kidney disorder renal tubular dysgenesis MONDO:0017609 primitive renal tubule syndrome Orphanet:3033 UMLS:C0266313 GARD:0000379 SCTID:702397002 renotubular dysgenesis MEDGEN:82738 Renal tubular dysgenesis is a rare disorder of the fetus characterized by absent or poorly developed proximal tubules of the kidneys, persistent oligohydramnios, leading to Potter sequence (facial dysmorphism with large and flat low-set ears, lung hypoplasia arthrogryposis and limb positioning defects), and skull ossification defects. It can be acquired during fetal development due to drugs taken by the mother or certain disorders (twin-twin transfusion syndrome, TTTS) or inherited in an autosomal recessive manner. icd11.foundation:191424358