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高剂量隔日疗法的泼尼松对特发性膜增生性肾小球肾炎自然病程的影响。

The effect of prednisone in a high-dose, alternate-day regimen on the natural history of idiopathic membranoproliferative glomerulonephritis.

作者信息

McEnery P T, McAdams A J, West C D

出版信息

Medicine (Baltimore). 1985 Nov;64(6):401-24. doi: 10.1097/00005792-198511000-00005.

Abstract

Experience in 45 children with diffuse proliferative MPGN of all three types has provided evidence that a high-dose, alternate-day regimen of prednisone alters the natural history of the disease. The experience has been gained over a 17-year period and the patients have been followed on the regimen for an average of 6.5 years. Evidence that the regimen has a salutary effect was provided by several observations: Survival was better than that in four other series in which the patients were not treated or treated sporadically. The difference was particularly marked when survival was compared with that in series in which the patients had diffuse proliferative lesions exclusively. Mesangial proliferation was less in biopsies obtained after 2 or more years of the alternate-day regimen. This was quantitated as a significant increase in the estimated percentage of open glomerular capillary lumens and a significant diminution in the prominence of PAS-positive mesangial matrix. In the second biopsy, as compared to the pre-regimen biopsy, no patient had a diminution in the estimated percent of open capillary lumens and 65% had a meaningful increase. Likewise, only 3% had an increase in prominence of PAS positive matrix and 68% had a diminution in matrix prominence. Of 32 patients who were hypoalbuminemic when the regimen started, the level rose into the normal range in 62%. The level became subnormal in none of the 13 who had a normal level at the start of the regimen. While receiving the regimen, renal function, as measured by serum creatinine levels, continued to be normal or improved in 73% and deteriorated in 27%. Hematuria disappeared in 80% of the 41 in whom it was present when the regimen started. Urinalysis became completely normal in 27% and none of these have relapsed while under observation. Comparison of data from 20 patients who did not receive the regimen for an average of 42 months after clinical onset with data for 25 patients who likewise were, on the average, 42 months from clinical onset but who had received the regimen for an average of 38 of those months provided the most convincing evidence that the regimen altered the natural history. In those receiving the regimen, the frequency of hematuria, proteinuria, and hypoalbuminemia was significantly less.(ABSTRACT TRUNCATED AT 400 WORDS)

摘要

对45例三种类型的弥漫性增生性MPGN患儿的治疗经验表明,高剂量隔日服用泼尼松的方案改变了该疾病的自然病程。这一经验是在17年期间积累的,患者按照该方案平均随访了6.5年。多项观察结果证明该方案具有有益效果:与其他四个未治疗或偶尔治疗的系列相比,生存率更高。与仅患有弥漫性增生性病变的系列相比,这种差异尤为明显。在隔日方案治疗2年或更长时间后所取的活检中,系膜增生较少。这通过肾小球毛细血管腔开放估计百分比的显著增加以及PAS阳性系膜基质突出程度的显著降低来量化。在第二次活检时,与治疗前活检相比,没有患者的毛细血管腔开放估计百分比降低,65%的患者有显著增加。同样,只有3%的患者PAS阳性基质突出程度增加,68%的患者基质突出程度降低。在开始治疗时为低白蛋白血症的32例患者中,62%的患者白蛋白水平升至正常范围。在开始治疗时白蛋白水平正常的13例患者中,没有一例水平降至正常以下。在接受该方案治疗期间,以血清肌酐水平衡量的肾功能在73%的患者中持续正常或改善,27%的患者恶化。在开始治疗时存在血尿的41例患者中,80%的患者血尿消失。27%的患者尿液分析完全正常,在观察期间这些患者均未复发。将20例临床发病后平均42个月未接受该方案治疗的患者的数据与25例同样平均距离临床发病42个月但在其中38个月接受了该方案治疗的患者的数据进行比较,提供了该方案改变自然病程的最有说服力的证据。接受该方案治疗的患者中,血尿、蛋白尿和低白蛋白血症的发生率显著降低。(摘要截取自400字)

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