Assadi Farahnak K
Department of Pediatrics, section of nephrology, Rush Presbyterian St. Luke's Medical Center, Rush University Medical College, Chicago, Illinois 60612, USA.
Med Sci Monit. 2004 Sep;10(9):CS54-6. Epub 2004 Aug 20.
Patients with Down syndrome have a variety of urinary tract abnormalities including obstructive hydronephrosis, dysplasia, reflux nephropathy and glomerular lesions. However, primary glomerulonephritis with prominent IgG deposits has not been reported previously in this syndrome.
A 17-year-old girl with Down syndrome presented with proteinuria and chronic renal insufficiency. A percutaneous renal biopsy revealed prominent IgG deposition without IgA or IgM deposits in the absence of an identifiable autoimmune disorder, or any other etiologic factors known to cause secondary IgG-associated glomerulonephritis.
The glomerular lesion identified in the present case demonstrates a distinct entity that further expands the spectrum of renal disease known to occur in Down syndrome. Early detection of renal disorder in Down syndrome may prevent or slow down the progression.
唐氏综合征患者存在多种泌尿系统异常，包括梗阻性肾积水、发育异常、反流性肾病和肾小球病变。然而，此前该综合征中尚未报道过以显著IgG沉积为主的原发性肾小球肾炎。
一名17岁唐氏综合征女孩出现蛋白尿和慢性肾功能不全。经皮肾活检显示，在无明确自身免疫性疾病或任何已知可导致继发性IgG相关肾小球肾炎的其他病因的情况下，有显著IgG沉积，而无IgA或IgM沉积。
本病例中发现的肾小球病变显示为一种独特的实体，进一步扩大了已知发生于唐氏综合征的肾脏疾病谱。唐氏综合征患者肾脏疾病的早期检测可能预防或减缓疾病进展。
