Renal tubular dysgenesis in twin-twin transfusion syndrome

In twin-twin transfusion syndrome (TTTS), the disparity in circulation is r eflected in discordant fetal growth, urine output, and amniotic fluid accum ulation. The effect of uneven shunting of the growth factor and nutrient-ri ch vasculature on development and differentiation of the kidney has not bee n well studied. We analyzed renal tubular growth and differentiation in 25 fetal autopsies with TTTS (13 donors and 12 recipients, including 9 sibling pairs) between 18 and 33 weeks gestation. Immunohistochemical markers for fumarylacetoacetate hydrolase (FAR), Leu-Mi, and Lotus tetragonolobus (LTA) were used to identify proximal convoluted tubules, and epithelial membrane antigen (EMA) was used to demonstrate distal convoluted and collecting tub ules. FAH appeared to be more specific and reliable than either Leu-Mi or L TA in the identification of proximal tubules. Donors tended to demonstrate a paucity of proximal tubules with crowding of glomeruli characteristic of renal tubular dysgenesis (RTD). The degree of dysgenesis was greater in lat er gestations and associated with more severe growth restriction. Donors in TTTS are at risk for the development of RTD. Several authors suggest ische mia as the underlying cause of "acquired" RTD. However, in this setting the re is no evidence of cell death or necrosis, and we suggest that hypoperfus ion leading to decreased glomerular filtration is the underlying etiology, with the severity of RTD related to the degree of shunting.