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J Nephropathol. 2017;6(4): 363-367.
doi: 10.15171/jnp.2017.60

Scopus ID: 85036578925
  Abstract View: 1754
  PDF Download: 805

Case Report

Rapidly progressive nephromegaly in a neonate with autosomal recessive poly cystic kidney disease

Mitra Naseri 1 * , Nona Zabolinejad 2, Syed Ali Alamdaran 3, Reza Shojaeian 4

1 Pediatric Nephrology Department, Mashhad University of Medical Sciences, Mashhad, Iran
2 Pediatric Pathology Section, Mashhad University of Medical Sciences, Mashhad, Iran
3 Pediatric Radiology Section, Mashhad University of Medical Sciences, Mashhad, Iran
4 Pediatric Surgery Department, Dr. Sheikh Children Hospital, Mashhad University of Medical Sciences, Mashhad, Iran
*Corresponding author: Mitra Naseri, Email address: Naserim@mums.ac.ir

Abstract

Background: Autosomal recessive polycystic kidney disease (ARPKD) associates with
significant renal and liver-related morbidity and mortality in children. Rarely severe
enlargement of kidneys necessitates nephrectomy and initiating dialysis.

Case Presentation: A 5-day-old newborn boy with prenatal ultrasonography suggestive of
ARPKD admitted in the hospital due to anuria from first day of life. One week after
admission, left nephrectomy was conducted because of severe enlargement of kidneys
accompanied by respiratory distress and repeated vomiting. Patient was placed on peritoneal
dialysis. Pathology of kidney revealed cystic lesions consisted of dilated collecting duct,
with lack of involvement of glomeruli and other parts of the nephrons. Patient died at age
17 days with respiratory hemorrhage.


Conclusions:
Our case is interesting because of progressive increase in kidney sizes requiring
nephrectomy. Such huge nephromegaly in ARPKD has not been reported yet.

Keywords: ADPKD, ARPKD, Childhood, Nephromegaly

Implication for health policy/practice/research/medical education:

The course of the disease in our case emphasizes on respiratory problems as the main medical concerns in neonatal onset
ARPKD, even if the patients have no respiratory symptoms at birth.

Please cite this paper as: Naseri M, Zabolinejad N, Alamdaran SA, Shojaeian R. Rapidly progressive nephromegaly in a neonate
with autosomal recessive poly cystic kidney disease. J Nephropathol. 2017;6(4):363-367. DOI: 10.15171/jnp.2017.60.

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Submitted: 05 Mar 2017
Accepted: 03 Jul 2017
ePublished: 19 Jul 2017
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