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MeSH Review:

Pulmonary Alveolar Proteinosis

Sato, K. et al., Schoch, O.D. et al., Usui, Y. et al., Chung, M.J. et al., Hattori, A. et al., et al.

Paschen, Reiter, Stanzel, Teschler, Griese, Meaney, Bonfield, Hansson, Babiker, Kavuru, Thomassen, Wylam, Ten, Prakash, Nadrous, Clawson, Anderson, Kuroki, Takahashi, Chiba, Akino,

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Disease relevance of Pulmonary Alveolar Proteinosis

Although the pathological features of the lung were similar to that of paediatric patients with lipoid pneumonia coexisting with pulmonary alveolar proteinosis (PAP), gastro-oesophageal reflux (GOR) and a diverse group of severe primary diseases, the patient lacked evidence for any of these [1].
An open lung biopsy was required to make the diagnosis of pulmonary alveolar proteinosis (PAP) and infection with Mycobacterium kansasii [2].
Metabolic lung disease includes pulmonary alveolar proteinosis (PAP), pulmonary amyloidosis, metastatic pulmonary calcification, dendritic pulmonary ossification, pulmonary alveolar microlithiasis, and storage diseases [3].
We describe the case of a 53-year-old Philadelphia-chromosome-positive woman with chronic myelogenous leukemia, who developed pulmonary alveolar proteinosis (PAP) [4].
BACKGROUND: Pulmonary alveolar proteinosis (PAP) is a rare disease, characterized by excessive intra-alveolar accumulation of surfactant lipids and proteins [5].

High impact information on Pulmonary Alveolar Proteinosis

Lack of GM-CSF as a cause of pulmonary alveolar proteinosis [6].
Pulmonary alveolar proteinosis (PAP), a disease characterised by accumulation of surfactant in alveoli, is diagnosed on the basis of invasive biopsy procedures [7].
We have previously shown that the purified preparation of surfactant protein A (SP-A) isolated from patients with pulmonary alveolar proteinosis (PAP) contains a very small amount of immunoglobulin G (IgG) [8].
Congenital pulmonary alveolar proteinosis (CPAP) is a fatal disease of full-term infants that is unresponsive to current medical therapy [9].
Recently, granulocyte-macrophage colony-stimulating factor (GM-CSF) auto-antibodies have been found in many patients with pulmonary alveolar proteinosis (PAP) [10].

Chemical compound and disease context of Pulmonary Alveolar Proteinosis

As an example of pulmonary cholesterol accumulation, a known disorder of surfactant homeostasis, pulmonary alveolar proteinosis (PAP), was studied [11].
Transbronchial lung biopsy (TBLB) and autopsy revealed PAS-positive granular materials characteristic of pulmonary alveolar proteinosis (PAP) [12].

Anatomical context of Pulmonary Alveolar Proteinosis

BACKGROUND: Idiopathic pulmonary alveolar proteinosis (PAP) has recently been recognised as a disease of impaired alveolar macrophage function caused by neutralising anti-granulocyte-macrophage colony-stimulating (anti-GM-CSF) autoantibodies [13].
The prominent increase of these proteins in BAL fluids and sputum is diagnostic for pulmonary alveolar proteinosis (PAP) [14].

Gene context of Pulmonary Alveolar Proteinosis

A case of severe pulmonary alveolar proteinosis (PAP) with interstitial involvement, mediastinal lymphadenopathy and an elevation of carcinoembryonic antigen (CEA) in the bronchoalveolar lavage (BAL) fluid and the serum is presented [15].
BACKGROUND: Auto-antibodies against granulocyte-macrophage colony stimulating factor (GM-CSF) may be central to the pathogenesis of adult sporadic pulmonary alveolar proteinosis (PAP) [16].
The immunoinhibitory activities of the lung lavage materials and sera from patients with pulmonary alveolar proteinosis (PAP) [17].

Analytical, diagnostic and therapeutic context of Pulmonary Alveolar Proteinosis

DESIGN: The study was based on five patients with pulmonary alveolar proteinosis (PAP) who underwent lung lavage [18].
The current mainstay of treatment for pulmonary alveolar proteinosis (PAP) is whole-lung lavage [19].

References

Diffuse progressive pulmonary interstitial and intra-alveolar cholesterol granulomas in childhood. Sato, K., Takahashi, H., Amano, H., Uekusa, T., Dambara, T., Kira, S. Eur. Respir. J. (1996) [Pubmed]
Disseminated Mycobacterium kansasii infection with pulmonary alveolar proteinosis in a patient with chronic myelogenous leukemia. Goldschmidt, N., Nusair, S., Gural, A., Amir, G., Izhar, U., Laxer, U. Am. J. Hematol. (2003) [Pubmed]
Metabolic lung disease: imaging and histopathologic findings. Chung, M.J., Lee, K.S., Franquet, T., Müller, N.L., Han, J., Kwon, O.J. European journal of radiology. (2005) [Pubmed]
Pulmonary alveolar proteinosis in a patient with chronic myelogenous leukemia. Tsushima, K., Koyama, S., Saitou, H., Takematsu, H., Ichiyoshi, T., Kubo, K. Respiration; international review of thoracic diseases. (1999) [Pubmed]
Therapeutic lung lavages in children and adults. Paschen, C., Reiter, K., Stanzel, F., Teschler, H., Griese, M. Respir. Res. (2005) [Pubmed]
Lack of GM-CSF as a cause of pulmonary alveolar proteinosis. Hallman, M., Merritt, T.A. J. Clin. Invest. (1996) [Pubmed]
Surfactant apoprotein-A concentration in sputum for diagnosis of pulmonary alveolar proteinosis. Masuda, T., Shimura, S., Sasaki, H., Takishima, T. Lancet (1991) [Pubmed]
Immunoglobulin G is associated with surfactant protein A aggregate isolated from patients with pulmonary alveolar proteinosis. Hattori, A., Kuroki, Y., Takahashi, H., Sohma, H., Akino, T. Am. J. Respir. Crit. Care Med. (1997) [Pubmed]
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, R.J., Bewig, B., Crystal, R.G. Hum. Gene Ther. (1995) [Pubmed]
Aerosol granulocyte-macrophage colony-stimulating factor for pulmonary alveolar proteinosis. Wylam, M.E., Ten, R., Prakash, U.B., Nadrous, H.F., Clawson, M.L., Anderson, P.M. Eur. Respir. J. (2006) [Pubmed]
Serum cholestenoic acid as a potential marker of pulmonary cholesterol homeostasis: increased levels in patients with pulmonary alveolar proteinosis. Meaney, S., Bonfield, T.L., Hansson, M., Babiker, A., Kavuru, M.S., Thomassen, M.J. J. Lipid Res. (2004) [Pubmed]
Rapidly progressive pulmonary alveolar proteinosis in a patient with chronic myelogenous leukemia. Ito, K., Iwabe, K., Okai, T., Kouda, S., Tadokoro, M., Isiko, T. Intern. Med. (1994) [Pubmed]
BAL findings in a patient with pulmonary alveolar proteinosis successfully treated with GM-CSF. Schoch, O.D., Schanz, U., Koller, M., Nakata, K., Seymour, J.F., Russi, E.W., Boehler, A. Thorax (2002) [Pubmed]
Surfactant proteins A and D: disease markers. Kuroki, Y., Takahashi, H., Chiba, H., Akino, T. Biochim. Biophys. Acta (1998) [Pubmed]
Interstitial lattice shadow and mediastinal lymphadenopathy with an elevation of carcinoembryonic antigen in severe pulmonary alveolar proteinosis. Usui, Y., Takayama, S., Nakayama, M., Miura, H., Kimula, Y. Intern. Med. (1992) [Pubmed]
Anti-GM-CSF antibodies in paediatric pulmonary alveolar proteinosis. Latzin, P., Tredano, M., Wüst, Y., de Blic, J., Nicolai, T., Bewig, B., Stanzel, F., Köhler, D., Bahuau, M., Griese, M. Thorax (2005) [Pubmed]
The immunoinhibitory activities of the lung lavage materials and sera from patients with pulmonary alveolar proteinosis (PAP). Stratton, J.A., Sieger, L., Wasserman, K. Journal of clinical & laboratory immunology. (1981) [Pubmed]
Use of CT morphometry to detect changes in lung weight and gas volume. Perez, A., Coxson, H.O., Hogg, J.C., Gibson, K., Thompson, P.F., Rogers, R.M. Chest (2005) [Pubmed]
Pulmonary alveolar proteinosis: treatment by bronchofiberscopic lobar lavage. Cheng, S.L., Chang, H.T., Lau, H.P., Lee, L.N., Yang, P.C. Chest (2002) [Pubmed]

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