eMedicine Specialties > Radiology > Chest

Alveolar Proteinosis: Follow-up

Author: Michael B Gotway, MD, Clinical Associate Professor of Diagnostic Radiology and Pulmonary/Critical Care Medicine, University of California at San Francisco School of Medicine; Consulting Staff, Scottsdale Medical Imaging, Ltd
Coauthor(s): Theodore J Lee, MD, Chief, Thoracic Radiology, San Francisco General Hospital; Assistant Professor of Clinical Radiology, University of California at San Francisco
Contributor Information and Disclosures

Updated: Dec 30, 2008

Intervention

Special Concerns

  • In children, pulmonary alveolar proteinosis (PAP) may be divided into congenital and childhood forms, which are not only different from each other but also different from adult PAP in several respects.
    • Congenital PAP presents as severe, unresponsive respiratory distress at birth and is usually fatal. This clinical entity is believed to be the result of an autosomal recessive condition and may be easily mistaken for severe hyaline membrane disease, although a more prolonged course can be a clue to the true diagnosis. PAP in older infants and children often presents with failure to thrive and recurrent infections. Patients are often immunocompromised, commonly with thymic alymphoplasia, diminished levels of immunoglobulin A, or other autoimmune diseases.
    • Generally, PAP in children is often more acute and severe than PAP in adults. Chest radiographic and HRCT scan findings in childhood forms of PAP are few but are likely similar to their adult counterparts. McCook et al suggested that a miliarylike pattern is encountered more frequently in the pediatric entity than in the adult form and that basilar predominant linear and reticular abnormalities may be observed more often on CT scans in children.65 High-resolution CT (HRCT) scan descriptions of childhood PAP by Zontsich et al suggest that the crazy-paving pattern may be seen in children as it is in adults.61
 
Acknowledgments

Samuel K Dawn, MD, is gratefully acknowledged for contributions made to this topic.



More on Alveolar Proteinosis

Overview: Alveolar Proteinosis
Imaging: Alveolar Proteinosis
Follow-up: Alveolar Proteinosis
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Keywords

alveolar proteinosis, pulmonary alveolar phospholipoproteinosis, alveolar lipoproteinosis, PAP, surfactant protein, SP-B, granulocyte macrophage-colony stimulating factor, GM-CSF, bronchoalveolar lavage, BAL, whole-lung lavage, diffuse air-space disease

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Contributor Information and Disclosures

Author

Michael B Gotway, MD, Clinical Associate Professor of Diagnostic Radiology and Pulmonary/Critical Care Medicine, University of California at San Francisco School of Medicine; Consulting Staff, Scottsdale Medical Imaging, Ltd
Michael B Gotway, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, Radiological Society of North America, and Society of Thoracic Radiology
Disclosure: Nothing to disclose.

Coauthor(s)

Theodore J Lee, MD, Chief, Thoracic Radiology, San Francisco General Hospital; Assistant Professor of Clinical Radiology, University of California at San Francisco
Theodore J Lee, MD is a member of the following medical societies: American Roentgen Ray Society, Radiological Society of North America, and Society of Thoracic Radiology
Disclosure: Nothing to disclose.

Medical Editor

Jeffrey A Miller, MD, Associate Professor of Clinical Radiology, University of Medicine and Dentistry of New Jersey; Associate Chief of Service, Department of Radiology, Veterans Affairs of New Jersey Health Care System
Jeffrey A Miller, MD is a member of the following medical societies: North American Society for Cardiac Imaging, Society for Health Services Research in Radiology, and Society of Thoracic Radiology
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

John D Newell, Jr, MD, FACR, FCCP, FASER, Co-Director of Thoracic Imaging, UCDHSC; Director of Lung Imaging Center, Professor of Radiology and Professor of Medicine, Department of Radiology, University of Colorado Health Sciences Center, National Jewish Medical and Research Center; Univ. Colorado Hospital
John D Newell, Jr, MD, FACR, FCCP, FASER is a member of the following medical societies: American College of Chest Physicians, American College of Radiology, American Roentgen Ray Society, American Thoracic Society, Association of University Radiologists, Radiological Society of North America, and Society of Thoracic Radiology
Disclosure: Siemens Medical Grant/research funds Consulting; Forevision Technologies Ownership interest Consulting; Vida Corporation Ownership interest Board membership; TeraRecon Grant/research funds Consulting; eMedicine Honoraria Consulting

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

Barry H Gross, MD, Professor, Department of Radiology, University of Michigan Medical School; Professor, University of Michigan Cancer Center
Barry H Gross, MD is a member of the following medical societies: American College of Chest Physicians, American College of Radiology, American Roentgen Ray Society, Association of University Radiologists, Michigan State Medical Society, Physicians for Social Responsibility, Radiological Society of North America, and Society of Thoracic Radiology
Disclosure: Nothing to disclose.

 
 
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