Therapeutic lung lavages in children and adults

Paschen Christian, Reiter Karl, Stanzel Franz, Teschler Helmut, Griese, Griese Matthias

Description

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. Conclusion 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.

Sujets

• Bal
• PAP
• Protein

Informations

• Publié par: biomed
• Publié le: 01 janvier 2005
• Nombre de lectures: 14
• Langue: English

Extrait

Respiratory Research

Research Therapeutic lung lavages in children and adults 1 12 Christian Paschen, Karl Reiter, Franz Stanzel, Helmut 1 Matthias Griese*

BioMedCentral

Open Access

3 Teschler and

1 2 Address: Dr.von Haunersches Kinderspital, University of Munich, Lindwurmstr. 4a, D80337 Munich, Germany,ASKLEPIOS Fachkliniken, 3 Zentrum für Pneumologie und Thoraxchirurgie, RobertKochAllee 2, D82131 MünchenGauting, Germany andRuhrlandklinik, Department Respiratory and Sleep Medicine, University of Essen, Tüschener Weg 40, Germany Email: Christian Paschen  christianpaschen@tonline.de; Karl Reiter  [e-mail]muenchen.de; Franz Stanzel  [e-mail]; Helmut Teschler  [e-mail]; Matthias Griese*  [e-mail]muenchen.de * Corresponding author

Published: 22 November 2005Received: 27 August 2005 Accepted: 22 November 2005 Respiratory Research2005,6:138 doi:10.1186/1465-9921-6-138 This article is available from: http://respiratory-research.com/content/6/1/138 © 2005 Paschen et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

BALPAPProtein

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. Conclusion: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.

Introduction Pulmonary alveolar proteinosis (PAP) is a rare respiratory disease characterized by the accumulation of surfactant derived material in the lung of patients [1]. Currently PAP is categorized into acquired, congenital, and secondary PAP [2,3].

The acquired form of PAP is clinically characterized by cough, dyspnea and progression to respiratory failure. The presence of antiGMCSF autoantibodies in serum and bronchoalveolar lavage (BAL) is of diagnostic value for this entity[3,4]. The congenital form of PAP is character ized by an acute onset immediately after birth with respi ratory distress and rapid progression[5]. Pathogenetically

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