title: Pulmonary interstitial glycogenosis: an unrecognized etiology of persistent pulmonary hypertension of the newborn in congenital heart disease?
abstract: 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.
authors.affiliation: Department of Pediatrics, University of California, 513 Parnassus Avenue, Box 1346, San Francisco, CA 94143-1346, USA.
authors.forename: Monique R
authors.initials: MR
authors.lastname: Radman
authors.forename: Patricia
authors.initials: P
authors.lastname: Goldhoff
authors.forename: Kirk D
authors.initials: KD
authors.lastname: Jones
authors.forename: Anthony
authors.initials: A
authors.lastname: Azakie
authors.forename: Sanjeev
authors.initials: S
authors.lastname: Datar
authors.forename: Ian
authors.initials: I
authors.lastname: Adatia
authors.forename: Peter E
authors.initials: PE
authors.lastname: Oishi
authors.forename: Jeffrey R
authors.initials: JR
authors.lastname: Fineman
mesh_terms: D001706:Biopsy; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D006008:Glycogen Storage Disease / Q000150:complications* / Q000175:diagnosis; D006330:Heart Defects, Congenital / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D006801:Humans; D007231:Infant, Newborn; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000151:congenital* / Q000175:diagnosis; D008297:Male; D010547:Persistent Fetal Circulation Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D012127:Respiratory Distress Syndrome, Newborn / Q000209:etiology*