Aviles D H, Irwin K C, Dublin L S, Vehaskari V M
Department of Pediatrics, Louisiana State University Medical Center and Children's Hospital, New Orleans 70118, USA.
Pediatr Nephrol. 1999 May;13(4):298-300. doi: 10.1007/s004670050612.
When focal segmental glomerulosclerosis (FSGS) has reached the stage of chronic renal insufficiency, further progression is usually considered inevitable. African-American patients are believed to exhibit a particularly aggressive form of FSGS. We have treated five African-American patients, aged 11-18 years, with FSGS and reduced renal function using intensive intravenous methylprednisolone protocol, combined with chlorambucil in three cases. All patients had a pretreatment creatinine clearance of less than 50 ml/min per 1.73 m2. Three patients responded with normalization of creatinine clearance and serum albumin levels and had no or only minimal proteinuria at latest follow-up. One patient showed no improvement and one patient progressed to end-stage renal disease. These findings indicate, for the first time, that even severe FSGS may respond to aggressive methylprednisolone with or without alkylating agent treatment, and that African-American race does not preclude a favorable response.
当局灶节段性肾小球硬化（FSGS）发展到慢性肾功能不全阶段时，通常认为病情会不可避免地进一步进展。据信非裔美国患者的FSGS表现出一种特别侵袭性的形式。我们使用强化静脉注射甲泼尼龙方案治疗了5名年龄在11至18岁之间、患有FSGS且肾功能减退的非裔美国患者，其中3例联合使用了苯丁酸氮芥。所有患者治疗前的肌酐清除率均低于每1.73平方米50毫升/分钟。3例患者的肌酐清除率和血清白蛋白水平恢复正常，在最近一次随访时无蛋白尿或仅有微量蛋白尿。1例患者无改善，1例患者进展至终末期肾病。这些发现首次表明，即使是严重的FSGS，无论是否联合烷化剂治疗，积极使用甲泼尼龙可能有效，而且非裔美国人种族并不排除良好的反应。
