Cogan's syndrome: an oculo-audiovestibular disease

Typical Cogan''s syndrome is a rare disease of young adults consisting of flares of interstitial keratitis and sudden onset of Ménière-like attacks (nausea, vomiting, tinnitus, vertigo and hearing loss). Life-threatening aortic insufficiency develops in 10% of reported cases. Atypical Cogan''s syndrome (audiovestibular dysfunction with other types of inflammatory eye disease) is associated with vasculitis in 20% of cases and has a less favourable prognosis than typical Cogan''s syndrome.


     

Schmidt's syndrome – Case report

When immune dysfunction affects two or more endocrine glands and other non-endocrine immune disorders are present, the polyglandular autoimmune (PGA) syndromes should be considered. The PGA syndromes are classified as two main types: PGA type I and PGA type II. We are reporting this case in which a patient had primary adrenal insufficiency, autoimmune hypothyroidism and insulin dependent diabetes mellitus and was diagnosed as "Schmidt''s syndrome" (PGA type II). This syndrome is a very rare autoimmune disorder and difficult to diagnose because the symptoms of this syndrome depends on the gland which gets involved first. Our patient was treated and improved with corticosteroid, thyroxine and insulin therapy.     

双侧肾上腺高功能皮质腺瘤致ACTH非依赖性库欣综合征3例临床诊疗探索

目的 探讨双侧肾上腺高功能皮质腺瘤致促肾上腺皮质激素（ACTH）非依赖性库欣综合征的临床特点、诊治思路,提高对该疾病的认知。 方法 收集2016～2021年四川大学华西医院内分泌代谢科收治的3例双侧肾上腺功能性皮质腺瘤致ACTH非依赖性库欣综合征患者行回顾性分析,分析患者的一般情况、症状及体征、辅助检查、诊治过程等临床资料,并结合文献进行复习和讨论。 结果 3例患者均表现为典型且严重的库欣综合征,并伴有显著的心脏和代谢并发症,影像学提示双侧肾上腺单发腺瘤;2例患者行双侧肾上腺静脉采血,均提示双侧肾上腺皮质醇高分泌且无优势侧;1例患者行双侧肾上腺肿物切除术,另外2例患者因为心功能不全严重,行分期双侧肾上腺肿物切除术,术后病理结果3例均提示双侧肾上腺皮质腺瘤,术后皮质醇浓度均明显降低,均给长期予糖激素替代治疗。 结论 双侧肾上腺高功能皮质腺瘤致ACTH非依赖性库欣综合征非常罕见,明确诊断并判断双侧功能定位非常重要,治疗上应根据患者的病情及并发症情况个体化的选择最适合患者的手术方式,术后应严密随访,及时给予并调整糖皮质激素替代治疗,并终身随访。     

Marjolin's ulcer: clinical and pathologic features of 83 cases and review of literature

Background

Marjolin''s ulcer is a rare, aggressive condition that arises on chronic skin lesions and diseases. Inthis article, we will report 83 cases of this disease.

Methods

Retrospectively, we retrieved 83 records of patients with cancer arising from chronic skin conditions.Data concerning demography, type of original skin insult, time interval between original lesion and cancer,cancer histology, and lymph node involvement were recorded.

Results

The mean age was 55.30 years (range: 21-90). There were 51 males (61.5%) and 32 females (38.5%).Foot was the most prevalent site of primary skin lesion (49.4%) followed by scalp (15.6%). Original skin insultswere burn (87.9%), osteomyelitis (2.4%), radiation (2.4%), electrical burn (1.2%), surgical scar (2.4%),pemphigus (1.2%), bite (1.2%), and bed sore (1.2%). Histologic diagnosis were well differentiated SCC(38.6%), SCC, differentiation not reported (24.1%), moderately differentiated SCC (13.2%), BCC (9.6%), poorlydifferentiated SCC (6.0%), melanoma (2.4%), verrucous carcinoma (2.4%), MFH (1.2%), mucoepidermoidcarcinoma (1.2%), and leiomyosarcoma (1.2%). Most of the cases occurred more than 20 years after the initialskin insult. There were 6 (7.2%) cases that developed within 1 year (acute Marjolin''s Ulcer). Forty three patients(69.3%) had palpable regional lymph nodes.

Conclusion

Data in this series were in confirmation with many other reports. Marjoln''s ulcer should be consideredas a significant post-skin injury complication.     

Bing-Neel综合征1例报告并文献复习

华氏巨球蛋白血症(Waldenstrom’s macroglobulinemia,WM)是一种以骨髓或肝、脾、淋巴结内浆细胞样淋巴细胞浸润和血清中存在大量单克隆Ig M为特征的恶性浆细胞病。Bing-Neel综合征是华氏巨球蛋白血症的一种罕见的疾病表现,由恶性浆细胞样淋巴细胞浸润中枢神经系统引起。本文报道1例Bing-Neel综合征并结合相关文献就其临床表现、辅助检查及治疗原则等进行讨论。     

Acute Pancreatitis Complicated by Sheehan's Syndrome: A Case Report and Literature Review

A 44-year-old woman was transferred to the ICU of the First Affiliated Hospital of Jinan University for 2 days of persistent epigastric pain and 7 hours of unconsciousness. Her admission diagnosis was severe acute necrotizing pancreatitis (hypertriglyceridemia type) with multiple organ dysfunctions. The results of CT revealed a small area of necrotizing pancreatitis, which was not consistent with the severe clinical manifestations. Considering lack of hair and history of postpartum hemorrhage, hormone examination was carried out. According to the results of the examination, she was further diagnosed as Sheehan's syndrome and pituitary crisis. After hormone replacement therapy, her condition improved rapidly.     

Herbal Medicine in the Treatment of Alzheimer’s Disease

Associated with the aging of our world population is a sharp increase in the incidence of Alzheimer’s disease,which not only poses a significant health issue but also presents a serious social problem.Although pharmacological treatments were developed based on existing hypotheses,the disease pathogenesis remains to be fully elucidated.Given the complexity of Alzheimer’s disease,Chinese herbal medicine appears to have therapeutic potential for Alzheimer’s disease through multi-target and multi-pathway approach at cellular and molecular levels and holistic adjustment of the body at organ system levels.Recently,a significant breakthrough has been made in the research of Chinese medicine for Alzheimer’s disease.In this article,we review the experimental research progress in understanding how Chinese medicine could be used for the treatment of Alzheimer’s disease.     

干燥综合征从瘀论治的研究进展

干燥综合征是一种以口眼干燥为主症的慢性自身免疫性疾病,中医古籍中并无"干燥综合征"一词,根据其临床表现等可归于"燥证"、"痹证"、"燥痹"的范畴;瘀血在本病的发生发展及预后中起着重要作用,它既是干燥综合征发展过程中的病理产物,也是致病因素,瘀去则气血条达,津液输布如常,官窍得以濡养;活血祛瘀法是治疗干燥综合征的重要方法,应贯穿本病治疗的始末;从瘀论治干燥综合征丰富了中医对其的认识,在临床诊疗中具有一定的借鉴和指导意义。     

Extrapulmonary lymphomatoid granulomatosis presenting as Pancoast's syndrome.

We report the case of a 55 year old man who presented as Pancoast''s syndrome associated with a left sided Horner''s syndrome, a mass in the left supraclavicular fossa and marked weakness with wasting of the left arm consistent with a left sided brachial plexus lesion. Biopsy revealed the typical features of lymphomatoid granulomatosis. Neurological involvement is a rare presenting manifestation of this uncommon disease. Lymphomatoid granulomatosis should be considered as a rare but potentially treatable cause of Pancoast''s syndrome.     

滋肾益髓方联合西药治疗帕金森氏病患者非运动症状的疗效观察

[目的]探讨滋肾益髓方联合西药治疗帕金森氏病患者非运动症状的疗效。[方法]选用证型为髓海不足证的帕金森氏病患为研究对象,进行前瞻性平行对照试验,按治疗组、对照组1∶1共计60例,其中治疗组30例,对照组30例。治疗组采用中西医结合疗法,给予滋肾益髓方药联合西药(标准左旋多巴剂量),对照组维持仅口服西药治疗,观察周期为1个月,停药后随访3个月。在治疗过程中通过帕金森病统一评定量表(UPDRS)及30项非运动症状筛查问卷(NMSQ)等国际公认的评价体系评估患者的临床症状,采用对比分析法评定疗效。[结果]与治疗前对比,治疗组病患治疗后不同时间内的UPDRS总评分值、NMSQ评分值均有明显下降,与治疗前相比具有统计学意义(P<0.05)。[结论]滋肾益髓方联合西药治疗帕金森氏病患非运动症状的疗效确切。     

蔡慎初从郁论治雷诺综合征经验

正雷诺综合征是指由血管神经功能紊乱所引起,遇冷或情绪紧张后出现阵发性肢端小动脉强烈收缩,从而导致肢端缺血改变的临床综合征。发病时,肢端皮肤出现阵发性、对称性的苍白-紫绀-潮红性改变,并伴有手脚冰凉、麻木或疼痛。女性发病率可达3%,病情缠绵难愈~([1])。蔡慎初教授为国家级名老中医药专家,从事中医相关临床、科研工作50余载,对雷诺综合征的诊治有丰富和独到的经验。笔者有幸侍诊于侧,现将蔡老师治疗雷诺综合征经验介绍如下,与同道共飨。     

Whipple's Disease in an Afro-Caribbean National

Whipple''s disease is a rare multi-organ infectious disease caused by Tropheryma whipplei. It is fatal without treatment. We report on a 40-year old Afro-Jamaican man who presented with a six-month history of weight loss and diarrhoea. Investigations revealed iron deficiency anaemia and hypoalbu-minaemia. Upper gastrointestinal endoscopy revealed white patchy lesions in the duodenum. The duodenal biopsy showed broadening and thickening of the villi by a dense infiltrate of foamy histiocytes within the lamina propria and focally extending into the attached submucosa. Periodic Acid-Schiff stains were positive. Electron microscopy was confirmatory and polymerase chain reaction testing conclusively identified the organisms as T whipplei. Antibiotic treatment resulted in resolution of symptoms. Although the diagnosis of Whipple''s disease is difficult, increased awareness should lead to an increase in reported cases with the improvements in diagnostic capabilities.     

以“转氨酶升高”为首发表现的飞行员干燥综合征1例

目的 报告 1 例以“转氨酶升高”为首发表现的飞行员干燥综合征，并讨论体检鉴定及航医工作。方法 报告 1 例以“转氨酶升高”为首发表现的飞行员干燥综合征的诊治过程及后续体检鉴定工作，讨论体检鉴定及航医工作。结果 该飞行员因“常规年度大体检发现转氨酶升高”入院，白细胞总数偏低；肝功能受损，抗“O”偏高、类风湿因子偏高。自身抗体谱：ANA、抗SSA、抗SSB、抗RO-52(+)；EBV壳抗原IgA、IgG(+)：巨细胞病Ig抗体(+)。结合唇腺活检结果，干燥综合征诊断明确，给予“暂时飞行不合格，地面观察1个月”结论。经过规范治疗后，抗“O”及类风湿因子降低，血液指标恢复正常，肝功能恢复正常。规范治疗和健康指导3个月后，血常规、肝肾功能均无异常。考虑其为青年男性，既往体健，飞行意愿强烈，无口干眼干、无腹痛黄疸等症状，予“飞行合格”结论。结论干燥综合征能够引起全身多器官免疫性损伤，早期诊断和治疗存在较多困难，常导致病情迁延难愈，累及各器官系统并造成功能损害，可能影响到飞行员飞行安全。全面的康复指导、细心的病情观察是预防并发症、决定患者预后的关键。规范治疗和健康指导后，若复查血常规、肝肾功能均无异常，无特异性症状，可予“飞行合格”结论。     

Botanical Therapeutics for Parkinson''s Disease

Parkinson''s disease (PD) was first formally described by James Parkinson in 1817, but the shaking of limbs was described in the literature of several ancient civilizations, such as ancient Chinese civilization and ancient Indian civilization. Historically, botanical drugs were used as the main source for the treatment of such kind of disorders. In Western countries, plant extracts also occupied an important place in the earlier medications of PD. With the adventure of synthetic drugs, the role of plant-derived drugs in management of PD has been diminished. Nowadays, there is still no cure for PD, dopaminergic (DA) medication is the treatment of choice, which is just designed to ameliorate symptoms of PD, and long-term use of DA medication will result in reduced efficacy and severe adverse reactions. It is necessary to explore new methods for the treatment of PD. Chinese medicine (CM) developed a holistic and unique theoretical system, and botanical drugs are widely used in practice for more than two millennia. Modern pharmacological studies have proved that Chinese herbs have potential therapeutic effects on PD, such as enhancing neurotrophic activity, clearing protein aggregates, regulating neuroinflammation, etc. All the advances provide us with hope for developing CM as a mainstream medication for treating PD.     

关于针对SARS的综合免疫治疗策略

采用鼻腔喷雾法(CCID50=105.7)研究了SARS冠状病毒(SARS-CoV)对成年和幼年布氏田鼠的感染效果.成年动物攻毒后出现死亡,表现为口鼻有出血,肠道出血;肺组织呈出血性间质性肺炎改变,肝、脾、肾、胰腺组织均呈淤血性改变;存活动物肺组织呈间质性肺炎,局灶出血及肺气肿改变,其他脏器未见明显病变.幼年动物攻毒后未见死亡但行动较为迟缓,主要脏器未见明显异常;早期肺组织有局限性肺炎改变,且病毒分离为阳性;同居对照组的一只动物有肺组织局灶性肺炎.结果表明,SARS-CoV可以很强地感染布氏田鼠;成年布氏田鼠比幼年动物对SARS-CoV更敏感;布氏田鼠有望成为一种比较理想的小型SARS动物模型。     

周郁鸿教授从脾肾论治免疫性血小板减少症的经验总结

[目的]总结周郁鸿教授从脾肾论治免疫性血小板减少症(immune thrombocytopenia,ITP)的辨证诊疗思维和临证拟方用药经验。[方法]通过概述ITP的现代医学认识、历代中医沿革,收集、整理、归纳周郁鸿教授治疗ITP的典型医案,从脾肾论治ITP的理论渊源、加味黄芪建中汤组方阐释及其药理学剖析等方面论述周教授从脾肾论治ITP的学术观点及临证诊疗思维,并举验案一则以佐证。[结果]现代医学认为,ITP是一种获得性自身免疫性疾病,而历代中医则认为ITP是一种血证。周郁鸿教授认为ITP患者常属"脾胃亏虚、肾精不足"之证,应以脾肾为着眼点辨证论治ITP,以"健脾补肾"为基本治则,以温中补虚立法。加味黄芪建中汤健脾补肾,现代药理研究证明其可能通过调节Th17/Treg细胞而有效治疗脾肾阳虚型慢性ITP。所举验案属脾肾阳虚型血证,方用加味黄芪建中汤,温补脾肾、养血止血,取得较好疗效,展示了该病的辨证诊疗思路。[结论]周郁鸿教授从脾肾论治临床上部分难治ITP患者,中医理论依据充分,临床效果显著,值得推广。     

Evidence and Expert Opinions: Drying Needling versus Acupuncture (Ⅰ)—The American Alliance for Professional Acupuncture Safety (AAPAS) White Paper 2016

In the last twenty years, in the United States and other Western countries, dry needling (DN) became a hot and debatable topic, not only in academic but also in legal fields. This White Paper is to provide the authoritative information of DN versus acupuncture to academic scholars, healthcare professional administrators, lawmakers, and the general public through providing the authoritative evidence and experts' opinions regarding critical issues of DN versus acupuncture, and then reach consensus. DN is the use of dry needles alone, either solid filiform acupuncture needles or hollow-core hypodermic needles, to insert into the body for the treatment of muscle pain and related myofascial pain syndrome. DN is sometimes also known as intramuscular stimulati on, trigger points (TrP) acupuncture, TrP DN, myofascial TrP DN, or biomedical acupuncture. In Western countries, DN is a form of simplified acupuncture using biomedical language in treating myofascial pain, a contemporary development of a portion of Ashi point acupuncture from Chinese acupuncture. It seeks to redefine acupuncture by reframing its theoretical principles in a Western manner. DN-like needling with filiform needles have been widely used in Chinese acupuncture practice over the past 2,000 years, and with hypodermic needles has been used in China in acupuncture practice for at least 72 years. In Eastern countries, such as China, since late of 1800s or earlier, DN is a common name of acupuncture among acupuncturists and the general public, which has a broader scope of indications, not limited to treating the myofascial pain.     

李墨林按摩正骨临床经验总结

李墨林先生是20世纪50、60年代河北一代推拿按摩名老中医,在推拿按摩方面造诣颇深,创立了"李氏按摩"流派,对当代按摩技术的发展有很深远的影响。"李氏按摩"流派传人李墨林长孙李建文先生多年运用"李氏按摩"技术于临床实践,对"李氏按摩"精华体会深刻,研究整理了李墨林先生生前的着作,对他的临床经验进行了总结。1)在中医基础理论的指导下,"李氏按摩"手法注重整体,辨证施治。2)"李氏按摩"以经络学说为指导,按摩手法以点穴手法为中心。3)手法点穴可以疏通经络,激发人体经气,促进机体自身机能修复。     

Marfan's syndrome presenting as bilateral spontaneous pneumothorax.

A case of bilateral spontaneous pneumothorax in a 14-year-old girl with previously undiagnosed Marfan''s syndrome is described. The pulmonary abnormalities of Marfan''s syndrome are not commonly encountered and bilateral pneumothorax is itself a rare event which, in most instances, has been reported following invasive procedures.     

Arthropathy in Dressler's syndrome.

Three patients developed a polyarthritis in association with Dressler''s (post-myocardial infarction) syndrome. Joint involvement was more pronounced in the upper limbs and persisted many months after other features of Dressler''s syndrome had settled. In 2 cases the findings in the joints were slight and might have been overlooked but for the persistently raised ESR. There was a prompt symptomatic response to prednisone.