难治性肾病综合征预测及治疗近况

<正> 难治性肾病综合征是指激素耐药、激素依赖或频繁复发型,病理类型大多为局灶节段性肾小球肾炎、膜性肾病、膜增殖性肾炎、系膜增殖型及部分微小病变型肾病(MCNS)。近年来,难治性肾病在预测以及治疗方面取得重大进展,现综述如下。 1.发病机理目前研究认为难治性肾病综合征可能与T细胞功能紊乱,部分与肾内免疫复合物沉积有关。频繁复发型肾病患儿由于IgG_1和IgG_2显著降低,尤其是IgG_2的减低,使体内抗病毒和细菌的抗体不足,致肾     

难治性肾病综合征发病机制研究进展

肾病综合征(NS)是以大量蛋白尿、低蛋白血症及高度水肿为主要表现的一组临床综合征.免疫异常为NS的重要病因之一,以肾上腺皮质激素治疗为主.难治性肾病综合征(RNS)包括微小病变及系膜增生性肾炎中的激素依赖或抵抗型,膜性肾病、局灶节段性肾小球硬化(FSGS)及系膜毛细血管增生性肾炎中的激素抵抗型.     

环胞素A对小儿肾病的治疗

<正> 小儿肾病综合征(肾病)大多数对肾上腺皮质激素(激素)治疗敏感;部分对激素+免疫抑制剂敏感,可致不同间期的缓解;少数却对激素及环磷酰胺依赖或耐药,而发展成难治性肾病。据154例肾病的肾活检示:微小病变性肾病占34.2％,局灶硬化性肾炎26.4％,膜增殖性肾炎12.1％,肾小管坏死19.2％,其它7.8％。任何形式的治疗都不能改变局灶性肾小球硬化、激素依赖性肾病的自然历史。肾病治疗的目的在于诱导缓解,预防复发。而肾病在初治中常有二、三次复发,在病程的4月、6月、12月中则有更多的复发,故预防复发已成为当前肾病治疗中一个重要的研究课题。     

小儿肾病综合征246例临床分析

目的 对小儿肾病综合征作临床分析。方法 运用病例回顾性分析方法，分析246例临床资料完整的肾病综合征。结果 单纯性肾病156例，肾炎性肾病90例；单独使用激素治疗单纯性肾病109例和肾炎性肾病52例达到完全缓解；联合使用免疫抑制剂治疗85例，59例达到完全缓解，18例部分缓解。32例复发病例中，有12例为单纯性肾病，20例为肾炎性肾病。结论 单纯用激素治疗可使大部分病例达到完全缓解，加用免疫抑制剂可提高疗效。单纯性肾病预后较肾炎性肾病好，复发率低。     

肾炎性肾病综合征32例临床分析

本文对32例肾炎性肾病综合征进行临床分析,其中血尿最为多见。发病年龄7岁内儿童占34·4%,最小者17/12岁。12例肾活检中,6例膜增生性肾小球肾炎,4例为增生性肾小球肾炎,1例膜性肾病,1例IgM 肾病。29例用激素治疗,初治敏感者占20.7%,且均有复发。22例加用免疫抑制剂,有14例获近期缓解。     

难治性肾病综合征的诊治探索

难治性肾病综合征是原发性肾病综合征中频繁发作激素依赖或耐药病例的总称.肾上腺皮质激素常为治疗肾病综合征的一线药物,然而对部分激素及其他免疫抑制剂不敏感的患者临床治疗颇为棘手.借鉴来氟米特治疗狼疮性肾炎疗效显著、副作用小的特点, 对于难治性肾病综合征病例可以试行用来氟米特联合氟吡格雷联合治疗,可以减少激素剂量、疗程,短期观察未见严重毒副作用.     

膜性肾病的中西医结合治疗进展

膜性肾病是成人肾病综合征常见的病理类型之一,临床上发病率较高。病变呈慢性的进行性发展,可逐渐出现肾功能不全、尿毒症。激素、细胞毒类药物疗效欠佳,且有较大的不良反应。近年来临床多采用中西医结合治疗膜性肾病,取得了较好的效果,且能有效减少激素及免疫抑制剂的不良反应。本文就有关中西医结合治疗膜性肾病的研究综述如下。     

儿童原发性肾病综合征40例临床分析

目的：探讨儿童原发性肾病综合征的临床特征。方法：对40例原发性。肾病综合征患儿的临床资料进行回顾性分析,其中单纯性肾病31例,肾炎性肾病9例。结果：患儿临床表现以水肿为主,且为就诊首原因。经休息、限制钠的摄入、利尿消肿,以及应用肾上腺皮质激素和细胞毒药物治疗,单纯性肾病完全缓解率70．1％,肾炎性肾病完全缓解率33．3％。合并感染9例均为呼吸道感染,低钠血症4例,甲状腺功能减低2例,急性肾功能不全2例,脑血管栓塞1例。结论：儿童原发性肾病综合征经泼尼松治疗单纯型肾病完全缓解率高于肾炎性肾病,对激素耐药的肾病综合征患儿采用免疫抑制剂有一定的疗效,治疗过程中应注意并发症及加强对患者及家属治疗依从性教育。     

66例成人原发肾病综合征临床治疗与病理分析

本对６６例原发肾病综合征患进行病理类型与临床疗效分析。最常见病理类型为系膜增殖性肾炎，占４２．５％，其次为膜性肾病，占３２．５％，ＩｇＡ肾病占１５．０％。微小病变型及系膜增殖性肾炎对激素及免疫抑制剂治疗效果好，膜性肾病治疗效果差。不同病理类型患病理损害程度与疗效相关。伴肾小管间质病变治疗效果差（Ｐ〈０．０５）。ＮＳⅠ型疗效明显优于ＮＳⅡ型（Ｐ〈０．０５）。５０岁以下原发肾病综合征（ＰＮＳ）     

中医药治疗膜性肾病研究进展

膜性肾病是成人肾病综合征最常见的病理类型,目前采用的激素及免疫抑制剂对一部分患者疗效欠佳,且长期应用的不良反应较多,而中医药在治疗膜性肾病方面的优势逐渐凸显,文中对近几年的膜性肾病的相关文献进行综述,目的在于为今后中医药治疗膜性肾病的研究提供思路,指导临床实践。     

肾病综合征不同治疗方案疗效的对比研究

目的:比较不同肾上腺糖皮质激素单独应用及激素与不同免疫抑制剂联合应用治疗原发性肾病综合征(PNS)的疗效。方法:140例原发性肾病综合症患者按所选治疗药物及方案不同分为6组,分别给予单独强的松口服、单独甲基强的松龙静点、强的松分别与长春新碱、环磷酰胺、来氟米特、雷公藤多甙合用,观察疗效及部分生化指标。结果:①原发性肾病综合征不同治疗方案之间末次随访时缓解率无明显差异,但达到缓解所需时间各不相同。①强的松口服治疗与甲基强的松龙静点治疗2组达到缓解所需时间存在显著性差异(P<0.001),前者所需时间较后者平均长13.1d,甲基强的松龙静点组治疗后血清白蛋白增长情况及24h尿蛋白减少情况均明显优于强的松口服组。③糖皮质激素与免疫抑制剂联合应用达到缓解所需时间较单用糖皮质激素治疗达到缓解所需时间平均短1￣3周,且前者治疗后血清白蛋白增长及24h尿蛋白减少情况明显优于后者。结论:在原发性肾病综合征的各种治疗方案中,糖皮质激素与长春新碱联合治疗最安全、最有效。     

Amyloidosis complicated by intractable postural hypotension

A patient is described in whom the nephrotic syndrome caused by amyloidosis was complicated by intractable postural hypotension. An account is given of his treatment with a combination of monoamine oxidase inhibitors and sympathomimetic pressor agents, after a failure of conventional therapy.     

小儿难治性肾病25例疗效观察与随访

对25例小儿难治性肾病(其中单纯型9例:肾炎型16例)应用激素中长程疗法治疗,小剂量长期维持,于激素减量时加用免疫抑制剂。同时在整个疗程中均服用中药“疫免糖浆”。经治疗及随访3年～5年,完全缓解20例,部分缓解3例,死亡1例,失访1例。认为用此法治疗小儿难治性肾病疗效较好。     

Subclinical acquired syphilis masquerading as membranous glomerulonephritis

Membranous glomerulonephritis (MGN) is one of the common forms of nephrotic syndrome in the adult population. The majority of MGN are idiopathic, but the secondary forms can be seen in the setting of autoimmune disease, neoplasia, infection and following exposure to certain therapeutic agents. Histologically, MGN is an immunologically mediated disease in which immune complexes deposit in the subepithelial space. Syphilis is a venereal disease that can also be acquired by exposure to infected blood. Untreated syphilis may progress and develop renal complications such as membranous glomerulonephritis (MGN) or diffuse endocapillary glomerulonephritis with or without crescent formation. Today, with increasing awareness of sexually transmitted diseases especially HIV infection coupled by the practice of protected sexual intercourse and advancement of medicine, we have seen fewer and fewer cases of acquired syphilis. Furthermore, majority will present with typical syphilitic symptoms of such as chancre, rash, fever and lymph node enlargement in which case the diagnosis is easily obtained. We are reporting a case of acquired syphilis masquerading as membranous glomerulonephritis without typical syphilitic symptoms.     

肾病综合征中医临床证治思路

肾病综合征(nephrotic syndrome,NS)是肾脏疾病常见类型之一,其确切发病机制尚未完全明确.中医药治疗本病历史悠久,具有独特的优势和鲜明的特色,目前临床上中医药联合激素及细胞毒药物应用治疗NS效果较为显著.本文对肾病综合征中医病因病机、证治规律等进行了分析阐述,在此基础上提出了肾病综合征的中医临床证治思路,并着重就脾肾双补、活血化瘀、分期辨证结合等思路方法进行了探讨.     

Platelet function in nephrotic syndrome patients at the Kenyatta National Hospital

Platelet function was assessed in 40 patients with nephrotic syndrome but without renal failure in order to establish whether or not there is any haemostatic disorder lending to hypercoagulable state. The findings were compared with those from 40 normal controls. There was no clinical evidence of thromboembolic phenomena in the patients. Values for the mean platelet counts and clot retraction were similar (P less than 0.05), whereas significant decrease of platelet adhesiveness (P less than 0.001) as well as prolonged platelet aggregation time (P less than 0.001) were noted. This is different from some reports in literature which have frequently reported enhanced platelet function. There may be a difference in the way platelets metabolise arachidonic acid to potent aggregating agents, in the African patients as compared to patients studied elsewhere. The hypercoagulable state in our nephrotic syndrome may be explained by alterations in other haemostatic parameters rather than in platelet function.     

Timing and predictive factors of developing chronic kidney disease in childhood-onset idiopathic nephrotic syndrome: an Asian experience

INTRODUCTIONIdiopathic nephrotic syndrome (INS) is the commonest type of nephrotic syndrome in children, and a majority of cases have favourable outcomes. A small proportion of INS cases progress to chronic kidney disease (CKD). We investigated the time to CKD and predictive risk factors associated with progression of CKD in these children.METHODSA retrospective review of medical records was done to investigate the demographic variables, and biochemical and histological changes in children with INS aged 12 months to 18 years between 2001 and 2016 at Hospital Universiti Sains Malaysia. The median renal survival time for progression to CKD stage III or higher was determined using survival curve analysis. Multiple Cox regression analysis was used to identify predictive factors for CKD.RESULTSThe total number of participants was 112 (boys: n = 71; girls: n = 41) and a majority had steroid-sensitive INS. Only about 10% of INS progressed to CKD Stage III or higher, with an overall median renal survival time of 19 years. Median renal survival time in steroid-resistant nephrotic syndrome (SRNS) was 13 years. Focal segmental glomerulosclerosis was predominant in SRNS. The predictors of progression to CKD were steroid resistance (adjusted hazard ratio [HR] [95% confidence interval (CI)] 23.8 [2.8–200.9]) and the presence of hypertension at presentation (adjusted HR [95% CI] 8.1 [1.2–55.7]).CONCLUSIONThe median renal survival time in our study was comparable to other studies. SRNS and the presence of hypertension at presentation were the main predictors for developing CKD in our population.     

中医药调节免疫机能减少肾病综合征复发

根据肾病综合征的发生发展与机体免疫功能失调密切相关，总结４８例治疗经验，认为和解枢机，升发少阳可调节免疫状态；增强稳定，健脾补肾护卫可提高免疫功能。以汤药治疗获得很好的疗效，其中显效１４例，好转３０例，无效４例，有效率达９１．６％，且大部分病人胳药后血清ＩｇＧ明显升高，尿蛋白减少。结果表明和解、扶正中药有调节免疫机能，减少复发的作用。     

儿童原发性肾病综合症治理方案

皮质激素、烷基化物如环磷酰胺、苯丁酸氮芥用于原发性肾病综合症近50年,改变了肾病综合症儿童的预后.然而,长期使用激素,尤其较高剂量却引起严重副作用.较新药物如环孢A、左旋咪唑、他克莫司、霉酚酸酯可免去不少激素副作用.在选择药物时,应衡量及平衡效益与副作用,并考虑药物价格.虽然大部份肾病综合症儿童都会复发,然而预后却良好,不少肾病综合症儿童成长后不再复发,或只间有复发,故治疗方案应以最少药物以维持缓解,以过渡儿童期,避免不必要的副作用.至于激素抵抗(SRNS)者,尤其局部节段性肾小球硬化(FSGS),预后可致终末期肾衰;治疗方面,用药可较为进取,多需较强免疫抑制剂如环孢A、他克莫司、霉酚酸酯等加以控制.治疗肾病综合症儿童的长期目标,还需考虑生长、教育与心理影响,尤其在过渡青春期时,能让他们正常地成长.     

异基因骨髓移植后肾病综合征的临床与病理分析

目的探讨异基因骨髓移植后肾病综合征的临床和病理特点。方法对1例异基因骨髓移植后发生肾病综合征患者的临床、病理资料及治疗进行综合分析。结果本例患者在骨髓移植后28个月发生肾病综合征,在此之前患者有皮肤、口腔和肝脏损害表现;肾脏病理表现为膜性肾病。糖皮质激素及霉酚酸酯治疗后肾病综合征完全缓解。结论异基因骨髓移植后肾病综合征是慢性移植物抗宿主病的肾脏表现,可能为自身免疫性疾病,是移植物对宿主产生的一种排斥反应。肾穿刺活检对该病的诊断和预后判断具有重要价值,免疫抑制剂治疗有效。