Voyer L E, Drut R, Méndez J H
Department of Paediatrics, Deutsche Hospital, Buenos Aires, Argentina.
Pediatr Nephrol. 1994 Oct;8(5):592-4. doi: 10.1007/BF00858136.
A female neonate, born by cesarean section at 37 weeks of gestation, presented with respiratory distress syndrome, right pneumothorax and anuria. A sonogram showed increased echogenicity, with neither hydronephrosis nor macroscopic cysts. Peritoneal dialysis was started on the 14th day because of renal insufficiency, but the newborn died on the 33rd day. Family history was unremarkable, except that the mother received piroxicam at about the 26th week of gestation. A sonogram at the 28th week showed oligohydramnios. Histopathological study of the kidneys revealed crowded glomeruli and only few differentiated proximal convoluted tubules in the inner cortex, abnormally differentiated microcystic tubules and microcystic glomeruli in the outer cortex. Periodic acid-Schiff staining showed only traces of brush border in the dilated tubules of the outer cortex. Immunoperoxidase staining for epithelial membrane antigen was positive in the luminal border of all tubules. Electron microscopy confirmed the presence of brush border remnants and other proximal tubular characteristics in some segments. The renal abnormality bears some similarities to that found in familiar renal tubular dysgenesis, but it fits better with those described after maternal use of angiotensin converting enzyme inhibitors and nonsteroidal anti-inflammatory drugs. The lesion in this case appears to have resulted from fetal exposure to piroxicam. Recently, a second pregnancy ended in a completely normal female newborn.
一名孕37周剖宫产出生的女新生儿，出现呼吸窘迫综合征、右侧气胸和无尿。超声检查显示回声增强，未见肾积水及肉眼可见的囊肿。因肾功能不全于第14天开始进行腹膜透析，但新生儿于第33天死亡。家族史无异常，只是母亲在妊娠约26周时服用过吡罗昔康。妊娠28周时的超声检查显示羊水过少。肾脏组织病理学研究显示，内皮质肾小球密集，仅有少数分化的近端曲管，外皮质有异常分化的微囊状小管和微囊状肾小球。过碘酸希夫染色显示外皮质扩张小管中仅有微量刷状缘。上皮膜抗原免疫过氧化物酶染色在所有小管的管腔边界均呈阳性。电子显微镜证实部分节段存在刷状缘残余及其他近端小管特征。肾脏异常与家族性肾小管发育不全有一些相似之处，但更符合母亲使用血管紧张素转换酶抑制剂和非甾体类抗炎药后所描述的情况。本例病变似乎是由于胎儿接触吡罗昔康所致。最近，第二次妊娠产下一名完全正常的女新生儿。
