儿童急性链球菌感染后肾炎致肾病综合征激素的治疗作用

目的 探讨糖皮质激素对儿童急性链球菌感染后肾炎(PSAGN)致肾病综合征(NS)的治疗作用.方法 采取开放对照研究的方法对63例呈NS表现的儿童PSAGN在常规治疗的基础上给予标淮中长程的泼尼松治疗.治疗结果与常规治疗组进行对比.结果 激素组治疗6个月末95.2%(60/63)尿蛋白阴转,12个月末100%(63/63)阴转;常规组在相同时期尿蛋白阴转率分别为74%(37/50)和80%(40/50).激素组治疗12个月末95.2%(60/63)镜下血尿消失,18个月末100%(63/63)消失;常规组在相同时期镜下血尿消失率分别为72%(36/50)和84%(42/50).激素组治疗2个月末血压恢复正常为92.1%(35/38),3个月末为100%(38/38);常规组在相同时期血压恢复正常分别为72.7%(24/33)和84.8%(28/33).激素组治疗1个月末肾功能恢复正常为91.7%(11/12),2个月末为100%(12/12);常规组在相同时期分别为42.9%(3/7)和57.1%(4/7).两组的上述治疗效果差异有显著性(P＜0.05或P＜0.01).结论 对PSAGN致NS患儿使用糖皮质激素治疗可有效减少尿蛋白及镜下血尿,促使肾功能、血压较快恢复,改善预后,减少肾脏慢性病变.     

糖皮质激素对小儿肾病综合征的治疗

在小儿肾病综合征的治疗中，糖皮质激素是最常应用的治疗方案之一，其临床的有效性是儿科临床医师所公认的，但在对糖皮质激素敏感的肾病综合征中，在发挥激素的最大临床效果而副作用最小的理想剂量和疗程上，一直存在争论。关于这类的临床研究很多，其中还包括一些多中心的临床研究，应用循证医学的理论和方法来分析、评价这些研究，以供儿科临床医师参考。     

促肾上腺皮质激素对频复发或激素依赖型肾病综合征患儿的疗效

目的:探讨促肾上腺皮质激素（ACTH）对儿童频复发或激素依赖型肾病综合征的疗效及安全性。方法:回顾性分析2015年1月至2020年12月就诊于浙江大学医学院附属儿童医院肾内科的38例频复发或激素依赖型肾病综合征患儿的临床资料。收集患儿的一般情况、临床表现、实验室检查、治疗、随访（至治疗后12个月）等资料。根据治疗方案分...     

原发性肾病综合征合并急性肾功能衰竭临床观察

分析 710例原发性肾病综合征 (NS)合并急性肾功能衰竭 2 8例 (ARF)的临床特点 ,其发病率为 3.9%。临床表现 :少尿 19例 (6 7.8% ) ,无尿 3例 (10 .71% ) ,合并昏迷抽搐 3例 (10 .71% ) ,腹水 16例(5 7.14% ) ,胸水 7例 (2 5 % ) ,高血压 9例 (32 .14% ) ,胃肠道症状 8例 (2 8.5 7% ) ,贫血 10例 (35 .71% ) ,出血6例 (2 1.42 % )。 3例以ARF为NS的首发症状 ,占 10 .71% ,诱因依次为感染 15例 (5 3.5 7% ) ,腹泻 6例(2 1.43% ) ,长期禁盐 2例 (7.14% ) ,原因不明 5例 (15 .86 % )。结果表明对NS应定期例行尿分析、2 4h尿蛋白定量、肝肾功能、血脂等检查。一旦并发ARF应尽早给予肾上腺皮质激素及对症等联合治疗。该组 2 8例中 2 7例逆转 (96 .43% )。NS并发ARF如及时发现治疗多数是可以逆转的。     

糖皮质激素对肾病综合征患儿成骨细胞功能的影响

目的　糖皮质激素是治疗肾病综合征的首选药物。但糖皮质激素可抑制成骨细胞功能,导致骨质疏松。该研究通过检测成骨细胞不同分化阶段的生化指标:I型前胶原羧基端前肽(PICP)、骨钙素(BGP)和总碱性磷酸酶(AKP),探讨糖皮质激素对肾病综合征(NS)患儿成骨细胞功能的影响。方法　测定正常对照组(n=30),未治NS患儿(n=30)和激素治疗后NS患儿(每日泼尼松2mg/kg治疗4 ~8周,n=30)血清PICP、BGP及AKP水平。结果　未治NS患儿血清PICP165 ±56μg/L,BGP15 ±9ng/L水平明显低于正常对照组205 ±81μg/L, 19 ±12ng/L(均P<0. 05),而血清总AKP198 ±71U/L与正常对照组202 ±46U/L比较差异无显著性。激素治疗后NS患儿血清PICP85 ±56μg/L、BGP8±5ng/L、AKP104 ±59 U/L均明显低于未治NS患儿(P<0. 01)。结论　NS患儿本身存在骨合成障碍,大剂量糖皮质激素治疗可进一步抑制NS患儿的成骨细胞合成功能。     

糖皮质激素受体基因多态性与肾病综合征患儿激素耐药相关性探讨

目的　筛选人类糖皮质激素受体基因 (NR3C1)的多态性 ,并分析其在激素耐药型、激素敏感型肾病综合征患儿以及参照人群 (随机抽取的脐血标本 )中的分布 ,以研究NR3C1多态性与肾病综合征患儿激素耐药的关系。方法　提取 3 9例激素耐药型、67例激素敏感型肾病综合征患儿以及64例参照人群血基因组DNA ,PCR扩增NR3C1中编码人类糖皮质激素受体全部功能区的第 2～ 9α外显子 ,以变性高效液相色谱 (DHPLC)分析检测PCR产物 ,对洗脱曲线异常者进行DNA测序。结果　在总计 170份基因组DNA样本中 ,DHPLC分析发现 12种多态性 ,均经DNA测序证实。另外 ,有 3组多态性呈紧密连锁的单倍型 ( [198G >A + 2 0 0G >A] ,[13 74A >G +IVSG 68_IVSG 63delAAAAAA +IVSH 9C >G + 2 3 82C >T] ,[1896C >T + 2 166C >T + 2 43 0T >C] )。后 2种单倍型为首次报道 ,它们在激素耐药型肾病综合征组的基因型频率 ( 10 .3 %和 15.4% )明显高于敏感型肾病综合征 ( 1.5%和 7.5% ) ,2种单倍型的OR值分别为 7.54和 2 .2 6。其余多态性在各组中出现频率相对较低。结论　在NR3C1基因中筛选出 12处多态性 ;而且新发现的 2种多位点紧密连锁的单倍型可能与肾病综合征患儿发生糖皮质激素耐药有关     

小儿原发性肾病综合征合并特发性急性肾功能衰竭的临床分析

目的 探讨小儿原发性肾病综合征(PNS)合并特发性急性肾功能衰竭(IARF)的临床病理特点、疗效及预后.方法 回顾性分析1997年1月至2007年11月入院的12例PNS合并IARF患儿的临床资料.结果 476例PNS患儿中,合并IARF12例(占2.52%),其中男9例,学龄儿童9例.全部病例均发生在PNS活动期,均为少尿型肾功能衰竭,且有较严重的低白蛋白血症[(20.13±5.35)g/L]、蛋白尿[(9.45±3.55)g/d]及明显水肿.合并胸腔、腹腔等浆膜腔积液及血压增高者7例,且均有外周血小板、纤维蛋白、纤维蛋白降解产物、D-二聚体增高,凝血酶原时间缩短等指标中一项或多项的异常.B超示双肾肿大10例.5例接受肾活检,其中微小病变3例,轻度系膜增生性肾炎2例,其共同病理变化示小管间质病变广泛.经甲泼尼龙、小剂量多巴胺联用等量甲磺酸酚妥拉明、必要时间断输注血浆或加用呋塞米及抗凝等综合治疗,肾功能均恢复正常.随访0.5～2年,IARF未见再发.结论 小儿PNS合并IARF以男性学龄儿童多见,临床表现为少尿型肾功能衰竭、严重的蛋白尿、低白蛋白血症及明显水肿,常合并浆膜腔积液、高血压及血液高凝状态;B超示肾肿大多见;肾病理诊断以肾小球轻微病变为主,且小管间质病变广泛;通过早期积极综合治疗,其预后良好.     

糖皮质激素对肾病综合征患儿成骨细胞功能的影响

目的　糖皮质激素是治疗肾病综合征的首选药物。但糖皮质激素可抑制成骨细胞功能,导致骨质疏松。该研究通过检测成骨细胞不同分化阶段的生化指标:I型前胶原羧基端前肽(PICP)、骨钙素(BGP)和总碱性磷酸酶(AKP),探讨糖皮质激素对肾病综合征(NS)患儿成骨细胞功能的影响。方法　测定正常对照组(n=30),未治NS患儿(n=30)和激素治疗后NS患儿(每日泼尼松2mg/kg治疗4 ~8周,n=30)血清PICP、BGP及AKP水平。结果　未治NS患儿血清PICP165 ±56μg/L,BGP15 ±9ng/L水平明显低于正常对照组205 ±81μg/L, 19 ±12ng/L(均P<0. 05),而血清总AKP198 ±71U/L与正常对照组202 ±46U/L比较差异无显著性。激素治疗后NS患儿血清PICP85 ±56μg/L、BGP8±5ng/L、AKP104 ±59 U/L均明显低于未治NS患儿(P<0. 01)。结论　NS患儿本身存在骨合成障碍,大剂量糖皮质激素治疗可进一步抑制NS患儿的成骨细胞合成功能。     

肾病综合征患儿血浆心钠素水平观察

心钠素(ANP)亦称心房肽和心房利钠多肽。它是心脏细胞所分泌的一种循环激素,具有强大的利钠、利尿、舒张血管、抑制肾素-血管紧张素系统的作用,在高血压、心、肾功能不全等疾病的发病中具有重要意义。本文对肾病综合征临床表现与血浆ANP相互关系进行了探讨。     

Increasing the dose of prednisolone during viral infections reduces the risk of relapse in nephrotic syndrome: a randomised controlled trial.

BACKGROUND: Relapses of nephrotic syndrome are often triggered by viral upper respiratory tract infections (URTIs), possibly mediated by cytokine release. OBJECTIVE: To test, in a randomised double-blind placebo-controlled crossover trial, the hypothesis that a small short-term increase in the dose of prednisolone will reduce the release of cytokines and thereby reduce the risk of relapse. METHODS: Sequential patients receiving low-dose (<0.6 mg/kg) prednisolone on alternate days as maintenance therapy were recruited. At the first sign of a presumed viral URTI, all children were examined and randomly allocated to take medicine A or B (containing either prednisolone (5 mg) or placebo) in the first viral URTI, and vice versa in the second. If the criteria for diagnosis of a viral URTI were met, the new medicine was prescribed on alternate days for 1 week at the same dose as that of the prednisolone being taken by the patient on an alternate-day basis. A freshly voided urine sample was tested each morning. The presence of 3+ proteinuria for 3 consecutive days was diagnostic of relapse. RESULTS: 48 patients were recruited, and 40 completed the trial (29 male; 11 female). Age at entry ranged from 1.5 to 13.2 (median 5.3) years. The relapse rate after viral URTI was 19/40 (48%) in the placebo group and 7/40 (18%) in the prednisolone group (p = 0.014; two-sided probability using Fisher's exact test). CONCLUSION: Prescribing prednisolone daily for 7 consecutive days at the same dose as that taken by the patient on an alternate-day basis at the onset of a presumed viral URTI significantly reduces the risk of relapse in children with steroid-dependent nephrotic syndrome.     

Low dose prednisolone in nephrotic syndrome.

Sixteen patients with steroid responsive nephrotic syndrome were treated on 29 separate occasions with a low dose of prednisolone (30 mg/m2/day). All went into remission within 14 days. The duration of remission in the six patients who had had previous relapses treated with a higher dose of prednisolone was similar.     

Adrenocortical suppression increases the risk of relapse in nephrotic syndrome

Objective

Children with nephrotic syndrome (NS) are usually treated with long‐term low dose alternate day prednisolone with or without glucocorticoid sparing therapy, such as levamisole or ciclosporin, to maintain remission. The degree of hypothalamic‐pituitary‐adrenal axis (HPA) suppression with such therapeutic strategies has not been studied systematically. HPA suppression could cause a relapse or adrenal crisis.

Study design

To study the risks of HPA suppression, a modified low dose synacthen test (0.5 μg) was administered to 32 patients (22 male,10 female) with a mean age of 9.7 years (range 3.8–17.6 years) with NS receiving long‐term alternate day prednisolone for over 12 months. Twelve patients received alternate day prednisolone, 11 alternate prednisolone+levamisole and nine alternate prednisolone+ciclosporin. All patients were followed up for 3 years and the relapse rate noted.

Results

20/32 (62.5%) patients had a peak serum cortisol concentration of <500 nmol/l, which suggested suboptimal cortisol secretion and possible HPA suppression. 10/12 children in the prednisolone group and 8/11 in the levamisole group had a suboptimal cortisol response compared with 2/9 in the ciclosporin group. During follow‐up, the 20 children who had a suboptimal cortisol response had significantly more relapses (95 relapses) compared to the 12 children with a normal cortisol response who had 24 relapses (p = 0.01).

Conclusions

Children with NS receiving long‐term alternate day prednisolone therapy are at risk of developing HPA suppression and should be evaluated using the modified synacthen test. Children with evidence of HPA suppression are at a greater risk of relapse.     

Adrenocortical suppression increases the risk of relapse in nephrotic syndrome.

OBJECTIVE: Children with nephrotic syndrome (NS) are usually treated with long-term low dose alternate day prednisolone with or without glucocorticoid sparing therapy, such as levamisole or ciclosporin, to maintain remission. The degree of hypothalamic-pituitary-adrenal axis (HPA) suppression with such therapeutic strategies has not been studied systematically. HPA suppression could cause a relapse or adrenal crisis. STUDY DESIGN: To study the risks of HPA suppression, a modified low dose synacthen test (0.5 mug) was administered to 32 patients (22 male,10 female) with a mean age of 9.7 years (range 3.8-17.6 years) with NS receiving long-term alternate day prednisolone for over 12 months. Twelve patients received alternate day prednisolone, 11 alternate prednisolone+levamisole and nine alternate prednisolone+ciclosporin. All patients were followed up for 3 years and the relapse rate noted. RESULTS: 20/32 (62.5%) patients had a peak serum cortisol concentration of <500 nmol/l, which suggested suboptimal cortisol secretion and possible HPA suppression. 10/12 children in the prednisolone group and 8/11 in the levamisole group had a suboptimal cortisol response compared with 2/9 in the ciclosporin group. During follow-up, the 20 children who had a suboptimal cortisol response had significantly more relapses (95 relapses) compared to the 12 children with a normal cortisol response who had 24 relapses (p = 0.01). CONCLUSIONS: Children with NS receiving long-term alternate day prednisolone therapy are at risk of developing HPA suppression and should be evaluated using the modified synacthen test. Children with evidence of HPA suppression are at a greater risk of relapse.     

肾病综合征并发DIC早期防治

弥散性血管内凝血 (ＤＩＣ)是一种继发于很多基础病变的综合征 ,近年对此进行了深入研究。肾脏疾病中并发ＤＩＣ以肾病综合症 (Ｎ Ｓ)较为多见 ,但国内儿科有关报道较少 ,为提高认识 ,结合有关文献对其早期防治作一介绍。1　Ｎ Ｓ并发ＤＩＣ的特点Ｎ Ｓ并发ＤＩＣ的病理生理基础是高凝状态 (Ｈｙｐｅｒｃｏ ａｇｕｌａｔｏｒＳｔａｔｅ)。其机制是由于 :①肝脏合成有关凝血物质增加 ,如纤维蛋白原 ,第Ⅴ、Ⅷ因子增加 ;②抗凝血酶Ⅲ由尿中丢失 ;③血浆纤维蛋白原活性下降 ;④高脂血症时血粘稠度增加 ,血小板聚集增强 ;⑤感染或血管壁损伤可激…     

免疫紊乱在原发性肾病综合征发病机制中的研究进展

原发性肾病综合征(primary nephritic syndrome,PNS)患者的Th1/Th2及其相关细胞因子的失衡,可以对其高敏体质、低丙种球蛋白血症等并发症做出适当的解释.T调节细胞(Treg)、Th17的失衡导致相关细胞因子的紊乱,从另一角度解释了 PNS的发病机制.利妥昔布可有效治疗难治性肾病并使Treg...     

羟已基淀粉代血浆在肾病综合征中的应用及远期疗效观察

以往临床医师常用人体白蛋白纠正肾病综合征（ＮＳ）的低蛋白血症,但易引起肾脏高灌注、高滤过,加重了已有病变肾脏的损害。我科自１９９３年以来,采用羟已基淀粉代血浆替代血浆制品用于肾病综合征低蛋白血症期的辅助治疗,收到了较好的疗效,现报道如下。１ 资料与方法１．