Multiple Cardiovascular Manifestations in a Patient with Autosomal Dominant Polycystic Kidney Disease

Autosomal dominant polycystic kidney disease (ADPKD) is a systemic disorder associated with various extrarenal complications. The major cardiovascular complications of ADPKD include valvulopathies and vascular ectasia. A 64-year-old man who was diagnosed with ADPKD seven years previously was admitted to our hospital for heart failure. Pelvic computed tomography revealed multiple variable-sized cysts in both kidneys. Transthoracic echocardiography showed enlargement of the left ventricle and left atrium. Severe mitral regurgitation and moderate aortic regurgitation with annuloaortic ectasia were observed. The left main coronary artery was dilated. The patient had various cardiovascular features associated with ADPKD.

Comparison of Computed Tomography Coronary Angiography and Exercise ECG Test for Diagnostic Accuracy in Real-World Practice / 대한내과학회지

BACKGROUND/AIMS: The exercise ECG test (XECG) and computed tomography coronary angiography (CTCA) have been used widely in initial evaluations of coronary artery disease (CAD) in real-world practice. In this study, we compared the diagnostic power of CTCA and XECG, based on conventional coronary angiography (CCA). METHODS: We enrolled 589 consecutive patients retrospectively who had been examined with both XECG and CTCA for the evaluation of CAD in outpatient clinics. Significant stenosis was defined as more than 50% diameter stenosis. Triage to CCA and/or revascularization treatment (RT) by the results of XECG and CTCA and the diagnostic accuracy of both exams, based on CCA, were investigated. RESULTS: In the 589 patients, 107 (19%) were triaged to CCA for further evaluation; in 77 (12.8%) significant stenosis was detected on CCA. Also, 65 (11%) patients underwent RT. In the CTCA results, 120 patients had significant stenosis. Of them, 58 (48%) and 75 (62%) patients were triaged to RT and CCA, respectively. Based on the XECG, 115 positive patients were triaged to RT and CCA (23 [20%]/41 [35%]). Among 107 patients with CCA, the sensitivity, specificity, positive predictive value, and negative predictive value for significant stenosis on CCA of CTCA were 89.9%, 74.0%, 90.6%, and 71.4%, respectively, and those of XECG were 50.0, 67.9, 78.0, and 37.3, respectively. The kappa value of CCA and CTCA was 0.62 (p < 0.001) and that of CCA and XECG was 0.145 (p = 0.113). CONCLUSIONS: In real-world practice, CCA was decided on more frequently, based on CTCA. CTCA showed better diagnostic accuracy than XECG.

The Clinicopathological Characteristics of Gastrointestinal Neuroendocrine Tumors; An Analysis of 65 Cases

Background : This study was designed to investigate gastrointestinal neuroendocrine tumors with an emphasis on their clinicopathological characteristics. Methods : Sixty-five cases were reviewed and classified as typical carcinoid (TC), atypical carcinoid (AC), large cell neuroendocrine carcinoma (LCNEC) and small cell carcinoma (SmCC). We performed immunohistochemistry to characterize the expression of the immunoreactivity for synaptophysin, chromogranin, gastrin, somatostatin, thyroid transcription factor-1, p53 and Ki-67. Results : Most commonly, the tumors were located in the rectum (54%), followed by the stomach (23%) and colon (9%). Histologically, the tumors were classified as 49 TCs, 4 ACs, 6 LCNECs and 6 SmCCs. Most tumors were stained positive for synaptophysin and/or chromogranin. Four LCNECs and one SmCC were p53-positive. The carcinoids revealed a low level ( or =30% of the cells showed reactivity for Ki-67 in the majority of LCNECs and SmCCs. Six patients with metastatic carcinoids were older than those patients without metastasis (64 vs 48 years, respectively, p=0.004). Furthermore, the size of tumors was larger for the patients with metastatic carcinoids than for the patients with nonmetastatic carcinoids (2.3 vs 0.5 cm, respectively, p=0.005). Conclusion : Old age, large tumor size and muscle invasion are associated with high grade neuroendocrine tumor and lymph node metastasis for those patients with carcinoids.