Alveolar proteinosis

Fine metallic aluminum powders inhaled by hamsters and guinea pigs caused no pulmonary fibrosis in rats that inhaled the dust, small scars resulted from foci of lipid pneumonitis. Alveolar proteinosis developed in all three species it resolved spontaneously, and the accumulated dust deposits cleared rapidly from the lungs after cessation of the exposure. The failure of inhaled aluminum powder to cause pulmonary fibrosis in experimental animals parallels the clinical experience in the United States, where pulmonary fibrosis has not been observed in aluminum workers. ... 

Alveolar proteinosis and marked increased lung weights were observed in rats exposed for life to 0.06 mg nickel/m as nickel oxide (Takenaka et al. 1985). At the end of the 31-month experiment, two... 

Survival time decreased from 125 weeks in controls to 88 weeks body weight loss after 13 months alveolar proteinosis and marked lung enlargement Pneumonia and bronchial epithelial metaplasia Bronchial gland hyperplasia 20 months after exposure... 

S.N. Bhattacharyya. S. Sahu and W.S. Lynn, Structural studies on a glycoprotein isolated from alveoli of patients with alveolar proteinosis, Biochem. Biophys. Acta 427 (1976) 91-106. 

Pulmonary alveolar proteinosis Surfactant protein C (SP-C) Local... 

Pulmonary alveolar proteinosis Lung surfactant protein C Local... 

In pulmonary alveolar proteinosis, the alveoli of the lung fill with an excess of a glycoprotein. The reaction may occur in response to dust or other irritants. 

A patient with acute myeloid leukemia developed pulmonary alveolar proteinosis during the period of leukopenia following an unrelated umbilical cord blood transplantation (49). 

Tomonari A, Shirafuji N, Iseki T, Ooi J, Nagayama H, Masunaga A, Tojo A, Tani K, Asano S. Acquired pulmonary alveolar proteinosis after umbilical cord blood transplantation for acute myeloid leukemia. Am J Hematol 2002 70(2) 154-7. 

Immunomodulatory effects of isotretinoin in the treatment of facial acne (40 mg/day for 4 weeks) were blamed for a recurrence of pulmonary alveolar proteinosis in a 16-year-old girl, in whom it had been in spontaneous remission for 2 years (107). 

Khurshid 1, Seymour IF, Nakata K, Downie GH. Recurrent manifestations of idiopathic pulmonary alveolar proteinosis after isotretinoin (Accutane) treatment. Chest 2001 120(Suppl) 335. 

Very rarely, respiratory distress in a term newborn can be caused by a mutation in the gene encoding for SP-B. This condition, termed congenital alveolar proteinosis, is often... 

Surfactant apoprotein A (SP-A) PE-10 Surfactant A Surfactant apoproteins isolated from lung lavages of patients with alveolar proteinosis Dako 1 100 HIER... 

Lung surfactant protein C Res 9-22 (WWVVVLVVVVIV) (deMeUo and Lin 2001 Hosia et al. 2002 Schmitt-Bemard et al. 2000 Seymour and PresneiU 2002 Trapnell et al. 2003) Pulmonary alveolar proteinosis... 

Seymour JF, Presneill JJ (2002) Pulmonary alveolar proteinosis progress in the first 44 years. Am J Respir Crit Care Med 166 215-235... 

Tracz SM, Abedini A, Driscoll M, Raleigh DP (2004) Role of aromatic interactions in amyloid formation by peptides derived from human Amylin. Biochemistry 43 15901-15908 Trapnell BC, Whitsett JA, Nakata K (2003) Pulmonary alveolar proteinosis. N Engl J Med 349 2527-2539 Tsuii S (1999) Dentatombral-pallidoluysian atrophy (DRPLA) clinical features and molecular genetics. Adv... 

Bhattacharya, S.N, and Lynn, W.S. (1977) Structural Studies of the Glycoprotein Isolated from the Alveoli of Patients with Alveolar Proteinosis , Journal of Biological Chemistry, 252, 1172-80... 

Other, more specific, transporter dysfunctions lead to distinct inborn errors of metabolism. Oxaluria and cystinuria, defects in oxalate and cysteine transport, respectively, manifest with renal stones. Cystinuria specifically presents with cysteine, ornithine, lysine, and arginine in the urine. The latter should not be confused with cystinosis. Lysinuric protein intolerance (LPI) is a defect in the dibasic amino acid transporter. This results in a specific amino aciduria pattern (ornithine, lysine, and arginine), which in turn results in secondary inhibition of the urea cycle. Individuals affected by LPI are at risk for hyperammonemia and also have a unique susceptibility to macrophage activation syndrome, an exaggerated systemic inflammatory response, and alveolar proteinosis. Renal damage... 

Respiratory Pulmonary alveolar proteinosis is a rare complication of treatment with sirolimus but has been reported in a 49-year-old female kidney transplant recipient [126 ]. A biopsy showed intact lung parenchyma and macrophages among alveoli filled with PAS-positive eosinophilic material which also stained positive for... 

Kadikoy H, Paolini M, Achkar K, Suki W, Gaber AO, Anwar N, Jeroudi A, Barrios R, Abdellatif A. Pulmonary alveolar proteinosis in a kidney transplant a rare complication of sirolimus. Nephrol Dial Transplant 2010 25(8) 2795-8. 

The electron microscopic findings were typical for alveolar proteinosis. Only rare alveolar macrophages were found, and their cytoplasm was distended with granules similar to those free in alveolar spaces. The cytoplasmic borders of the macrophages were abnormally smooth and rounded and only occasional primary lysosomes were present. 

In rats, illite and muscovite, but not kaolin produced lesions of alveolar proteinosis (Martin et al. 1977). Reisner et al. (1982) and Rosmanith et al. (1982) emphasised that the relation between the ka-olinite content of the lymph nodes and that of the lungs six months after an intratracheal injection of different West German coal mine dusts showed an exponential regression function by the method of least squares. 

Respiratory Bronchiolitis-Associated Interstitial Lung Disease 341 Desquamative Interstitial Pneumonia 341 Lymphoid Interstitial Pneumonia 342 Acute Interstitial Pneumonia 342 Sarcoidosis 343 Miscellaneous Rare Forms of Interstitial Lung Disease of Unknown Etiology 345 Pulmonary Langerhans Cell Histiocytosis 345 Lymphangioleiomyomatosis 345 Eosinophilic Pneumonia 346 Pulmonary Alveolar Proteinosis 348 Pulmonary Microlithiasis 348... 

Fig. 26.20. Alveolar proteinosis in 40-year-old man with myelogenous leukemia presenting with cough and dyspnea. Axial CT image displays bilateral geographical areas of ground-glass opacity. Interlobular septa are thickened and within these areas, a fine reticular network of interlobular lines can be seen. These changes are referred to as the typical crazy-paving appearance of alveolar proteinosis...

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