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Eur Respir J 2004; 23:783-785
Copyright ©ERS Journals Ltd 2004


Pulmonary infiltrates in Costello Syndrome

N. Waldburg1, F. Buehling2, M. Evert3, O. Burkhardt1 and T. Welte1

1 Dept of Pulmonary and Intensive Care Medicine, 2 Institute of Immunology, and 3 Institute of Pathology, Otto-von-Guericke-University, Magdeburg, Germany

CORRESPONDENCE: N. Waldburg, Klinik für Pneumologie und Intersivmedizin, Universitätsklinikum Magdeburg, Leipziger Str. 44, 39112 Magdeburg, Germany. Fax: 49 3916715420. E-mail: tobias.welte@medizin.uni-magdeburg.de

Keywords: Costello, infiltrate, lipid pneumonia, lung

Received: June 27, 2003
Accepted October 6, 2003

Abstract

This paper reports on a patient with diffuse pulmonary infiltrates directly related to Costello Syndrome.

This congenital disorder is characterised by multiple congenital abnormalities, such as psychomotor retardation, short stature, redundant skin, papillomata, curly hair, relative macroencephaly, distinctive face and various defects of internal organs.

This study is the first to document the histopathological findings in the lungs.

Most conspicuous was the depositing of abnormal collagen and elastic fibres and the development of endogenous lipid pneumonia.







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Copyright © 2004 by the European Respiratory Society.