title: Pulmonary maturational arrest and death in a patient with pulmonary interstitial glycogenosis.
abstract: We present the clinical presentation and pathological findings from a term infant with atypical neonatal lung disease. A full term Caucasian male presented at birth with signs of respiratory distress. The respiratory condition continued to deteriorate despite maximum intervention and the patient was placed on ECMO for further cardiorespiratory assistance. An open lung biopsy demonstrated findings consistent with severe lung growth abnormality with non-uniform pulmonary interstitial glycogenosis. The patient consequently developed a pulmonary hemorrhage that required discontinuation of ECMO. The patient died shortly after decannulation. Most literature suggests that PIG is one of the few pediatric interstitial lung diseases that has a favorable prognosis with rare mortality in the absence of co-morbidities. However, the current case suggests prognosis may depend more on the underlying diagnosis than on the histological finding of PIG. In addition, this case may provide insight into the pathogenesis and potential modifiers of this idiopathic disorder.
authors: The Perinatal Institute, Section of Neonatology, Perinatal and Pulmonary Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH45229-3039, USA. paul.kingma@cchmc.org
authors: Brooke A
authors: BA
authors: King
authors: J Todd
authors: JT
authors: Boyd
authors: Paul S
authors: PS
authors: Kingma
references: Pediatr Radiol. 2007 Jan;37(1):47-56
references: 17063351
references: Am J Respir Crit Care Med. 2002 Jun 1;165(11):1557-65
references: 12045133
references: Chest. 1992 Apr;101(4):1065-9
references: 1555422
references: Pediatr Pulmonol. 2005 Oct;40(4):362-6
references: 16082691
references: Am J Respir Crit Care Med. 2007 Dec 1;176(11):1120-8
references: 17885266
references: Pediatr Dev Pathol. 2009 Nov-Dec;12(6):475-80
references: 19320535
references: Clin Perinatol. 2009 Jun;36(2):391-416, x
references: 19559327