Zusammenfassung
Das Krankheitsbild der pulmonalen Alveolarproteinosen (PAP) wurde erstmals 1958 detailliert beschrieben. Es handelt sich um eine Gruppe von ungewöhnlichen diffusen Lungenerkrankungen, die durch die Anhäufung großer Mengen phospholipid- und proteinhaltiger Materialien in den Alveolen der Lungen gekennzeichnet ist. Treffend ist daher die Bezeichnung ▸ alveoläre Füllungssyndrome (Abb. 1).
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
Literatur
Ballard PL, Nogee LM, Beers MF, Ballard RA, Planer BC, Polk L, deMello DE, Moxley MA, Longmore WJ (1995) Partial deficiency of surfactant protein B in an infant with chronic lung disease. Pediatrics 96: 1046–1052
Costabel U, Guzman J (2000) Alveolar proteinosis. In: Olivieri D, du Bois RM (eds) Interstitial lung diseases. Eur Respir Monograph, pp 194–205
Dirksen U, Nishinakamura R, Groneck P, Hattenhorst U, Nogee L, Murray R, Burdach S (1997) Human pulmonary alveolar proteinosis associated with a defect in GM-GSF/IL-3/IL-5 receptor common ß chain expression. J Clin Invest 100: 2211–2217
Dunbar III AE, Wert SE, Ikegami M, Whitsett JA, Hamvas A, White FV, Piedboeuf B, Jobin C, Guttentag S, Nogee LM (2000) Prolonged surival in hereditary surfactant protein B (SP-B) deficiency associated with a novel splicing mutation. Pediatr Res 48:275–282
Griese M (1992) Surfactanthomöostase — Grundlagen für die Surfactantsubstitutionstherapie. Monatsschr Kinderhkd 140: F2–F12
Griese M (1999) Pulmonary surfactant in health and lung diseases: state of the art. Eur Respir J13: 1455–1476
Hamvas A, Nogee LM, deMello DE, Cole FS (1995) Pathophysiology and treatment of surfactant protein-B deficiency. Biol Neonate [Suppl 1] 67:18–31
Kitamura T, Uchida K, Tanaka N, Tsuchiya T, Watanabe J, Yamada Y, Hanaoka K, Seymour JF, Schoch OD, Doyle IR, Inoue Y, Sakatani M, Kudoh S, Azuma A, Nukiwa T, Tomita T, Katagiri M, Fujita A, Kurashima T, Kanegasaki S, Nakata K (2000) Serological diagnosis of idiopathic pulmonary alveolar proteinosis. Am J Respir Crit Care Med 162:658–662
Klein J, Thompson M, Snyder J, George T, Whitsett JA, Bell E, McCray P, Nogee L (1998) Transient surfactant protein B deficiency in a term infant with severe respiratory failure. J Pediatr 132:244–248
Nogee LM, de Mello DE, Dehner LP, Colten HR (1993) Brief-report: deficiency of pulmonary surfactant protein B in congenital alveolar proteinosis. N Engl J Med 328:406–410
Nogee LM, Wert SE, Proffit SA, Hull WM, Whitsett JA (2000) Allelic heterogeneity in hereditary surfactant protein B (SP-B) deficiency. Am J Respir Crit Care Med 161:973–981
Shah PL, Hansell D, Lawson PR, Reid K, Morgan C (2000) Pulmonary alveolar proteinosis: clinical aspects and current concepts on pathogenesis. Thorax 55:67–77
Tredano M, Elburg RM van, Kaspers AG, Zimmermann LJ, Houdayer C, Aymard P, Hull WM, Whitsett JA, Elion J, Griese M, Bahuau M (1999) Compound SFTPB1549C-GAA (121ins2) and 457delC heterozygosity in severe congenital lung disease and surfactant protein B (SP-B) deficiency. Hum Mutat 14:502–509
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2001 Springer-Verlag Berlin Heidelberg
About this chapter
Cite this chapter
Griese, M., Tredano, M., Bahuau, M. (2001). Pulmonale Alveolarproteinosen. In: Koletzko, B., Reinhardt, D., Stöckler-Ipsiroglu, S. (eds) Pädiatrie upgrade 2002. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-56123-8_8
Download citation
DOI: https://doi.org/10.1007/978-3-642-56123-8_8
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-540-43401-6
Online ISBN: 978-3-642-56123-8
eBook Packages: Springer Book Archive