Pulmonary interstitial glycogenosis in identical twins

W. Onland; J. J. Molenaar; R. J. Leguit; J. C. Van Nierop; L. A. Noorduyn; R. R. van Rijn; V. G. M. Geukers

doi:https://doi.org/10.1002/ppul.20255

Pulmonary interstitial glycogenosis in identical twins

W. Onland, J. J. Molenaar, R. J. Leguit, J. C. Van Nierop, L. A. Noorduyn, R. R. van Rijn, V. G. M. Geukers

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Abstract

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 interstititial 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

Original language	English
Pages (from-to)	362-366
Journal	Pediatric pulmonology
Volume	40
Issue number	4
DOIs	https://doi.org/10.1002/ppul.20255
Publication status	Published - 2005

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https://doi.org/10.1002/ppul.20255

Cite this

@article{bb5846ec622341909be07fe2f28a8a16,

title = "Pulmonary interstitial glycogenosis in identical twins",

abstract = "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 interstititial 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",

author = "W. Onland and Molenaar, {J. J.} and Leguit, {R. J.} and {Van Nierop}, {J. C.} and Noorduyn, {L. A.} and {van Rijn}, {R. R.} and Geukers, {V. G. M.}",

year = "2005",

doi = "https://doi.org/10.1002/ppul.20255",

language = "English",

volume = "40",

pages = "362--366",

journal = "Pediatric pulmonology",

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TY - JOUR

T1 - Pulmonary interstitial glycogenosis in identical twins

AU - Onland, W.

AU - Molenaar, J. J.

AU - Leguit, R. J.

AU - Van Nierop, J. C.

AU - Noorduyn, L. A.

AU - van Rijn, R. R.

AU - Geukers, V. G. M.

PY - 2005

Y1 - 2005

N2 - 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 interstititial 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

AB - 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 interstititial 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

U2 - https://doi.org/10.1002/ppul.20255

DO - https://doi.org/10.1002/ppul.20255

M3 - Article

C2 - 16082691

SN - 8755-6863

VL - 40

SP - 362

EP - 366

JO - Pediatric pulmonology

JF - Pediatric pulmonology

IS - 4

ER -