Uchida Takahiro, Oda Takashi, Watanabe Atsushi, Higashi Keishi, Katsurada Yuka, Shimazaki Hideyuki, Tamai Seiichi, Kumagai Hiroo
Department of Nephrology , National Defense Medical College , Tokorozawa, Saitama , Japan.
Department of Laboratory Medicine , National Defense Medical College , Tokorozawa, Saitama , Japan.
Clin Kidney J. 2012 Oct;5(5):445-8. doi: 10.1093/ckj/sfs108.
A 53-year-old woman with a history of dyslipidemia presented with medium-grade proteinuria and several years of progressive renal dysfunction. Renal biopsy showed diffuse and global Kimmelstiel-Wilson nodule like nodular mesangial sclerosis, but she had no history of diabetes mellitus, no diabetic retinopathy and normal oral glucose tolerance. Congo red staining was negative, and immunofluorescence staining showed no immunoglobulin deposition including kappa or lambda light chains. Electron microscopy showed no electron dense deposits or organized deposits. Thus, we diagnosed idiopathic nodular glomerulosclerosis (ING). ING is a recently established clinicopathologic disease entity linked to longstanding cigarette smoking and hypertension. Obesity is also listed as a contributing factor. However, none of these factors was documented in this case. This is a valuable case of ING that suggests the existence of as-yet unknown causative factors of ING other than smoking, hypertention or obesity.
一名有血脂异常病史的53岁女性出现中度蛋白尿及数年进行性肾功能不全。肾活检显示弥漫性和全球性的类似Kimmelstiel-Wilson结节的结节性系膜硬化,但她无糖尿病病史、无糖尿病视网膜病变且口服葡萄糖耐量正常。刚果红染色阴性,免疫荧光染色显示无免疫球蛋白沉积,包括κ或λ轻链。电子显微镜检查未见电子致密沉积物或有组织的沉积物。因此,我们诊断为特发性结节性肾小球硬化(ING)。ING是一种最近确立的临床病理疾病实体,与长期吸烟和高血压有关。肥胖也被列为一个促成因素。然而,该病例中未记录到这些因素中的任何一项。这是一例有价值的ING病例,提示除吸烟、高血压或肥胖外,还存在尚未知晓的ING致病因素。