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Thorakale Veränderungen bei zystischer Fibrose im Kindesalter

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Zusammenfassung

Die zystische Fibrose (Mukoviszidose) ist die häufigste angeborene Stoffwechselerkrankung in Europa. Sie wird autosomal-rezessiv vererbt und hat in Europa eine Inzidenz von 1:2500.

Die Lungenbeteiligung beeinflusst die Morbidität und die Mortalität am deutlichsten. Durch die verbesserte Diagnostik und Therapie ist die Lebenserwartung in den letzten Jahrzehnten gestiegen. Die zugrundeliegende Pathologie besteht in einem Gendefekt, welcher für einen gestörten Transport von Chlorid verantwortlich ist. In der Folge bilden exokrine Drüsen einen wasserarmen, zähen Schleim, welcher u. a. die Atemwege verlegt und Entzündungen begünstigt. Dadurch entsteht ein Circulus vitiosus, der in der Zerstörung des Lungengewebes mündet. Die konventionelle Röntgenaufnahme stellt die derzeit wichtigste radiologische Untersuchungsmethode dar, die Computertomographie erfasst die Morphologie jedoch besser und früher. Die Magnetresonanztomographie stellt einen aktuellen Forschungsschwerpunkt dar und bietet durch die Möglichkeit einer zusätzlichen funktionellen Bildgebung interessante Aspekte für die Zukunft.

Abstract

Cystic fibrosis is the most common autosomal recessive metabolic disease in Europe with an incidence of 1:2500. The severity of the lung disease is the most important factor of morbidity and mortality in CF-patients. Because of the better diagnostic and therapeutic modalities life expectancy has increased significantly. The underlying pathology is a defect of chromosome 7, which encodes the regulation of the fluid balance across the cell membrane which effects chloride as well as sodium. The exocrine glands produce a viscous mucus which obstructs the airways and promotes infections. The result is the destruction of lung parenchyma. In daily routine, chest x-ray is still the most important radiological tool, although computed tomography depicts changes in morphology earlier and more exactly. Recent research studies show that MRI has—because of its additional functional options—interesting aspects for the future.

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Danksagung

Ich danke Herrn Prof. Dr. Kauczor, DKFZ Heidelberg, für das freundliche Überlassen des MRT-Bildes (Abb. 5) und Herrn Dr. Hansmann, Abt. Radiodiagnostik Universität Heidelberg, für das CT-Bild (Abb. 3).

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  1. Abteilung Radiologie, Vestische Kinder- und Jugendklinik Datteln

    R. Wunsch & C. Wunsch

  2. Abteilung Radiologie, Vestische Kinder- und Jugendklinik, Universität Witten/Herdecke, 45711 , Datteln

    R. Wunsch

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Wunsch, R., Wunsch, C. Thorakale Veränderungen bei zystischer Fibrose im Kindesalter. Radiologe 43, 1103–1108 (2003). https://doi.org/10.1007/s00117-003-0987-3

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