AGGRESSIVE SURGICAL AND MEDICAL-MANAGEMENT OF AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY-DISEASE

The treatment of autosomal recessive polycystic kidney disease (ARPKD) is evolving. Overall prognosis is difficult to determine, but most ne onates without severe pulmonary hypoplasia will survive. It is difficu lt to predict which neonates with ARPKD requiring immediate ventilator y support have pulmonary hypoplasia incompatible with survival. Massiv ely enlarged kidneys restricting diaphragmatic excursion can cause res piratory distress in addition to pulmonary hypoplasia. Three infants w ith ARPKD underwent bilateral nephrectomies and institution of periton eal dialysis in an attempt to improve ventilatory status and assess lo ng-term pulmonary function. Two children are alive at twelve and thirt y months postoperatively and awaiting renal transplantation. One infan t died of acute cardiorespiratory complications.