A Case of Prenatal Diagnosed Polycystic Kidney / 대한주산의학회잡지

ABSTRACT

The infantile polycystic kidney disease is rare urinary tract anomaly.It is inherited with an autosomal recessive pattern and recurrence rate is about 25%. The gene locus is on chromosome 6p. The pathogenesis of infantile polycystic kidney is the primary defect of the collecting ducts. The ultrasonographic findings of infantile polycystic kidney are oligohydroamnios, bilaterally symmetrical enlarged kidneys with maintenance of their reinform shape. The differential diagnosis with adult polycystic kidney disease and the examination of the parents and other members of the family is helpful to confirm the adult polycystic kidney disease. If there is severe renal involvements, stillbirth or neonatal death secondary to pulmonary hypoplasia will be developed. If it is diagnosed before viability, termination of pregnancy would be recommended. In a fetus diagnosed after viability, pregnancy termmination is also recommended since this condition is uniformly fatal. We present a case of infantile polycystic kidney.