Polycythemia, capillary rarefaction, and focal glomerulosclerosis in two adolescents born extremely low birth weight and premature
Polycythemia, capillary rarefaction, and focal glomerulosclerosis in two adolescents born extremely low birth weight and premature
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DOI：
10.1007/s00467-017-3654-z
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发表时间：
2017-07-01
影响因子：
3
通讯作者：
Awazu, Midori
中科院分区：
文献类型：
--
作者：
Asada, Nariaki;Tsukahara, Takanori;Awazu, Midori
低出生体重儿的肾单位数量减少，患慢性肾脏疾病的风险很高。早产和/或宫内生长受限（IUGR）也可能影响肾小管周围毛细血管的发展，已被证明在其他orgas.We报告的早产和极低出生体重的历史，谁表现出红细胞增多症和肾毛细血管稀疏的两个病人。患者1和2，出生于妊娠25周，出生体重分别为728和466克，分别在8岁和6岁时出现轻度蛋白尿。除了增加蛋白尿，血红蛋白水平升高，接近青春期，他们的血清促红细胞生成素（EPO）高，尽管红细胞增多症。肾活检标本的光镜检查显示肾小球肥大，局灶节段性肾小球硬化，仅轻度肾小管间质纤维化。肾活检标本中CD 31和CD 34内皮细胞免疫组化染色的减少与肾小管周围毛细血管稀疏一致，由于肾功能几乎正常，纤维化不严重，我们认为与EPO水平升高相关的毛细血管稀疏和红细胞增多症在很大程度上归因于早产和/或IUGR。
Low birthweight infants have a reduced number of nephrons and are at high risk of chronic kidney disease. Preterm birth and/or intrauterine growth restriction (IUGR) may also affect peritubular capillary development, as has been shown in other organs.We report two patients with a history of preterm birth and extremely low birthweight who showed polycythemia and renal capillary rarefaction. Patient 1 and 2, born at 25 weeks of gestation with a birthweight of 728 and 466 g, showed mild proteinuria at age 8 and 6 years, respectively. In addition to increasing proteinuria, hemoglobin levels became elevated towards adolescence and their serum erythropoietin (EPO) was high despite polycythemia. Light microscopic examination of renal biopsy specimens showed glomerular hypertrophy, focal segmental glomerulosclerosis, and only mild tubulointerstitial fibrosis. A decrease in the immunohistochemical staining of CD31 and CD34 endothelial cells in renal biopsy specimens was consistent with peritubular capillary rarefaction.Since kidney function was almost normal and fibrosis was not severe, we consider that the capillary rarefaction and polycythemia associated with elevated EPO levels were largely attributable to preterm birth and/or IUGR.