All major glycosaminoglycans except keratan sulphate are found in animal lung and pleura (Wusteman, 1972). Pleural fluids from patients with several pulmonary diseases contain mostly hyaluronic acid (Friman, Hellstrom, Juvani & Riska, 1977). The high concentration of hyaluronic acid in pleural fluid associated with mesothelioma is considered diagnostic of this disease (Havez, Degand, Boersma & ...

To determine the molecular defect accounting for the deficiency of pulmonary surfactant protein B (SP-B) in full-term neonates who died from respiratory failure associated with alveolar proteinosis, the sequence of the SP-B transcript in affected infants was ascertained. A frameshift mutation consisting of a substitution of GAA for C in codon 121 of the SP-B cDNA was identified. The three affec...

Although BAL had been used for therapeutic purposes prior to its use as a diagnostic procedure and the value of BAL in the exploration and management of some interstitial lung diseases is now well established, its place in therapy is controversially reported. As early as 1963, RAMIREz et al. [280] were the frrst to perform a whole lung lavage (WLL) using a large volume of fluid in patients with...

mechanisms, and applications. Genes Dev 2010;24:2239–2263. 3. Takahashi K, Tanabe K, Ohnuki M, Narita M, Ichisaka T, Tomoda K, Yamanaka S. Induction of pluripotent stem cells from adult human fibroblasts by defined factors. Cell 2007;131: 861–872. 4. Yu J, Vodyanik MA, Smuga-Otto K, Antosiewicz-Bourget J, Frane JL, Tian S, Nie J, Jonsdottir GA, Ruotti V, Stewart R, et al. Induced pluripotent st...

Description of a “New” Disease Nature of the Accumulated Alveolar Material Published Features of Patients with PAP Presentation Demographic Features Smoking Age Arterial Oxygen Pressure Serum LDH Spirometric and Radiographic Features Diagnostic Procedures Histopathology Pathogenesis and Classification Acquired PAP Congenital PAP Secondary PAP Development of Effective Treatment Application and E...

Pulmonary alveolar proteinosis (PAP) is a rare pulmonary syndrome characterized by surfactant accumulation in the alveoli, which results impaired gas exchange. There are three types of PAP: primary, secondary and congenital. Autoimmune PAP, under category primary most common form, caused an impairment granulocyte–macrophage colony-stimulating factor (GM-CSF) signalling, due to presence anti-GM-...