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Klassifikation der Alveolarproteinosen im Neugeborenen-, Säuglings- und Kindesalter

Classification of pulmonary alveolar proteinosis in newborns, infants, and children

  • Schwerpunkt: Kinderpathologie
  • Published:
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Zusammenfassung

Alveolarproteinosen sind seltene Lungenerkrankungen, die durch eine intraalveoläre Akkumulation des Surfactantprotein (SP-) A charakterisiert sind. Folgende Subtypen können unterschieden werden:

Typ-I-Alveolarproteinosen: schwere, ohne Lungentransplantation letal verlaufende respiratorische Insuffizienz bei reifen Neugeborenen; hereditärer Mangel an SP-B und intraalveoläre Akkumulation eines N-terminal inkomplett prozessierten SP-C.

Typ-II-Alveolarproteinosen: bei reifen Neugeborenen und Säuglingen; häufig letaler Verlauf; intraalveoläre Akkumulation von Prozessierungszwischenstufen des SP-B und reifem SP-B sowie begleitende, variabel ausgeprägte, interstitielle Lungenerkrankung.

Typ-III-Alveolarproteinosen: bei Säuglingen und Kindern; in der Regel nicht letal; intraalveoläre Akkumulation von Prozessierungszwischenstufen des SP-B sowie reifem SP-B ohne eine begleitende interstitielle Lungenerkrankung; „kryptogen“ kongenitale, erworbene (idiopathische) und sekundäre Typ-III-Alveolarproteinosen werden unterschieden.

Bei Neugeborenen, Säuglingen und Kindern mit Alveolarproteinosen sollte eine detaillierte pathologisch-anatomische Begutachtung unter Einsatz immunhistochemischer und molekulargenetischer Verfahren erfolgen, um eine Optimierung der Therapie zu erzielen.

Abstract

Pulmonary alveolar proteinoses are rare pulmonary diseases characterised by an intraalveolar accumulation of surfactant protein A. Subtyping of alveolar proteinoses:

Type I alveolar proteinoses: severe respiratory insufficiency in newborns, which will take a lethal course without lung transplant; hereditary SP-B deficiency and an intraalveolar accumulation of N-terminal incompletely processed SP-C.

Type II alveolar proteinoses: occur in newborns and infants; often take a lethal course; show intraalveolar accumulation of precursors of SP-B and mature SP-B as well as an accompanying interstitial lung disease of variable severity.

Type III alveolar proteinoses: in infants and children; do not generally take a lethal course; they are characterised by an intraalveolar accumulation of precursors of SP-B and mature SP-B without accompanying interstitial lung disease. “Cryptogenic” congenital, acquired (idiopathic), and secondary type III alveolar proteinoses can be distinguished.

In newborns, infants, and children with pulmonary alveolar proteinosis, a detailed pathological-anatomical examination including immunohistochemical and molecular genetic analyses, should be performed in order to optimise the therapeutical management.

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  1. Institut für Pathologie, Berufsgenossenschaftliche Kliniken Bergmannsheil Bochum

    F. Brasch & K.-M. Müller

  2. Institut für Pathologie, Berufsgenossenschaftliche Kliniken Bergmannsheil, Ruhr-Universität Bochum, Bürkle-de-la-Camp Platz 1, 44789, Bochum

    F. Brasch

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  1. F. Brasch
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Correspondence to F. Brasch.

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Dieser Beitrag wurde in Auszügen bereits publiziert: Brasch F, Müller K-M (2004) Alveolarproteinosen. In: Rieger C, Hardt H v d, Sennhauser FH, Wahn U, Zach M (Hrsg) Pädiatrische Pneumologie, 2. Aufl. Springer, Berlin Heidelberg New York Tokyo, S 945–956.

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Brasch, F., Müller, KM. Klassifikation der Alveolarproteinosen im Neugeborenen-, Säuglings- und Kindesalter. Pathologe 25, 299–310 (2004). https://doi.org/10.1007/s00292-004-0706-5

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