title: Therapeutic lung lavages in children and adults.
abstract: BACKGROUND
  Pulmonary alveolar proteinosis (PAP) is a rare disease, characterized by excessive intra-alveolar accumulation of surfactant lipids and proteins. Therapeutic whole lung lavages are currently the principle therapeutic option in adults. Not much is known on the kinetics of the wash out process, especially in children.
  
  
  METHODS
  In 4 pediatric and 6 adult PAP patients 45 therapeutic half lung lavages were investigated retrospectively. Total protein, protein concentration and, in one child with a surfactant protein C mutation, aberrant pro-SP-C protein, were determined during wash out.
  
  
  RESULTS
  The removal of protein from the lungs followed an exponential decline and averaged for adult patients 2-20 g and <0.5 to 6 g for pediatric patients. The average protein concentration of consecutive portions was the same in all patient groups, however was elevated in pediatric patients when expressed per body weight. The amount of an aberrant pro-SP-C protein, which was present in one patient with a SP-C mutation, constantly decreased with ongoing lavage. Measuring the optical density of the lavage fluid obtained allowed to monitor the wash out process during the lavages at the bedside and to determine the termination of the lavage procedure at normal protein concentration.
  
  
  CONCLUSIONS
  Following therapeutic half lung lavages by biochemical variables may help to estimate the degree of alveolar filling with proteinaceous material and to improve the efficiency of the wash out, especially in children.
authors.affiliation: Dr. von Haunersches Kinderspital, University of Munich, Lindwurmstr. 4a, D-80337 Munich, Germany. christian-paschen@t-online.de
authors.forename: Christian
authors.initials: C
authors.lastname: Paschen
authors.forename: Karl
authors.initials: K
authors.lastname: Reiter
authors.forename: Franz
authors.initials: F
authors.lastname: Stanzel
authors.forename: Helmut
authors.initials: H
authors.lastname: Teschler
authors.forename: Matthias
authors.initials: M
authors.lastname: Griese
mesh_terms: D000328:Adult; D018893:Bronchoalveolar Lavage / Q000379:methods*; D001992:Bronchoalveolar Lavage Fluid / Q000737:chemistry*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D011649:Pulmonary Alveolar Proteinosis / Q000378:metabolism* / Q000628:therapy*; D037721:Pulmonary Surfactant-Associated Protein C / Q000302:isolation & purification* / Q000378:metabolism*; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D037721:Pulmonary Surfactant-Associated Protein C