雷公藤多苷治疗儿童肾病综合征的机制探讨

目的 :探讨雷公藤多苷在治疗儿童肾病综合征中的作用及其机制。　方法 :对 16年来在我科住院明确病理类型的 5 5 0例中 10 5例肾病综合征患儿 ,在激素逐渐减量的同时用雷公藤多苷 ( 1m g/ kg· d- 1 )治疗。　结果 :81例肾病综合征获得缓解 ,总缓解率达 77.1% ,其中 MCNS( 2 4例 )、Ms PGN ( 46例 )、MPGN ( 17例 )、MN ( 13例 )和FSGS( 5例 )的缓解率分别为 91.7%、89.1%、76 .5 %、38.5 %和 0 % ,FSGS效果最差 ;14例改善 ,10例无效。　结论 :由于病理类型不同疗效相差较大 ,临床上对激素治疗无效或耐药的肾病综合征患儿 ,应及时行肾活检以明确病理诊断 ,对病理类型为 MCNS和 Ms PGN者可单用雷公藤多苷治疗 ,以避免长期应用激素的不良反应 ,这是治疗儿童肾病综合征的有效方法之一     

甲基强的松龙冲击治疗难治性肾病综合征疗效分析

目的探讨甲基强的松龙冲击疗法对难治性肾病综合征的疗效。方法选择38例难治性肾病综合征患儿,给予甲基强的松龙每次20－30mg／kg,加入10％葡萄糖溶液100ml中静滴、1／d,连用3d,第4－7d口服强的松2mg／(kg·d)。未完全缓解者重复第二疗程。结果冲击治疗后,血浆白蛋白有不同程度的升高,24h尿蛋白定量、血胆固醇、血肌酐(Cr)、尿素氮(BUN)有明显降低,冲击治疗前后相差有高度显著性(P<0．01)。随着MP冲击疗程增加,完全及部分缓解例数增多。结论甲基强的松龙冲击疗法可以使难治性肾病达到较稳定的缓解及保护肾功能,尤其对早期病例效果较好,但必须通过前瞻性对照研究加以证实。     

Extracellular fluid and its proteins: dehydration, shock, and recovery

This review highlights characteristics of extracellular fluid (ECF) that are often overlooked. ECF has, in addition to plasma and interstitial fluid (ISF) surrounding cells, a third large compartment, the ISF of skin and connective tissue. This acts as a reservoir that gives up ECF to plasma volume (PV) in order to sustain circulation in the event of either shock or dehydration. While Starling forces drive filtration, ECF is returned to PV more by lymph and less by Starling forces than previously appreciated. Lymph return to PV is dependent on physical activity and muscle contraction to overcome gravity. Regional change in metabolic rate alters the need for oxygen and nutrients that is met by a regional increase in capillary blood flow. Blood flow is controlled by vasoactive compounds released in response to a drop in PO₂; these relax capillary smooth muscle to increase blood flow and delivery of oxygen and nutrients. Plasma proteins, including albumin, are filtered into the interstitium through larger pores than those filtering ECF. The rate of protein filtration is set by size and charge of these larger endothelial pores and by size and charge of proteins. Charge of these pores, hence albumin permeability, is regulated by many of the same vasoactive compounds that control capillary flow. As a consequence, in response to gravitational stress and other forms of shock that reduce effective circulation, albumin as well as ECF is rapidly shifted from plasma and sequestered in ISF. When this has occurred, as in burn shock, restoration is better effected by generous expansion of ECF with Ringer’s solution alone, rather than with Ringer’s solution supplemented with human serum albumin or other colloid. Restoring both PV and ISF volume restores lymph circulation and returns sequestered albumin to PV. Received: 12 November 1998 / Revised: 30 March 1999 / Accepted: 2 April 1999     

Mycoplasma pneumoniae-associated nephritis in children

 Mycoplasma pneumoniae infection is a rare cause of acute nephritis. Six children (2 girls) aged 5–10 years, admitted for nephritis, had serological tests showing recent Mycoplasma pneumoniae infection. The diagnosis of Mycoplasma pneumoniae infection was based on the presence of serum IgM, detected either by immunofluorescence (IF) (n=1) or enzyme-linked immunosorbent assay (n=5). Four children had a renal biospy, with analysis of parenchymal Mycoplasma pneumoniae components by indirect IF and polymerase chain reaction. Extrarenal symptoms were: respiratory (n=3), ear, nose, and throat (n=2), gastrointestinal (n=3), hepatic (n=1), neurological (n=1), articular (n=1), and hematological (n=3). The patients presented with acute nephritis (1 had a nephrotic syndrome) or with acute renal failure and proteinuria. Pathological findings included type 1 membranoproliferative glomerulonephritis (MPGN, n=1), proliferative endocapillary glomerulonephritis (n=2), and minimal change disease (n=1). The patient with type 1 MPGN progressed rapidly towards end-stage renal failure because of a congenital solitary kidney. Among the patients with endocapillary glomerulonephritis, 1 relapsed 6 months later and remained proteinuric, while the other recovered, as did the child with minimal change disease. The search for Mycoplasma pneumoniae antigens and nucleic acids in renal tissue was negative. However, the absence of the microorganism in the kidney is a common feature of post-streptococcal glomerulonephritis. We conclude that Mycoplasma pneumoniae is a rare yet potential cause of acute glomerulonephritis. Received: 13 September 1996 / Revised: 16 June 1998 / Accepted: 18 June 1998     

Influence of serum albumin on renal function in nephrotic syndrome

 Glomerular filtration rate (GFR) and effective renal plasma flow (ERPF), determined by the clearances of inulin and para-aminohippuric acid, were evaluated in 119 children with different types of nephrotic syndrome and in different stages: the nephrotic stage (serum albumin <25 g/l), recovery stage (25–35 g/l), and remission (>35 g/l). GFR in the nephrotic stage was significantly lower than in remission and in controls, and was lowest at onset of the disease (84±6, 111±4, and 119±2 ml/min per 1.73 m²). ERPF was higher in the nephrotic stage than in recovery, especially in children with histological lesions. Thus the filtration fraction (FF) was greatly decreased in the nephrotic stage. In patients investigated both in the nephrotic and the remission phase, GFR and FF increased significantly. There was a direct correlation between the serum albumin concentration and FF and an inverse correlation between mean arterial pressure (MAP) and GFR and FF in all patients, a direct correlation between the serum albumin concentration and GFR in minimal change nephrotic syndrome patients, and an inverse correlation between ERPF and serum albumin in children with histological lesions. In conclusion, GFR and FF were decreased and ERPF increased in the nephrotic stage, normalizing in remission. The low GFR in the nephrotic stage was thus not dependent on hypoperfusion. We suggest that the low GFR is dependent on a very low ultrafiltration coefficient. The direct correlation between GFR and serum albumin and the indirect correlation between GFR and MAP suggest compensatory mechanisms that increase the ultrafiltration pressure to counteract the severely reduced ultrafiltration coefficient. Received: 19 November 1997 / Revised: 11 April 1998 / Accepted: 14 April 1998     

肾病综合征、肾小球肾炎患儿血清铜、锌、铁、镁与血清蛋白质的测定与分析

采用原子吸收分光光度法,测定50 例肾病综合征患儿、28 例肾小球肾炎患儿血清铜、锌、铁、镁元素,并探讨这些元素与上述疾病的相互关系。结果显示,肾病综合征组和肾小球肾炎组血清锌均极显著低于正常组( P< 0-01) ,血清铁、镁与正常组比较无显著性差异,血清锌浓度与血清白蛋白、球蛋白含量呈显著正相关。肾病综合征组血清铜极显著低于正常组(P< 0-01) ,肾小球肾炎组血清铜与正常组比较无显著差异,血清铜浓度与血清白蛋白、球蛋白含量呈显著正相关。提示这些变化与疾病的发生和疾病状态有关     

Pulmonary thrombo-embolism in nephrotic syndrome treated with tissue plasminogen activator

We describe the case of a boy with steroid sensitive nephrotic syndrome and left pulmonary artery thrombo-embolism. Clinical presentation initially suggested sepsis and respiratory signs were minor. Treatment with tissue plasminogen activator infused into the pulmonary artery was successful. Conclusion Pulmonary thrombo-embolism should be considered in unwell children with nephrotic syndrome. Received: 6 September 1996 / Accepted: 17 December 1996     

甲泼尼龙冲击治疗儿童难治性肾病的临床与病理

目的 :探讨小儿难治性肾病的有效治疗措施、疗效与病理类型的关系。　方法 :总结了使用甲泼尼龙冲击治疗的 30例小儿难治性肾病的经验。甲泼尼龙 15～ 30 mg/ ( kg· d) ,共 3天 ,间歇 4～ 7天后用第二个疗程 ,疗程间隙期用泼尼松 1.5～ 2 mg/ ( kg· d)口服 ,疗程结束后 ,泼尼松按中长程疗法治疗。　结果 :30例肾病综合征 14例完全缓解 ,4例部分缓解 ,总有效率为 6 0 % ,无一例出现严重的不良反应。　结论 :甲泼尼龙冲击治疗小儿难治性肾病是一种安全有效的方法 ,特别是对 Ms PGN、MPGN、MCNS更佳     

原发性肾病综合征患儿肾组织中pRb的表达及意义

目的探讨肾病综合征患儿肾组织视网膜母细胞瘤基因的蛋白产物pRb的表达水平及其与肾脏固有细胞增生之间的关系。方法以39例原发性肾病综合征患儿肾活检石蜡包埋肾组织及6例肾肿瘤肾切除患儿的正常肾组织作为研究对象,用免疫组织化学方法检测了pRb在肾组织的表达水平,并与肾病综合征患儿的病理类型、肾组织病理学积分及肾组织中增殖细胞核抗原(PCNA)的表达水平进行比较。结果肾病综合征组肾小球内PCNA和pRb阳性细胞百分率、肾小管-间质内的PCNA阳性细胞百分率均明显高于对照组,且不同病理类型肾病综合征患儿的肾小球内PCNA和pRb阳性细胞百分率存在显著差异。肾小球内PCNA和pRb的阳性细胞百分率分别与肾小球的病理积分呈正相关,肾小球内pRb的阳性细胞百分率和PCNA阳性细胞百分率呈正相关。结论肾病综合征患儿肾组织pRb表达水平增加促进了肾脏固有细胞的异常增生。     

通肾汤对阿霉素肾病综合征大鼠红细胞膜上负电荷的影响

目的观察通肾汤对阿霉素肾病综合征大鼠模型红细胞膜表面负电荷含量的影响.方法采用阿霉素肾病综合征大鼠模型及Alcain蓝(AB)染色方法.结果阿霉素肾病大鼠模型组红细胞膜结合AB的量与正常组相比,P＜0.01,而激素组、通肾汤组、激通组红细胞膜结合AB的量与阿霉素肾病大鼠模型组相比明显改善(P＜0.01),改善程度为:激通组＞激素组＞通肾汤组.各组红细胞经神经氨酸酶处理后,其表面结合AB含量的下降程度无显著性差异(P＞0.05).结论阿霉素肾病大鼠模型红细胞结合AB含量下降,而通肾汤能逆转这一改变,使红细胞结合AB含量达正常水平。同时，阿霉素肾病大鼠模型虽有红细胞表面结合AB含量减少，但其表面主要的负电荷物质-涎酸的含量并无变化。