TY - JOUR
T1 - Pulmonary interstitial glycogenosis
T2 - An unrecognized etiology of persistent pulmonary hypertension of the newborn in congenital heart disease?
AU - Radman, Monique R.
AU - Goldhoff, Patricia
AU - Jones, Kirk D.
AU - Azakie, Anthony
AU - Datar, Sanjeev
AU - Adatia, Ian
AU - Oishi, Peter E.
AU - Fineman, Jeffrey R.
N1 - Funding Information:
This research was supported in part by HL61284 (to J.R.F.) from the National Institutes of Health.
PY - 2013/6
Y1 - 2013/6
N2 - Background: Pulmonary interstitial glycogenosis (PIG) arises from a developmental disorder of the pulmonary mesenchyme and presents clinically with reversible neonatal respiratory distress and/or persistent pulmonary hypertension of the newborn (PPHN). Objective: We report two cases of PIG in patients with congenital heart disease (CHD) and evidence of PPHN. Results: Both cases demonstrated the hallmark PIG histologic finding of diffuse, uniform interstitial thickening due to the presence of immature interstitial cells containing abundant cytoplasmic glycogen. Conclusions: We report the second and third patients with PIG associated with CHD. Because histologic examination is required to establish the diagnosis, we speculate that PIG, although rare, may be underrecognized in neonates presenting with PPHN in the setting of CHD.
AB - Background: Pulmonary interstitial glycogenosis (PIG) arises from a developmental disorder of the pulmonary mesenchyme and presents clinically with reversible neonatal respiratory distress and/or persistent pulmonary hypertension of the newborn (PPHN). Objective: We report two cases of PIG in patients with congenital heart disease (CHD) and evidence of PPHN. Results: Both cases demonstrated the hallmark PIG histologic finding of diffuse, uniform interstitial thickening due to the presence of immature interstitial cells containing abundant cytoplasmic glycogen. Conclusions: We report the second and third patients with PIG associated with CHD. Because histologic examination is required to establish the diagnosis, we speculate that PIG, although rare, may be underrecognized in neonates presenting with PPHN in the setting of CHD.
KW - Congenital heart defects
KW - Glycogen storage diseases
KW - Interstitial lung diseases
KW - Neonatal respiratory distress syndrome
KW - Persistent pulmonary hypertension of the newborn
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U2 - 10.1007/s00246-012-0371-z
DO - 10.1007/s00246-012-0371-z
M3 - Article
C2 - 22614905
AN - SCOPUS:84878020527
SN - 0172-0643
VL - 34
SP - 1254
EP - 1257
JO - Pediatric Cardiology
JF - Pediatric Cardiology
IS - 5
ER -
