Cabot Dalmau A, Callis L, Lara E, Carreras M
Servicio de Pediatría, Hospital S. Jaime y Sta. Magdalena de Mataró, Barcelona.
An Esp Pediatr. 1993 Sep;39(3):227-34.
We have reviewed 22 cases of Berger's disease in children (glomerular nephritis with mesangial IgA deposits), all of which were diagnosed by renal biopsy between 1976 and the present time. We describe the clinical and pathological findings in these patients. In addition, we put special emphasis on the evolution of the disease in relationship to some of the parameters that have been reported in the literature as being related to a bad prognosis of glomerular function such as, massive proteinuria at the onset of the disease, histological classification, presence of deposits of IgM or fibrinogen derivatives and glomerular sclerosis. All of the patients started with hematuria, 21 of which had gross hematuria (95%). Fourteen patients (63%) showed proteinuria (2 of which also had a temporary nephrotic syndrome). Five children showed some transient decrease in glomerular filtration rate and another patient rapidly developed renal failure and then end stage renal disease. We were able to follow 15 children for 3 years: 8 (53%) still showed outbreaks of gross hematuria, 5 (33%) only had microhematuria and 2 (14%) showed no signs of hematuria. Four children (27%) still had proteinuria. The glomerular filtration rate was still normal in all but two children (one with rapid evolution to end stage renal disease and another with a glomerular filtration decrease of 20%). Ten children were followed for 6 to 13 years. After 6 years, 2 (20%) still showed outbreaks of gross hematuria, 1 (10%) still had proteinuria.(ABSTRACT TRUNCATED AT 250 WORDS)
我们回顾了22例儿童伯杰氏病(系膜IgA沉积性肾小球肾炎),所有病例均于1976年至目前期间经肾活检确诊。我们描述了这些患者的临床和病理表现。此外,我们特别强调了疾病的演变与文献中报道的一些与肾小球功能预后不良相关的参数之间的关系,如疾病发作时的大量蛋白尿、组织学分类、IgM或纤维蛋白原衍生物沉积的存在以及肾小球硬化。所有患者均以血尿起病,其中21例为肉眼血尿(95%)。14例患者(63%)出现蛋白尿(其中2例还曾有过短暂的肾病综合征)。5名儿童出现肾小球滤过率的一些短暂下降,另1例患者迅速发展为肾衰竭,进而进入终末期肾病。我们对15名儿童进行了3年的随访:8例(53%)仍有肉眼血尿发作,5例(33%)仅有镜下血尿,2例(14%)无血尿迹象。4例儿童(27%)仍有蛋白尿。除2名儿童外(1例迅速发展为终末期肾病,另1例肾小球滤过率下降20%),所有儿童的肾小球滤过率仍正常。10名儿童随访了6至13年。6年后,2例(20%)仍有肉眼血尿发作,1例(10%)仍有蛋白尿。(摘要截选至250字)