Clinical Course of Intestinal Behcet's Disease according to the Characteristics of Ulcer in Colonoscopy

BACKGROUND/AIMS: Intestinal involvement with bleeding and perforation is one of the main causes of morbidity and mortality in patients with Behcet's disease (BD); however, the clinical course of intestinal BD has not been defined. The aim of this study was to determine the clinical course of intestinal BD based on the characteristics of ulcers visualized during colonoscopy. METHODS: We retrospectively reviewed the medical records and colonoscopic findings of 41 patients with intestinal BD. All patients were classified into subgroups according to ulcer depth, size, and number, and we analyzed the clinical manifestations, subset type of BD, medications, surgical procedures, and relapse rate among the subgroups. RESULTS: The median age at the time of diagnosis was 38 years (range, 18-74 years); 25 patients were females (M:F=1:1.56). Abdominal pain (n=40), diarrhea (n=27), and RLQ tenderness (n=21) were common symptoms. The number of complete, incomplete, and suspected types was 3, 17, and 21, respectively. In an analysis according to ulcer depth, the rate of steroid use and intestinal surgeries was more frequent in the deep ulcer group compared with the aphthous and shallow ulcer groups (50.0% vs. 0% vs. 17.6%; P=0.012; 42.9% vs. 0% vs. 23.5%: P=0.013) The rate of steroid use and relapse tended to be higher as the size of the ulcer increased; however, there was no difference in the rates of steroid use, surgeries, and relapse based on the number of intestinal ulcers. CONCLUSIONS: The prognosis was worse in patients with intestinal BD who have deeper and larger ulcers. Therefore, we suggest that such patients need to be treated aggressively.

Clinical Course of Intestinal Behcet's Disease according to the Characteristics of Ulcer in Colonoscopy

BACKGROUND/AIMS: Intestinal involvement with bleeding and perforation is one of the main causes of morbidity and mortality in patients with Behcet's disease (BD); however, the clinical course of intestinal BD has not been defined. The aim of this study was to determine the clinical course of intestinal BD based on the characteristics of ulcers visualized during colonoscopy. METHODS: We retrospectively reviewed the medical records and colonoscopic findings of 41 patients with intestinal BD. All patients were classified into subgroups according to ulcer depth, size, and number, and we analyzed the clinical manifestations, subset type of BD, medications, surgical procedures, and relapse rate among the subgroups. RESULTS: The median age at the time of diagnosis was 38 years (range, 18-74 years); 25 patients were females (M:F=1:1.56). Abdominal pain (n=40), diarrhea (n=27), and RLQ tenderness (n=21) were common symptoms. The number of complete, incomplete, and suspected types was 3, 17, and 21, respectively. In an analysis according to ulcer depth, the rate of steroid use and intestinal surgeries was more frequent in the deep ulcer group compared with the aphthous and shallow ulcer groups (50.0% vs. 0% vs. 17.6%; P=0.012; 42.9% vs. 0% vs. 23.5%: P=0.013) The rate of steroid use and relapse tended to be higher as the size of the ulcer increased; however, there was no difference in the rates of steroid use, surgeries, and relapse based on the number of intestinal ulcers. CONCLUSIONS: The prognosis was worse in patients with intestinal BD who have deeper and larger ulcers. Therefore, we suggest that such patients need to be treated aggressively.

A Case of Primary Malignant Melanoma of the Esophagus Treated by Esophagectomy / 대한소화기내시경학회지

A primary malignant melanoma of the esophagus is a very rare disease representing approximately 0.1~0.2% of all esophageal neoplasms and 0.5% of all mucosal melanomas. Despite a surgical resection, a primary malignant melanoma of the esophagus has a poor prognosis, mainly due to the fast development of hematogenous and lymphatic metastases. However, a resection of the tumor appears to be the treatment of choice. Local excision does not prolong the survival of patients. After a radical excision, the mean survival time is 7~12 months and the estimated 5 year survival is 4.2%. However, after a local excision, the average survival time is 9 months and there is no report of a patient who survived more than 1year. We encountered a 55-year-old woman with a primary malignant melanoma of the esophagus, who underwent a radical esophageal resection with a lymph node dissection. We report this case with a review of the relevant literature.

Comparison of Patients' Satisfaction for Transnasal or Transoral Endoscopy in Unsedated Patients / 대한소화기내시경학회지

BACKGROUND/AIMS: The aim of this study was to compare the patient's satisfaction and tolerance of transnasal (TN) and transoral (TO) endoscopy prospectively. METHODS: 120 patients were assigned to undergo TN (age: 49.5, M : F=65 : 55) or TO (age: 47.4, M : F=63 : 57) endoscopy according to their wishes. RESULTS: The patients' satisfaction was higher in the TN group than in the TO group (8.45 vs. 4.95, p <0.05). The degree of choking sensation, nausea, and throat soreness was lower in the TN group than the TO group. There was no difference in endoscopists' satisfaction between the two groups. The duration for TN was longer than for TO. Epistaxis and nasal pain were the common complications in the TN group. All complications were mild and were relieved spontaneously. An endoscopic examination could be completed in all patients in the TO group. In the TN group, examination failure was quite common in the early phase but the incidence decreased with increasing experience. It took the experience of at least 20 cases for the endoscopist to adapt to the TN route. CONCLUSIONS: TN endoscopy is believed to be a comfortable and safe procedure for improving the patients' satisfaction and for reducing the level of inconvenience.

A Case Report of von Hippel-Lindau Disease Manifested in a Monozygous Twin / 대한내분비학회지

Von Hippel-Lindau (VHL) disease is an autosomal dominant disease, which forms hypervascular tumors in multiple organs, such as hemangioblastomas in the retina and central nervous system, renal cell carcinomas, pheochromocytomas and cysts in various organs. Recent advances in gene testing have made it possible to screen family members for VHL disease. We experienced a 28 year-old male, who was diagnosed with bilateral pheochromocytomas through a family screening test when his elder monozygous twin brother was diagnosed with a pheochromocytoma. He received no treatment until December, 2004, when he visited the Emergency room due to a headache. A hemangioma of the cerebellum was seen in the brain MR study, leading to the diagnosis of type 2A VHL disease. An abdominal CT scan revealed no lesions of the pancreas or kidney. There was no evidence of a hemangioma in the retinal scan. The subsequent gene testing showed a germline mutation in exon 3 codon 167 of the VHL gene. The mother of the patient was revealed to have the same mutation of the VHL gene, but the elder brother of the patient did not.