Department of Pediatric Surgery and Urology, Necker-Enfants Malades Hospital, APHP, Paris, France.
EA FETUS 7328-PACT, Imagine Institute, Paris University, Paris, France.
Ultrasound Obstet Gynecol. 2022 Apr;59(4):513-521. doi: 10.1002/uog.23718.
To identify favorable renal histology in fetuses with early severe lower urinary tract obstruction (LUTO) and determine the best timing and selection criteria for prenatal surgery.
This multicenter, retrospective study included male fetuses with severe LUTO which died before 24 weeks of gestation during the period January 2000 to December 2018. Age-matched controls were used as reference standard for renal histology. Prenatal ultrasound features and fetal serum and/or urine β2microglobulin level were retrieved and kidney histology slides (hematein-eosin-safran and α-smooth-muscle-actin (αSMA) immunostaining) were prepared and reviewed. αSMA-positive staining of the blastema is due to its aberrant differentiation into myofibroblastic cells. Cases were sorted into histopathologic groups (favorable or unfavorable) according to the blastema's morphology and αSMA labeling and the data of these groups were compared.
Included in the study were 74 fetuses with a median gestational age at outcome of 17 + 6 (range, 13 + 0 to 23 + 5) weeks. Parenchymal organization was preserved in 48% of the kidneys. A blastema was present in 90% of the kidneys, but it was morphologically normal in only 9% and αSMA-negative in only 1% of them. Most (82%) fetuses had an unfavorable prognosis, and 36% of fetuses died ≤ 18 weeks and had severe renal lesions detected on histology (early unfavorable prognosis). A favorable renal prognosis was associated with an earlier gestational age (P = 0.001). Fetuses with LUTO had a significantly lower number of mature glomeruli (P < 0.001) compared with controls. However, there was no significant difference in the number of glomeruli generations between the early-unfavorable-prognosis group (≤ 18 weeks) and the group with a favorable prognosis (P = 0.19). A comparison of prenatal ultrasound features and biochemical markers between groups could not identify any prenatal selection criteria.
Before 18 weeks, around 30% of fetuses with severe LUTO still have potential for kidney development. Identification of these cases would enable them to be targeted for prenatal therapy. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.
确定早期严重下尿路梗阻(LUTO)胎儿中有利的肾组织学表现,并确定产前手术的最佳时机和选择标准。
本多中心回顾性研究纳入了 2000 年 1 月至 2018 年 12 月期间因严重 LUTO 而在 24 孕周前死亡的男性胎儿。年龄匹配的对照组作为肾组织学的参考标准。检索了产前超声特征以及胎儿血清和/或尿液β2 微球蛋白水平,并制备和评估了肾组织学切片(苏木精-伊红-固绿和α-平滑肌肌动蛋白(αSMA)免疫染色)。成纤维细胞芽的αSMA 阳性染色归因于其异常分化为肌成纤维细胞。根据成纤维细胞芽的形态和αSMA 标记,将病例分为组织病理学组(有利或不利),并比较这些组的数据。
本研究纳入了 74 例结局为 17+6(范围 13+0 至 23+5)周的胎儿。48%的肾脏保持了肾实质组织的结构。90%的肾脏存在成纤维细胞芽,但形态正常的仅占 9%,αSMA 阴性的仅占 1%。大多数(82%)胎儿预后不良,36%的胎儿在 18 周内死亡,组织学检查显示严重的肾脏病变(早期预后不良)。有利的肾脏预后与较早的胎龄相关(P=0.001)。与对照组相比,LUTO 胎儿成熟肾小球的数量明显减少(P<0.001)。然而,早期预后不良组(≤18 周)和预后良好组之间的肾小球世代数无显著差异(P=0.19)。对两组之间的产前超声特征和生化标志物进行比较,无法确定任何产前选择标准。
在 18 周之前,约 30%严重 LUTO 胎儿仍有肾脏发育的潜力。识别这些病例将使他们能够成为产前治疗的目标。