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CC BY-NC-ND 4.0 · Klin Padiatr 2024; 236(02): 64-72
DOI: 10.1055/a-2194-3467
Original Article

Ten-year experience of whole lung lavage in pediatric Pulmonary Alveolar Proteinosis

Zehn-jährige Erfahrung mit Ganzlungenwaschung bei kindlicher Alveolar-Proteinose
Katja Nickel   *
1   Clinic of Anesthesiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany
,
Katharina Schütz   *
2   Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany
,
Julia Carlens
2   Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany
,
Simon Grewendorf
2   Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany
,
Martin Wetzke
2   Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany
4   Biomedical Research in Endstage and Obstructive Lung Disease (BREATH), Member of the German Center for Lung Research (DZL)
,
Oliver Keil
1   Clinic of Anesthesiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany
,
Nils Dennhardt
1   Clinic of Anesthesiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany
,
Vanessa Rigterink
1   Clinic of Anesthesiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany
,
Harald Köditz
3   Department of Pediatric Cardiology and Intensive Medicine, Hannover Medical School, Hannover, Germany
,
Michael Sasse
3   Department of Pediatric Cardiology and Intensive Medicine, Hannover Medical School, Hannover, Germany
,
Christine Happle
2   Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany
4   Biomedical Research in Endstage and Obstructive Lung Disease (BREATH), Member of the German Center for Lung Research (DZL)
5   RESIST Cluster of Excellence Infection Research, Hannover Medical School, Hannover, Germany
,
Christiane E. Beck   *
1   Clinic of Anesthesiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany
,
Nicolaus Schwerk   *
2   Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany
4   Biomedical Research in Endstage and Obstructive Lung Disease (BREATH), Member of the German Center for Lung Research (DZL)
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Abstract

Background Pulmonary Alveolar Proteinosis (PAP) is extremely rare and can be caused by hereditary dysfunction of the granulocyte macrophage colony-stimulating factor receptor (GM-CSF) receptor, autoantibodies against GM-CSF, or other diseases leading to alveolar macrophage (AM) dysfunction. This leads to protein accumulation in the lung and severe dyspnea and hypoxemia. Whole lung lavage (WLL) is the first line treatment strategy.

Methods Here, we present data from more than ten years of WLL practice in pediatric PAP. WLL performed by the use of a single lumen or double lumen tube (SLT vs. DLT) were compared for technical features, procedure time, and adverse events.

Results A total of n=57 procedures in six PAP patients between 3.5 and 14.3 years of age were performed. SLT based WLL in smaller children was associated with comparable rates of adverse events but with longer intervention times and postprocedural intensive care treatment when compared to DLT based procedures.

Discussion Our data shows that WLL is feasible even in small children. DLT based WLL seems to be more effective, and our data supports the notion that it should be considered as early as possible in pediatric PAP.

Conclusion WLL lavage is possible in small PAP patients but should performed in close interdisciplinary cooperation and with age appropriate protocols.

Zusammenfassung

Hintergrund Die pulmonale Alveolarproteinose (PAP) ist eine sehr seltene Lungenerkrankung welche durch Mutationen im Granulozyten-Makrophagen-Kolonie-stimulierenden Faktor (GM-CSF)- Rezeptorgen, durch GM-CSF-Autoantikörper oder durch andere Erkrankungen mit Schädigung der Alveolarmakrophagenfunktion hervorgerufen wird. Dies führt zu Proteinakkumulation in der Lunge und schwerer Hypoxämie und Atemnot. Die Ganzlungenlavage stellt die Therapie der ersten Wahl dar.

Methoden Hier zeigen wir Daten einer Zehnjahresbeobachtung von Ganzlungenlavagen bei Kindern mit PAP. Wir vergleichen Ganzlungenlavage-Prozeduren mittels Einzellumen- versus Doppellumen-Tubus im Hinblick auf Durchführung, Dauer und Komplikationen.

Resultate Insgesamt wurden n=57 Prozeduren bei sechs PAP Patient:innen im Alter zwischen 3,5 und 14,3 Jahren ausgewertet. Einzellumen-basierte Lavagen wurden bei jüngeren Kindern durchgeführt und zeigten vergleichbare Komplikationsraten, aber längere Interventions- und längere postinterventions-Behandlungsdauern auf der Kinderintensivstation.

Diskussion Wir zeigen, dass Ganzlungenlavagen auch bei kleinen Patient:innen durchführbar sind, wobei Doppellumentubus-basierte Prozeduren bei PAP im Kindesalter schneller zu sein scheinen und daher, wenn möglich, frühzeitig versucht werden sollten.

Schlussfolgerung Ganzlungenlavagen sind möglich und relativ sicher bei jungen PAP-Patient:innen, aber sollten in einem erfahrenen Zentrum in enger interdisziplinärer Kollaboration und mit alters-angepassten Protokollen durchgeführt werden.

Key words

Whole lung lavage - single lung ventilation - bronchoscopy - Pulmonary Alveolar Proteinosis - pediatric anaesthesia

Schlüsselwörter

Ganzlungenlavage - Einlungenventilation - Bronchoskopie - Pulmonale Alveolar Proteinose - Kinderanästhesie

* These authors contributed equally to this work: Katja Nickel, Katharina Schütz, Christiane E. Beck, Nicolaus Schwerk


Additional material



Publication History

Article published online:
23 January 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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