The Tohoku journal of experimental medicine
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Tohoku J. Exp. Med. · Sep 2020
Review Case ReportsManagement of a Preterm Infant with Renal Tubular Dysgenesis: A Case Report and Review of the Literature.
Renal tubular dysgenesis (RTD) is the absence or poor development of the renal proximal tubules caused by gene mutations in the renin-angiotensin system. Although RTD has been considered fatal, improving neonatal intensive care management has enhanced survival outcomes. However, little has been reported on the survival of extremely preterm infants. ⋯ Currently, there is no established treatment for RTD. Considering cases reported to date, treatment with vasopressin and fludrocortisone appears to be most effective for survival and maintenance of renal function in patients with RTD. This study presents the successful management of RTD using this strategy in an extremely preterm infant.