Familial Mediterranean fever (FMF) is an inherited autosomal recessive disorder, ethnically restricted and commonly found among populations surrounding the Mediterranean Sea. FMF is the most prevalent autoinflammatory disease; is characterized by recurrent, self-limited episodes of fever with serositis; and is caused by Mediterranean fever gene (MEFV) mutations on chromosome 16. We describe a case of adult-onset FMF with complete symptomatic remission during pregnancy, without the use of colchicine. A 25-year-old woman had presented with periodic fever, abdominal pain, and vomiting since she was 21. Her abdominal computed tomography scan showed intestinal nonrotation. She underwent exploratory laparotomy and appendectomy for her symptoms 1 year prior. She had a symptom-free pregnancy period, but abdominal pain and fever recurred after delivery. Mutation analysis of the MEFV gene revealed two point mutations (p.Leu110Pro and p.Glu148Gln). We report an adult female patient with FMF in Korea with complete symptomatic remission during pregnancy.
Abdominal Pain ; Adult ; Appendectomy ; Chromosomes, Human, Pair 16 ; Colchicine ; Familial Mediterranean Fever* ; Female ; Fever ; Humans ; Korea ; Laparotomy ; Mediterranean Sea ; Point Mutation ; Pregnancy* ; Serositis ; Vomiting

Stenotrophomonas maltophilia is an ubiquitous aerobic Gram-negative bacillus. Hospitalization and prior antibiotic therapy are risk factors for S. maltophilia infection. This organism is isolated with increasing frequency from hospitalized patients and may cause therapeutic problems because of its intrinsic resistance to common antibiotics and the immunodeficiency status of the affected host. S. maltophilia has been reported to be commonly associated with pneumonia and urinary tract infection. However, biliary infection caused by S. maltophilia is very rare. Herein, we report on a case of acute cholecystitis that developed secondary to S. maltophilia bacteremia in a patient with hepatitis-B related liver cirrhosis and gallbladder stone.
Anti-Bacterial Agents ; Bacillus ; Bacteremia* ; Cholecystitis, Acute* ; Gallbladder ; Hospitalization ; Humans ; Liver Cirrhosis ; Pneumonia ; Risk Factors ; Stenotrophomonas maltophilia* ; Urinary Tract Infections

Pulmonary embolism is a common clinical problem in patients with immobilization, cancer, indwelling central venous catheter and surgery. However, although rare, it may occur in patients with inherited thrombophilia. Protein S deficiency is known to increase the risk of venous thrombosis and pulmonary embolism. There are many reports of venous thrombosis with protein S deficiency, but there are few reports of arterial thrombosis, especially recurrent acute pulmonary embolism. Here, we report a case of recurrent pulmonary embolism associated with type II protein S deficiency.
Blood Coagulation Disorders, Inherited ; Central Venous Catheters ; Humans ; Immobilization ; Protein S ; Protein S Deficiency ; Pulmonary Embolism ; Thrombophilia ; Thrombosis ; Venous Thrombosis

BACKGROUND/AIMS: Colonoscopic polypectomy is an effective tool for the treatment of colonic polyps. With the recent widespread use of colonoscopic polypectomy, there is a growing concern about iatrogenic complications, such as bleeding and perforation. The aim of this study is to analyze the clinical presentation and management of complications during colonoscopic polypectomy. METHODS: We retrospectively reviewed the medical records of colonoscopic examinations performed at 5 hospitals of Hallym University Medical Center between June 2005 and June 2008. RESULTS: A total of 26,773 colonoscopies and 4,123 colonoscopic polypectomy were performed. The overall rate of complication was 1.04% (43/4,123). Perforation occurred in 0.19% (8/4,123) of cases, and bleeding occurred in 0.85% (35/4,123) of cases. Perforation occurred in 0.05% of snare polypectomies, 1.09% of endoscopic mucosal resections (EMR), and 7.14% of endoscopic submucosal dissections (ESD). Bleeding occurred in 0.8% of snare polypectomies, 1.09% of EMRs, and 3.57% of ESDs. The complication rate of ESDs was higher than in EMRs and snare polypectomies (P<0.001). Endoscopic clippings were performed in 25% of perforation and 66.7% of bleeding cases. Medical treatment was successful in 75% of perforation and 100% of bleeding cases. CONCLUSIONS: Colonoscopic polypectomy is a safe procedure for the treatment of colonic polyps, but rarely, serious complications occur. Compared to a snare polypectomy or an EMR, the complication of an ESD occurs more frequently. Endoscopic treatment and further conservative management seems to be appropriate in most cases with complication.
Academic Medical Centers ; Colonic Polyps ; Colonoscopy ; Hemorrhage ; Medical Records ; Retrospective Studies ; SNARE Proteins

Central venous stenosis or occlusion occurs in 11-50% of hemodialysis patients with prior subclavian vein cannulation and ipsilateral fistula or shunt. Most patients are asymptomatic but some require treatment to reduce the risk of thrombosis and improve inadequate hemodialysis pressure. In these cases, endovascular intervention, including ballooning and stenting, is a feasible strategy for selected patents. We report an unusual case of a 40-year-old man on hemodialysis that underwent endovascular stenting to treat right subclavian vein stenosis and experienced stent migration to the right ventricle, requiring surgical removal.
Adult ; Catheterization ; Constriction, Pathologic ; Fistula ; Heart Ventricles ; Humans ; Renal Dialysis ; Stents ; Subclavian Vein ; Thrombosis

Central venous catheterization is commonly used for supplying large amounts of fluids, total parenteral nutrition and for monitoring central venous pressure. Numerous complications exist with the technique, including pneumothorax, arterial puncture with vessel injury, catheter embolus, mediastinal hematoma, hydrothorax, and the thrombus of the vein. We reported an uncommon case of pleural effusion, due to catheter tip migration and penetration, which occurred 4 days after central venous catheterization.
Catheterization, Central Venous ; Catheters ; Central Venous Catheters ; Central Venous Pressure ; Embolism ; Glycosaminoglycans ; Hematoma ; Hydrothorax ; Parenteral Nutrition, Total ; Pleural Effusion ; Pneumothorax ; Punctures ; Subclavian Vein ; Thrombosis ; Veins

BACKGROUND: Stomach cancer is the most common cancer in Korea, and it can be easily diagnosed by performing endoscopy. Helicobacter pylori (H. pylori) is associated with stomach cancer, and there has been much recent interest in the epidemiology of stomach cancer. Therefore, we estimated the incidence of stomach cancer in Chuncheon-si during 2000-2002. METHODS: From July 1st, 2000 through June 30th, 2002, we investigated the subjects who were confined to be newly diagnosed cases living in Chuncheon-si. The data was collected from the medical records from all the medical facilities located in Chuncheon-si. RESULTS: The total number of cases of newly diagnosed stomach cancer during this period (2000-2002) was 186 (117 males and 69 females). The annual crude incidence rate of stomach cancer was estimated to be 37.0 per 100,000 persons overall, and 47.0 and 27.2 in the males and females, respectively. The annual age standardized incidence rate for the Korean population was 33.7 per 100,000 persons overall, and 42.2 and 25.1 for the males and females, respectively. The major symptoms of stomach cancer patients were epigastric pain (61.8%) and weight loss (31.2%). The proportion of early gastric cancer was 34.9% (60 among 172 persons) of the cases of stomach cancer. 64 patients (74%) were H. pylori positive. CONCLUSIONS: We report here on the estimated incidence rate of the stomach cancer and the characteristics of the stomach cancer patients in Chuncheon-si during 2000-2002. This study has generated basic epidemiologic data to identify the effect of H. pylori eradication on the future incidence rate of stomach cancer.
Endoscopy ; Epidemiology ; Female ; Gangwon-do* ; Helicobacter pylori ; Humans ; Incidence* ; Korea ; Male ; Medical Records ; Stomach Neoplasms* ; Stomach* ; Weight Loss

PURPOSE: Ampullary carcinoid tumors are rare and therefore the clinicopathologic characteristics and prognosis after radical surgery have yet to be clarified. The goal of this study was to analyze the outcome of ampullary carcinoid tumors in patients who underwent radical curative resection. METHODS: From January 1998 to December 2005, 10 patients (3.4%) were diagnosed with an ampullary carcinoid tumor among 294 patients who underwent pancreatoduodenectomy for various ampullary neoplasms. The clinical findings from these 10 patients were retrospectively analyzed. RESULTS: The mean patient age was 58.0 +/- 13.4 years and seven were male. A standard pancreatoduodenectomy was performed in three patients and pylorus-preserving pancreatoduodenectomy in seven. An R0 resection was achieved in all 10 patients. The mean tumor size was 2.1 +/- 1.3 cm. Synaptophysin staining was positive in 10 and chromogranin staining was positive in eight patients. The overall and disease-free survival rates were 90% and 80% at 1 year and 64% and 56% at 3 years, respectively. Univariate analyses revealed that a maximum tumor diameter > or = 2 cm and tumor invasion beyond the ampulla were significant risk factors for tumor recurrence. CONCLUSIONS: The results of this study showed that performing a radical resection is the treatment of choice, with the intention of total tumor removal and the possibility of cure.
Carcinoid Tumor* ; Carcinoma, Neuroendocrine ; Disease-Free Survival ; Humans ; Intention ; Male ; Pancreaticoduodenectomy ; Prognosis ; Recurrence ; Retrospective Studies ; Risk Factors ; Synaptophysin

BACKGROUND: Type 2 diabetes develops because of defects in both insulin secretion and action. The half-life of insulin in uremia is prolonged because the metabolic clearance rate of insulin in diabetic end stage renal disease (ESRD) patients is reduced with consequence that the dose of insulin and/or oral hypoglycemic agent (OHA) administered in normal renal function make them increase the risk of hypoglycemia. Therefore, we should usually reduce the dose of insulin and/or OHA, or stop administration of insulin and/or OHA if type 2 diabetic patients are progressed to ESRD. But in some patients, that is not true. The aim of this study was to test the hypothesis that insulin resistance plays an important role in (re)evaluation of optimal insulin and/or OHA dose for glycemic control after type 2 diabetic patients are progressed to ESRD. METHODS: Insulin resistance was examined in 23 type 2 diabetic ESRD patients with tight control of glycemia using the K index of the insulin tolerance test (Kitt). We divided 23 patients into three groups. Group 1 (n=10) was defined as patients who were administered neither insulin nor OHA after ESRD. Group 2 (n=9) was defined as patients who were changed from insulin to OHA as drug for glycemic control after ESRD. Group 3 (n=4) was defined as patients in whom insulin or OHA was continuously administered after ESRD without a change of them for glycemic control. We compared the degree of insulin resistance among these three groups. RESULTS: Insulin resistance determined by Kitt was significantly different between group 1 (Kitt, 2.1422/0.94-4.01%/min), group 2 (Kitt, 1.3811/0.79- 3.90%/min) and group 3 (Kitt, 0.8550/0.44-1.81%/min) by using Kruskal-Wallis test (p=0.048). Kitt in group 3 was significantly lower than in group 1 by using Mann-Whitney test (p=0.016). CONCLUSION: Although metabolic clearance of insulin is reduced by renal failure, demand of insulin/ OHA for optimal glycemic control is not reduced in higher insulin-resistant type 2 diabetic ESRD patients on hemodialysis. Insulin resistance plays an important role in determination of optimal insulin/ OHA dose for glycemic control after type 2 diabetic patients are progressed to ESRD.
Half-Life ; Humans ; Hypoglycemia ; Insulin Resistance* ; Insulin* ; Kidney Failure, Chronic* ; Metabolic Clearance Rate ; Renal Dialysis* ; Renal Insufficiency ; Uremia

PURPOSE: To make a histological study of a urethral plate using light and transmission electron microscopies to find evidence responsible for ventral curvature in hypospadias. MATERIALS AND METHODS: Full thickness biopsies of the urethral plate were obtained from 5 patients with proximal hypospadias; including 4 children and 1 adult. All specimens were examined with light and transmission electron microscopies. RESULTS: With the light microscope, all specimens demonstrated well vascularized connective tissue, comprised of smooth muscle and collagen. With the transmission electron microscope, all specimens demonstrated intact endothelial cells and fibroblasts, with dispersed chromatin distribution, intercellular loose collagen and a regular arrangement of collagen fibrils in intercellular spaces. No histological evidence of fibrous cord could be found in any of the specimens. CONCLUSIONS: No histological evidence of fibrous cord could be historically found that could be considered responsible for ventral penile curvature in hypospadias.
Adult ; Biopsy ; Child ; Chromatin ; Collagen ; Connective Tissue ; Endothelial Cells ; Extracellular Space ; Female ; Fibroblasts ; Humans ; Hypospadias ; Male ; Microscopy ; Microscopy, Electron ; Microscopy, Electron, Transmission* ; Muscle, Smooth ; Urethra