Chronic atherosclerotic occlusion of the abdominal aorta is a rare disease in clinical practice. In this disease, juxtarenal aortic occlusion is defined as the condition extended, adjacent to the renal arteries. The treatment of juxtarenal aortic occlusion is more difficult than a 'simple' abdominal aortic occlusion. Vascular surgery of a juxtarenal aortic occlusion-specifically aortic endarterectomy and bypass grafting-is a challenging procedure that almost invariably requires aortic cross-clamping above the level of the renal arteries, and may be associated with significant morbidity and mortality. With the advent of endovascular treatment, percutaneous transluminal angioplasty and stent implantation have been used increasingly as an alternative to conventional surgery in the management of patients with aortoiliac disease. However, endovascular treatment for juxtarenal aortic occlusion is not common and, also, special attention is needed with regard to possible renal complications. Here, we report the successful revascularization of a case of chronic juxtarenal aortic occlusion with endovascular treatment and adjunctive anticoagulation.
Angioplasty ; Aorta, Abdominal ; Endarterectomy ; Endovascular Procedures ; Humans ; Leriche Syndrome ; Rare Diseases ; Renal Artery ; Stents

Hematopoietic cell transplantation recipients have a high risk of subsequent malignant neoplasms. Among post-transplantation lymphoproliferative disorders (PTLDs) after hematopoietic cell transplantation, Hodgkin's lymphoma is rare and distinct from the majority of other PTLDs because of its later onset and relatively good prognosis. It is known to be associated with exposure to the Epstein-Barr virus, and the mixed cellularity subtype is the most common. We herein describe two cases of Hodgkin's lymphoma that developed after allogeneic hematopoietic cell transplantation.
Cell Transplantation ; Hematopoietic Stem Cell Transplantation ; Herpesvirus 4, Human ; Hodgkin Disease ; Lymphoproliferative Disorders ; Prognosis ; Transplants

Renal artery pseudoaneurysm is an uncommon disease. A 47-year-old man complained of recently developed gross hematuria and left flank pain. The initial laboratory study and radiographic evaluation revealed acute pyelonephritis. After the treatment with antibiotics, gross hematuria was resolved and the patient was discharged. However, he revisited with repeated gross hematuria and throbbing left flank pain. The renal angiography revealed left intra-renal arterial pseudoaneurysm. Angiographic embolization by glue injection was successfully performed. Thereafter, gross hematuria and flank pain were disappeared. We report a successful glue embolization of intra-renal artery pseudoaneurym associated with acute pyelonephritis.
Adhesives ; Aneurysm, False ; Angiography ; Anti-Bacterial Agents ; Arteries ; Flank Pain ; Hematuria ; Humans ; Middle Aged ; Pyelonephritis ; Renal Artery

Colonic lipoma, a very rare form of benign tumor, is typically detected incidentally in asymptomatic patients. The size of lipoma is reported variously from 2 mm to 30 cm, with higher likelihood of symptoms as the size is bigger. Cases with symptom or bigger lesion are surgically resected in principle; endoscopic resection, which has developed recently with groundbreaking advance of endoscopic excision technology, is being used more often but with rare report of success due to high chance of complications such as bowel perforation or bleeding. The authors report here, together with a literature review, our experiences of three cases of giant colonic lipomas showing complete remission after aggressive unroofing technique, at certain intervals, using snare catheter at the origin of the lipoma so that the remaining lipoma could be drained out of the exposed surface spontaneously, in order to reduce complications.
Catheters ; Colon ; Colonoscopy ; Hemorrhage ; Humans ; Lipoma ; SNARE Proteins

PURPOSE: The aim of the present study was to investigate the clinical features and outcome of eosinophilic gastroenteritis (EGE) in children. METHODS: Our study enrolled 24 children who were diagnosed with EGE from 1993 to 2014 at the Department of Pediatrics, Seoul National University Children's Hospital. The patients' clinical manifestations, treatments, and outcomes were reviewed from the medical records. RESULTS: The mean age at diagnosis was 5.3 years. Most patients had gastrointestinal symptoms including diarrhea (54.2%) and abdominal pain (45.8%). Peripheral eosinophilia was present in 91.7% of the patients. Thirteen patients (54.2%) showed anemia, and 15 patients (62.5%) had hypoalbuminemia. EGE was classified as mucosal, subserosal, or muscular in 75.0%, 20.8%, and 4.2% of cases, respectively. Three patients showed gastroduodenal ulcers upon endoscopic analysis. A history of allergy was reported in 13 patients, including atopic dermatitis, allergic rhinitis, and asthma. Five patients (20.8%) improved with food restrictions. Among the 19 patients treated with steroids, 11 (57.9%) discontinued steroid treatment without subsequent relapse, 4 (21.1%) relapsed after ceasing steroid treatment, and 4 (21.1%) showed no response to steroids. Two patients who were resistant to steroids underwent therapeutic surgery. The presence of gastroduodenal ulcers was significantly associated with relapse and steroid resistance. CONCLUSION: A high suspicion of EGE is warranted when children have nonspecific gastrointestinal symptoms and peripheral eosinophilia. Most patients improved with food restrictions or steroid treatment, although one-third of patients showed a relapse or steroid resistance.
Abdominal Pain ; Anemia ; Asthma ; Child* ; Dermatitis, Atopic ; Diagnosis ; Diarrhea ; Eosinophilia ; Eosinophils* ; Gastroenteritis* ; Humans ; Hypersensitivity ; Hypoalbuminemia ; Medical Records ; Pediatrics ; Peptic Ulcer ; Recurrence ; Rhinitis ; Seoul ; Steroids ; Ulcer

PURPOSE: Parenteral nutrition (PN) not only provides nutritional support but also plays a crucial role in the treatment of children with intestinal failure. The aim of this study was to evaluate the clinical significance and clinical outcomes of long-term PN. METHODS: Retrospective cohort study was conducted using the medical records of patients treated at Seoul National University Children's Hospital. This study included 19 patients who received PN for over six months. Most patients received home PN. RESULTS: The indications for PN included short bowel syndrome, chronic intestinal pseudo-obstruction, and intractable diarrhea of infancy. The median age of PN initiation was 1.3 years, and the median treatment duration was 2.9 years. Two patients were weaned from PN; 14 continued to receive PN with enteral feedings; and 3 patients died. The overall survival rates at 2 and 5 years were 93.3% and 84.0%, respectively. The incidence of catheter-related bloodstream infections was 2.7/1,000 catheter-days and was associated with younger age at PN initiation and lower initial height Z-score. Six patients developed catheter-related central vein thrombosis, with an incidence of 0.25/1,000 catheter-days. Eleven patients experienced PN-associated liver disease (PNALD), and one patient underwent multi-visceral transplant. The patients with PNALD exhibited lower final heights and body weight Z-scores. All patients experienced micronutrient deficiencies transiently while receiving PN. CONCLUSION: PN is an important and safe treatment for pediatric intestinal failure. PNALD was linked to final anthropometric poor outcomes. Micronutrient deficiencies were common. Anthropometric measurements and micronutrient levels must be monitored for successful PN completion.
Body Weight ; Catheter-Related Infections ; Child ; Cholestasis ; Cohort Studies ; Diarrhea ; Humans ; Incidence ; Intestinal Pseudo-Obstruction ; Liver Diseases ; Medical Records ; Micronutrients ; Nutritional Support ; Parenteral Nutrition ; Retrospective Studies ; Seoul ; Short Bowel Syndrome ; Survival Rate ; Thrombosis ; Veins

PURPOSE: The aim of our study was to establish a safe and convenient diagnostic method for acute gastrointestinal (GI) graft-versus-host disease (GVHD) in children by determining the sensitivity and negative predictive values of upper and lower endoscopic biopsies for children suspected of GI GVHD. METHODS: Patients suspected of GI GVHD who received endoscopic evaluation within 100 days after stem cell transplantation and endoscopies between January 2012 and March 2014 in Seoul National University Children's Hospital were included in our study. RESULTS: Fifteen patients with a total of 20 endoscopic procedures were included in our study. Sensitivity at the esophagus, stomach, and duodenum were 22.2%, 30.0%, and 80.0%, respectively. Negative predictive values at the esophagus, stomach, and duodenum were 22.2%, 30.0%, and 60.0%, respectively. Overall sensitivity and negative predictive values of upper endoscopic biopsy for GVHD were 77.8% and 50.0%, respectively. Overall sensitivity and negative predictive values of lower endoscopic biopsy for GVHD were 88.9% and 66.7%, respectively. CONCLUSION: We recommend flexible sigmoidoscopy as a safe and accurate diagnostic tool for GVHD, similar to other studies reported previously. However, if there is no evidence of GVHD on sigmoidoscopy with high index of suspicion of GI bleeding, full colonoscopy and upper endoscopy should be considered.
Biopsy ; Child ; Colonoscopy ; Duodenum ; Endoscopy* ; Esophagus ; Graft vs Host Disease* ; Hemorrhage ; Humans ; Seoul ; Sigmoidoscopy* ; Stem Cell Transplantation ; Stomach

Anomalous origin of a coronary artery is rare and does not generally lead to myocardial infarction and paroxysmal supraventricular tachycardia (PSVT). We report an uncommon case of anomalous origin of the right coronary artery (RCA) originating from the left sinus of Valsalva with PSVT and myocardial ischemia. A 58-year-old man presented with PSVT. After arrhythmia subsided, electrocardiogram showed ST and T wave abnormalities, and transient cardiac enzymes were found to be elevated. Coronary CT angiography confirmed that there was anomalous origin of the RCA originating from the left sinus of Valsalva and no intracoronary stenotic lesion. He was managed with conservative treatment, having no symptoms on clinical follow-up for 4 years.
Angiography ; Arrhythmias, Cardiac ; Coronary Vessel Anomalies ; Coronary Vessels ; Electrocardiography ; Follow-Up Studies ; Humans ; Multidetector Computed Tomography ; Myocardial Infarction ; Myocardial Ischemia ; Sinus of Valsalva ; Tachycardia, Paroxysmal ; Tachycardia, Supraventricular

Anomalous origin of a coronary artery is rare and does not generally lead to myocardial infarction and paroxysmal supraventricular tachycardia (PSVT). We report an uncommon case of anomalous origin of the right coronary artery (RCA) originating from the left sinus of Valsalva with PSVT and myocardial ischemia. A 58-year-old man presented with PSVT. After arrhythmia subsided, electrocardiogram showed ST and T wave abnormalities, and transient cardiac enzymes were found to be elevated. Coronary CT angiography confirmed that there was anomalous origin of the RCA originating from the left sinus of Valsalva and no intracoronary stenotic lesion. He was managed with conservative treatment, having no symptoms on clinical follow-up for 4 years.
Angiography ; Arrhythmias, Cardiac ; Coronary Vessel Anomalies ; Coronary Vessels ; Electrocardiography ; Follow-Up Studies ; Humans ; Multidetector Computed Tomography ; Myocardial Infarction ; Myocardial Ischemia ; Sinus of Valsalva ; Tachycardia, Paroxysmal ; Tachycardia, Supraventricular

Cetuximab, a chimeric mouse-human immunoglobulin, is an antiepidermal growth factor receptor monoclonal antibody. It has been approved by the U.S. Food and Drug Administration for the treatment of metastatic colorectal and head/neck cancer, but can cause fatal hypersensitivity reactions in some patients. A 66-year-old male with metastatic sigmoid cancer had cetuximab-induced anaphylaxis when the first dose of cetuximab was administered. Cetuximab was safely readministered for another 15 cycles based on the rapid desensitization protocol. We experienced a case of cetuximab-induced anaphylaxis on the first exposure which was successfully managed by rapid desensitization.
Aged ; Anaphylaxis* ; Desensitization, Immunologic ; Humans ; Hypersensitivity ; Immunoglobulins ; Male ; Sigmoid Neoplasms ; United States Food and Drug Administration ; Cetuximab