Prenatally diagnosed autosomal recessive polycystic kidney disease: initial postnatal management

Munding, Matthew; Al-Uzri, A.; Gralnek, Daniel; Riden, D.

doi:10.1016/s0090-4295(99)00362-3

Cited by 11 publications

(3 citation statements)

References 5 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Presentation in the perinatal period carries the highest mortality (2). Improved survival may result from continued improvements in medical care including early bilateral nephrectomies followed by dialysis for salvaging severely affected neonates (13). Increased numbers of children with end‐stage renal disease will result in a proportionate increase in transplantation and as a result long‐term survivors may differ from those reported in previous series (4, 9–11).…”

Section: Discussionmentioning

confidence: 99%

Morbidity from Congenital Hepatic Fibrosis after Renal Transplantation for Autosomal Recessive Polycystic Kidney Disease

Khan¹,

Schwarzenberg²,

Sharp³

et al. 2002

American Journal of Transplantation

View full text Add to dashboard Cite

Presentation of autosomal recessive polycystic kidney disease (ARPKD) ranges from severe renal impairment and a high mortality rate in infancy to older children and adolescents with minimal renal disease and complications of congenital hepatic fibrosis (CHF), cholangitis and portal hypertension. Renal transplantation improves prognosis but it is unclear whether CHF in transplanted children follows the same clinical course as in older children with less severe renal disease. The aim of this study was to evaluate morbidity from CHF in ARPKD post renal transplantation. Data were analyzed for six males and eight females, transplanted for ARPKD (mean age 8.3years, range 1-22.3years) at the University of Minnesota between 1972 and 1998. Follow-up was for a mean of 14.5years (range 3.1-33.6years). One and 5years patient survival rates were 93% and 86%, respectively. Overall five patients (36%) died; 4/5 deaths were related to CHF. Causes of death were hepatic failure immediately post transplant (n Ω 1), septicemia related to bile duct dilatation (n Ω 3) and multiorgan failure (n Ω 1). One and 5years graft survival rates were 87% and 70%, respectively. One patient had a combined liver-kidney transplant and two were re-transplanted. Initial signs of CHF were splenomegaly (n Ω 5), hepatosplenomegaly (n Ω 4) and gastrointestinal bleed (n Ω 2). Progression of CHF through childhood included hypersplenism (n Ω 7), esophageal varices with gastrointestinal bleeding (n Ω 5) and bile duct dilatation (n Ω 5). Portal hypertension was treated with portosystemic shunt (n Ω 3), sclerotherapy (n Ω 2), banding of varices (n Ω 1) and transjugular intrahepatic portosystemic shunt (n Ω 1). Of the nine survivors (mean age 12.8years) 78% have functioning grafts (one liver-kidney transplant), 63% have portal hypertension and 22% have asymptomatic biliary dilatation. Complications of CHF developed in 79% of children who received a renal transplant for ARPKD. Mortality related to CHF occurred in 29% and accounted for 80% (4/5) of the deaths.

show abstract

Section: Discussionmentioning

confidence: 99%

Morbidity from Congenital Hepatic Fibrosis after Renal Transplantation for Autosomal Recessive Polycystic Kidney Disease

Khan¹,

Schwarzenberg²,

Sharp³

et al. 2002

American Journal of Transplantation

View full text Add to dashboard Cite

show abstract

“…Bilateral nephrectomy in the early postpartum period has been reported in 3 previous cases with good results when adequate ventilation was impossible due to subdiaphragmatic compression from the ARPKD affected kidneys. 1,3 In our case although the infant had regained respiratory function, unilateral left nephrectomy was reluctantly performed due to severely restricted gastrointestinal capacity and the failure of other nutritional management options. This ap- proach improved nutritional tolerance and allowed the infant to thrive.…”

Section: Case Reportmentioning

confidence: 77%

Unilateral Nephrectomy as Palliative Therapy in an Infant With Autosomal Recessive Polycystic Kidney Disease

2004

View full text Add to dashboard Cite

Prenatal diagnosis of autosomal recessive polycystic kidney disease (ARPKD) implies a dire prognosis. Neonates affected by the more severe variants of ARPKD suffer respiratory failure caused by massive kidneys that restrict diaphragmatic expansion and result in pulmonary hypoplasia. Afflicted infants who survive the neonatal period and gain adequate respiratory function may subsequently suffer from an inability to tolerate enteral nutrition due to abdominal compression from the massive kidney and the systemic effects of renal compromise. Palliative unilateral or bilateral nephrectomy may be considered in rare instances to facilitate pulmonary expansion and gastrointestinal function. We report on an infant with severe ARPKD who was able to tolerate enteral nutrition only after left nephrectomy.

show abstract

“…Two of theses patients had unilateral nephrectomy, seven had bilateral nephrectomy and PD, two of which had a subsequent KT. Yet, no long‐term follow‐up following nephrectomy with or without KT is reported (11–16). In our experience, patients who underwent aggressive enteral feedings, early bilateral nephrectomy with PD had no mortality in the first year of life.…”

Section: Discussionmentioning

confidence: 99%

Optimizing outcomes for neonatal ARPKD

Beaunoyer

Snehal

et al. 2007

Pediatric Transplantation

View full text Add to dashboard Cite

A retrospective analysis was conducted on 10 consecutive cases of neonatal ARPKD, 9 of whom received kidney transplants (KT). All were diagnosed antenatally (n = 6) or at birth. In the first month of life 70% required ventilatory support. Pre-emptive bilateral nephrectomy and peritoneal dialysis (PD) catheter placement were performed in 9 at a mean age of 7.8 +/- 11.9 months. The indications for nephrectomy were massive kidneys, resulting in suboptimal nutrition and respiratory compromise. All patients received assisted enteral nutrition, with significant increase in mean tolerated feeds following nephrectomy (p < 0.05), with increase in mean normalized weight and height (0.92 and 1.2 delta SDS respectively), by one year post-transplantation. KT was performed at a mean age and weight of 2.5 +/- 1.4 years and 13.3 +/- 6.1 kg. The mean creatinine clearance at one year post-KT was 91.3 +/- 38.1 mls/min/1.73 m(2), with a projected graft life expectancy of 18.4 years. Patient survival was 89% and death censored graft survival was 100%, at a mean follow-up of 6.1 +/- 4.5 years post-transplant. Six patients demonstrated evidence of hepatic fibrosis, one of which required liver transplantation. In patients with massive kidneys from ARPKD, pre-emptive bilateral nephrectomy, supportive PD and early aggressive nutrition, can minimize early infant mortality, so that subsequent KT can be performed with excellent patient and graft survival.

show abstract

Prenatally diagnosed autosomal recessive polycystic kidney disease: initial postnatal management

Cited by 11 publications

References 5 publications

Morbidity from Congenital Hepatic Fibrosis after Renal Transplantation for Autosomal Recessive Polycystic Kidney Disease

Morbidity from Congenital Hepatic Fibrosis after Renal Transplantation for Autosomal Recessive Polycystic Kidney Disease

Unilateral Nephrectomy as Palliative Therapy in an Infant With Autosomal Recessive Polycystic Kidney Disease

Optimizing outcomes for neonatal ARPKD

Contact Info

Product

Resources

About