RENAL TUBULAR DYSGENESIS WITHOUT PULMONARY HYPOPLASIA

Only a few cases of renal tubular dysgenesis (RTD) have been diagnosed . RTD is associated with a very early death, always due to lethal pulm onary hypoplasia. We report an additional patient, interesting for two reasons: (1) the rarity of RTD, (2) the absence of microscopic and ma croscopic anatomical alterations of the lungs. The infant had only a m ild, transient pulmonary maladaptation; after a few days of life the p ulmonary function returned to normal. Although renal function dramatic ally improved after institution of peritoneal dialysis at 6 days of li fe, the patient died after 2 weeks with an overwhelming Candida sepsis . We suggest that the lack of pulmonary hypoplasia might be due to a s hort exposure to oligohydramnios.