Aggressive respiratory support and unilateral nephrectomy for infants with severe perinatal autosomal recessive polycystic kidney disease☆,☆☆,★
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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Cited by (26)
Unilateral or bilateral early nephrectomy in infants with autosomal recessive polycystic kidney disease? Weighing risks and benefits
2021, Journal of Pediatric SurgeryEarly nephrectomy in neonates with symptomatic autosomal recessive polycystic kidney disease
2021, Journal of Pediatric SurgeryCitation Excerpt :For patients who fall into the latter category, early surgical intervention for nephrectomy, dialysis catheter placement, and feeding access are often required. To this point, the literature surrounding the optimal surgical management of these patients has been limited to case reports, which have reported success with unilateral [6,9,13] and bilateral [7,8,10] nephrectomies. Recent recommendations from an international multidisciplinary conference state that there is insufficient evidence to demonstrate the benefit of nephrectomy for respiratory compromise or severe hypertension [11].
Laparoscopic-assisted retroperitoneal nephrectomy in autosomal recessive polycystic kidney disease
2015, Journal of Pediatric UrologyCitation Excerpt :In these situations, unilateral or bilateral nephrectomy may be recommended. All previous reports of nephrectomy in ARPKD have described an open approach, generally via a transperitoneal route, with the risk of compromising future PD [2–5]. Here, we demonstrate laparoscopic-assisted retroperitoneal nephrectomy (LARN), which has been used successfully in two patients.
The Spectrum of Polycystic Kidney Disease in Children
2011, Advances in Chronic Kidney DiseaseCitation Excerpt :Enteral feedings through nasogastric or gastrostomy tubes are often needed to ensure delivery of sufficient calories. In some instances, unilateral or bilateral nephrectomies have been performed because of severe feeding intolerance and/or respiratory distress associated with massive kidney enlargement.53,54 Patients with ARPKD may have evidence of hematuria, but gross hematuria and cyst bleeding do not occur as they do in patients with ADPKD.
Prenatal Diagnosis of Fetal Renal Abnormalities
2010, Pediatric Urology
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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.
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0022-3476/95/$3.00 + 0 9/24/64607