多学科联合救治地震灾害后挤压综合征合并急性肾损伤

2008年5月12日四川省汶川地区发生了里氏8.0级大地震,地震灾害后的挤压综合征合并急性肾损伤是肾科医生面临的重要救治任务.本研究总结了多学科联合救治地震灾害后挤压综合征合并急性肾损伤的经验.除了骨科对挤压伤、骨筋膜室综合征的处理,肾科对急性肾损伤及其相关并发症的肾脏替代治疗外,康复、心理卫生、感染、ICU等多学科的早期协作、综合治疗也是成功治疗地震灾害后挤压综合征合并急性肾损伤必不可少的重要保障.     

地震伤挤压综合征导致急性肾衰竭救治体会和思考

挤压综合征是地震相关损伤中除直接外伤导致死亡以外的第2位死亡原因。1995年日本阪神地震挤压综合征发病率为13·6%,病死率为13·4%;1999年土耳其马尔马拉地震挤压综合征病死率为15·2%。因此有效救治挤压综合征是降低重症地震伤员病死率的关键。“5·12汶川地震”发生后,作为     

脑梗死伴发挤压综合征2例分析

挤压综合征(crush syndrome,CS)是严重挤压伤可能发生的并发症,即于伤处解除挤压后,出现以肌红蛋白尿、高血钾、酸中毒及氮质血症等为特点的以急性肾功能衰竭(acute renal failure,ARF)为主的综合征[1]。在自然灾害(如地震)、生产事故中均有CS病例报道[2]。脑梗死后伴发CS临床罕见,     

地震致挤压综合征的临床救治

2008年5月12日的汶川大地震导致大规模的人员伤亡,其中以挤压综合征最常见。现回顾、整理地震致挤压综合征伤员的救治情况,报告如下。     

锁骨下挤压综合征(181)

<正>起搏器及起搏心电图领域中,锁骨下挤压综合征(subclavian crush syndrome)并非少见,也是造成起搏器起搏与感知功能障碍的常见原因。[定义]锁骨下挤压综合征是引起起搏功能障碍的一个明确原因,这是起搏电极导线经锁骨下静脉     

腕管综合征30例患者的临床和神经电生理资料分析

腕管综合征(CTS)是周围神经卡压综合征中最常见的一种疾病,亦称腕正中神经挤压症;为各种因素所引起的腕管内压力增高,致使正中神经受压、缺血而产生的系列症状及体征,如不及时治疗,常造成手功能受损.2003～2006年,我院收治30例CTS患者,现对其临床和神经电生理资料作回顾性分析.     

关节镜下闭合松解髌骨外侧支持带联合关节清理术治疗髌骨外侧挤压综合征

目的观察关节镜下闭合松解髌骨外侧支持带联合关节清理术治疗髌骨外侧挤压综合征的疗效。方法对56例髌骨外侧挤压综合征患者行关节镜下髌骨外侧支持带闭合松解加关节清理术。采用Lysholm膝关节功能评分评价疗效。结果术后随访时间12～24个月,平均14个月。优40例、良12例、可1例、差3例,优良率为92.86%。结论关节镜下闭合松解髌外侧支持带联合关节清理术治疗外侧髌骨挤压综合征效果好,具有创伤小、康复快等优点。     

脑梗死致挤压伤患者的临床特点分析

正挤压伤(crush injury)指肌肉受到挤压引起的损伤,导致肾功能衰竭时称为挤压综合征(crush syndrome,CS),挤压伤和CS是同一疾病的不同发展阶段。挤压伤的病理机制之一为肌细胞膜完整性受损,释放肌酸激酶(CK)和肌红蛋白(Mb)等细胞内物质入血,当CK1000 U/L、且合并Mb尿时称为横纹肌溶解症(RM)。挤压伤和CS多见于地震、     

提高对挤压综合征的认识建立多学科联合的灾难救治队伍——汶川大地震救治启示

5·12汶川地震是建国以来损伤程度最严重的地震灾害之一,造成了重大人员伤亡.在党中央、中央军委的领导下,经解放军、武警官兵、专业救援人员、医务人员和志愿者的浴血奋战,成功救治了近4万名地震伤员,受到社会的高度赞誉.地震后继发的挤压综合征是广大医务工作者面临的最大挑战.地震伤挤压综合征导致的急性肾损害具有不同于其他急性肾损害的特点:(1)肾功能恢复与挤压伤部位、时间及骨科合理治疗密切相关;(2)合并其他器官复合伤影响患者的预后;(3)治疗过程中经常合并心肺功能不全、出血及感染等合并症,影响患者肾功能恢复及预后;(4)伴有急性肾功能不全的危重地震伤员实施持续性肾脏替代治疗(CRRT)的时间常常需要3～4周以上.由于危重地震伤员的临床表现复杂,救治难度大,存在特殊性与危重性,因此需要深入研究,积累经验,提高我国灾难医学救治水平.     

关节镜下外侧支持带松解治疗外侧髌骨挤压综合征合并氟骨症临床研究

正外侧髌骨挤压综合征属肌肉骨骼疾病,发病机制复杂,主要是由于外侧软组织与髌骨的超负荷不平衡而致横向压力增大,导致病发,通常伴有疼痛感。合并氟骨症则可致病情加重,骨质可出现病理改变,膝关节疼痛加剧,需手术治疗。临床主要采取关节镜下髌骨外侧支持带松解术治疗外侧髌骨挤压综合征,但对于合并氟骨症患者治疗难度增加。本研究探讨关节镜下外侧支持带松解治疗外侧髌骨挤压综合征合并氟骨症的临床应用价值。     

Mitral valve diseases in Williams syndrome-case report and review of the literature

Williams syndrome is a genetic syndrome involving an unusual facies, short stature, developmental delay and heart defects. There is a genetic marker for this disease. Williams syndrome is frequently associated with congenital heart defects. The most common cardiac diagnoses are supravalve aortic stenosis, supravalve pulmonic stenosis, and arterial hypertension. In contrast, the association of mitral valve prolapse with Williams syndrome is less well defined. We present a case of a 15-year-old girl with Williams syndrome who underwent successful mitral valve repair. Review of the echocardiographic database of our institution over a 10-year period identified 26 other patients with Williams syndrome. Overall, 10 of the 27 children with Williams syndrome had mitral valve disease (37%) including 9 patients with mitral valve prolapse and one with mitral insufficiency. In conclusion, patients with Williams syndrome should be examined for mitral valve disease. Mitral valve repair is feasible and may be considered in the growing child with Williams syndrome.     

Metabolic aspects of the polycystic ovary syndrome

The author reviews the history of the term polycystic ovaries syndrome. He emphasizes the importance of insulin resistance in this disease. According to more recent criteria for the definition of the syndrome suffices the finding of hyperandrogenism, an irregular cycle (after elimination of other classical causes of this condition) and insulin resistance. The frequency of the disease varies in different populations up to 10%. It is significantly associated in particular with type 2 diabetes and obesity. The molecular biology of the syndrome is obscure. The metabolic syndrome as well as the polycystic ovaries syndrome have partly a genetic pathogenesis as well as an environmentally induced participation caused by stress. The polycystic ovaries syndrome is nowadays unequivocally an atherogenic syndrome and is a unit very close to Reaven's metabolic syndrome X or is part of this syndrome.     

Acquired hypophosphatemia osteomalacia associated with Fanconi’s syndrome in Sjögren’s syndrome

Sjögren’s syndrome is an autoimmune disorder involving exocrine glands that occurs alone or in association with various autoimmune and connective tissue diseases. The severity of Sjögren’s syndrome ranges from isolated sicca syndrome to severe complications such as vasculitis, lung and renal involvement. Overt or latent renal tubular acidosis caused by autoimmune tubulointerstitial nephritis, is a common extraglandular manifestation in Sjögren’s syndrome. Osteomalacia is a rare complication of renal tubular acidosis, and it was reported to be associated with distal renal tubular acidosis in Sjögren’s syndrome. We report a 60-year-old woman who presented with multiple bone deformity and general muscle weakness. Osteomalacia was secondary to Fanconi’s syndrome, and the Fanconi’s syndrome was a result of renal involvement in Sjögren’s syndrome. Fanconi’s syndrome is a rare kidney manifestation in Sjögren’s syndrome. It may be latent and may precede the subjective sicca symptoms. These findings suggest that evidence for Sjögren’s syndrome should be sought in adult patients with unexplained osteomalacia and renal tubular acidosis, even in the absence of subjective sicca syndrome. Conversely, in patients with Sjögren’s syndrome, early investigation and treatment of renal tubular dysfunction may prevent future complications, such as osteomalacia.     

A chronic myeloid leukemia-like syndrome case with del (12) (p12) in a Li-Fraumeni syndrome family

Li-Fraumeni syndrome is a familial cancer syndrome characterized by different tumors and hereditary p53 mutations. Here, a chronic myeloid leukemia-like syndrome case in a Li-Fraumeni syndrome family with del (12) (p12) cytogenetic abnormality was presented. A hereditary p53 mutation (pro309ser) supported the Li-Fraumeni syndrome diagnosis in this family. This syndrome was characterized by the clonal myeloproliferative accumulation in bone marrow and peripheral blood with negative bcr/abl gene rearrangement finding. The etiology of this rare syndrome is still unclear. This is the only chronic myeloid leukemia-like syndrome case reported in a Li-Fraumeni syndrome family. Del (12)(p12) was observed in leukemias except chronic myeloid leukemia-like syndrome. The deletion in chromosome 12p12 with hereditary p53 mutation should have a critical role in chronic myeloid leukemia-like syndrome etiology in our case.     

Hepatopulmonary syndrome: role of nitric oxide and clinical aspects.

Hepatopulmonary syndrome is defined by oxygenation impairment due to abnormal intrapulmonary vascular dilatations in patients with liver disease. The implication of enhanced pulmonary production of nitric oxide in the pathogenesis of intrapulmonary vascular dilatations has been demonstrated both in murine models and in human hepatopulmonary syndrome. The diagnosis of hepatopulmonary syndrome in chronic liver disease is of paramount importance, considering the fact that severe hypoxemia related to hepatopulmonary syndrome may occur in patients with well compensated liver disease and that survival is reduced in patients with hepatopulmonary syndrome relative to non hepatopulmonary syndrome patients. Priority for liver transplantation, which is presently the only cure, has been recently increased in patients with advanced hepatopulmonary syndrome.     

Morvan Syndrome Converted from Isaacs' Syndrome after Thymectomy with Positivity for Both Anti-LGI1 and Anti-CASPR2 Antibodies

Anti-voltage-gated potassium channel complex antibodies-mediated disorder includes Isaacs'' syndrome, which is characterized by neuromyotonia, and Morvan syndrome, which is characterized by neuromyotonia, encephalopathy and autonomic dysfunction. We herein report a patient with Morvan syndrome that converted from Isaacs'' syndrome after thymectomy. The patient first presented with myospasm in all extremities and positivity for both anti-leucine-rich glioma inactivated 1 (LGI1) and anti-contactin-associated protein like 2 (CASPR2) antibodies and subsequently developed encephalopathy after thymectomy, which was successfully improved by immunotherapy. This is the first case of Morvan syndrome wherein thymectomy worsened Isaacs'' syndrome, suggesting that immunotherapy should be considered for Isaacs'' syndrome accompanied by positivity for both anti-LGI1 and anti-CASPR2 antibodies to prevent worsening to Morvan syndrome.     

病证方药相应及其意义

病证方药作为医疗过程中的基本要素,存在纷繁复杂的关系,临床诊疗的目的在于妥善处理它们之间的关系,因而在方证、病证相关命题的基础上,展开病证方药相应理论的探讨,认为它涵盖了病证关系、方证关系、药证关系、专方专药等内容。病证方药相应真实反映了临床诊疗过程,拓展了中医辨证论治的诊疗思路,强调了对整体的重视,对临床疗效、诊疗规律的研究均有理论指导意义。     

基于证候要素建立慢性乙型肝炎证候诊断标准的思路与设想

本文探讨慢性乙型肝炎中医证候诊断标准研究的思路.认为以证候要素为核心,通过开展文献研究、临床调查及发挥专家集体智慧等手段,建立证候要素辨证量表,通过证侯要素辨证及证侯要素间的应证组合,实现符合临床实际的中医证候量化诊断是建立慢性乙型肝炎证候诊断标准的重要途径.     

心肾综合征的临床研究进展

心肾综合征一词已广泛用于进行性充血性心力衰竭引起的肾功能下降。为了概括心脏与肾脏之间复杂的因果关系,心肾综合征分为5种临床亚型:Ⅰ型:急性心肾综合征;Ⅱ型:慢性心肾综合征;Ⅲ型:急性肾心综合征;Ⅳ型:慢性肾心综合征;Ⅴ型:继发性心肾综合征。心肾综合征是慢性肾功能不全和慢性心力衰竭中治疗的难题,现就心肾综合征近年来临床研究进展进行综述。     

Severe jaundice due to coexistence of Dubin-Johnson syndrome and hereditary spherocytosis: a case report

Dubin-Johnson syndrome is a chronic, benign, intermittent jaundice, mostly of conjugated hyperbilirubinemia. The level of bilirubin is not expected to be more than 20 mg/dl in this syndrome. In this article, we report a patient who was evaluated for hyperbilirubinemia and liver function test abnormalities and diagnosed with Dubin-Johnson syndrome coexisting with hereditary spherocytosis. We suggest that other diseases should be investigated if patients with Dubin-Johnson syndrome present with severe hyperbilirubinemia. Dubin-Johnson syndrome accompanied by hemolytic diseases might also have high coproporphyrin levels (as in Rotor's syndrome) than expected in pure Dubin-Johnson syndrome.