Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder that leads to mucocutaneous telangiectasias, epistaxis, and gastrointestinal bleeding. Depending on the severity and manifestation of the disease, various therapeutic modalities have been used, from local bleeding control to surgery or concomitant drug therapy. Several articles under review have presented guidelines for treatment of HHT with bevacizumab as a direct anti-angiogenesis strategy. Still, neither the exact optimal dose nor the minimum effective dose of intravenous bevacizumab in patients with severe HHT has been reported. A 55-year-old man presented with long-standing epistaxis, recent melena, dizziness, and a three-generation family history of chronic epistaxis, anemia, and regular blood transfusions. Treatment with argon plasma coagulation (APC) for the gastrointestinal bleeding failed to raise hemoglobin levels, we considered using the bevacizumab. We report a patient with severe HHT, who was treated with low-dose bevacizumab (2 mg/kg) and improved substantially.

Superior mesenteric artery (SMA) syndrome is an uncommon disease that results from SMA compression of the third portion of the duodenum. Patients with SMA syndrome present with upper gastrointestinal symptoms, such as nausea, vomiting, and abdominal pain. The diagnosis is usually made from an upper barium study or computed tomography. Typically, SMA syndrome is caused by a decreased aortomesenteric angle of 6° to 25°. An underweight body mass index (BMI) is a risk factor for development of SMA syndrome. There are few reports of the role of linear endoscopic ultrasound (EUS) in the diagnosis of SMA syndrome. We report a case of SMA syndrome, with normal BMI, that was diagnosed with the aid of linear EUS. Although SMA syndrome is not typically within the scope of practice of endosonographers, it is useful to get familiar with the findings.

Background/Aims:While surgical resection remains the standard of care in the treatment of upper urinary tract malignancies, nephrectomy is a risk factor for the development of chronic kidney disease (CKD). The aim of this study was to determine whether histologic evaluation of non-neoplastic kidney could enable early identification of unrecognized kidney disease and could be of prognostic value in predicting postoperative renal outcomes.Methods:We retrospectively analyzed 51 patients with upper urinary tract malignancies who received uninephrectomy or uninephroureterectomy. A thorough pathologic evaluation of non-neoplastic kidney including special stains, immunofluorescence, and electron microscopic studies was performed. The degree of parenchymal changes was graded from 0 to 15.Results:Of 51 patients, only 13 showed normal kidney pathology. Fifteen patients showed glomerular abnormalities, 14 showed diabetic nephropathy, and 11 showed vascular nephropathy. There was one case each of reflux nephropathy and chronic pyelonephritis. The median histologic score was 5 points. Only 25.4% of patients had ≤ 3 points. Score more than 5 was observed in 47.1% of patients. Postoperative estimated glomerular filtration rate (eGFR) at 3 to 36 months were obtained from 90.2% of patients, and of those, 34.8% had de novo CKD. Since no one had CKD in partial nephrectomized patients, we determined risk factors for CKD in radical nephrectomized patients. Cox regression analysis revealed that postoperative AKI, preoperative eGFR, and histologic score of non-neoplastic kidney were the independent predictors for CKD.Conclusions:We conclude that routine pathologic evaluation of non-neoplastic kidney provides valuable diagnostic and prognostic information.

A 52-year-old woman presented with atypical chest pain. Coronary angiography demonstrated multiple microfistulae between left coronary artery and left ventricle (LV) cavity, extensive enough to produce an LV angiogram. This LV angiogram revealed apical hypertrophic cardiomyopathy (HCM) which was confirmed by echocardiography. Coronary steal phenomenon by coronary artery microfistulae and HCM might have a role for developing of angina in patient with apical HCM.

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