eMedicine Specialties > Pediatrics: General Medicine > Pulmonology

Children's Interstitial Lung Disease (ChILD): Multimedia

Author: James S Hagood, MD, Director, Pediatric Pulmonary Center, Professor of Pediatrics, Cell Biology, Pathology and Biochemistry and Molecular Genetics, Department of Pediatrics, University of Alabama School of Medicine
Coauthor(s): Gulnur Com, MD, Pediatric Pulmonologist, University of Arkansas for Medical Sciences Children's Hospital; David J Vaughan, MBBCh, Consultant Pediatrician, Department of Pediatrics, Our Lady of Lourdes Hospital, Ireland; Daniel William Young, MD, FACR, Clinical Professor of Radiology, Clinical Professor of Pediatrics, University of Alabama School of Medicine; Active Staff, Department of Pediatric Imaging, Children's Hospital of Alabama; Vice-President, Pediatric Radiology Associates, PC; Elizabeth C Mroczek-Musulman, MD, Clinical Associate Professor of Pathology, Associate Pathologist, Department of Pathology, University of Alabama Schools of Medicine and Dentistry, The Children's Hospital of Alabama; Lisa R Young, MD, Assistant Professor, Pediatric Pulmonary Medicine and Pulmonary Critical Care and Sleep Medicine, University of Cincinnati; Director of Pediatric Rare Lung Diseases Program and Consulting Physician, Cincinnati Children's Hospital Medical Center; Consulting Physician, University Hospital, Cincinnati
Contributor Information and Disclosures

Updated: Sep 11, 2009

Multimedia

Interstitial lung disease (ILD) due to <EM>ABCA3<...
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Media file 1: Interstitial lung disease (ILD) due to ABCA3 gene mutations.

(A) High-resolution CT (HRCT) scan from a 4-month-old infant with ABCA3 mutations. The CT scan was performed with controlled ventilation under general anesthesia. Diffuse bilateral ground glass opacities and thickened interlobular septae are present. This "crazy paving" pattern suggests alveolar proteinosis or ILD due to genetic mutations affecting surfactant function and metabolism.

(B) Histopathology (hematoxylin and eosin) shows diffuse alveolar septal thickening with uniform prominent type II cell hyperplasia. Accumulations of alveolar macrophages and granular proteinosis are also present in the alveolar spaces.

(C) Electron microscopy demonstrates abnormal lamellar bodies with dense inclusions (arrows).
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Interstitial lung disease (ILD) due to <EM>ABCA3<...

Interstitial lung disease (ILD) due to ABCA3 gene mutations.

(A) High-resolution CT (HRCT) scan from a 4-month-old infant with ABCA3 mutations. The CT scan was performed with controlled ventilation under general anesthesia. Diffuse bilateral ground glass opacities and thickened interlobular septae are present. This "crazy paving" pattern suggests alveolar proteinosis or ILD due to genetic mutations affecting surfactant function and metabolism.

(B) Histopathology (hematoxylin and eosin) shows diffuse alveolar septal thickening with uniform prominent type II cell hyperplasia. Accumulations of alveolar macrophages and granular proteinosis are also present in the alveolar spaces.

(C) Electron microscopy demonstrates abnormal lamellar bodies with dense inclusions (arrows).

Pulmonary interstitial glycogenosis (PIG). <BR><B...
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Media file 2: Pulmonary interstitial glycogenosis (PIG).

(A) Lung histopathology from a 5-week-old infant shows diffuse interstitial widening and cellularity with bland-appearing vacuolated foamy cells that contain glycogen (periodic acid-Schiff [PAS] stain). These cells seen in PIG are strongly immunoreactive with vimentin (not shown). Pigmented alveolar macrophages were an additional finding in this infant with history of meconium aspiration.

(B) Electron microscopy demonstrates that these mesenchymal cells contain abundant monoparticulate glycogen.
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Pulmonary interstitial glycogenosis (PIG). <BR><B...

Pulmonary interstitial glycogenosis (PIG).

(A) Lung histopathology from a 5-week-old infant shows diffuse interstitial widening and cellularity with bland-appearing vacuolated foamy cells that contain glycogen (periodic acid-Schiff [PAS] stain). These cells seen in PIG are strongly immunoreactive with vimentin (not shown). Pigmented alveolar macrophages were an additional finding in this infant with history of meconium aspiration.

(B) Electron microscopy demonstrates that these mesenchymal cells contain abundant monoparticulate glycogen.

Neuroendocrine cell hyperplasia of infancy (NEHI)...
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Media file 3: Neuroendocrine cell hyperplasia of infancy (NEHI)

(A) Chest high-resolution CT (HRCT) scanning (at total lung capacity) in a 6-month-old infant with tachypnea, hypoxemia, and failure to thrive. Sharply defined areas of ground glass opacity are seen most prominent in the right middle lobe and lingual. Diffuse air-trapping was seen on expiratory images (not shown). No additional abnormalities were identified.

(B) Hematoxylin and eosin staining of the lung biopsy reveals near-normal lung architecture.

(C) Bombesin immunostaining reveals increased numbers of neuroendocrine cells.
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Neuroendocrine cell hyperplasia of infancy (NEHI)...

Neuroendocrine cell hyperplasia of infancy (NEHI)

(A) Chest high-resolution CT (HRCT) scanning (at total lung capacity) in a 6-month-old infant with tachypnea, hypoxemia, and failure to thrive. Sharply defined areas of ground glass opacity are seen most prominent in the right middle lobe and lingual. Diffuse air-trapping was seen on expiratory images (not shown). No additional abnormalities were identified.

(B) Hematoxylin and eosin staining of the lung biopsy reveals near-normal lung architecture.

(C) Bombesin immunostaining reveals increased numbers of neuroendocrine cells.

Follicular bronchiolitis <BR><BR>(A) Chest high-r...
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Media file 4: Follicular bronchiolitis

(A) Chest high-resolution CT (HRCT) scan from a 6-year-old infant with common variable immunodeficiency with history of anemia, thrombocytopenia, recurrent pneumonia, chronic cough, and exercise intolerance. Mosaic attenuation is present diffusely throughout the lungs. Extensive hilar and mediastinal lymphadenopathy is also present. Air-trapping was seen on expiratory images (not shown).

(B) Lung histopathology demonstrates severe airway-centric lymphocytic inflammation with reactive follicles, which infiltrates and obscures most bronchioles.
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Follicular bronchiolitis <BR><BR>(A) Chest high-r...

Follicular bronchiolitis

(A) Chest high-resolution CT (HRCT) scan from a 6-year-old infant with common variable immunodeficiency with history of anemia, thrombocytopenia, recurrent pneumonia, chronic cough, and exercise intolerance. Mosaic attenuation is present diffusely throughout the lungs. Extensive hilar and mediastinal lymphadenopathy is also present. Air-trapping was seen on expiratory images (not shown).

(B) Lung histopathology demonstrates severe airway-centric lymphocytic inflammation with reactive follicles, which infiltrates and obscures most bronchioles.

Bronchiolitis obliterans. <BR><BR>(A) Chest CT sc...
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Media file 5: Bronchiolitis obliterans.

(A) Chest CT scanning from an 8-year-old demonstrates irregular large mosaic regions of ground-glass opacity and air-trapping, as well as the presence of peribronchial thickening and bronchiectasis.

(B) Pathology demonstrates focal areas of fibrosis with polypoid plugs of fibroblastic cells and fibrin filling distal bronchioles and airspaces (hematoxylin and eosin).
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Bronchiolitis obliterans. <BR><BR>(A) Chest CT sc...

Bronchiolitis obliterans.

(A) Chest CT scanning from an 8-year-old demonstrates irregular large mosaic regions of ground-glass opacity and air-trapping, as well as the presence of peribronchial thickening and bronchiectasis.

(B) Pathology demonstrates focal areas of fibrosis with polypoid plugs of fibroblastic cells and fibrin filling distal bronchioles and airspaces (hematoxylin and eosin).

Nonspecific interstitial pneumonitis. <BR><BR>(A)...
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Media file 6: Nonspecific interstitial pneumonitis.

(A) Chest high-resolution CT (HRCT) scanning from a 10-year-old with systemic sclerosis and progressive exercise intolerance.

(B) Lung biopsy showed multiple abnormalities including a relatively diffuse interstitial process with mild chronic inflammation, abundant fibroblastic tissue and patchy dense interstitial fibrosis. Accumulation of alveolar macrophages is seen in the airspaces, with rare foci of organizing pneumonia. Pulmonary arteries demonstrated focal intimal hyperplasia and medial hypertrophy, and the pleura contains patchy chronic inflammation. This overall constellation of findings is generally classified as mixed cellular and fibrotic nonspecific interstitial pneumonia (NSIP) and is a pattern most commonly seen in the setting of underlying collagen vascular disease.
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Nonspecific interstitial pneumonitis. <BR><BR>(A)...

Nonspecific interstitial pneumonitis.

(A) Chest high-resolution CT (HRCT) scanning from a 10-year-old with systemic sclerosis and progressive exercise intolerance.

(B) Lung biopsy showed multiple abnormalities including a relatively diffuse interstitial process with mild chronic inflammation, abundant fibroblastic tissue and patchy dense interstitial fibrosis. Accumulation of alveolar macrophages is seen in the airspaces, with rare foci of organizing pneumonia. Pulmonary arteries demonstrated focal intimal hyperplasia and medial hypertrophy, and the pleura contains patchy chronic inflammation. This overall constellation of findings is generally classified as mixed cellular and fibrotic nonspecific interstitial pneumonia (NSIP) and is a pattern most commonly seen in the setting of underlying collagen vascular disease.

More on Children's Interstitial Lung Disease (ChILD)

Overview: Children's Interstitial Lung Disease (ChILD)
Differential Diagnoses & Workup: Children's Interstitial Lung Disease (ChILD)
Treatment & Medication: Children's Interstitial Lung Disease (ChILD)
Follow-up: Children's Interstitial Lung Disease (ChILD)
Multimedia: Children's Interstitial Lung Disease (ChILD)
References
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Further Reading

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Keywords

children’s interstitial lung disease, ChILD, ILD, diffuse infiltrative lung disease, pulmonary disease, lung disease, interstitial disease, idiopathic pulmonary fibrosis, IPF, nonidiopathic interstitial pulmonary fibrosis, usual interstitial pneumonia, UIP, desquamative interstitial pneumonia, DIP, bronchiolitis obliterans with interstitial pneumonia, BIP, lymphoid interstitial pneumonia, lymphocytic interstitial pneumonia, LIP, giant cell interstitial pneumonia, giant-cell interstitial pneumonia, GIP, respiratory bronchiolitis interstitial lung disease, RBILD

nonspecific interstitial pneumonia, NSIP, bronchiolitis obliterans organizing pneumonia, BOOP, cryptogenic organizing pneumonia, COP, cryptogenic fibrosing alveolitis, CFA, pulmonary histiocytosis X, eosinophilic granuloma, Langerhans cell histiocytosis, LCH, acute interstitial pneumonia, AIP, idiopathic BOOP, nonclassifiable ILD, neuroendocrine cell hyperplasia of infancy, NEHI, pulmonary interstitial glycogenosis, PIG, idiopathic interstitial pneumonia, cryptogenic fibrosing alveolitis, chronic pneumonitis of infancy, cellular interstitial pneumonitis

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Contributor Information and Disclosures

Author

James S Hagood, MD, Director, Pediatric Pulmonary Center, Professor of Pediatrics, Cell Biology, Pathology and Biochemistry and Molecular Genetics, Department of Pediatrics, University of Alabama School of Medicine
James S Hagood, MD is a member of the following medical societies: American Thoracic Society
Disclosure: Nothing to disclose.

Coauthor(s)

Gulnur Com, MD, Pediatric Pulmonologist, University of Arkansas for Medical Sciences Children's Hospital
Gulnur Com, MD is a member of the following medical societies: American Academy of Pediatrics, American Thoracic Society, and Cystic Fibrosis Foundation
Disclosure: Nothing to disclose.

David J Vaughan, MBBCh, Consultant Pediatrician, Department of Pediatrics, Our Lady of Lourdes Hospital, Ireland
David J Vaughan, MBBCh is a member of the following medical societies: American College of Chest Physicians, American Thoracic Society, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

Daniel William Young, MD, FACR, Clinical Professor of Radiology, Clinical Professor of Pediatrics, University of Alabama School of Medicine; Active Staff, Department of Pediatric Imaging, Children's Hospital of Alabama; Vice-President, Pediatric Radiology Associates, PC
Daniel William Young, MD, FACR is a member of the following medical societies: Alpha Omega Alpha, American College of Radiology, Radiological Society of North America, and Society for Pediatric Radiology
Disclosure: Nothing to disclose.

Elizabeth C Mroczek-Musulman, MD, Clinical Associate Professor of Pathology, Associate Pathologist, Department of Pathology, University of Alabama Schools of Medicine and Dentistry, The Children's Hospital of Alabama
Elizabeth C Mroczek-Musulman, MD is a member of the following medical societies: American Society for Clinical Pathology and College of American Pathologists
Disclosure: Nothing to disclose.

Lisa R Young, MD, Assistant Professor, Pediatric Pulmonary Medicine and Pulmonary Critical Care and Sleep Medicine, University of Cincinnati; Director of Pediatric Rare Lung Diseases Program and Consulting Physician, Cincinnati Children's Hospital Medical Center; Consulting Physician, University Hospital, Cincinnati
Lisa R Young, MD is a member of the following medical societies: American College of Chest Physicians, American Thoracic Society, Central Society for Clinical Research, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Medical Editor

Susanna A McColley, MD, Director of Cystic Fibrosis Center; Head, Division of Pulmonary Medicine; Associate Professor, Department of Pediatrics, Children's Memorial Medical Center of Chicago, Northwestern University
Susanna A McColley, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Sleep Disorders Association, and American Thoracic Society
Disclosure: Genentech Honoraria Speaking and teaching; Genentech Honoraria Consulting; Novartis Honoraria Consulting; Altus  Consulting fee Consulting; Axcan Scandi Consulting fee Consulting; Boston Scientific Consulting fee Consulting; Gilead  Speaking and teaching

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Heidi Connolly, MD, Associate Professor of Pediatrics and Psychiatry, University of Rochester; Director, Pediatric Sleep Medicine Services, Strong Sleep Disorders Center
Heidi Connolly, MD is a member of the following medical societies: American Academy of Pediatrics, American Thoracic Society, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

CME Editor

Mary E Cataletto, MD, Associate Director, Division of Pediatric Pulmonology, Winthrop University Hospital; Professor of Clinical Pediatrics, State University of New York at Stony Brook; Director of Children's Sleep Services, Winthrop University Hospital
Mary E Cataletto, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Chest Physicians
Disclosure: Shering Plough Pharmaceuticals Honoraria Consulting

Chief Editor

Michael R Bye, MD, Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons; Attending Physician, Pediatric Pulmonary Medicine, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center
Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, and American Thoracic Society
Disclosure: Merck Honoraria Speaking and teaching

 
 
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