Diagnosing Pulmonary Alveolar Proteinosis

Wang, Bennet M.; Stern, Eric J.; Schmidt, Rodney A.; Pierson, David J.

doi:10.1378/chest.111.2.460

Cited by 173 publications

(119 citation statements)

References 35 publications

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“…123 A confident diagnosis of pulmonary alveolar proteinosis can be made with the combination of milky and opaque BALF and characteristic findings on highresolution CT. 12 Tumor markers, mucin-like glycoprotein, and surfactant proteins also occur in the BALF, but their diagnostic utility is limited, as they are also present in other pulmonary diseases. 124,125 …”

Section: Bronchoalveolar Lavagementioning

confidence: 99%

Pulmonary Alveolar Proteinosis

Khan

Agarwal

2011

Respir Care

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Section: Bronchoalveolar Lavagementioning

confidence: 99%

Pulmonary Alveolar Proteinosis

Khan

Agarwal

2011

Respir Care

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“…WLL remains the treatment of choice for PAP and is a safe procedure that provides lasting benefits with more than 70% of patients free of recurrence for seven years [13][14][15][16][17][18] .…”

Section: Discussionmentioning

confidence: 99%

“…Currently the WLL technique with a double-lumen tube and lung isolation is well established for adult patients 3,4,[13][14][15][16][17][18][19] . However, pediatric patients present a technical challenge for WLL due to the absence of DLET compatible with the airway caliber of these patients [20][21][22][23] .…”

Section: Discussionmentioning

confidence: 99%

Anestesia para lavagem pulmonar em paciente pediátrico portador de proteinose alveolar pulmonar

B¹,

Teixeira²,

Bittencourt³

2012

Rev. Bras. Anestesiol.

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“…The chest x-ray, performed for respiratory distress, which is most often unexplained, shows alveolar opacities, as well as reactional interstitial infiltrates (honeycombing). The pathological examination of lung elicits periodic acid-Schiff (PAS)-positive, protein and lipid, material filling the alveoli (41). PAP is diagnosed prevalently in the adult but pediatric or neonatal forms are also seen (42,43).…”

Section: Alveolar Proteinosismentioning

confidence: 99%

Clinical Biological and Genetic Heterogeneity of the Inborn Errors of Pulmonary Surfactant Metabolism

Tredano¹,

Blic²,

Griese³

et al. 2001

Clinical Chemistry and Laboratory Medicine

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Pulmonary surfactant is a multimolecular complex located at the air-water interface within the alveolus to which a range of physical (surface-active propert i e s ) and immune functions has been assigned. This complex consists of a surface-active lipid layer (consisting mainly of phospholipids), and of an aqueous subphase. From discrete surfactant sub-fractions one can isolate strongly hydrophobic surfactant proteins B (SP-B) and C (SP-C) as well as collectins SP-A and SP-D, which were shown to have specific structural, metabolic, or immune properties. Inborn or acquired abnormalities of the s u rfactant, qualitative or quantitative in nature, account for a number of human diseases. Beside hyaline membrane disease of the preterm neonate, a cluster of hereditary or acquired lung diseases has been characterized by periodic acid-Schiff-positive material filling the alveoli. From this heterogeneous nosologic group, at least two discrete entities presently emerge. The first is the SP-B deficiency, in which an essentially proteinaceous material is stored within the alveoli, and which represents an autosomal recessive Mendelian entity linked to the S F T P B gene (MIM 1786640). The disease usually generally entails neonatal respiratory distress with rapid fatal outcome, although partial or transient deficiencies have also been observed. The second is alveolar proteinosis, characterized by the storage of a mixed protein and lipid material, which constitutes a relatively heterogeneous clinical and biological syndrome, especially with regard to age at onset (from the neonate through to adulthood) as well as the severity of associated signs. Murine models, with a targeted mutation of the gene encoding granulocyte macrophage colonystimulating factor (GM-CSF) (C s f g m) or the ␤ subunit of its receptor (I l 3 r b 1) support the hypothesis of an abnormality of surfactant turnover in which the alveolar macrophage is a key player. Apart from SP-B deficiency, in which a near-consensus diagnostic chart can be designed, the ascertainment of other abnormalities of surfactant metabolism is not straightforward. The disentanglement of this disease cluster is however essential to propose specific therapeutic procedures: repeated broncho-alveolar lavages, GM-CSF replacement, bone marrow grafting or lung transplantation.

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Diagnosing Pulmonary Alveolar Proteinosis

Cited by 173 publications

References 35 publications

Pulmonary Alveolar Proteinosis

Pulmonary Alveolar Proteinosis

Anestesia para lavagem pulmonar em paciente pediátrico portador de proteinose alveolar pulmonar

Clinical Biological and Genetic Heterogeneity of the Inborn Errors of Pulmonary Surfactant Metabolism

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