伴心脏浸润的高级别B细胞淋巴瘤1例并文献复习

回顾性分析1例合并心脏浸润的高级别B细胞淋巴瘤诊治经过并对相关文献进行复习。患者经骨髓MICM、骨髓活检、淋巴结活检确诊,增强CT提示心脏浸润,病情进展迅速,接受"RP"方案治疗过程中出现胸闷气急加重,频发房早、短阵房速等心律失常,后出现血压、心率下降,经抢救无效死亡。     

肾功能衰竭患者肾活检的可行性及临床意义

肾活检作为诊断、指导治疗肾实质性疾病的重要手段其临床意义已得到充分肯定.但对于肾功能衰竭患者,由于多合并高血压、不同程度贫血、病情相对严重以及B超双肾结构显示不清等因素,肾活检后并发症发生率较高,部分学者将肾功能衰竭患者作为肾活检的相对禁忌症,也有学者认为肾衰竭患者行肾活检意义不大[1].我院自1993年8月～2002年6月共行肾活检686例,其中肾功能衰竭失代偿期病例69例,本文总结这69例患者肾活检的病理类型以及术后并发症,探讨肾功能衰竭患者肾活检的可行性及临床意义.     

甲型副伤寒并发急性肾功能衰竭7例临床分析

目的:探讨甲型副伤寒(甲副)并发急性肾功能衰竭的临床发生规律及致病机制.方法:回顾性总结甲副并发急性肾功能衰竭的临床资料.结果:甲副并发急性肾功能衰竭发生率为 0.61%,全身中毒症状及消化系统症状出现早,而泌尿系统症状多数出现在极期,较前两者要迟.实验室检查发现既有肾小球又有肾小管功能受损,合并脾肿大及肝功能损害发生率为 71.43%,经综合治疗,临床症状和实验室指标均在 4周内恢复正常.细胞免疫功能检测 CD4下降,1例肾活检病理为 IgA肾病(第Ⅲ型).结论:甲副可并发急性肾功能衰竭,但发生率低至 0.61%,泌尿系统症状多见于极期且易被其它症状所掩盖,造成延误诊治,提醒人们甲副患者一旦病情加重,需考虑到是否合并急性肾功能衰竭存在而作相应检查,对及时避免肾毒性药物的使用,有着独到的临床价值.7例合并脾肿大,发病率高,CD4的下降以及肾活检免疫病理均在很大程度上支持免疫损伤参与致病机制.     

老年糖尿病低血糖症40例分析

目的探讨老年糖尿病低血糖症的临床特点及防治。方法通过回顾性分析老年糖尿病患者发生低血糖症的临床表现,并对其原因进行分析,提出防治对策。结果 40例中37例得到及时治疗病情好转,1例遗留轻度痴呆,1例因同时患有大面积脑梗死,经积极抢救无效死亡。1例合并糖尿病肾病、心力衷竭死亡。结论老年糖尿病患者低血糖症临床表现多样,易误诊而延误抢救时机,造成大脑不可逆性损害,甚至危及生命,所以临床医生应提高对低血糖症认识。     

系统性硬化症患者的护理

总结35例系统性硬化症患者的护理。系统性硬化症容易合并肺、心、肾、消化道等多脏器功能受损，护理过程中需密切观察病情，注意血氧饱和度、血压、尿量等变化，发现病情变化，及时予以对症护理和治疗，防止脏器功能进一步损害。经治疗及护理，33例好转，另1例自动出院、1例死亡。     

硝酸甘油鼻腔用药治疗血压急性升高的效果观察及体会

临床护理中，当患者血压急性升高时，必须紧急降压抢救治疗；若不及时对症处理，极易形成高血压危象而造成颅内出血、急性心功能衰竭、急性心肌梗死、肾功能衰竭及严重器官损害等，甚至危及生命。我院针对此类患者，采用硝酸甘油经鼻腔用药，以迅速控制血压急性升高，效果良好。     

慢性肾脏病患者并发急性肾损伤的相关因素及护理

分析62例慢性肾脏病并发急性肾损伤患者的相关因素有：严重感染,血压、血糖控制不良,使用肾损害药物。教育患者在感染早期应积极治疗,切勿一拖再拖,或自行使用药物;向患者提供肾性高血压的健康教育知识,纠正患者的不良生活习惯、控制水钠摄入,以更好地控制血压水平;避免使用肾损害药物,抗菌药物使用时强调个体化,根据肾小球滤过率减量或延长给药间隔,必须使用造影剂时应注意充分水化,并在使用过程中严密监测肾功能变化;积极控制血糖及避免各种应激状态对肾脏的损害。提出加强对慢性肾功能不全患者的管理和宣教对延缓慢性肾功能不全患者病情进展,改善患者的临床症状有重要意义。     

妊娠致肾脏损害特点临床和肾活检病理的研究

目的　为探讨妊娠致肾脏损害的临床特点及病理特征。方法　对 46例妊娠致肾脏损害住院患者进行了临床和肾活检病理的分析研究。结果　 46例终止妊娠 3个月后仍有蛋白尿 ,合并肾功能不全者 2 6例 ( 56 .52 % ) ,肾活检免疫病理示 Ig G32例 ,Ig A36例 ,Ig M2 8例及 C338例肾小球血管袢及系膜区沉积 ;病理表现系膜增生性肾炎 2 8例、膜增生性肾炎 1 0例、局灶节段性肾小球硬化 6例、膜性肾病 2例 ,46例中合并小球硬化者 2 8例 ;胎儿死亡 36例 ( 76 .6 0 % )。结论　妊娠激发了免疫反应导致肾脏病变是肾小球疾病发病机制之一 ,积极诊治该类患者是临床不可忽视的问题。     

心肺复苏术成功后合并急性肾功能损伤3例行CRRT的临床效果观察

目的观察连续性血液净化治疗(CRRT)对心肺复苏术成功后合并急性肾功能损伤患者临床效果。方法采用CRRT的主要模式连续性静脉—静脉血液滤过(CVVH)治疗3例心肺复苏术成功后合并急性肾功能损伤患者,对病情、主要抢救措施和结果等临床资料进行回顾性分析。结果例1、例2患者分别经CRRT治疗298h和148h后,尿量逐渐开始恢复,转为隔日进行CRRT治疗,每天治疗6～8h,分别治疗6次和10次后转为普通血液透析滤过治疗,复查肾功能基本正常后停止血液净化治疗;第3例患者经CRRT连续治疗18h后,因血压呈持续下降趋势,达不到CRRT治疗所需要求,经抢救无效临床死亡。结论心肺复苏术成功后部分患者常合并急性肾功能损伤,尽早进行CRRT治疗,可使患者安全度过急性应激期,提高患者的生存率。     

高血压对慢性肾功能衰竭的影响

为观察高血压对慢性肾功能衰竭的影响,我们以１０５例慢性肾功能衰竭患者的血压进行监测３个月,同时观察患者的病情变化,肾功能变化。结果：１０５例慢性肾功能衰竭者中血压增高９７例（９２．４％）经治疗有效控制６８例（７０．１％）,其肾功能和全身症状不同程度好转,而２９例（２９．９０％）,控制血压无效者,肾功能呈进行性损害,全身症状加重,结论：高血压是影响慢性肾功能衰竭的重要因素,在综合治疗慢性肾功能衰竭时     

Progressive systemic sclerosis: Part 1--The disease and medical management

The incidence of scleroderma is approximately 700,000 people in the United States. Pathologic changes occur in a variety of body systems and can alter lifestyle, especially for people with diffuse scleroderma. Information from clinical practice and research concerning the nature, incidence, pathophysiology, complications, and medical management is discussed. Although there is no known cure for scleroderma, life expectancy has improved as management of renal hypertensive crisis and renal failure has improved.     

Cerebral infarction caused by systemic sclerosis: a case report

Systemic sclerosis, also known as scleroderma, is a rare multisystem autoimmune disease characterized by vascular lesions caused by collagen deposition in the skin and viscera and damage to the endothelium. Endothelial injury and microvascular occlusion result in Raynaud’s phenomenon, finger ischemia, pulmonary hypertension, and scleroderma renal crisis. Scleroderma itself is a rare disease with an incidence ranging from 0.1 to 14 per 100,000 people in the general population. Cerebral involvement is not considered a common manifestation of systemic sclerosis, although studies have shown that the brain can be involved. Therefore, to deepen the understanding of this disease, we herein report a case of cerebral infarction associated with systemic sclerosis.     

Different patterns of endothelial cell activation in renal and pulmonary vascular disease in scleroderma

Abnormal endothelial cell function is implicated in the development of scleroderma, and in major life-threatening complications of the disease. The nature of the stimulus leading to abnormal endothelial cell function in scleroderma, scleroderma renal crisis, and scleroderma- associated pulmonary hypertension was investigated by measurement of soluble adhesion molecules, shed by activated endothelial cells, in sera from patients with these conditions. In scleroderma renal crisis, mean levels of soluble E-selectin (p < 0.05 limited scleroderma, p < 0.0001 diffuse scleroderma), sVCAM-1 (soluble vascular cell adhesion molecule-1) (p < 0.05 limited scleroderma, p < 0.05 diffuse scleroderma), and sICAM-1 (soluble intercellular adhesion molecule-1) (p < 0.0001 limited scleroderma, p < 0.0001 diffuse scleroderma) were raised, supporting a model of endothelial cell activation in this complication. Evidence for endothelial cell activation in scleroderma- associated pulmonary hypertension was inconsistent, with normal sE- selectin and normal sVCAM-1 in sera from patients with limited scleroderma and pulmonary hypertension. The endothelial cell phenotype in scleroderma-associated pulmonary hypertension may resemble that of unstimulated cells. The pulmonary vascular and renal vascular lesions associated with scleroderma may arise by distinct pathogenic mechanisms.      

Life-threatening complications of systemic sclerosis

Pulmonary arterial hypertension is common in patients with SSc. Fig. 1 shows the diagnostic and therapeutic approach to PAH in SSc. Doppler echocardiography may suggest the diagnosis, but RHC is necessary to confirm PAH and to measure vasoreactivity. Therapy is directed at the underlying connective tissue disease. Vasoreactive patients often benefit from therapy with high-dose calcium-channel [figure: see text] blockers, but most patients are not vasoreactive. Intravenous epoprostenol and oral endothelin-1 receptor antagonists improve hemodynamic measurements and symptoms in SSc-associated PAH. The therapy of right ventricular failure is focused on vasodilators, inotropes, and diuretics with careful attention to avoiding systemic hypotension. The scleroderma pulmonary-renal syndrome and the scleroderma renal crisis are distinct syndromes with different clinical presentations, histopathologic manifestations, treatments, and outcomes. The scleroderma pulmonary renal syndrome is an autoimmune vasculitis of kidney and lung associated with normal blood pressure. Treatment is supportive, and prognosis is dismal. In contrast, scleroderma renal crisis is associated with systemic hypertension, onion skinning of afferent arterioles, and response to ACE inhibition and renal replacement therapy. Pericardial effusions are common but only occasionally lead to tamponade. Esophageal dysmotility is often associated with aspiration, leading to pulmonary fibrosis, pneumonia, or ARDS. Diffuse bowel involvement may result in pseudo-obstruction, bacterial overgrowth, or malabsorption. Prokinetic agents, antibiotics, and parenteral nutrition may be required.     

Successful treatment of the crisis of scleroderma with enalapril maleate (MK-421)

A 74-year-old man has been known to have scleroderma for 5 years. Two months before the hospitalization, his blood pressure was rapidly elevated, and progressive renal impairment and ulcerations of the fingers manifested. Then he was diagnosed to have a crisis of scleroderma. The treatment with a new long-acting angiotensin I converting enzyme inhibitor, enalapril maleate (MK-421), normalized the blood pressure and protected the progress of renal impairment without side effects.     

Three rheumatologic emergencies: a sore toe, a cough, hypertension

Patients often present to internists with symptoms that can represent a minor illness or a rheumatologic emergency. This paper discusses the recognition and treatment of three rheumatologic emergencies: septic arthritis, alveolar hemorrhage as a complication of systemic lupus erythematosus (SLE), and scleroderma renal crisis.     

Systemic sclerosis/scleroderma: a treatable multisystem disease

Systemic sclerosis (systemic scleroderma) is a chronic connective tissue disease of unknown etiology that causes widespread microvascular damage and excessive deposition of collagen in the skin and internal organs. Raynaud phenomenon and scleroderma (hardening of the skin) are hallmarks of the disease. The typical patient is a young or middle-age woman with a history of Raynaud phenomenon who presents with skin induration and internal organ dysfunction. Clinical evaluation and laboratory testing, along with pulmonary function testing, Doppler echocardiography, and high-resolution computed tomography of the chest, establish the diagnosis and detect visceral involvement. Patients with systemic sclerosis can be classified into two distinct clinical subsets with different patterns of skin and internal organ involvement, autoantibody production, and survival. Prognosis is determined by the degree of internal organ involvement. Although no disease-modifying therapy has been proven effective, complications of systemic sclerosis are treatable, and interventions for organ-specific manifestations have improved substantially. Medications (e.g., calcium channel blockers and angiotensin-II receptor blockers for Raynaud phenomenon, appropriate treatments for gastroesophageal reflux disease) and lifestyle modifications can help prevent complications, such as digital ulcers and Barrett esophagus. Endothelin-1 receptor blockers and phosphodiesterase-5 inhibitors improve pulmonary arterial hypertension. The risk of renal damage from scleroderma renal crisis can be lessened by early detection, prompt initiation of angiotensin-converting enzyme inhibitor therapy, and avoidance of high-dose corticosteroids. Optimal patient care includes an integrated, multidisciplinary approach to promptly and effectively recognize, evaluate, and manage complications and limit end-organ dysfunction.     

Scleroderma renal crisis: patient characteristics and long-term outcomes

BACKGROUND: Scleroderma renal crisis (SRC) is an important complication of systemic sclerosis, causing acute renal failure, and usually hypertension. AIMS: To review the clinical and pathological features of SRC, and correlate them with renal outcomes and mortality. DESIGN: Retrospective case series. METHODS: We identified 110 cases of SRC managed at a single centre between 1990 and 2005. RESULTS: SRC occurred in 5% of scleroderma cases under follow-up. Cases were predominantly female (81%), with diffuse cutaneous disease (78%). RNA polymerase antibodies were found in 59% of cases tested. Almost all (108/110) received treatment with ACE inhibitors (ACEIs). Dialysis was not required in 36%, was required temporarily (for up to 3 years) in 23%, was required permanently in 41%. Patients not on dialysis showed improvement in estimated glomerular filtration rate after SRC (mean change +23 ml/min over 3 years). Poor renal outcome was associated with lower blood pressure at presentation, and with higher age in those requiring dialysis. Steroid use, microangiopathic haemolytic anaemia, and antibody profile were not related to renal outcome. In the 58 renal biopsies available for clinical correlation, acute changes of mucoid intimal thickening in arteries and fibrinoid necrosis in arterioles were associated with a poorer renal outcome. Mortality was high (59% survival at 5 years), and was higher in men. DISCUSSION: Despite the efficacy of ACEIs in managing SRC, the poor long-term outcome warrants evaluation for additional treatments for this devastating complication of systemic sclerosis.     

系统性硬皮病并发急性肾功衰1例报告

系统性硬皮病并发急性肾功衰报告甚少。本文报告1例,其皮肤症状为潮红、水肿和硬化,发病9个月之后出现硬皮病肾性高血压和氮质血症,经皮质激素治疗无效。本文讨论了诊治意见。     

系统性硬化新进展

系统性硬化(systemic sclerosis,SSc)是一种原因不明的复杂的系统性结缔组织病,系统性硬化有多种亚型,它们的临床表现和预后各不相同。虽然已表明SSc发病主要是由于血管炎和纤维化异常,应用血管抑制制剂可以缓解诸如硬皮病肾危象、继发肺动脉高压等病情,近几年随着对SSc发病机制的进一步研究,发现了一些治疗新视点,本文就SSc这些新进展做一综述。