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[肾病综合征的临床谱]

[Clinical spectrum of nephrotic syndrome].

作者信息

Bökenkamp A, Hoyer P F, Ehrich J H, Brodehl J

机构信息

Abteilung Pädiatrische Nieren- und Stoffwechselerkrankungen, Kinderklinik der Medizinischen Hochschule, Hannover, Bundesrepublik Deutschland.

出版信息

Monatsschr Kinderheilkd. 1992 Feb;140(2):122-7.

PMID:1557055
Abstract

AIM OF STUDY

A wide spectrum of glomerular diseases manifests as a nephritic syndrome with haematuria, proteinuria, hypertension, edema, and impaired renal function. Little is known about the presentation of each symptom and the distribution of the underlying glomerular diseases.

METHODS

In order to delineate the clinical spectrum of glomerular disease presenting as a nephritic syndrome, we examined the records of 192 patients who had been followed in our paediatric nephrological outpatient clinic between 1973 and 1988 for a nephritic syndrome.

RESULTS

Oligosymptomatic courses with microhaematuria and proteinuria predominated. The mean age at presentation was 8.1 years. In a broad spectrum of 29 diagnoses, postinfectious glomerulonephritis and Henoch-Schönlein nephritis were the most prevalent. End-stage renal failure developed in 16% of the patients. Prognosis was poor in cases of rapidly progressive glomerulonephritis, focal segmental glomerulosclerosis, Henoch-Schönlein nephritis and in autoimmune disease, with end-stage renal failure developing in more than 20% of each of these groups.

CONCLUSIONS

The nephritic syndrome often manifests oligosymptomatically. An intensive nephrological workup is indicated in order to early diagnose serious--and potentially treatable--glomerular diseases.

摘要

研究目的

多种肾小球疾病表现为肾炎综合征,伴有血尿、蛋白尿、高血压、水肿及肾功能损害。对于每种症状的表现以及潜在肾小球疾病的分布情况,我们知之甚少。

方法

为了描述表现为肾炎综合征的肾小球疾病的临床谱,我们查阅了1973年至1988年间在我们儿科肾病门诊接受随访的192例肾炎综合征患者的病历。

结果

以镜下血尿和蛋白尿为主的轻症病程较为常见。就诊时的平均年龄为8.1岁。在29种广泛的诊断中,感染后肾小球肾炎和过敏性紫癜性肾炎最为常见。16%的患者发展为终末期肾衰竭。快速进展性肾小球肾炎、局灶节段性肾小球硬化、过敏性紫癜性肾炎以及自身免疫性疾病患者的预后较差,这些组中每组超过20%的患者发展为终末期肾衰竭。

结论

肾炎综合征常表现为轻症。为了早期诊断严重且可能可治疗的肾小球疾病,需要进行全面的肾病检查。

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