Kakkar Nandita, Menon Santosh, Radotra B D
Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Fetal Pediatr Pathol. 2006 Jan-Feb;25(1):35-49. doi: 10.1080/15227950600701446.
Pediatric developmental and genetic renal lesions are a known cause of mortality in the perinatal/neonatal period. These lesions are associated with a wide range of extrarenal congenital malformations that influence the outcome of the patients. In this autopsy study, we have analyzed the spectrum of pediatric developmental and genetic renal lesions and their associated congenital malformations. A total of 4,099 autopsies (20 weeks of gestation to 1 year of life) were reviewed, of which 158 cases (3.85%) of pediatric developmental (143 cases) and genetic renal lesions (15 cases) were found. Autosomal recessive polycystic kidney disease was the commonest genetic lesion. Primitive ducts with cuffing of immature mesenchyme--the sine qua non of renal dysplasia--was found in all cases of dysplasia. Associated congenital malformations were seen in all cases and thus a thorough search for them is mandatory. Ductal plate malformation was found in all cases of autosomal recessive polycystic kidney disease and in 1 case of bilateral multicystic dysplasia.
小儿发育性和遗传性肾脏病变是围产期/新生儿期已知的死亡原因。这些病变与多种影响患者预后的肾外先天性畸形有关。在这项尸检研究中，我们分析了小儿发育性和遗传性肾脏病变的范围及其相关的先天性畸形。共回顾了4099例尸检（妊娠20周至1岁），其中发现158例（3.85%）小儿发育性（143例）和遗传性肾脏病变（15例）。常染色体隐性多囊肾病是最常见的遗传性病变。在所有发育异常病例中均发现有原始导管伴有未成熟间充质套叠——这是肾发育异常的必要条件。所有病例均可见相关的先天性畸形，因此必须对其进行全面检查。在所有常染色体隐性多囊肾病病例以及1例双侧多囊性发育异常病例中均发现了导管板畸形。
