Pulmonary arterial aneurysm with CATCH 22 syndrome--a case report.

An adult case of CATCH 22 syndrome with a pulmonary arterial aneurysm is presented. The chest radiograph showed a large massive lesion, which should be differentiated from lung cancer, mediastinal tumor, or aortic aneurysm. CATCH 22 syndrome is a developmental malformation caused by hemizygous deletion of 22q11 chromosome, which usually presents with cardiac malformation including tetralogy of Fallot, truncus arteriosus, patent arterial duct, and ventricular septal defect. Most patients with this syndrome usually die early in life.

Ultrastructural studies of concentric lamellar bodies in the bronchus of patients with sarcoidosis.

Biopsy specimens of the major bronchus of sixty-nine patients with sarcoidosis were investigated electronmicroscopically. 22 patients (12 males, 10 females), 32% of the cases, showed concentric lamellar or similar kinds of dense bodies in the bronchial wall. Typically, the concentric lamellar bodies were observed in various numbers, scattered in the collagen-increased tunica propria or superficial submucosal layer, where adjacent or deeply located blood capillary vessels were mostly obliterated. They were generally of 0.2 to 1.8 micron in size, but occasionally smaller ones were observed. We also found large swollen and irregular lamellated bodies greater than 2 microns in the cell cytoplasm. Six cases with particularly large numbers of these bodies in the bronchial wall showed "capped" figures, in which the concentric lamellar bodies were partly attached to other demilunar dense bodies. These figures were assumed to be the very early stage of formation of conchoidal bodies at the LM level, so-called Schaumann bodies. Two cases (5%) (chronic bronchitis, lung cancer) among 40 control patients with other diseases disclosed the same small lamellated bodies in the bronchial wall. We were unable to resolve the problem of the origin of these deposited in them, but assume that they will initially occur in the cell cytoplasm in some patients with sarcoidosis and eventually develop into typical concentric lamellar bodies.