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Previous Volume 349:2527-2539 December 25, 2003 Number 26 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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Pulmonary alveolar proteinosis is a rare disorder in which lipoproteinaceous material accumulates within alveoli.¹ The clinical course of the disease is variable, ranging from respiratory failure to spontaneous resolution. An important feature of the disease is susceptibility to pulmonary infections, sometimes with opportunistic organisms.

Pulmonary alveolar proteinosis occurs in three clinically distinct forms: congenital, secondary, and acquired. The congenital form comprises a heterogeneous group of disorders² caused by mutations in the genes encoding surfactant protein B or C or the _C chain of the receptor for granulocyte–macrophage colony-stimulating factor (GM-CSF).^3,4,5,6,7 Secondary pulmonary alveolar proteinosis develops in association with conditions involving functional . . . [Full Text of this Article]

Epidemiology

Clinical, Radiographic, and Laboratory Manifestations

Clinical Presentation

Laboratory Findings

Pulmonary Function

Characteristics of Bronchoalveolar-Lavage Fluid

Pathological Features

Natural History

Surfactant Homeostasis

Mouse Models

GM-CSF and Surfactant Homeostasis

            Effect of GM-CSF Replacement

            Cellular Target of GM-CSF

Immune Functions of Alveolar Macrophages and GM-CSF

Role of the Transcription Factor PU.1

Lessons from Animal Models

Pathogenesis in Humans

Role of Autoimmunity

            Inhibition of Alveolar Macrophages

            Role of GM-CSF

            Autoantibodies against GM-CSF

Pulmonary Cytokines

Therapeutic Approaches

Current Approaches

GM-CSF Therapy

Conclusions

Source Information

From the Divisions of Pulmonary Biology (B.C.T., J.A.W.) and Neonatology (J.A.W.), Children's Hospital Medical Center, Cincinnati; and the Department of Respiratory Diseases, International Medical Center of Japan, Tokyo (K.N.).

Address reprint requests to Dr. Trapnell at the Division of Pulmonary Biology, Children's Hospital Medical Center, 3333 Burnet Ave., Cincinnati, OH 45229, or at bruce.trapnell@cchmc.org.

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