Ronkainen J, Ala-Houhala M, Huttunen N P, Jahnukainen T, Koskimies O, Ormälä T, Nuutinen M
Department of Pediatrics, Oulu University Hospital, Oulu, Finland.
Clin Nephrol. 2003 Aug;60(2):80-4. doi: 10.5414/cnp60080.
All children with Henoch-Schoenlein glomerulonephritis (HSP-GN) and nephrotic-range proteinuria (> 40 mg/h/m2), treated at 5 university hospitals and in 1 central hospital in Finland during in 1990-1997, were analyzed retrospectively. The mean age of these 19 patients (8 girls, 11 boys) at the time of diagnosis was 9.9 years (range 4.6-15.1 years). A renal biopsy had been performed in all cases, giving findings according to the classification used in the International Study of Kidney Diseases in Children (ISKDC) of grade II (4 patients), grade III (10), grade IV (4) and grade V (1). Six patients underwent a second biopsy.
The yearly incidence of nephrotic-range HSP-GN in Finland was 2 per 1 million children under 15 years of age. After a mean follow-up of 4.6 years (range 9 months-9.1 years), 3 patients (15.7%) had no signs of nephritis, 11 (57.9%) had proteinuria < 1 g/day or microscopic hematuria, 2 (10.5%) had proteinuria > 1 g/day, and 3 (15.7%) had developed ESRD or uremia. 47% of the patients needed medication for proteinuria at the time of the latest follow-up. The first kidney biopsy did not predict the outcome of HSP-GN, since all the patients with the poorest outcome had only ISKDC II-III findings in their first biopsy.
According to our series, the morbidity in cases of HSP-GN with nephrotic-range proteinuria is high and a close clinical follow-up is needed. The treatment of HSP-GN patients should be based on the clinical presentation rather than on the biopsy findings.
对1990年至1997年期间在芬兰5所大学医院和1所中心医院接受治疗的所有患有过敏性紫癜性肾小球肾炎(HSP - GN)且蛋白尿达到肾病范围(>40mg/h/m²)的儿童进行回顾性分析。这19例患者(8名女孩,11名男孩)诊断时的平均年龄为9.9岁(范围4.6 - 15.1岁)。所有病例均进行了肾活检,根据儿童肾脏病国际研究(ISKDC)使用的分类得出结果:II级(4例)、III级(10例)、IV级(4例)和V级(1例)。6例患者接受了第二次活检。
芬兰15岁以下儿童中肾病范围的HSP - GN年发病率为每100万儿童中有2例。平均随访4.6年(范围9个月 - 9.1年)后,3例患者(15.7%)无肾炎迹象,11例(57.9%)蛋白尿<1g/天或镜下血尿,2例(10.5%)蛋白尿>1g/天,3例(15.7%)发展为终末期肾病(ESRD)或尿毒症。在最近一次随访时,47%的患者需要针对蛋白尿进行药物治疗。首次肾活检未能预测HSP - GN的预后,因为所有预后最差的患者在首次活检中仅表现为ISKDC II - III级结果。
根据我们的系列研究,伴有肾病范围蛋白尿的HSP - GN病例发病率较高,需要密切的临床随访。HSP - GN患者的治疗应基于临床表现而非活检结果。