title: Aggressive respiratory support and unilateral nephrectomy for infants with severe perinatal autosomal recessive polycystic kidney disease.
abstract: Newborn infants with severe autosomal recessive polycystic kidney disease often receive minimal intervention because poor respiratory and renal outcomes are anticipated. We describe two patients whose respiratory failure was successfully treated with aggressive intervention. Massive kidneys restricted gastrointestinal capacity and limited feedings. Uninephrectomy allowed adequate enteral feedings and preserved sufficient renal function for homeostasis and growth.
authors.affiliation: Division of Neonatology, Medical Center of Central Massachusetts, USA.
authors.forename: S A
authors.initials: SA
authors.lastname: Bean
authors.forename: F J
authors.initials: FJ
authors.lastname: Bednarek
authors.forename: W A
authors.initials: WA
authors.lastname: Primack
mesh_terms: D004750:Enteral Nutrition; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009392:Nephrectomy; D017044:Polycystic Kidney, Autosomal Recessive / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D012121:Respiration, Artificial; D012131:Respiratory Insufficiency / Q000209:etiology / Q000628:therapy*