Clinical Significance of Incidentally Detected Eosinophilic Esophagitis with Pathologic Review / 대한소화기학회지

BACKGROUND/AIMS: Eosinophilic esophagitis (EE) is a chronic inflammatory disorder characterized by abnormal dense eosinophilic infiltration of esophageal mucosa and results in dysphasia and food impaction. EE is being increasingly recognized in adults. The prevalence is largely unknown. This study was performed to evaluate the detection rate of EE diagnosed based on pathologic criteria and to define the clinical characteristics of EE in Korea. METHODS: We reviewed biopsy specimen of the 1,609 patients who underwent esophageal biopsy from January 2006 till August 2008. The presence of more than 20 eosinophils per high power field in biopsy specimens was considered cases of EE. Clinical information and endoscopic findings were obtained. RESULTS: 7 (0.4%) patients were diagnosed as EE based on pathologic criteria retrospectively. Clinical symptoms were epigastric pain (43%), regurgitation (29%), dyspepsia (14%), and no symptom (14%). Endoscopic findings were whitish exudates or granules (57%), esophageal polyp (29%), and hyperemic change (14%). Two patients received treatment. One patient with bronchial asthma improved after treatment with inhaled corticosteroid, and one patient improved after 8 week proton pump inhibitor therapy. CONCLUSIONS: Eosinophilic esophagitis was found in 0.4% of the total esophageal biopsied cases. Our results suggest that Korean patients with eosinophilic esophagitis showed symptoms mimicking gastroesophageal reflux disease and atypical endoscopic findings. Therefore, regardless of the gross appearance of the mucosa, meticulous diagnostic approaches are needed for patients with swallowing difficulty and lack of response to proton pump inhibitor.

Congenital Angiodysplasia in a Woman Presenting with Idiopathic Jejunal Varicosis on Angiography

Angiography is a useful diagnostic tool in cases with massive gastrointestinal bleeding such as angiodysplasia and varicosis when endoscopy is not available. Angiodysplasia and varicosis have distinguishable characteristic features on angiography, such as the presence of a nidus, visible late-draining veins, and the typical vascular tuft. We recently treated a rare case of congenital angiodysplasia without the characteristic angiodysplasia features on angiography. Instead, the patient presented with a very rare case of idiopathic jejunal varicosis. A 42-year-old woman visited the emergency room with the chief complaint of melena for three days and a hemoglobin level of 5.9 g/dL. An abdominal CT angiogram showed varicosis at the jejunal mesentery. Angiography of the superior and inferior mesenteric arteries showed tortuous and dilated jejunal and ileal branches during the venous phase, suggesting a vascular malformation such as varicosis of the jejunum. Surgical exploration with intraoperative endoscopy revealed diffuse engorged veins and a 1.0-cm-diameter superficial ulcer covered with a blood clot that was 70 cm from the ligament of Treitz. A 100-cm segment of jejunum was resected. Histological examination revealed that the lesion was angiodysplasia, not varicosis. The final diagnosis was congenital angiodysplasia.

Effects of Pamidronate Treatment on Osteogenesis Imperfecta / 대한내분비학회지

BACKGROUND: Osteogenesis imperfecta (OI) is a congenital disorder of type I collagen, with variable phenotypes, due to increased bone fragility and low bone mass. Previous pharmacological treatments for OI have been attempted with calcitonin and growth hormone but with little beneficial effects. Recently, Glorieux reported the beneficial effects of bisphosphonates in OI. METHODS: In this study, the effects of pamidronate treatment were evaluated in 9 patients with OI. All patients received intravenous pamidronate infusions, which was dose adjusted according to the patients' age. The outcome measures included the biochemical bone markers; serum alkaline phosphatase, urine deoxy-pyridinoline, urine Ca/Cr ratio, and bone mineral density (BMD). RESULTS: Serum alkaline phosphatase, urine deoxypyridinoline, and urine Ca/Cr ratio were slightly decreased after 1 year of therapy, although these changes were not statistically significant. The BMDs of the lumbar spine and proximal femur were significantly increased after 1-year of pamidronate treatment. No fractures were reported during the 1 year treatment periods. CONCLUSION: Pamidronate treatment had an effect on the BMD in osteogenesis imperfecta, probably due to decreasing bone resorption

A Case of Primary Signet Ring Cell Carcinoma of Lung with Multiple Bone Metastasis / 결핵및호흡기질환

Signet ring cell carcinoma of lung is an unique variant of mucin producing adenocarcinoma which is characterized by abundant intracellular mucin accumulation. Only a few cases of primary signet ring cell carcinoma of lung have been reported in the world wide literature. And we have, recently experienced one case of primary signet ring cell carcinoma of lung. A 55 years old man was evaluated for paralysis of lower extremities and was found to have lung cancer in the left upper and lower lobe with pleural, multiple spinal, bone and liver metastases. Signet ring tumor cells were revealed by cytologic examination of pleural fluids. And there were no evidence of signet ring cell carcinoma of other organs. Primary signet ring cell carcinoma of lung seems to have an aggressive behavior and therapeutic modalities could be different from those for signet ring cell carcinomas from other organs. Therefore it is important to separate primary signet ring cell adenocarcinoma of lung from metastatic tumors.

Two Cases of Microscopic Polyangiitis / 결핵

Microscopic polyangiitis is a systemic small-vessel vasculitis that is primarily associated with necrotizing glomerulonephritis and pulmonary capillaritis. Lung involvement is characterized by a diffuse alveolar hemorrhage. However, rarely central nervous system involvement has been reported to be occurred with the microscopic polyangiitis. Relapse of microscopic polyangiitis are reported to be more frequent than those of polyarteritis nodosa, often after a reduction or discontinuation of the therapy. We would like to report two patients with microscopic polyangiitis. One presented with clinical manifestations of both lung and central nervous system involvements and the other was a case of recurrence during steroid tapering following the steroid pulse therapy.