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  • International Scientific Advisory Board
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International Scientific Advisory Board
Scientific Advisory Board
Bruce C. Trapnell, M.D. (Chair)
Mani Kavuru, M.D.
John Seymour, M.D.
Francis X. McCormack, M.D.
Koh Nakata, M.D. Ph.D.
Michael Beers, M.D.

 
Milestones in PAP Research

History and Scientific Background
Acquired PAP has only been recognized in the literature since 1958 when an article published in The New England Journal of Medicine and authored by doctors Rosen, Castleman, and Liebow, commented on the abnormal yet distinctive histological appearance of lung tissue specimens marked by accumulation of lipoproteinaceous material. In 1965, Larson and Gordinier first suggested that this material was an abnormal accumulation of surfactant.

For many years, the cause of the accumulation of lipoproteinaceous material in the alveoli of the lungs was debated. Was it due to abnormalities in the production, break-down, or actual structure of surfactant? It wasn’t until1994 that the discovery of a pulmonary disorder in knock-out mice (mice in which a gene has been silenced or made non-functional) played a key role in understanding the true nature of the abnormalities. These mice were knock-outs for a gene that encoded for a growth factor called GM-CSF, thought to be responsible for stimulating the growth and differentiation of blood cells derived from bone marrow. The mice, deficient in GM-CSF, developed a condition that mirrored the alveolar lipoproteinosis in humans.
 
Genetically Altered Mice Deficient in GM-CSF Develop PAP
Researchers, seeking to determine if the GM-CSF deficiency was in fact the cause of alveolar proteinosis in the knock-out mice, tried replacing GM-CSF by multiple methods and consistently were able to reverse the lung disorder, indicating the key role that this growth factor plays in the development of the disease. With this new information, research soon turned to examining why lack of GM-CSF caused alveolar proteinosis in mice.
 
Researchers first investigated to see if GM-CSF deficiency was somehow causing an overproduction of surfactant in the lungs of the knock-out mice, but studies of gene expression in the mice indicated that there was no increased production of surfactant proteins or phospholipids as compared to normal mice. However, in other studies severe impairments in the clearance of proteins and phospholipids were observed. The scientists soon began to suspect that perhaps some sort of defect in the lungs’ alveolar macrophages was resulting in inadequate clearance of surfactant from the lungs of the GM-CSF knock-out mice. Supporting this idea was the observation of abnormal, enlarged alveolar macrophages with a foamy appearance that were obtained from the lungs of the knock-out mice.
 
Follow up studies examining alveolar macrophages demonstrated that in the absence of GM-CSF, the alveolar macrophages are capable of engulfing (ingesting) surfactant, but are unable to properly break it down, possibly explaining the enlarged, foamy appearance of the alveolar macrophages and the accumulation of lipoproteinaceous material in the lungs. This also supported the previously hypothesized idea that inadequate clearance of surfactant from the lungs of GM-CSF knock-out mice was due to impairment of alveolar macrophage function. Subsequent studies showed that GM-CSF stimulated a key growth factor for macrophages and indicated that lack of GM-CSF leads to defective maturation of the alveolar macrophages and, consequently, malfunction.
 
The Role of GM-CSF in Primary PAP In Humans
One key difference that makes the alveolar lipoproteinosis in mice different from in the acquired human disease is that in the human version, there is no deficiency in GM-CSF production. What then accounts for the disorder in humans?
 
In September of 1999, a ground-breaking paper by Kitamura et al. was published in The Journal of Experimental Medicine announcing the discovery that acquired PAP is an autoimmune disease. An autoimmune disease is a disorder that occurs when the body mistakenly mounts an immune response against its own self-made substances, cells, or tissues. Typically in an autoimmune disease, the body produces antibodies against whatever it has misidentified as foreign. In the case of acquired PAP, the researchers discovered the presence of an antibody against GM-CSF in both the blood and in Bronchoalveolar Lavage Fluid (BALF, fluid that is obtained when the lungs are rinsed with saline). This "neutralizing" antibody was not found in healthy patients.
 
More recently, investigations into the effects of the autoantibody obtained from acquired PAP patients demonstrated that the presence of the antibody inhibits the growth and development of alveolar macrophages, further supporting the concept that GM-CSF plays an important role in the maturation of these immune cells. In addition, studies have also demonstrated that the autoantibody is present in high enough quantities to neutralize the activity of GM-CSF in the lungs, indicating that this autoantibody is likely key in the development of acquired pulmonary alveolar proteinosis.




 

PAP Publications


Please click on a link below to see the articles on the subject:

Review Articles of Primary (IDIOPATHIC) PAP
  • Epidemiology- The Study of the Determinants of Primary PAP
  • Clinical, Radiographic & Laboratory Manifestations of Primary PAP
  • Natural History, Background, and Associations of PAP
  • Surfactant Homeostasis in PAP
  • Mouse Models of PAP
  • Pathogenesis of Primary (Idiopathic) PAP
  • Therapeutic Approaches to Treat Primary PAP
Congenital Pulmonary Alveolar Proteinosis
Published Literature on SECONDARY PAP

 
Review Articles of Primary (IDIOPATHIC) PAP

Pulmonary alveolar proteinosis
Trapnell BC, Whitsett JA and Nakata K
N Engl J Med 2003;349:2527-39
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=14695413

Pulmonary alveolar proteinosis: progress in the first 44 years

Seymour JF and Presneill JJ
Am J Respir Crit Care Med 2002;166:215-35
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=12119235

GM-CSF regulates pulmonary surfactant homeostasis and alveolar macrophage-mediated innate host defense
Trapnell BC and Whitsett JA
Annu Rev Physiol 2002;64:775-802
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11826288

Pulmonary alveolar proteinosis: a review

deMello DE and Lin Z
Pediatr Pathol Mol Med 2001;20:413-32
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11552740

Diagnosing pulmonary alveolar proteinosis. A review and an update

Wang BM, Stern EJ, Schmidt RA and Pierson DJ
Chest 1997;111:460-6
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=9041997

[Primary pulmonary alveolar proteinosis--clinical observation of 68 patients in Japan]
Asamoto H, Kitaichi M, Nishimura K, Itoh H and Izumi T
Nihon Kyobu Shikkan Gakkai Zasshi 1995;33:835-45
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=7474563

Pulmonary alveolar phospholipoproteinosis: experience with 34 cases and a review
Prakash UB, Barham SS, Carpenter HA, Dines DE and Marsh HM
Mayo Clin Proc 1987;62:499-518
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=3553760

Pulmonary alveolar proteinosis: prospective clinical experience in 23 patients for 15 years
Kariman K, Kylstra JA and Spock A
Lung 1984;162:223-31
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=6492867

Pulmonary alveolar proteinosis in four siblings
Teja K, Cooper PH, Squires JE and Schnatterly PT
N Engl J Med 1981;305:1390-2
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=7300857

Pulmonary alveolar proteinosis
Davidson JM and Macleod WM
Br J Dis Chest 1969;63:13-28
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=4886263

Pulmonary Alveolar Proteinosis. Report of Six Cases, Review of the Literature, and Formulation of a New Theory
Larson RK and Gordinier R
Ann Intern Med 1965;62:292-312
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=14259213

Pulmonary alveolar proteinosis
Rosen SH, Castleman B and Liebow AA
N Engl J Med 1958;258:1123-42
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=13552931
 
Epidemiology- The Study of the Determinants of Primary PAP

Pulmonary alveolar proteinosis: progress in the first 44 years
Seymour JF and Presneill JJ
Am J Respir Crit Care Med 2002;166:215-35
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=12119235

Pulmonary alveolar proteinosis: a review

deMello DE and Lin Z
Pediatr Pathol Mol Med 2001;20:413-32
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11552740

Pulmonary alveolar proteinosis in Israel: ethnic clustering
Ben-Dov I, Kishinevski Y, Roznman J, Soliman A, Bishara H, Zelligson E, Grief J, Mazar A, Perelman M, Vishnizer R and Weiler-Ravel D
Isr Med Assoc J 1999;1:75-8
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10731299

Pulmonary alveolar proteinosis: clinical features and outcomes
Goldstein LS, Kavuru MS, Curtis-McCarthy P, Christie HA, Farver C and Stoller JK
Chest 1998;114:1357-62
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=9824014

Lung transplantation for treatment of infants with surfactant protein B deficiency
Hamvas A, Nogee LM, Mallory GB, Jr., Spray TL, Huddleston CB, August A, Dehner LP, deMello DE, Moxley M, Nelson R, Cole FS and Colten HR
J Pediatr 1997;130:231-9
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=9042125

Secondary alveolar proteinosis is a reversible cause of respiratory failure in leukemic patients

Cordonnier C, Fleury-Feith J, Escudier E, Atassi K and Bernaudin JF
Am J Respir Crit Care Med 1994;149:788-94
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=8118651

Pulmonary alveolar phospholipoproteinosis: experience with 34 cases and a review

Prakash UB, Barham SS, Carpenter HA, Dines DE and Marsh HM
Mayo Clin Proc 1987;62:499-518
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=3553760

 
Clinical, Radiographic & Laboratory Manifestations of Primary PAP

Pulmonary alveolar proteinosis: progress in the first 44 years
Seymour JF and Presneill JJ
Am J Respir Crit Care Med 2002;166:215-35
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=12119235

BAL findings in a patient with pulmonary alveolar proteinosis successfully treated with GM-CSF

Schoch OD, Schanz U, Koller M, Nakata K, Seymour JF, Russi EW and Boehler A
Thorax 2002;57:277-80
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11867836

Therapeutic efficacy of granulocyte-macrophage colony-stimulating factor in patients with idiopathic acquired alveolar proteinosis
Seymour JF, Presneill JJ, Schoch OD, Downie GH, Moore PE, Doyle IR, Vincent JM, Nakata K, Kitamura T, Langton D, Pain MC and Dunn AR
Am J Respir Crit Care Med 2001;163:524-31
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11179134

Change in cytokeratin 19 fragment level according to the severity of pulmonary alveolar proteinosis

Minakata Y, Kida Y, Nakanishi H, Nishimoto T and Yukawa S
Intern Med 2001;40:1024-7
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11688827

Serological diagnosis of idiopathic pulmonary alveolar proteinosis
Kitamura T, Uchida K, Tanaka N, Tsuchiya T, Watanabe J, Yamada Y, Hanaoka K, Seymour JF, Schoch OD, Doyle I, Inoue Y, Sakatani M, Kudoh S, Azuma A, Nukiwa T, Tomita T, Katagiri M, Fujita A, Kurashima A, Kanegasaki S and Nakata K
Am J Respir Crit Care Med 2000;162:658-62
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10934102

Crazy-paving appearance at thin-section CT: spectrum of disease and pathologic findings

Johkoh T, Itoh H, Muller NL, Ichikado K, Nakamura H, Ikezoe J, Akira M and Nagareda T
Radiology 1999;211:155-60
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10189465

Elevated bronchoalveolar concentrations of MCP-1 in patients with pulmonary alveolar proteinosis

Iyonaga K, Suga M, Yamamoto T, Ichiyasu H, Miyakawa H and Ando M
Eur Respir J 1999;14:383-9
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10515418

Serum and bronchoalveolar fluid KL-6 levels in patients with pulmonary alveolar proteinosis
Takahashi T, Munakata M, Suzuki I and Kawakami Y
Am J Respir Crit Care Med 1998;158:1294-8
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=9769294

Surfactant proteins A and D: disease markers

Kuroki Y, Takahashi H, Chiba H and Akino T
Biochim Biophys Acta 1998;1408:334-45
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=9813383

Pulmonary alveolar proteinosis: clinical features and outcomes

Goldstein LS, Kavuru MS, Curtis-McCarthy P, Christie HA, Farver C and Stoller JK
Chest 1998;114:1357-62
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=9824014

Diagnosing pulmonary alveolar proteinosis. A review and an update

Wang BM, Stern EJ, Schmidt RA and Pierson DJ
Chest 1997;111:460-6
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=9041997

Pulmonary alveolar proteinosis: high-resolution CT, chest radiographic, and functional correlations

Lee KN, Levin DL, Webb WR, Chen D, Storto ML and Golden JA
Chest 1997;111:989-95
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=9106579

Six cases of pulmonary alveolar proteinosis: presentation of unusual associations
Garcia Rio F, Alvarez-Sala R, Caballero P, Prados C, Pino JM and Villamor J
Monaldi Archives for Chest Disease 1995;50:12-5
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=7742818&dopt=Abstract

Increased carcinoembryonic antigen concentrations in sera and bronchoalveolar lavage fluids of patients with pulmonary alveolar proteinosis
Fujishima T, Honda Y, Shijubo N, Takahashi H and Abe S
Respiration 1995;62:317-21
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=8552862

[Primary pulmonary alveolar proteinosis--clinical observation of 68 patients in Japan]
Asamoto H, Kitaichi M, Nishimura K, Itoh H and Izumi T
Nihon Kyobu Shikkan Gakkai Zasshi 1995;33:835-45
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=7474563

Pulmonary alveolar phospholipoproteinosis: experience with 34 cases and a review
Prakash UB, Barham SS, Carpenter HA, Dines DE and Marsh HM
Mayo Clin Proc 1987;62:499-518
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=3553760

The clinical and physiological effect of whole-lung lavage in pulmonary alveolar proteinosis: a ten-year experience
Selecky PA, Wasserman K, Benfield JR and Lippmann M
Ann Thorac Surg 1977;24:451-61
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=921378

Diagnosis and management of alveolar proteinosis: the role of electron microscopy

Costello JF, Moriarty DC, Branthwaite MA, Turner-Warwick M and Corrin B
Thorax 1975;30:121-32
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=1179307

Pulmonary alveolar proteinosis
Preger L
Radiology 1969;92:1291-5
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=5790638

Lactate dehydrogenase isoenzymes in alveolar proteinosis
Fountain FF, Jr.
Jama 1969;210:1283
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=5394655

Pulmonary alveolar proteinosis. A review of the findings and theories to date, with a digression on Pneumocystis carinii pneumonia
De Sanctis PN
Bmq 1962;13:19-35
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=13884520

Physiologic and clinical aspects of pulmonary alveolar proteinosis

Fraimow W, Cathcart RT and Taylor RC
Ann Intern Med 1960;52:1177-94
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=13824313

Pulmonary alveolar proteinosis

Rosen SH, Castleman B and Liebow AA
N Engl J Med 1958;258:1123-42
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=13552931

 
Natural History, Background, and Associations of PAP

Pulmonary alveolar proteinosis: progress in the first 44 years
Seymour JF and Presneill JJ
Am J Respir Crit Care Med 2002;166:215-35
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=12119235

Primary cerebellar nocardiosis and alveolar proteinosis

Oerlemans WG, Jansen EN, Prevo RL and Eijsvogel MM
Acta Neurol Scand 1998;97:138-41
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=9517865

Secondary alveolar proteinosis in cancer patients
Ladeb S, Fleury-Feith J, Escudier E, Tran Van Nhieu J, Bernaudin JF and Cordonnier C
Support Care Cancer 1996;4:420-6
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=8961472

Pulmonary alveolar proteinosis in a painter with elevated pulmonary concentrations of titanium
Keller CA, Frost A, Cagle PT and Abraham JL
Chest 1995;108:277-80
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=7606971

Secondary alveolar proteinosis is a reversible cause of respiratory failure in leukemic patients
Cordonnier C, Fleury-Feith J, Escudier E, Atassi K and Bernaudin JF
Am J Respir Crit Care Med 1994;149:788-94
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=8118651

Pulmonary alveolar phospholipoproteinosis: experience with 34 cases and a review

Prakash UB, Barham SS, Carpenter HA, Dines DE and Marsh HM
Mayo Clin Proc 1987;62:499-518
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=3553760

Pulmonary alveolar proteinosis complicated by cerebral abscess: report of a case

Walker DA and McMahon SM
J Am Osteopath Assoc 1986;86:447-50
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=3528083

Secondary pulmonary alveolar proteinosis occurring in two patients with acquired immune deficiency syndrome

Ruben FL and Talamo TS
Am J Med 1986;80:1187-90
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=3014876

Alveolar proteinosis as a consequence of immunosuppression. A hypothesis based on clinical and pathologic observations

Bedrossian CW, Luna MA, Conklin RH and Miller WC
Hum Pathol 1980;11:527-35
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=7429503

Pulmonary alveolar proteinosis and Nocardia brain abscess. Report of a case

Supena R, Karlin D, Strate R and Cramer PG
Arch Neurol 1974;30:266-8
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=4204855

Acute silico-proteinosis. A new pathologic variant of acute silicosis in sandblasters, characterized by histologic features resembling alveolar proteinosis

Buechner HA and Ansari A
Dis Chest 1969;55:274-8
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=5775743

The Association of Nocardiosis and Pulmonary Alveolar Proteinosis. A Case Study

Andriole VT, Ballas M and Wilson GL
Ann Intern Med 1964;60:266-75
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=14114445

Pulmonary Alveolar Proteinosis with Hematologic Disorders

Doyle AP, Balcerzak SP, Wells CL and Crittenden JO
Arch Intern Med 1963;112:940-6
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=14065004

Pulmonary alveolar proteinosis

Rosen SH, Castleman B and Liebow AA
N Engl J Med 1958;258:1123-42
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=13552931

 
Surfactant Homeostasis in PAP

Hydrophobic surfactant proteins in lung function and disease
Whitsett JA and Weaver TE
N Engl J Med 2002;347:2141-8
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=12501227

Gm-CSF regulates pulmonary surfactant homeostasis and alveolar macrophage-mediated innate host defense
Trapnell BC and Whitsett JA
Annu Rev Physiol 2002;64:775-802
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11826288

A mutation in the surfactant protein C gene associated with familial interstitial lung disease

Nogee LM, Dunbar AE, 3rd, Wert SE, Askin F, Hamvas A and Whitsett JA
N Engl J Med 2001;344:573-9
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11207353

Surfactant metabolism in SP-D gene-targeted mice

Ikegami M, Whitsett JA, Jobe A, Ross G, Fisher J and Korfhagen T
Am J Physiol Lung Cell Mol Physiol 2000;279:L468-76
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10956621

Collectins and pulmonary host defense
Crouch EC
Am J Respir Cell Mol Biol 1998;19:177-201
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=9698590

A mutation in the surfactant protein B gene responsible for fatal neonatal respiratory disease in multiple kindreds
Nogee LM, Garnier G, Dietz HC, Singer L, Murphy AM, deMello DE and Colten HR
J Clin Invest 1994;93:1860-3
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=8163685

Aggregation and opsonization of type A but not type B Hemophilus influenzae by surfactant protein A
McNeely TB and Coonrod JD
Am J Respir Cell Mol Biol 1994;11:114-22
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=8018334

Brief report: deficiency of pulmonary surfactant protein B in congenital alveolar proteinosis
Nogee LM, de Mello DE, Dehner LP and Colten HR
N Engl J Med 1993;328:406-10
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=8421459

Surfactant protein-A concentration in bronchoalveolar lavage fluids of patients with pulmonary alveolar proteinosis

Honda Y, Takahashi H, Shijubo N, Kuroki Y and Akino T
Chest 1993;103:496-9
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=8432143

Function and regulation of expression of pulmonary surfactant-associated proteins
Weaver TE and Whitsett JA
Biochem J 1991;273(Pt 2):249-64
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=1991023

Pulmonary alveolar proteinosis. Staining for surfactant apoprotein in alveolar proteinosis and in conditions simulating it

Singh G, Katyal SL, Bedrossian CW and Rogers RM
Chest 1983;83:82-6
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=6336692

Diagnosis and management of alveolar proteinosis: the role of electron microscopy
Costello JF, Moriarty DC, Branthwaite MA, Turner-Warwick M and Corrin B
Thorax 1975;30:121-32
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=1179307

Pulmonary alveolar proteinosis

McClenahan JB
Arch Intern Med 1974;133:284-7
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=4405909

Pulmonary alveolar proteinosis

Davidson JM and Macleod WM
Br J Dis Chest 1969;63:13-28
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=4886263

Pulmonary alveolar proteinosis. Nature and origin of alveolar lipid

Ramirez J and Harlan WR, Jr.
Am J Med 1968;45:502-12
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=5678094

Pulmonary Alveolar Proteinosis. Report of Six Cases, Review of the Literature, and Formulation of a New Theory

Larson RK and Gordinier R
Ann Intern Med 1965;62:292-312
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Lipoprotein composition of the film lining the lung

Pattle RE and Thomas LC
Nature 1961;189:844
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Composition of surface-active material isolated from beef lung
Klaus MH, Clements JA and Havel RJ
Proc Natl Acad Sci U S A 1961;47:1858-9
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The surface membrane of pulmonary alveolar walls

Chase WH
Exp Cell Res 1959;18:15-28
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Pulmonary alveolar proteinosis

Rosen SH, Castleman B and Liebow AA
N Engl J Med 1958;258:1123-42
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Properties, function and origin of the alveolar lining layer

Pattle RE
Nature 1955;175:1125-6
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Mouse Models of PAP

GM-CSF regulates pulmonary surfactant homeostasis and alveolar macrophage-mediated innate host defense

Trapnell BC and Whitsett JA
Annu Rev Physiol 2002;64:775-802
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GM-CSF, via PU.1, regulates alveolar macrophage Fcgamma R-mediated phagocytosis and the IL-18/IFN-gamma -mediated molecular connection between innate and adaptive immunity in the lung

Berclaz PY, Shibata Y, Whitsett JA and Trapnell BC
Blood 2002;100:4193-200
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Endocytic internalization of adenovirus, nonspecific phagocytosis, and cytoskeletal organization are coordinately regulated in alveolar macrophages by GM-CSF and PU.1

Berclaz PY, Zsengeller Z, Shibata Y, Otake K, Strasbaugh S, Whitsett JA and Trapnell BC
J Immunol 2002;169:6332-42
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GM-CSF regulates protein and lipid catabolism by alveolar macrophages

Yoshida M, Ikegami M, Reed JA, Chroneos ZC and Whitsett JA
Am J Physiol Lung Cell Mol Physiol 2001;280:L379-86
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11159019

GM-CSF regulates alveolar macrophage differentiation and innate immunity in the lung through PU.1

Shibata Y, Berclaz PY, Chroneos ZC, Yoshida M, Whitsett JA and Trapnell BC
Immunity 2001;15:557-67
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11672538

Impaired functional activity of alveolar macrophages from GM-CSF-deficient mice

Paine R, 3rd, Morris SB, Jin H, Wilcoxen SE, Phare SM, Moore BB, Coffey MJ and Toews GB
Am J Physiol Lung Cell Mol Physiol 2001;281:L1210-8
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11597913

Granulocyte-macrophage colony-stimulating factor in the innate immune response to Pneumocystis carinii pneumonia in mice
Paine R, 3rd, Preston AM, Wilcoxen S, Jin H, Siu BB, Morris SB, Reed JA, Ross G, Whitsett JA and Beck JM
J Immunol 2000;164:2602-9
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10679099

Regulation of B lymphocyte and macrophage development by graded expression of PU.1

DeKoter RP and Singh H
Science 2000;288:1439-41
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Aerosolized GM-CSF ameliorates pulmonary alveolar proteinosis in GM-CSF-deficient mice

Reed JA, Ikegami M, Cianciolo ER, Lu W, Cho PS, Hull W, Jobe AH and Whitsett JA
Am J Physiol 1999;276:L556-63
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The key role of PU.1/SPI-1 in B cells, myeloid cells and macrophages

Lloberas J, Soler C and Celada A
Immunol Today 1999;20:184-9
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10203717

GM-CSF-deficient mice are susceptible to pulmonary group B streptococcal infection

LeVine AM, Reed JA, Kurak KE, Cianciolo E and Whitsett JA
J Clin Invest 1999;103:563-9
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Adenovirus-mediated granulocyte-macrophage colony-stimulating factor improves lung pathology of pulmonary alveolar proteinosis in granulocyte-macrophage colony-stimulating factor-deficient mice

Zsengeller ZK, Reed JA, Bachurski CJ, LeVine AM, Forry-Schaudies S, Hirsch R and Whitsett JA
Hum Gene Ther 1998;9:2101-9
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Emerging applications of recombinant human granulocyte-macrophage colony-stimulating factor

Armitage JO
Blood 1998;92:4491-508
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Myeloid development is selectively disrupted in PU.1 null mice

Anderson KL, Smith KA, Conners K, McKercher SR, Maki RA and Torbett BE
Blood 1998;91:3702-10
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=9573007

GM-CSF enhances lung growth and causes alveolar type II epithelial cell hyperplasia in transgenic mice

Huffman Reed JA, Rice WR, Zsengeller ZK, Wert SE, Dranoff G and Whitsett JA
Am J Physiol 1997;273:L715-25
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Granulocyte-macrophage colony-stimulating factor and the immune system

Tarr PE
Med Oncol 1996;13:133-40
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Targeted disruption of the PU.1 gene results in multiple hematopoietic abnormalities

McKercher SR, Torbett BE, Anderson KL, Henkel GW, Vestal DJ, Baribault H, Klemsz M, Feeney AJ, Wu GE, Paige CJ and Maki RA
Embo J 1996;15:5647-58
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Surfactant metabolism in transgenic mice after granulocyte macrophage-colony stimulating factor ablation

Ikegami M, Ueda T, Hull W, Whitsett JA, Mulligan RC, Dranoff G and Jobe AH
Am J Physiol 1996;270:L650-8
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Pulmonary epithelial cell expression of GM-CSF corrects the alveolar proteinosis in GM-CSF-deficient mice

Huffman JA, Hull WM, Dranoff G, Mulligan RC and Whitsett JA
J Clin Invest 1996;97:649-55
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The transcription factor PU.1 is involved in macrophage proliferation

Celada A, Borras FE, Soler C, Lloberas J, Klemsz M, van Beveren C, McKercher S and Maki RA
J Exp Med 1996;184:61-9
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Hematopoietic and lung abnormalities in mice with a null mutation of the common beta subunit of the receptors for granulocyte-macrophage colony-stimulating factor and interleukins 3 and 5

Robb L, Drinkwater CC, Metcalf D, Li R, Kontgen F, Nicola NA and Begley CG
Proc Natl Acad Sci U S A 1995;92:9565-9
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Mice deficient for the IL-3/GM-CSF/IL-5 beta c receptor exhibit lung pathology and impaired immune response, while beta IL3 receptor-deficient mice are normal

Nishinakamura R, Nakayama N, Hirabayashi Y, Inoue T, Aud D, McNeil T, Azuma S, Yoshida S, Toyoda Y, Arai K and et al.
Immunity 1995;2:211-22
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Granulocyte/macrophage colony-stimulating factor-deficient mice show no major perturbation of hematopoiesis but develop a characteristic pulmonary pathology

Stanley E, Lieschke GJ, Grail D, Metcalf D, Hodgson G, Gall JA, Maher DW, Cebon J, Sinickas V and Dunn AR
Proc Natl Acad Sci U S A 1994;91:5592-6
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Requirement of transcription factor PU.1 in the development of multiple hematopoietic lineages

Scott EW, Simon MC, Anastasi J and Singh H
Science 1994;265:1573-7
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Involvement of granulocyte-macrophage colony-stimulating factor in pulmonary homeostasis

Dranoff G, Crawford AD, Sadelain M, Ream B, Rashid A, Bronson RT, Dickersin GR, Bachurski CJ, Mark EL, Whitsett JA and et al.
Science 1994;264:713-6
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Hematopoietic lineage- and stage-restricted expression of the ETS oncogene family member PU.1

Hromas R, Orazi A, Neiman RS, Maki R, Van Beveran C, Moore J and Klemsz M
Blood 1993;82:2998-3004
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A model for the interaction of the GM-CSF, IL-3 and IL-5 receptors with their ligands

Goodall GJ, Bagley CJ, Vadas MA and Lopez AF
Growth Factors 1993;8:87-97
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Granulocyte-macrophage colony-stimulating factor: pleiotropic cytokine with potential clinical usefulness

Ruef C and Coleman DL
Rev Infect Dis 1990;12:41-62
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The macrophage and B cell-specific transcription factor PU.1 is related to the ets oncogene

Klemsz MJ, McKercher SR, Celada A, Van Beveren C and Maki RA
Cell 1990;61:113-24
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=2180582

Molecular cloning of a second subunit of the receptor for human granulocyte-macrophage colony-stimulating factor (GM-CSF): reconstitution of a high-affinity GM-CSF receptor

Hayashida K, Kitamura T, Gorman DM, Arai K, Yokota T and Miyajima A
Proc Natl Acad Sci U S A 1990;87:9655-9
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Expression cloning of a receptor for human granulocyte-macrophage colony-stimulating factor

Gearing DP, King JA, Gough NM and Nicola NA
Embo J 1989;8:3667-76
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Structure of the chromosomal gene for granulocyte-macrophage colony stimulating factor: comparison of the mouse and human genes

Miyatake S, Otsuka T, Yokota T, Lee F and Arai K
Embo J 1985;4:2561-8
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Purification and properties of colony-stimulating factor from mouse lung-conditioned medium

Burgess AW, Camakaris J and Metcalf D
J Biol Chem 1977;252:1998-2003
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Pathogenesis of Primary (Idiopathic) PAP

High-affinity autoantibodies specifically eliminate granulocyte-macrophage colony-stimulating factor activity in the lungs of patients with idiopathic pulmonary alveolar proteinosis

Uchida K, Nakata K, Trapnell BC, Terakawa T, Hamano E, Mikami A, Matsushita I, Seymour JF, Oh-Eda M, Ishige I, Eishi Y, Kitamura T, Yamada Y, Hanaoka K and Keicho N
Blood 2004;103:1089-98
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=14512323

Autoantibodies against granulocyte macrophage colony-stimulating factor are diagnostic for pulmonary alveolar proteinosis

Bonfield TL, Russell D, Burgess S, Malur A, Kavuru MS and Thomassen MJ
Am J Respir Cell Mol Biol 2002;27:481-6
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=12356582

GM-CSF regulates alveolar macrophage differentiation and innate immunity in the lung through PU.1

Shibata Y, Berclaz PY, Chroneos ZC, Yoshida M, Whitsett JA and Trapnell BC
Immunity 2001;15:557-67
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11672538

Therapeutic efficacy of granulocyte-macrophage colony-stimulating factor in patients with idiopathic acquired alveolar proteinosis

Seymour JF, Presneill JJ, Schoch OD, Downie GH, Moore PE, Doyle IR, Vincent JM, Nakata K, Kitamura T, Langton D, Pain MC and Dunn AR
Am J Respir Crit Care Med 2001;163:524-31
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11179134

Impaired functional activity of alveolar macrophages from GM-CSF-deficient mice

Paine R, 3rd, Morris SB, Jin H, Wilcoxen SE, Phare SM, Moore BB, Coffey MJ and Toews GB
Am J Physiol Lung Cell Mol Physiol 2001;281:L1210-8
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11597913

Serological diagnosis of idiopathic pulmonary alveolar proteinosis

Kitamura T, Uchida K, Tanaka N, Tsuchiya T, Watanabe J, Yamada Y, Hanaoka K, Seymour JF, Schoch OD, Doyle I, Inoue Y, Sakatani M, Kudoh S, Azuma A, Nukiwa T, Tomita T, Katagiri M, Fujita A, Kurashima A, Kanegasaki S and Nakata K
Am J Respir Crit Care Med 2000;162:658-62
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10934102

Exogenous granulocyte-macrophage colony-stimulating factor administration for pulmonary alveolar proteinosis

Kavuru MS, Sullivan EJ, Piccin R, Thomassen MJ and Stoller JK
Am J Respir Crit Care Med 2000;161:1143-8
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10764303

Detection of granulocyte-macrophage colony-stimulating factor in patients with pulmonary alveolar proteinosis

Carraway MS, Ghio AJ, Carter JD and Piantadosi CA
Am J Respir Crit Care Med 2000;161:1294-9
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10764326

GM-CSF and GM-CSF beta c receptor in adult patients with pulmonary alveolar proteinosis

Bewig B, Wang XD, Kirsten D, Dalhoff K and Schafer H
Eur Respir J 2000;15:350-7
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10706504

Lungs of patients with idiopathic pulmonary alveolar proteinosis express a factor which neutralizes granulocyte-macrophage colony stimulating factor

Tanaka N, Watanabe J, Kitamura T, Yamada Y, Kanegasaki S and Nakata K
FEBS Lett 1999;442:246-50
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=9929010

Idiopathic pulmonary alveolar proteinosis as an autoimmune disease with neutralizing antibody against granulocyte/macrophage colony-stimulating factor

Kitamura T, Tanaka N, Watanabe J, Uchida, Kanegasaki S, Yamada Y and Nakata K
J Exp Med 1999;190:875-80
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10499925

Elevated bronchoalveolar concentrations of MCP-1 in patients with pulmonary alveolar proteinosis

Iyonaga K, Suga M, Yamamoto T, Ichiyasu H, Miyakawa H and Ando M
Eur Respir J 1999;14:383-9
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Defective expression of granulocyte-macrophage colony-stimulating factor/interleukin-3/interleukin-5 receptor common beta chain in children with acute myeloid leukemia associated with respiratory failure

Dirksen U, Hattenhorst U, Schneider P, Schroten H, Gobel U, Bocking A, Muller KM, Murray R and Burdach S
Blood 1998;92:1097-103
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Human pulmonary alveolar proteinosis associated with a defect in GM-CSF/IL-3/IL-5 receptor common beta chain expression

Dirksen U, Nishinakamura R, Groneck P, Hattenhorst U, Nogee L, Murray R and Burdach
J Clin Invest 1997;100:2211-7
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Efficacy of granulocyte-macrophage colony-stimulating factor in acquired alveolar proteinosis

Seymour JF, Dunn AR, Vincent JM, Presneill JJ and Pain MC
N Engl J Med 1996;335:1924-5
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=8965913

Involvement of granulocyte-macrophage colony-stimulating factor in pulmonary homeostasis

Dranoff G, Crawford AD, Sadelain M, Ream B, Rashid A, Bronson RT, Dickersin GR, Bachurski CJ, Mark EL, Whitsett JA and et al.
Science 1994;264:713-6
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=8171324

A macrophage-suppressing 40-kD protein in a case of pulmonary alveolar proteinosis

Muller-Quernheim J, Schopf RE, Benes P, Schulz V and Ferlinz R
Klin Wochenschr 1987;65:893-7
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Pulmonary alveolar proteinosis. Further evaluation of abnormal alveolar macrophages

Gonzalez-Rothi RJ and Harris JO
Chest 1986;90:656-61
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Macrophage function in pulmonary alveolar proteinosis

Nugent KM and Pesanti EL
Am Rev Respir Dis 1983;127:780-1
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The immunoinhibitory activities of the lung lavage materials and sera from patients with pulmonary alveolar proteinosis (PAP)

Stratton JA, Sieger L and Wasserman K
J Clin Lab Immunol 1981;5:81-6
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Pulmonary alveolar proteinosis: abnormal in vitro function of alveolar macrophages

Harris JO
Chest 1979;76:156-9
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Alveolar proteinosis and the overfed macrophage

Golde DW
Chest 1979;76:119-20
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Defective lung macrophages in pulmonary alveolar proteinosis
Golde DW, Territo M, Finley TN and Cline MJ
Ann Intern Med 1976;85:304-9
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Pulmonary Alveolar Proteinosis. Report of Six Cases, Review of the Literature, and Formulation of a New Theory

Larson RK and Gordinier R
Ann Intern Med 1965;62:292-312
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Pulmonary alveolar proteinosis. A review of the findings and theories to date, with a digression on Pneumocystis carinii pneumonia
De Sanctis PN
Bmq 1962;13:19-35
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=13884520

 
Pathogenesis of Primary (Idiopathic) PAP

High-affinity autoantibodies specifically eliminate granulocyte-macrophage colony-stimulating factor activity in the lungs of patients with idiopathic pulmonary alveolar proteinosis

Uchida K, Nakata K, Trapnell BC, Terakawa T, Hamano E, Mikami A, Matsushita I, Seymour JF, Oh-Eda M, Ishige I, Eishi Y, Kitamura T, Yamada Y, Hanaoka K and Keicho N
Blood 2004;103:1089-98
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=14512323

Autoantibodies against granulocyte macrophage colony-stimulating factor are diagnostic for pulmonary alveolar proteinosis

Bonfield TL, Russell D, Burgess S, Malur A, Kavuru MS and Thomassen MJ
Am J Respir Cell Mol Biol 2002;27:481-6
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=12356582

GM-CSF regulates alveolar macrophage differentiation and innate immunity in the lung through PU.1

Shibata Y, Berclaz PY, Chroneos ZC, Yoshida M, Whitsett JA and Trapnell BC
Immunity 2001;15:557-67
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11672538

Therapeutic efficacy of granulocyte-macrophage colony-stimulating factor in patients with idiopathic acquired alveolar proteinosis

Seymour JF, Presneill JJ, Schoch OD, Downie GH, Moore PE, Doyle IR, Vincent JM, Nakata K, Kitamura T, Langton D, Pain MC and Dunn AR
Am J Respir Crit Care Med 2001;163:524-31
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11179134

Impaired functional activity of alveolar macrophages from GM-CSF-deficient mice

Paine R, 3rd, Morris SB, Jin H, Wilcoxen SE, Phare SM, Moore BB, Coffey MJ and Toews GB
Am J Physiol Lung Cell Mol Physiol 2001;281:L1210-8
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11597913

Serological diagnosis of idiopathic pulmonary alveolar proteinosis

Kitamura T, Uchida K, Tanaka N, Tsuchiya T, Watanabe J, Yamada Y, Hanaoka K, Seymour JF, Schoch OD, Doyle I, Inoue Y, Sakatani M, Kudoh S, Azuma A, Nukiwa T, Tomita T, Katagiri M, Fujita A, Kurashima A, Kanegasaki S and Nakata K
Am J Respir Crit Care Med 2000;162:658-62
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10934102

Exogenous granulocyte-macrophage colony-stimulating factor administration for pulmonary alveolar proteinosis

Kavuru MS, Sullivan EJ, Piccin R, Thomassen MJ and Stoller JK
Am J Respir Crit Care Med 2000;161:1143-8
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10764303

Detection of granulocyte-macrophage colony-stimulating factor in patients with pulmonary alveolar proteinosis

Carraway MS, Ghio AJ, Carter JD and Piantadosi CA
Am J Respir Crit Care Med 2000;161:1294-9
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10764326

GM-CSF and GM-CSF beta c receptor in adult patients with pulmonary alveolar proteinosis

Bewig B, Wang XD, Kirsten D, Dalhoff K and Schafer H
Eur Respir J 2000;15:350-7
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10706504

Lungs of patients with idiopathic pulmonary alveolar proteinosis express a factor which neutralizes granulocyte-macrophage colony
stimulating factor
Tanaka N, Watanabe J, Kitamura T, Yamada Y, Kanegasaki S and Nakata K
FEBS Lett 1999;442:246-50
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=9929010

Idiopathic pulmonary alveolar proteinosis as an autoimmune disease with neutralizing antibody against granulocyte/macrophage
colony-stimulating factor
Kitamura T, Tanaka N, Watanabe J, Uchida, Kanegasaki S, Yamada Y and Nakata K
J Exp Med 1999;190:875-80
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Elevated bronchoalveolar concentrations of MCP-1 in patients with pulmonary alveolar proteinosis

Iyonaga K, Suga M, Yamamoto T, Ichiyasu H, Miyakawa H and Ando M
Eur Respir J 1999;14:383-9
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Defective expression of granulocyte-macrophage colony-stimulating factor/interleukin-3/interleukin-5 receptor common beta chain in children with acute myeloid leukemia associated with respiratory failure

Dirksen U, Hattenhorst U, Schneider P, Schroten H, Gobel U, Bocking A, Muller KM, Murray R and Burdach S
Blood 1998;92:1097-103
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Human pulmonary alveolar proteinosis associated with a defect in GM-CSF/IL-3/IL-5 receptor common beta chain expression

Dirksen U, Nishinakamura R, Groneck P, Hattenhorst U, Nogee L, Murray R and Burdach S
J Clin Invest 1997;100:2211-7
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Efficacy of granulocyte-macrophage colony-stimulating factor in acquired alveolar proteinosis

Seymour JF, Dunn AR, Vincent JM, Presneill JJ and Pain MC
N Engl J Med 1996;335:1924-5
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Involvement of granulocyte-macrophage colony-stimulating factor in pulmonary homeostasis

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A macrophage-suppressing 40-kD protein in a case of pulmonary alveolar proteinosis

Muller-Quernheim J, Schopf RE, Benes P, Schulz V and Ferlinz R
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Macrophage function in pulmonary alveolar proteinosis

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Am Rev Respir Dis 1983;127:780-1
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The immunoinhibitory activities of the lung lavage materials and sera from patients with pulmonary alveolar proteinosis (PAP)

Stratton JA, Sieger L and Wasserman K
J Clin Lab Immunol 1981;5:81-6
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Pulmonary alveolar proteinosis: abnormal in vitro function of alveolar macrophages

Harris JO
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Alveolar proteinosis and the overfed macrophage

Golde DW
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Defective lung macrophages in pulmonary alveolar proteinosis

Golde DW, Territo M, Finley TN and Cline MJ
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Pulmonary Alveolar Proteinosis. Report of Six Cases, Review of the Literature, and Formulation of a New Theory

Larson RK and Gordinier R
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Pulmonary alveolar proteinosis. A review of the findings and theories to date, with a digression on Pneumocystis carinii pneumonia

De Sanctis PN
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Therapeutic Approaches to Treat Primary PAP

Pulmonary alveolar proteinosis: progress in the first 44 years

Seymour JF and Presneill JJ
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BAL findings in a patient with pulmonary alveolar proteinosis successfully treated with GM-CSF

Schoch OD, Schanz U, Koller M, Nakata K, Seymour JF, Russi EW and Boehler A
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Pulmonary alveolar proteinosis: treatment by bronchofiberscopic lobar lavage

Cheng SL, Chang HT, Lau HP, Lee LN and Yang PC
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Therapeutic efficacy of granulocyte-macrophage colony-stimulating factor in patients with idiopathic acquired alveolar proteinosis

Seymour JF, Presneill JJ, Schoch OD, Downie GH, Moore PE, Doyle IR, Vincent JM, Nakata K, Kitamura T, Langton D, Pain MC and Dunn AR
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Pulmonary alveolar proteinosis: a review

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Exogenous granulocyte-macrophage colony-stimulating factor administration for pulmonary alveolar proteinosis

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Lung transplantation for treatment of infants with surfactant protein B deficiency

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Secondary alveolar proteinosis in cancer patients

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Improvement in alveolar macrophage migration after therapeutic whole lung lavage in pulmonary alveolar proteinosis
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[Alveolar proteinosis: restoration of the function of the alveolar macrophages after therapeutic lavage]

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Pulmonary alveolar proteinosis: prospective clinical experience in 23 patients for 15 years
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Pulmonary Alveolar Proteinosis: A New Technique and Rationale for Treatment

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Congenital Pulmonary Alveolar Proteinosis

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The genetics of neonatal respiratory disease

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[Autoantibody against granulocyte-macrophage colony-stimulating factor and other serum markers in pulmonary alveolar proteinosis]

Xu KF, Chen Y, Guo ZJ and Zhu YJ
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Mutation of SFTPC in infantile pulmonary alveolar proteinosis with or without fibrosing lung disease

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Rare infiltrative lung diseases: a challenge for clinicians

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Congenital alveolar proteinosis

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Inherited interstitial lung disease

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Pulmonary pathology

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[Classification of pulmonary alveolar proteinosis in newborns, infants, and children]

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Analysis of 40 sporadic or familial neonatal and pediatric cases with severe unexplained respiratory distress: relationship to SFTPB

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[Pulmonary alveolar proteinosis]

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Pulmonary alveolar proteinosis: progress in the first 44 years

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[Pulmonary alveolar proteinosis and its treatment with total pulmonary lavage in the Czech Republic]

Jansa P, Stritesky M and Homolka J
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A mutation in the surfactant protein C gene associated with familial interstitial lung disease

Nogee LM, Dunbar AE, 3rd, Wert SE, Askin F, Hamvas A and Whitsett JA
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[Clinical, biological and genetic heterogeneity of the inborn errors of pulmonary surfactant metabolism: SP-B deficiency and alveolar proteinosis]

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Congenital surfactant protein B deficiency--emphasis on imaging

Newman B, Kuhn JP, Kramer SS and Carcillo JA
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Focal congenital alveolar proteinosis associated with abnormal surfactant protein B messenger RNA

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Pulmonary alveolar proteinosis: a review

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Pediatr Pathol Mol Med 2001;20:413-32
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[Constitutional deficiency of pulmonary surfactant protein B: clinical presentation, histologic and molecular diagnosis]

Tredano M, Cneude F, Denamur E, Truffert P, Capron F, Manouvrier S, Feldmann D, Couderc R, Elion J and Lacaze-Masmonteil T
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Aberrant SP-B mRNA in lung tissue of patients with congenital alveolar proteinosis (CAP)

Lin Z, deMello DE, Batanian JR, Khammash HM, DiAngelo S, Luo J and Floros J
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Computed tomography of diffuse interstitial lung disease in children

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Serological diagnosis of idiopathic pulmonary alveolar proteinosis

Kitamura T, Uchida K, Tanaka N, Tsuchiya T, Watanabe J, Yamada Y, Hanaoka K, Seymour JF, Schoch OD, Doyle I, Inoue Y, Sakatani M, Kudoh S, Azuma A, Nukiwa T, Tomita T, Katagiri M, Fujita A, Kurashima A, Kanegasaki S and Nakata K
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Diagnostic accuracy of thin-section CT and chest radiography of pediatric interstitial lung disease

Copley SJ, Coren M, Nicholson AG, Rubens MB, Bush A and Hansell DM
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Normal lung function in subjects heterozygous for surfactant protein-B deficiency

Yusen RD, Cohen AH and Hamvas A
Am J Respir Crit Care Med 1999;159:411-4
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Surfactant protein B deficiency: clinical, histological and molecular evaluation

Williams GD, Christodoulou J, Stack J, Symons P, Wert SE, Murrell MJ and Nogee LM
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Congenital alveolar proteinosis caused by a novel mutation of the surfactant protein B gene and misalignment of lung vessels in consanguineous kindred infants

Wallot M, Wagenvoort C, deMello D, Muller KM, Floros J and Roll C
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Pulmonary alveolar proteinosis in infants

Sakai Y, Abo W, Yoshimura H, Sano H, Kuroki Y, Satoh M and Kaimori M
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Imaging of medical disease of the newborn lung

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An alternatively spliced surfactant protein B mRNA in normal human lung: disease implication

Lin Z, Wang G, Demello DE and Floros J
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Idiopathic pulmonary alveolar proteinosis as an autoimmune disease with neutralizing antibody against granulocyte/macrophage colony-stimulating factor

Kitamura T, Tanaka N, Watanabe J, Uchida, Kanegasaki S, Yamada Y and Nakata K
J Exp Med 1999;190:875-80
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Molecular confirmation retrospectively of a diagnosis of surfactant protein B deficiency

Gattas M, Harvey P and Adsett D
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Fanconi anemia and beta c deficiency-associated pulmonary alveolar proteinosis as two hereditary diseases of childhood which are potentially curable by stem cell gene therapy but require different therapeutic approaches

Dirksen U, Moritz T, Burdach S, Flasshove M and Hanenberg H
Klin Padiatr 1999;211:329-35
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Prenatal diagnosis of congenital alveolar proteinosis (surfactant protein B deficiency)

Stuhrmann M, Bohnhorst B, Peters U, Bohle RM, Poets CF and Schmidtke J
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Attenuated hematopoietic response to granulocyte-macrophage colony-stimulating factor in patients with acquired pulmonary alveolar proteinosis

Seymour JF, Begley CG, Dirksen U, Presneill JJ, Nicola NA, Moore PE, Schoch OD, van Asperen P, Roth B, Burdach S and Dunn AR
Blood 1998;92:2657-67
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An SP-B gene mutation responsible for SP-B deficiency in fatal congenital alveolar proteinosis: evidence for a mutation hotspot in exon a

Lin Z, deMello DE, Wallot M and Floros J
Mol Genet Metab 1998;64:25-35
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Surfactant proteins: molecular genetics of neonatal pulmonary diseases

Floros J and Kala P
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Pulmonary alveolar proteinosis in an HIV-infected child

Nachajon RV, Rutstein RM, Rudy BJ and Collins MH
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Inherited surfactant protein-B deficiency

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Surfactant protein B deficiency: insights into inherited disorders of lung cell metabolism

Hamvas A
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Human pulmonary alveolar proteinosis associated with a defect in GM-CSF/IL-3/IL-5 receptor common beta chain expression

Dirksen U, Nishinakamura R, Groneck P, Hattenhorst U, Nogee L, Murray R and Burdach S
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Congenital alveolar proteinosis in the Netherlands: a report of five cases with immunohistochemical and genetic studies on surfactant apoproteins

de la Fuente AA, Voorhout WF and deMello DE
Pediatr Pathol Lab Med 1997;17:221-31
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The pulmonary alveolar proteinosis in granulocyte macrophage colony-stimulating factor/interleukins 3/5 beta c receptor-deficient mice is reversed by bone marrow transplantation

Nishinakamura R, Wiler R, Dirksen U, Morikawa Y, Arai K, Miyajima A, Burdach S and Murray R
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[Neonatal pulmonary alveolar proteinosis and SP-B deficiency: pulmonary surfactant at the time of molecular pathology]

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In vitro and in vivo transfer and expression of human surfactant SP-A- and SP-B-associated protein cDNAs mediated by replication-deficient, recombinant adenoviral vectors

Korst RJ, Bewig B and Crystal RG
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Molecular and cellular pathobiology of pulmonary surfactant protein B deficiency

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A mutation in the surfactant protein B gene responsible for fatal neonatal respiratory disease in multiple kindreds

Nogee LM, Garnier G, Dietz HC, Singer L, Murphy AM, deMello DE and Colten HR
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[The pulmonary surfactant factor. Current knowledge, research trends and use in clinical practice]

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Surfactant protein B deficiency: antenatal diagnosis and prospective treatment with surfactant replacement

Hamvas A, Cole FS, deMello DE, Moxley M, Whitsett JA, Colten HR and Nogee LM
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Molecular and phenotypic variability in the congenital alveolar proteinosis syndrome associated with inherited surfactant protein B deficiency

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Ultrastructure of lung in surfactant protein B deficiency

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Treatment of congenital alveolar proteinosis

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J Pediatr 1993;123:495-6
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=8355136

Brief report: deficiency of pulmonary surfactant protein B in congenital alveolar proteinosis

Nogee LM, de Mello DE, Dehner LP and Colten HR
N Engl J Med 1993;328:406-10
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=8421459

Surfactant protein B deficiency: radiographic manifestations

Herman TE, Nogee LM, McAlister WH and Dehner LP
Pediatr Radiol 1993;23:373-5
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=8233692

Congenital pulmonary alveolar proteinosis: failure of treatment with extracorporeal life support

Moulton SL, Krous HF, Merritt TA, Odell RM, Gangitano E and Cornish JD
J Pediatr 1992;120:297-302
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=1341413

Glutaric aciduria type II: autopsy study of a case with electron-transferring flavoprotein dehydrogenase deficiency
Kamiya M, Eimoto T, Kishimoto H, Tsudzuki T, Morishita H, Wada Y, Wakabayashi T, Hashimoto T, Goodman SI, Frerman FE and et al.
Pediatr Pathol 1990;10:1007-19
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=2082330

Pulmonary alveolar proteinosis in four siblings
Teja K, Cooper PH, Squires JE and Schnatterly P
N Engl J Med 1981;305:1390-2
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=7300857

 
Published Literature on SECONDARY PAP

Increased NO production in lysinuric protein intolerance

Mannucci L, Emma F, Markert M, Bachmann C, Boulat O, Carrozzo R, Rizzoni G and Dionisi-Vici C
J Inherit Metab Dis 2005;28:123-9
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=15877200

Pulmonary alveolar proteinosis undergoing whole lung lavage in south Taiwan--a case report

Tsai TC, Hung CC, Chen MS, He CC, Kuo MC and Hsieh SW
Acta Anaesthesiol Taiwan 2004;42:49-54
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=15148695

Imaging of interstitial lung disease

Pipavath S and Godwin JD
Clin Chest Med 2004;25:455-65, v-vi
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=15331186

[Alveolar proteinosis after professional exposure to cotton and linen dust, successfully treated with whole lung lavage--a case report]

Kosacka M, Dyla T and Jankowska R
Pneumonol Alergol Pol 2004;72:217-20
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=15757263

[Classification of pulmonary alveolar proteinosis in newborns, infants, and children]

Brasch F and Muller KM
Pathologe 2004;25:299-309
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=15241624

Effects of various forms of surfactant protein C on tidal volume in ventilated immature newborn rabbits

Tashiro K, Ohta K, Cui X, Nishizuka K, Yamamoto K, Konzaki T, Kobayashi T and Suzuki Y
J Appl Physiol 2003;94:1519-26
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=12433871

Secondary pulmonary alveolar proteinosis associated with myelodysplastic syndrome

Ohnishi T, Yamada G, Shijubo N, Takagi-Takahashi Y, Itoh T, Takahashi H, Satoh M, Koba H, Nakata K and Abe S
Intern Med 2003;42:187-90
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=12636240

Molecular determinants for amyloid fibril formation:lessons from lung surfactant protein C

Johansson J
Swiss Med Wkly 2003;133:275-82
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=12844270

[Pulmonary alveolar proteinosis: a report of 2 cases, with literature review]

Gao ZC, Xue Q, Xu Y, Li YP, Shen DH and He QY
Zhongguo Wei Zhong Bing Ji Jiu Yi Xue 2003;15:411-4
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=12857494

[Pulmonary alveolar proteinosis]

Floarea-Strat A, Stanciu A and Creteanu M
Rev Med Chir Soc Med Nat Iasi 2003;107:518-23
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=14756054

Deacylated pulmonary surfactant protein SP-C transforms from alpha-helical to amyloid fibril structure via a pH-dependent mechanism: an infrared structural investigation

Dluhy RA, Shanmukh S, Leapard JB, Kruger P and Baatz JE
Biophys J 2003;85:2417-29
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=14507705

Human parainfluenza virus giant cell pneumonia following cord blood transplant associated with pulmonary alveolar proteinosis

Butnor KJ and Sporn TA
Arch Pathol Lab Med 2003;127:235-8
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=12562244

Increased circulating CD16+ CD14dim monocytes in a patient with pulmonary alveolar proteinosis

Yoshioka Y, Ohwada A, Harada N, Satoh N, Sakuraba S, Dambara T and Fukuchi Y
Respirology 2002;7:273-9
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=12153694

[Secondary pulmonary alveolar proteinosis associated with myelodysplastic syndrome]

Yokomura K, Chida K, Suda T, Miwa S, Nakano H, Kuwata H, Suzuki K, Matsuda H, Asada K, Nakamura Y, Inui N, Shirai M and Nakamura H
Nihon Kokyuki Gakkai Zasshi 2002;40:599-604
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=12382426

Pulmonary alveolar proteinosis as a terminal complication in myelodysplastic syndromes: a report of four cases detected on autopsy

Shoji N, Ito Y, Kimura Y, Nishimaki J, Kuriyama Y, Tauchi T, Yaguchi M, Payzulla D, Ebihara Y and Ohyashiki K
Leuk Res 2002;26:591-5
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=12007507

Protein content and biophysical properties of tracheal aspirates form neonates with respiratory failure

Landmann E, Gortner L, Reiss I, Weller E and Tegtmeyer FK
Klin Padiatr 2002;214:1-7
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11823947

[Expression of GM-CSF in patients with pulmonary alveolar proteinosis]

Wang X, Luo F and Liu F
Zhonghua Jie He He Hu Xi Za Zhi 2001;24:417-20
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11802999

T-cadherin (CDH13, H-cadherin) expression downregulated surfactant protein D in bronchioloalveolar cells

Takeuchi T, Misaki A, Fujita J, Sonobe H and Ohtsuki
Virchows Arch 2001;438:370-5
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11355171

Pulmonary alveolar proteinosis: a spectrum of cytologic, histochemical, and ultrastructural findings in bronchoalveolar lavage fluid

Maygarden SJ, Iacocca MV, Funkhouser WK and Novotny DB
Diagn Cytopathol 2001;24:389-95
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11391819

Diagnosis of pulmonary alveolar proteinosis: usefulness of papanicolaou-stained smears of bronchoalveolar lavage fluid

Chou CW, Lin FC, Tung SM, Liou RD and Chang SC
Arch Intern Med 2001;161:562-6
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11252115

Exogenous lipid pneumonia related to smoking weed oil following cadaveric renal transplantation

Vethanayagam D, Pugsley S, Dunn EJ, Russell D, Kay JM and Allen C
Can Respir J 2000;7:338-42
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10980460

Serological diagnosis of idiopathic pulmonary alveolar proteinosis

Kitamura T, Uchida K, Tanaka N, Tsuchiya T, Watanabe J, Yamada Y, Hanaoka K, Seymour JF, Schoch OD, Doyle I, Inoue Y, Sakatani M, Kudoh S, Azuma A, Nukiwa T, Tomita T, Katagiri M, Fujita A, Kurashima A, Kanegasaki S and Nakata K
Am J Respir Crit Care Med 2000;162:658-62
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10934102

[Digital clubbing associated with primary alveolar proteinosis: possible implication of growth factors]

Hazouard E, Jacquemain C, Rivoire B, Besnier JM, de Muret A and Diot P
Presse Med 2000;29:999
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10862250

Cholesterol granulomas of the lungs associated with microangiopathic hemolytic anemia and thrombocytopenia in pulmonary hypertension

Fischer EG, Marek JM, Morris A and Nashelsky MB
Arch Pathol Lab Med 2000;124:1813-5
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=11100063

Pulmonary alveolar proteinosis: a complication in patients with hematologic malignancy

Birsak CA, van Rossem RN, Nijhuis-Heddes JM and Maartense E
Neth J Med 2000;56:193-7
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10781712

Imaging of medical disease of the newborn lung

Newman B
Radiol Clin North Am 1999;37:1049-65
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10546665

Idiopathic pulmonary alveolar proteinosis as an autoimmune disease with neutralizing antibody against granulocyte/macrophage colony-stimulating factor

Kitamura T, Tanaka N, Watanabe J, Uchida, Kanegasaki S, Yamada Y and Nakata K
J Exp Med 1999;190:875-80
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10499925

Acute respiratory failure caused by secondary alveolar proteinosis in a patient with acute myeloid leukemia: a case report

Gacouin A, Le Tulzo Y, Suprin E, Briens E, Bernard M, Camus C and Thomas R
Intensive Care Med 1998;24:265-7
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=9565812

Defective expression of granulocyte-macrophage colony-stimulating factor/interleukin-3/interleukin-5 receptor common beta chain in children with acute myeloid leukemia associated with respiratory failure

Dirksen U, Hattenhorst U, Schneider P, Schroten H, Gobel U, Bocking A, Muller KM, Murray R and Burdach S
Blood 1998;92:1097-103
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=9694696

[Pulmonary alveolar proteinosis: ultrastructural study of 4 cases]

Garrido L, Gledhill T, Martin M, Caleiras E, Parada D and Garcia Tamayo J
Invest Clin 1997;38:25-37
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=9235071

Secondary alveolar proteinosis in cancer patients

Ladeb S, Fleury-Feith J, Escudier E, Tran Van Nhieu J, Bernaudin JF and Cordonnier C
Support Care Cancer 1996;4:420-6
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=8961472

[Histologic changes in the lungs and heart in children with extrahepatic blockade of the portal circulation]

Klochkov SA, Alekseevskikh Iu G and Seniakovich VM
Arkh Patol 1996;58:54-8
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=8967847

Pulmonary alveolar proteinosis in a painter with elevated pulmonary concentrations of titanium

Keller CA, Frost A, Cagle PT and Abraham JL
Chest 1995;108:277-80
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=7606971

Six cases of pulmonary alveolar proteinosis: presentation of unusual associations

Garcia Rio F, Alvarez-Sala R, Caballero P, Prados C, Pino JM and Villamor J
Monaldi Arch Chest Dis 1995;50:12-5
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=7742818

A patient with multiple myeloma and respiratory insufficiency due to accumulation of paraprotein in the alveolar space

Meijer WG, Van Marwijk Kooy M and Ladde BE
Br J Haematol 1994;87:663-5
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=7993818

Secondary alveolar proteinosis is a reversible cause of respiratory failure in leukemic patients

Cordonnier C, Fleury-Feith J, Escudier E, Atassi K and Bernaudin JF
Am J Respir Crit Care Med 1994;149:788-94
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=8118651

[An autopsy case of acute lymphocytic leukemia associated with secondary pulmonary alveolar proteinosis and systemic aspergillosis]

Kita H, Muro S, Nakano Y, Hattori N, Mizutani T, Kagioka H and Fujita M
Nihon Kyobu Shikkan Gakkai Zasshi 1993;31:374-8
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=8315908

[A case of pulmonary alveolar proteinosis with invasion of macrophages in alveolar interstitial region]

Ishida K, Hatakeyama S, Koumura K, Ebe T and Arai O
Nihon Kyobu Shikkan Gakkai Zasshi 1992;30:1852-7
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=1464988

[Lactate dehydrogenase isoenzymes and alveolar inflammation in acute severe bronchiolitis in infants]

Baldini G, Cappuccio A, Ruschi A, Bertelloni C and Pifferi M
Pediatr Med Chir 1992;14:597-600
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=1298931

Hemodynamic changes during whole bronchoalveolar lavage in two cases of pulmonary alveolar proteinosis

Aguinaga MA, Santos P, Renes E, Alvaro PF, Lorente JA, Maudes A, Diaz RR, Landin L and Liste D
Intensive Care Med 1991;17:421-3
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=1774397

Pulmonary alveolar proteinosis associated with Pneumocystis carinii. Ultrastructural identification in bronchoalveolar lavage in AIDS and immunocompromised non-AIDS patients

Tran Van Nhieu J, Vojtek AM, Bernaudin JF, Escudier E and Fleury-Feith J
Chest 1990;98:801-5
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=2209133

Glutaric aciduria type II: autopsy study of a case with electron-transferring flavoprotein dehydrogenase deficiency

Kamiya M, Eimoto T, Kishimoto H, Tsudzuki T, Morishita H, Wada Y, Wakabayashi T, Hashimoto T, Goodman SI, Frerman FE and et al.
Pediatr Pathol 1990;10:1007-19
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=2209133

Pulmonary complications of leukemia

Hildebrand FL, Jr., Rosenow EC, 3rd, Habermann TM and Tazelaar HD
Chest 1990;98:1233-9
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=2225971

[A case of pulmonary alveolar proteinosis accompanied with the elevation of CEA (carcinoembryonic antigen) in bronchoalveolar lavage fluid]

Tanaka F, Nakamura M, Nakashima H, Suga M, Sugimoto M, Ando M, Araki S and Higuchi S
Nihon Kyobu Shikkan Gakkai Zasshi 1989;27:968-73
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=2615113

Pulmonary alveolar proteinosis. Occurrence with metastatic melanoma to lung

Schiller V, Aberle DR and Aberle AM
Chest 1989;95:466-7
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=2914503

Pulmonary alveolar proteinosis: primary and secondary, a report of three cases

Gilligan DM, McCabe MM and FitzGerald MX
Ir J Med Sci 1989;158:14-7
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=2737853

Alveolar proteinosis treated with a single bronchial lavage

Wilson JW, Rubinfeld AR, White A and Mullerworth M
Med J Aust 1986;145:158-60
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=3736481

Secondary pulmonary alveolar proteinosis occurring in two patients with acquired immune deficiency syndrome

Ruben FL and Talamo TS
Am J Med 1986;80:1187-90
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=3014876

Pulmonary alveolar proteinosis. Staining for surfactant apoprotein in alveolar proteinosis and in conditions simulating it

Singh G, Katyal SL, Bedrossian CW and Rogers RM
Chest 1983;83:82-6
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=6336692

Pulmonary alveolar proteinosis: its association with hematologic malignancy and lymphoma

Carnovale R, Zornoza J, Goldman AM and Luna M
Radiology 1977;122:303-6
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=834862

Defective lung macrophages in pulmonary alveolar proteinosis

Golde DW, Territo M, Finley TN and Cline MJ
Ann Intern Med 1976;85:304-9
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=183578

Pulmonary alveolar proteinosis, bronchiectasis and secondary amyloidosis: a case report

Ganguli PC, Lynne-Davies P and Sproule BJ
Can Med Assoc J 1972;106:569 passim
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=5027640

Acute silico-proteinosis. A new pathologic variant of acute silicosis in sandblasters, characterized by histologic features resembling alveolar proteinosis

Buechner HA and Ansari A
Dis Chest 1969;55:274-8
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=5775743

[Alveolar proteinosis secondary to pulmonary adenocarcinoma (secondary pneumopathy with amylaceous bodies). Anatomoclinical
and histochemical study of a case]
Bisetti A and Barbolini G
Arch De Vecchi Anat Patol 1965;46:199-236
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=5331417

Pulmonary Alveolar Proteinosis with Hematologic Disorders
Doyle AP, Balcerzak SP, Wells CL and Crittenden JO
Arch Intern Med 1963;112:940-6
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=14065004




 
Current Clinical Trials

LIST OF CURRENT CLINICAL TRIALS RELATED TO PAP

The trial descriptions are for informational purposes only. Individuals with PAP should discuss with their health care providers whether or not participation in a clinical trial is appropriate and advisable. Your health care provider can also discuss this with the Principal Investigator (PI) of the study in order to determine if you are eligible and if it is advisable for you to participate. Only PAP clinical trials and clinical studies that have obtained Institutional Review Board approval are listed below.

1. EXAMPLE


Official Title:

Purpose: Rationale

Eligibility: Ages Eligible for Study: 16 Years - 65 Years Genders Eligible for Study: Both

Location and Contact Information:
(coming soon)

Study chairs or principal investigators:


For more information about this clinical trial, visit:


Under Construction.
 
Research Resources

PAP Registry
Under Construction

Tissue Bank
Under Construction

Knockout mice available
Under Construction

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