Leumann E P, Briner J, Donckerwolcke R A, Kuijten R, Largiadèr F
Nephron. 1980;25(2):65-71. doi: 10.1159/000181755.
The clinical and renal biopsy findings in 3 patients with recurrent focal and segmental glomerulosclerosis (FSG) are reported, as well as the results of a survey among 17 European centers totaling 27 kidney-transplanted children with FSG. From these 27 patients, 10 had recurrent FSG. The duration of the original disease was less than 3 years in 9 of them. In contrast, this rapid progression of the disease was observed in only 7 of 17 patients in the group without recurrences. Heavy proteinuria was noted within the 1st day, or week, in all but 2 patients where it was detected at 3 and 7 weeks. All developed the nephrotic syndrome. From the 10 kidneys (mostly from cadaver donors) which functioned for more than 1 month, 5 were lost 2--24 months after transplantation because of recurrence (3 patients) or infection (2 patients). It appears that the risk of recurrence can best be predicted by the duration of the original disease: 50% or more if the duration was short (less than 3 years), and only 10--20% when the duration was longer.
报告了3例复发性局灶节段性肾小球硬化(FSG)患者的临床及肾活检结果,以及对17个欧洲中心共27例肾移植FSG患儿的调查结果。这27例患者中,10例出现复发性FSG。其中9例原发病病程不到3年。相比之下,在无复发组的17例患者中,只有7例观察到疾病的这种快速进展。除2例在3周和7周时检测到蛋白尿外,其余所有患者在第1天或第1周内均出现大量蛋白尿。所有患者均发展为肾病综合征。在功能超过1个月的10个肾脏(大多来自尸体供体)中,5个在移植后2至24个月因复发(3例)或感染(2例)而丢失。似乎复发风险最好通过原发病病程来预测:病程短(不到3年)时复发风险为50%或更高,病程较长时则仅为10%至20%。