TY - JOUR
T1 - Bilateral renal dysplasia, nephroblastomatosis, and bronchial stenosis. A new syndrome?
AU - Rodriguez, Maria Matilde
AU - Correa-Medina, Mayrin
AU - Whittington, Elizabeth E.
N1 - Publisher Copyright:
© 2014 Informa Healthcare USA, Inc.
Copyright:
Copyright 2015 Elsevier B.V., All rights reserved.
PY - 2015/6/1
Y1 - 2015/6/1
N2 - Bilateral nephroblastomatosis (NB) is an uncommon renal anomaly characterized by multiple confluent nephrogenic rests scattered through both kidneys, with only a limited number of cases reported in the medical literature. Some of these children may have associated either Perlman or Beckwith-Wiedemann syndrome and others do not demonstrate syndromic features. We report a full-term boy with anteverted nose, bilateral bronchial stenosis due to lack of cartilage, bilateral obstructive renal dysplasia and NB with glomeruloid features. The infant had visceromegaly, but neither gigantism nor hemihypertrophy. Immunohistochemistry for PAX2 (Paired box gene-2) and WT-1 (Wilms Tumor 1) were strongly positive in the areas of NB. GLEPP-1 (Glomerular Epithelial Protein) did not stain the areas of NB with a glomeruloid appearance, but was positive in the renal glomeruli as expected. We found neither associated bronchial stenosis nor the histology of NB resembling giant glomeruli in any of the reported cases of NB.
AB - Bilateral nephroblastomatosis (NB) is an uncommon renal anomaly characterized by multiple confluent nephrogenic rests scattered through both kidneys, with only a limited number of cases reported in the medical literature. Some of these children may have associated either Perlman or Beckwith-Wiedemann syndrome and others do not demonstrate syndromic features. We report a full-term boy with anteverted nose, bilateral bronchial stenosis due to lack of cartilage, bilateral obstructive renal dysplasia and NB with glomeruloid features. The infant had visceromegaly, but neither gigantism nor hemihypertrophy. Immunohistochemistry for PAX2 (Paired box gene-2) and WT-1 (Wilms Tumor 1) were strongly positive in the areas of NB. GLEPP-1 (Glomerular Epithelial Protein) did not stain the areas of NB with a glomeruloid appearance, but was positive in the renal glomeruli as expected. We found neither associated bronchial stenosis nor the histology of NB resembling giant glomeruli in any of the reported cases of NB.
KW - Bronchial stenosis
KW - Nephroblastomatosis
KW - Nephrogenic rests
KW - Renal dysplasia
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U2 - 10.3109/15513815.2015.1014952
DO - 10.3109/15513815.2015.1014952
M3 - Article
C2 - 25871299
AN - SCOPUS:84929878291
VL - 34
SP - 190
EP - 196
JO - Fetal and Pediatric Pathology
JF - Fetal and Pediatric Pathology
SN - 1551-3815
IS - 3
ER -