evaluations showed the presence of SCS on the right adrenal adenoma. A laparoscopic right adrenalectomy was then performed. The histopathological examination of the resected right adrenal revealed the presence of a cortisol-producing adenoma, while CYP11B2 immunoreactivity was absent in this adenoma. However, in the adjacent non-neoplastic adrenal, multiple CYP11B2-positive adrenocortical micronodules were detected, showing the presence of aldosterone-producing adrenocortical micronodules. CONCLUSIONCareful clinical and pathological examination should be performed when a patient harboring AI presents with concomitant SCS and PA.     

原发性骨膜增生厚皮症伴重度贫血1例

患者男,39岁,面部皮肤肥厚、四肢肢端肥大20年,间断头晕乏力1年。查体:重度贫血貌,睑结膜、口唇及甲床苍白;头面部皮肤肥厚、皱褶、呈回状颅皮;双下肢皮肤肥厚,手指及足趾呈杵状;皮损颜色正常,触之略韧;踝关节肿胀无压痛、活动自如。X线检查提示右腓骨中段髓腔密度增多,腓骨中段及胫骨下段后缘骨皮质增厚。血常规重度正常细胞低色素性贫血,骨髓活检提示骨髓造血衰竭,骨髓纤维化不明显。诊断:原发性骨膜增生厚皮症伴重度贫血。     

Neurovascular-modulation: A review of primary vascular responses to transcranial electrical stimulation as a mechanism of action

BackgroundThe ubiquitous vascular response to transcranial electrical stimulation (tES) has been attributed to the secondary effect of neuronal activity forming the classic neurovascular coupling. However, the current density delivered transcranially concentrates in: A) the cerebrospinal fluid of subarachnoid space where cerebral vasculature resides after reaching the dural and pial surfaces and B) across the blood-brain-barrier after reaching the brain parenchyma. Therefore, it is anticipated that tES has a primary vascular influence.ObjectivesFocused review of studies that demonstrated the direct vascular response to electrical stimulation and studies demonstrating evidence for tES-induced vascular effect in coupled neurovascular systems.ResultstES induces both primary and secondary vascular phenomena originating from four cellular elements; the first two mediating a primary vascular phenomenon mainly in the form of an immediate vasodilatory response and the latter two leading to secondary vascular effects and as parts of classic neurovascular coupling: 1) The perivascular nerves of more superficially located dural and pial arteries and medium-sized arterioles with multilayered smooth muscle cells; and 2) The endothelial lining of all vessels including microvasculature of blood-brain barrier; 3) Astrocytes; and 4) Neurons of neurovascular units.ConclusionA primary vascular effect of tES is highly suggested based on various preclinical and clinical studies. We explain how the nature of vascular response can depend on vessel anatomy (size) and physiology and be controlled by stimulation waveform. Further studies are warranted to investigate the mechanisms underlying the vascular response and its contribution to neural activity in both healthy brain and pathological conditions – recognizing many brain diseases are associated with alteration of cerebral hemodynamics and decoupling of neurovascular units.     

原发性干燥综合征生物标志物的研究进展

目前原发性干燥综合征(pSS)诊断仍然依赖于侵入性小涎腺活检手术,常导致其早期诊断异常艰难,因此筛选特定生物标志物可能对pSS诊断和个体化治疗极为有益。本文回顾传统标志物及与B细胞活化和表观遗传相关的新标志物,并综述组学标志物研究进展。尽管生物标志物前景较好,但其可靠性仍需扩大样本量进行验证。     

The role of orthodontics in management of obstructive sleep apnea

Dental sleep medicine is the field of dental practice that deals with the management of sleep-related breathing disorders, which includes obstructive sleep apnea (OSA) in adults and children. Depending on the developmental age of the patient and the cause of the apnea dental treatment options may vary. For adult patients, treatment modalities may include oral appliance therapy (OAT), orthognathic surgery and surgical or miniscrew supported palatal expansion. While for children, treatment may include non-surgical maxillary expansion and orthodontic functional appliances. Many physicians and dentists are unaware of the role dentistry, particularly orthodontics, may play in the interdisciplinary management of these disorders. This review article is an attempt to compile evidence-based relevant information on the role of orthodontists/sleep dentists in the screening, diagnosis, and management of sleep apnea. Oral sleep appliance mechanisms of action, selective efficacy, and the medical physiological outcomes are discussed. The purpose of this review is to provide a comprehensive understanding of how orthodontists and sleep physicians can work in tandem to maximize the benefits and minimize the side effects while treating patients with OSA.     

2022年上海市医师协会全科医师分会年会主题报告（一）

本文内容来源于2022年7月22日由上海市医师协会、上海市医师协会全科医师分会主办的"第二届海上全科医师论坛"，主要包括《基层医疗在整合医疗中的作用》《创建全科医学科为国家临床重点专科》《上海市基层卫生发展趋势》《拥抱变化，更好更强》四部分。其中，《基层医疗在整合医疗中的作用》从整合程度、整合层面、整合方向3个维度阐释了"整合医疗"的概念与内涵，并就整合医疗在制度、管理、支付、服务模式、技术应用5个层面的实现路径做了详细介绍；在分析基层医疗和整合医疗共性特征的基础上，阐释基层医疗在上述5个层面对整合医疗的助推作用。《创建全科医学科为国家临床重点专科》从全科医学科在卫生保健改革、满足居民卫生服务需求中的重要作用出发，阐述了创建全科医学科为国家临床重点专科的必要性，认为创建临床重点专科需做到：提高全科医学科学术地位，推进全科医学人才建设，提升全科医学科区域协同能力，形成全方位全周期健康照护专科特色，加快全科医学科研创新发展。《上海市基层卫生发展趋势》肯定了基层卫生对城市健康发展的贡献，提出在"大健康"的背景下进一步的重点工作为推进健康老龄化和实现出生人口提升计划；上海基层卫生的发展趋势在于创新，而基层卫生高质量发展工作的创新需要由全科医生作为推动者和牵头者，创造出新的健康服务模式。《拥抱变化，更好更强》，认为当前社区卫生服务的"薄弱"之处，并非发展之初的硬件或人员配置不足，而是体现在全科学科建设方面。全科医学领域工作者应主动面对和接纳当前国家健康战略的变化、健康需求的变化、医学模式的变化、自身发展/管理的变化，开展主动健康，应对多病共存，加强全-专结合，关注需方角度的服务评价，以助力全科医学学科建设。