Arant B S, Sotelo-Avila C, Bernstein J
J Pediatr. 1979 Dec;95(6):931-9. doi: 10.1016/s0022-3476(79)80279-6.
Severe segmental renal atrophy with loss of parenchymal elements in small kidneys is commonly known as segmental hypoplasia. The scars are seen as cortical depressions overlying shrunken medullary pyramids and their dilated calyces, and are characterized histologically by colloid-filled tubular microcysts and a paucity or absence of glomeruli. This lesion has been identified in 17 patients, 11 female and 6 male, between 6 and 23 years of age. Eleven patients had hypertension, which developed in six while they were under observation. Thirteen had histories of urinary tract infection, and 16 had evidence of vesicoureteric reflux. Seven patients had impaired renal function (GFR less than 40 ml/minute/1.73 m2). Abnormal metanephric differentiation (dysplasia) in two specimens, one in association with posterior urethral valves, suggested an occasional intrauterine origin of the abnormality. Twelve patients had radiographic evidence of decreasing renal size over two to five years of observation, even after surgical correction of reflux, in four of them unaccompanied by infection. We conclude that segmental "hypoplasia" is an acquired lesion, although it sometimes has intrauterine origins, and that it is commonly associated with vesicoureteric reflux, even in the absence of demonstrable infection.
小肾脏实质成分丧失的严重节段性肾萎缩通常被称为节段性发育不全。瘢痕表现为覆盖在萎缩肾髓质锥体及其扩张肾盏上的皮质凹陷，组织学特征为充满胶体的肾小管微囊肿以及肾小球稀少或缺失。该病变已在17例患者中得到确认，其中11例为女性，6例为男性，年龄在6至23岁之间。11例患者患有高血压，其中6例在观察期间发病。13例有尿路感染病史，16例有膀胱输尿管反流证据。7例患者肾功能受损（肾小球滤过率低于40 ml/分钟/1.73 m²）。两个标本中存在异常的后肾分化（发育异常），其中一个与后尿道瓣膜有关，提示该异常偶尔起源于子宫内。12例患者在两至五年的观察期内有肾脏大小减小的影像学证据，即使在反流手术矫正后也是如此，其中4例无感染伴随。我们得出结论，节段性“发育不全”是一种后天性病变，尽管它有时起源于子宫内，并且通常与膀胱输尿管反流相关，即使在没有明显感染的情况下也是如此。
