Takanami I, Morota A, Shikata J
First Department of Surgery, Teikyo University School of Medicine, Tokyo, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1991 Sep;29(9):1186-9.
A 19-year-old male was admitted to Teikyo University Hospital because of dyspnea. The chest film revealed pneumothorax of the right side. The patient was tall and thin, with long tapered extremities. The lens showed ectopia lentis. Echography of the heart showed dilatation of the aorta. A diagnosis of Marfan's syndrome was established on the basis of characteristic skeletal, ocular and cardiovascular findings. At thoracotomy, several bullae were removed by wedge resection. Collagen type distribution in the lung was investigated by an indirect immunohistochemical study using type-special antibody of type I collagen. There were no significant differences in collagen type I in the lung between Marfan's syndrome and control. This suggests that type I collagen in Marfan's syndrome cannot be distinguished from normal collagen I by indirect immunochemical study.