Aggressive respiratory support and unilateral nephrectomy for infants with severe perinatal autosomal recessive polycystic kidney disease,☆☆,

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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. (J PEDIATR 1995;127:311-3)

Section snippets

Patient 1

Patient 1, a 2120 gm boy, was born in 1990 at 36 weeks of gestation by cesarean section because of preterm labor with rupture of membranes and breech presentation. Prenatal ultrasonography revealed oligohydramnios and massively enlarged fetal kidneys, findings strongly suggestive of ARPKD. Marked respiratory distress at birth led to immediate tracheal intubation. Apgar scores were 4 and 6 at 1 and 5 minutes, respectively. In the first hours of life the patient required ventilator settings to

DISCUSSION

We believe that aggressive intervention should be considered for infants with severe perinatal ARPKD. It is difficult to predict which of these patients have lethal pulmonary insufficiency, the leading cause of death in neonates with ARPKD.8, 9 Potentially reversible causes of respiratory compromise include infection, pulmonary air leaks, respiratory distress syndrome, persistent pulmonary hypertension, and upward compression of the diaphragm by massive kidneys. For patients with suspected

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From the Division of Neonatology, Medical Center of Central Massachusetts, and the Departments of Pediatrics, Fallon Clinic and the University of Massachusetts Medical Center, Worcester

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Reprint requests: Stephen A. Bean, MD, The Med Center-Memorial, 119 Belmont St., Worcester, MA 01605.

0022-3476/95/$3.00 + 0 9/24/64607

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