Pulmonary interstitial glycogenosis: A new variant of neonatal interstitial lung disease
Identifieur interne : 002252 ( Main/Exploration ); précédent : 002251; suivant : 002253Pulmonary interstitial glycogenosis: A new variant of neonatal interstitial lung disease
Auteurs : Anne-Marie Canakis [Canada] ; Ernest Cutz [Canada] ; David Manson [Canada] ; Hugh O'Brodovich [Canada]Source :
- American journal of respiratory and critical care medicine [ 1073-449X ] ; 2002.
Descripteurs français
- KwdFr :
- Association thérapeutique, Femelle, Glycogénose (), Glycogénose (diagnostic), Glycogénose (mortalité), Hormones corticosurrénaliennes (administration et posologie), Humains, Immunohistochimie, Indice de gravité médicale, Mâle, Nouveau-né, Pneumopathies interstitielles (), Pneumopathies interstitielles (anatomopathologie), Pneumopathies interstitielles (imagerie diagnostique), Pneumopathies interstitielles (mortalité), Ponction-biopsie à l'aiguille, Taux de survie, Tomodensitométrie, Ventilation artificielle, Études de suivi.
- MESH :
- administration et posologie : Hormones corticosurrénaliennes.
- anatomopathologie : Pneumopathies interstitielles.
- diagnostic : Glycogénose.
- imagerie diagnostique : Pneumopathies interstitielles.
- mortalité : Glycogénose, Pneumopathies interstitielles.
- Pascal (Inist)
- Association thérapeutique, Femelle, Glycogénose, Humains, Immunohistochimie, Indice de gravité médicale, Mâle, Nouveau né, Etude cas, Nouveau-né, Pneumopathie interstitielle, Physiopathologie, Diagnostic, Exploration, Chronique, Anatomopathologie, Immunohistochimie, Glucide, Pneumopathies interstitielles, Ponction-biopsie à l'aiguille, Taux de survie, Tomodensitométrie, Ventilation artificielle, Études de suivi.
English descriptors
- KwdEn :
- Adrenal Cortex Hormones (administration & dosage), Biopsy, Needle, Carbohydrate, Case study, Chronic, Combined Modality Therapy, Diagnosis, Exploration, Female, Follow-Up Studies, Glycogen Storage Disease (diagnosis), Glycogen Storage Disease (mortality), Glycogen Storage Disease (therapy), Glycogenosis, Humans, Immunohistochemistry, Infant, Newborn, Interstitial pneumonitis, Lung Diseases, Interstitial (congenital), Lung Diseases, Interstitial (diagnostic imaging), Lung Diseases, Interstitial (mortality), Lung Diseases, Interstitial (pathology), Male, Newborn, Pathology, Pathophysiology, Respiration, Artificial, Severity of Illness Index, Survival Rate, Tomography, X-Ray Computed.
- MESH :
- chemical , administration & dosage : Adrenal Cortex Hormones.
- congenital : Lung Diseases, Interstitial.
- diagnosis : Glycogen Storage Disease.
- diagnostic imaging : Lung Diseases, Interstitial.
- mortality : Glycogen Storage Disease, Lung Diseases, Interstitial.
- pathology : Lung Diseases, Interstitial.
- therapy : Glycogen Storage Disease.
- Biopsy, Needle, Combined Modality Therapy, Female, Follow-Up Studies, Humans, Immunohistochemistry, Infant, Newborn, Male, Respiration, Artificial, Severity of Illness Index, Survival Rate, Tomography, X-Ray Computed.
Abstract
We present the clinical, radiologic, and pathologic findings in lung biopsies from seven infants with atypical neonatal lung disease. All seven infants presented with tachypnea, hypoxemia, and diffuse interstitial infiltrates with overinflated lungs on chest radiographs in the first month of life. Lung biopsies from all cases showed similar pathology, with expansion of the interstitium by spindle-shaped cells containing periodic acid-Schiff positive diastase labile material consistent with glycogen. Immunohistochemical staining showed these cells to be vimentin positive but negative for leucocyte common antigen, lysozyme, and other macrophage markers. Electron microscopy revealed primitive interstitial mesenchymal cells with few cytoplasmic organelles and abundant monoparticulate glycogen. Minimal or no glycogen was seen in the alveolar lining cells. Five cases were treated with pulse corticosteroids; hydroxychloroquine was added in one case. Six of seven infants have shown a favorable clinical outcome. One infant died from complications of extreme prematurity and bronchopulmonary dysplasia. Three cases that have been followed for at least 6 years have shown clinical resolution and radiographic improvement. We propose the term "pulmonary interstitial glycogenosis" of the neonate for this new entity to be differentiated from other forms of interstitial lung disease. Because abundant glycogen is not normally found in pulmonary interstitial cells, we postulate an abnormality in lung cytodifferentiation involving interstitial mesenchymal cells.
Affiliations:
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Le document en format XML
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uniqKey="Canakis A" first="Anne-Marie" last="Canakis">Anne-Marie Canakis</name><affiliation wicri:level="4"><inist:fA14 i1="01"><s1>Division of Respiratory Medicine, Departments of Paediatrics and Physiology, Division of Pathology, Departments of Paediatric Laboratory Medicine and Diagnostic Imaging of the Hospital for Sick Children, University of Toronto</s1><s2>Toronto, Ontario</s2><s3>CAN</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>4 aut.</sZ></inist:fA14><country>Canada</country><placeName><settlement type="city">Toronto</settlement><region type="state">Ontario</region></placeName><orgName type="university">Université de Toronto</orgName></affiliation></author><author><name sortKey="Cutz, Ernest" sort="Cutz, Ernest" uniqKey="Cutz E" first="Ernest" last="Cutz">Ernest Cutz</name><affiliation wicri:level="4"><inist:fA14 i1="01"><s1>Division of Respiratory Medicine, Departments of Paediatrics and Physiology, Division of Pathology, Departments of Paediatric Laboratory Medicine and Diagnostic Imaging of the Hospital for Sick Children, University of Toronto</s1><s2>Toronto, Ontario</s2><s3>CAN</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>4 aut.</sZ></inist:fA14><country>Canada</country><placeName><settlement type="city">Toronto</settlement><region type="state">Ontario</region></placeName><orgName type="university">Université de Toronto</orgName></affiliation></author><author><name sortKey="Manson, David" sort="Manson, David" uniqKey="Manson D" first="David" last="Manson">David Manson</name><affiliation wicri:level="4"><inist:fA14 i1="01"><s1>Division of Respiratory Medicine, Departments of Paediatrics and Physiology, Division of Pathology, Departments of Paediatric Laboratory Medicine and Diagnostic Imaging of the Hospital for Sick Children, University of Toronto</s1><s2>Toronto, Ontario</s2><s3>CAN</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>4 aut.</sZ></inist:fA14><country>Canada</country><placeName><settlement type="city">Toronto</settlement><region type="state">Ontario</region></placeName><orgName type="university">Université de Toronto</orgName></affiliation></author><author><name sortKey="O Brodovich, Hugh" sort="O Brodovich, Hugh" uniqKey="O Brodovich H" first="Hugh" last="O'Brodovich">Hugh O'Brodovich</name><affiliation wicri:level="4"><inist:fA14 i1="01"><s1>Division of Respiratory Medicine, Departments of Paediatrics and Physiology, Division of Pathology, Departments of Paediatric Laboratory Medicine and Diagnostic Imaging of the Hospital for Sick Children, University of Toronto</s1><s2>Toronto, Ontario</s2><s3>CAN</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>4 aut.</sZ></inist:fA14><country>Canada</country><placeName><settlement type="city">Toronto</settlement><region type="state">Ontario</region></placeName><orgName type="university">Université de Toronto</orgName></affiliation></author></analytic><series><title level="j" type="main">American journal of respiratory and critical care medicine</title><title level="j" type="abbreviated">Am. j. respir. crit. care med.</title><idno type="ISSN">1073-449X</idno><imprint><date when="2002">2002</date></imprint></series></biblStruct></sourceDesc><seriesStmt><title level="j" type="main">American journal of respiratory and critical care medicine</title><title level="j" type="abbreviated">Am. j. respir. crit. care med.</title><idno type="ISSN">1073-449X</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adrenal Cortex Hormones (administration & dosage)</term><term>Biopsy, Needle</term><term>Carbohydrate</term><term>Case study</term><term>Chronic</term><term>Combined Modality Therapy</term><term>Diagnosis</term><term>Exploration</term><term>Female</term><term>Follow-Up Studies</term><term>Glycogen Storage Disease (diagnosis)</term><term>Glycogen Storage Disease (mortality)</term><term>Glycogen Storage Disease (therapy)</term><term>Glycogenosis</term><term>Humans</term><term>Immunohistochemistry</term><term>Infant, Newborn</term><term>Interstitial pneumonitis</term><term>Lung Diseases, Interstitial (congenital)</term><term>Lung Diseases, Interstitial (diagnostic imaging)</term><term>Lung Diseases, Interstitial (mortality)</term><term>Lung Diseases, Interstitial (pathology)</term><term>Male</term><term>Newborn</term><term>Pathology</term><term>Pathophysiology</term><term>Respiration, Artificial</term><term>Severity of Illness Index</term><term>Survival Rate</term><term>Tomography, X-Ray Computed</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Association thérapeutique</term><term>Femelle</term><term>Glycogénose ()</term><term>Glycogénose (diagnostic)</term><term>Glycogénose (mortalité)</term><term>Hormones corticosurrénaliennes (administration et posologie)</term><term>Humains</term><term>Immunohistochimie</term><term>Indice de gravité médicale</term><term>Mâle</term><term>Nouveau-né</term><term>Pneumopathies interstitielles ()</term><term>Pneumopathies interstitielles (anatomopathologie)</term><term>Pneumopathies interstitielles (imagerie diagnostique)</term><term>Pneumopathies interstitielles (mortalité)</term><term>Ponction-biopsie à l'aiguille</term><term>Taux de survie</term><term>Tomodensitométrie</term><term>Ventilation artificielle</term><term>Études de suivi</term></keywords><keywords scheme="MESH" type="chemical" qualifier="administration & dosage" xml:lang="en"><term>Adrenal Cortex Hormones</term></keywords><keywords scheme="MESH" qualifier="administration et posologie" xml:lang="fr"><term>Hormones corticosurrénaliennes</term></keywords><keywords scheme="MESH" qualifier="anatomopathologie" xml:lang="fr"><term>Pneumopathies interstitielles</term></keywords><keywords scheme="MESH" qualifier="congenital" xml:lang="en"><term>Lung Diseases, Interstitial</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Glycogen Storage Disease</term></keywords><keywords scheme="MESH" qualifier="diagnostic" xml:lang="fr"><term>Glycogénose</term></keywords><keywords scheme="MESH" qualifier="diagnostic imaging" xml:lang="en"><term>Lung Diseases, Interstitial</term></keywords><keywords scheme="MESH" qualifier="imagerie diagnostique" xml:lang="fr"><term>Pneumopathies interstitielles</term></keywords><keywords scheme="MESH" qualifier="mortality" xml:lang="en"><term>Glycogen Storage Disease</term><term>Lung Diseases, Interstitial</term></keywords><keywords scheme="MESH" qualifier="mortalité" xml:lang="fr"><term>Glycogénose</term><term>Pneumopathies interstitielles</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Lung Diseases, Interstitial</term></keywords><keywords scheme="MESH" qualifier="therapy" xml:lang="en"><term>Glycogen Storage Disease</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Biopsy, Needle</term><term>Combined Modality Therapy</term><term>Female</term><term>Follow-Up Studies</term><term>Humans</term><term>Immunohistochemistry</term><term>Infant, Newborn</term><term>Male</term><term>Respiration, Artificial</term><term>Severity of Illness Index</term><term>Survival Rate</term><term>Tomography, X-Ray Computed</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Association thérapeutique</term><term>Femelle</term><term>Glycogénose</term><term>Humains</term><term>Immunohistochimie</term><term>Indice de gravité médicale</term><term>Mâle</term><term>Nouveau né</term><term>Etude cas</term><term>Nouveau-né</term><term>Pneumopathie interstitielle</term><term>Physiopathologie</term><term>Diagnostic</term><term>Exploration</term><term>Chronique</term><term>Anatomopathologie</term><term>Immunohistochimie</term><term>Glucide</term><term>Pneumopathies interstitielles</term><term>Ponction-biopsie à l'aiguille</term><term>Taux de survie</term><term>Tomodensitométrie</term><term>Ventilation artificielle</term><term>Études de suivi</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">We present the clinical, radiologic, and pathologic findings in lung biopsies from seven infants with atypical neonatal lung disease. All seven infants presented with tachypnea, hypoxemia, and diffuse interstitial infiltrates with overinflated lungs on chest radiographs in the first month of life. Lung biopsies from all cases showed similar pathology, with expansion of the interstitium by spindle-shaped cells containing periodic acid-Schiff positive diastase labile material consistent with glycogen. Immunohistochemical staining showed these cells to be vimentin positive but negative for leucocyte common antigen, lysozyme, and other macrophage markers. Electron microscopy revealed primitive interstitial mesenchymal cells with few cytoplasmic organelles and abundant monoparticulate glycogen. Minimal or no glycogen was seen in the alveolar lining cells. Five cases were treated with pulse corticosteroids; hydroxychloroquine was added in one case. Six of seven infants have shown a favorable clinical outcome. One infant died from complications of extreme prematurity and bronchopulmonary dysplasia. Three cases that have been followed for at least 6 years have shown clinical resolution and radiographic improvement. We propose the term "pulmonary interstitial glycogenosis" of the neonate for this new entity to be differentiated from other forms of interstitial lung disease. Because abundant glycogen is not normally found in pulmonary interstitial cells, we postulate an abnormality in lung cytodifferentiation involving interstitial mesenchymal cells.</div></front></TEI><affiliations><list><country><li>Canada</li></country><region><li>Ontario</li></region><settlement><li>Toronto</li></settlement><orgName><li>Université de Toronto</li></orgName></list><tree><country name="Canada"><region name="Ontario"><name sortKey="Canakis, Anne Marie" sort="Canakis, Anne Marie" uniqKey="Canakis A" first="Anne-Marie" last="Canakis">Anne-Marie Canakis</name></region><name sortKey="Cutz, Ernest" sort="Cutz, Ernest" uniqKey="Cutz E" first="Ernest" last="Cutz">Ernest Cutz</name><name sortKey="Manson, David" sort="Manson, David" uniqKey="Manson D" first="David" last="Manson">David Manson</name><name sortKey="O Brodovich, Hugh" sort="O Brodovich, Hugh" uniqKey="O Brodovich H" first="Hugh" last="O'Brodovich">Hugh O'Brodovich</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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