Pulmonary infiltrates in Costello Syndrome.
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.
Waldburg
N
N
Dept of Pulmonary and Intensive Care Medicine, Otto-von-Guericke-University, Magdeburg, Germany.
Buehling
F
F
Evert
M
M
Burkhardt
O
O
Welte
T
T
D000015:Abnormalities, Multiple; D000328:Adult; D003094:Collagen / Q000378:metabolism; D004547:Elastic Tissue / Q000473:pathology; D006801:Humans; D008168:Lung / Q000378:metabolism / Q000473:pathology; D008171:Lung Diseases / Q000150:complications* / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology; D008297:Male; D011017:Pneumonia, Lipid / Q000150:complications; D013902:Radiography, Thoracic; D013577:Syndrome; D014057:Tomography, X-Ray Computed
D003094:Collagen