title: Renal tubular dysgenesis in twin-twin transfusion syndrome.
abstract: In twin-twin transfusion syndrome (TTTS), the disparity in circulation is reflected in discordant fetal growth, urine output, and amniotic fluid accumulation. The effect of uneven shunting of the growth factor and nutrient-rich vasculature on development and differentiation of the kidney has not been 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 (FAH), Leu-M1, and Lotus tetragonolobus (LTA) were used to identify proximal convoluted tubules, and epithelial membrane antigen (EMA) was used to demonstrate distal convoluted and collecting tubules. FAH appeared to be more specific and reliable than either Leu-M1 or LTA 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 later gestations and associated with more severe growth restriction. Donors in TTTS are at risk for the development of RTD. Several authors suggest ischemia as the underlying cause of "acquired" RTD. However, in this setting there is no evidence of cell death or necrosis, and we suggest that hypoperfusion leading to decreased glomerular filtration is the underlying etiology, with the severity of RTD related to the degree of shunting.
authors.affiliation: Department of Pathology, Texas Children's Hospital, Baylor College of Medicine, Houston 77030, USA.
authors.forename: K C
authors.initials: KC
authors.lastname: Oberg
authors.forename: J P
authors.initials: JP
authors.lastname: Pestaner
authors.forename: L
authors.initials: L
authors.lastname: Bielamowicz
authors.forename: E P
authors.initials: EP
authors.lastname: Hawkins
mesh_terms: D005314:Embryonic and Fetal Development / Q000187:drug effects; D005260:Female; D005330:Fetofetal Transfusion / Q000150:complications*; D005865:Gestational Age; D006801:Humans; D007150:Immunohistochemistry; D007687:Kidney Tubules, Proximal / Q000002:abnormalities* / Q000196:embryology; D018811:Maternal Exposure / Q000009:adverse effects; D011247:Pregnancy; D019966:Substance-Related Disorders / Q000150:complications