Pulmonary interstitial glycogenosis in identical twins.
We present the clinical, radiological, and pathological findings of open lung biopsies from monozygotic prematurely born male twins with respiratory distress at ages 6 and 8 weeks postnatally. Radiological examination showed a reticular nodular interstitial pattern on chest radiography. High-resolution computed tomography (HRCT) revealed ground-glass opacification and thickened interstitial septae in both infants. Lung biopsies showed a similar histology. There was diffuse interstitial thickening of the alveolar septa by mesenchymal cells, without prominent hyperplasia of type 2 pneumocytes, and without airspace exudates. Sections were periodic acid-Schiff (PAS)-positive within the cytoplasm of interstitial cells, indicating the presence of glycogen. Thus the diagnosis of pulmonary interstitial glycogenosis was made. Both infants were treated with glucocorticoids and had a favorable outcome. We speculate that pulmonary interstitial glycogenosis could be a histopathological form of chronic lung disease (CLD) of infancy.
Onland
W
W
Department of Pediatrics, Emma Children's Hospital, Academic Medical Center, Amsterdam, The Netherlands.
Molenaar
J J
JJ
Leguit
R J
RJ
van Nierop
J C
JC
Noorduyn
L A
LA
van Rijn
R R
RR
Geukers
V G M
VG
D004200:Diseases in Twins; D006008:Glycogen Storage Disease / Q000000981:diagnostic imaging* / Q000473:pathology; D006801:Humans; D007223:Infant; D008171:Lung Diseases / Q000000981:diagnostic imaging* / Q000473:pathology; D008297:Male; D011859:Radiography; D014430:Twins, Monozygotic