Congenital partial defect of the left pericardium presenting as chest pain.

Congenital pericardial defect is a rare cardiac defect with variable clinical presentations. It is usually an unexpected finding during cardiac surgery or autopsy. The clinical detection of congenital absence of pericardium is important because of its life-threatening complications such as fatal myocardial strangulation, myocardial ischaemia and sudden death. We present a patient with the incidental finding of left-sided partial defect of the pericardium during evaluation of chest pain.

A large pericardial cyst compressing the left atrium presenting as a pericardiopleural efussion.

Pericardial cysts are rare congenital abnormalities with a reported incidence rate of 1:100,000, accounting for about 7.6% of all mediastinal masses. These cysts frequently occur in the right cardiophrenic angle and their diagnosis is usually suspected after an abnormal chest X-ray is obtained. Herein, we present a case of pericardial cyst compressing the left atrium complicated by a pericardial effusion and pleural effusion in a 62 year-old man with chest discomfort and dyspnoea. After the pericardial cyst was surgically removed, the histopathological examination revealed an inflamed pericardial cyst lined with mesothelial cells.

A case of pemphigus vulgaris associated with ulcerative colitis.

Pemphigus vulgaris is an autoimmune bullous disorder characterized by the production of autoantibodies against the intercellular space of the epithelium. It has rarely been reported in association with inflammatory bowel disease. Ulcerative colitis is one of the forms of inflammatory bowel disease. A 62-year-old woman who had been treated for ulcerative colitis for 16 years developed pruritic bullae on the skin of her face and body. Histological findings and direct immunofluorescence examination of the skin showed pemphigus vulgaris. She was treated with systemic steroids, mesalazine, and azathioprine. Her cutaneous lesions have remained in remission and her ulcerative colitis has remained well-controlled. The relationship between pemphigus vulgaris and ulcerative colitis is unclear. An autoimmune response has been suspected in the pathogenesis of ulcerative colitis. Pemphigus vulgaris is also associated with an autoimmune mechanism. To our knowledge, this is the first case of ulcerative colitis associated with pemphigus vulgaris reported in Korea. The association may be causal.

Childhood colon cancer in a patient with ataxia telangiectasia.

BACKGROUND: Ataxia-telangiectasia (AT) is a rare autosomal recessive disease characterized by progressive neurologic impairment and cerebellar ataxia. In addition, patients with this disease are known to have an inherent increased susceptibility to the development of cancer, predominantly hematologic malignancies. METHODS: We report the case of a young boy with AT from Russia, who had abdominal pain. Laboratory tests and radiologic examinations were performed to him. RESULTS: After abdominal computed tomography (CT), colonoscopy and surgical interventions, the young boy was diagnosed with colon cancer that had signet ring cell features. CONCLUSIONS: It is known that the patient with AT appeared to be predisposed to various tumors, including leukemia or lymphoma, which are more common in childhood. Even if the patient with AT could have solid tumor such as stomach cancer or breast cancer, it is less likely to have colon cancer, especially signet ring cell type. Actually, no case of colon cancer has ever been reported, especially in young patient and hence, we have focused on this point and are hereby reporting this unique case.