Von Recklinghausen's disease is an autosomal dominant hereditary disease associated with characteristic cafeau-lait spots of skin and multiple neurofibromatosis. It is complicated by malignancies, which in most cases is neurofibrosarcoma. The development of lung cancer in von Recklinghausen's disease is rare. A 61-year-old male was admitted for cough and sputum for 20 days. He had multiple cafe-au-lait spots and subcutaneous neurofibromas in whole body area and Lisch nodules in both iris and he had been diagnosed von Recklinghausen s disease 35 years ago. Chest radiography showed emphysematous buliae in both upper lung field and mass in right upper lung field. Chest CT scan revealed subcarinal lymph node enlargement. Bronchoscopic biopsy was done in mass in superior segment of right lower lobe and the results showed squamous cell carcinoma. The presence of von Recklinghausen's disease and lung cancer are noteworthy.
Biopsy ; Cafe-au-Lait Spots ; Carcinoma, Squamous Cell ; Cough ; Genetic Diseases, Inborn ; Humans ; Iris ; Lung Neoplasms* ; Lung* ; Lymph Nodes ; Male ; Middle Aged ; Neurofibroma ; Neurofibromatoses ; Neurofibromatosis 1* ; Neurofibrosarcoma ; Radiography ; Skin ; Sputum ; Thorax ; Tomography, X-Ray Computed

Measlazine(5-Aminosalicylic acid, 5-ASA), a component of Sulfasalazine (sulfapyridine bound to 5-ASA), is used to treat inflammatory bowel disease. Most adverse pulmonary effects caused by sulfasalazine have been attributed to sulfapyridine. However, lung toxicity associated with measlazine(5-ASA) is rare. Here we report a case of eosinophilic pneumonia in a 44-year-old woman with ulcerative colitis, who was treated with mesalazine. She developed symptoms of a dry cough, mild night fever, and evertional dyspnea. Bilateral peripheral pulmonary infiltrates, peripheral blood eosinophilia, and histologic features were consistent with eosinophilic pneumonia. The symptoms improved quite rapidly after the discontinuation of mesalazine and initiation of steroid therapy. Therefore, adverse pulmonary reactions to mesalazine must be considered in a differential diagnosis of pulmonary involvement in patients with inflammatory bowel disease who are receiving with measlazine therapy.
Adult ; Colitis, Ulcerative* ; Cough ; Diagnosis, Differential ; Dyspnea ; Eosinophilia ; Eosinophils* ; Female ; Fever ; Humans ; Inflammatory Bowel Diseases ; Lung ; Mesalamine ; Pulmonary Eosinophilia* ; Sulfapyridine ; Sulfasalazine ; Ulcer*

NSAIDs are now the most widely prescribed of all drugs for the therapy of a large variety of disorders including rheumatologic disorders, and so the population of patients who are at risk for adverse effects of these drugs is rapidly expanding. A number of renal and electrolyte problems have been associated with the use of NSAIDs, including alterations in glome-rular filtration rate, hyperkalemia, acute interstitial nephritis and papillary necrosis. While the use of NSAIDs has also been associated with minimal change nephrotic syndrome, this complication has almost invariably occured in association with an acute interstitial nephritis. Recently, we experienced a case of minimal change nephrotic syndrome without significant interstitial inflammation associated with the use of NSAIDs. This patient is a 64-year-old female who developed the generalized edema and about 10kg of weight gain since three days ago. She had taken the anti-inflammatory drugs for five years intermittently and started taking diclofenac sodium, 25mg orally three times a day, 10 days before admission for increasing pain in her knees. Laboratory findings disclosed the following values WBC 5,200/mm3 with only 0.6% eosinophils, total serum protein 4.0g/dL, albumin 1.2 g/dL, BUN 17mg/dL, creatinine 0.8mg/dL, sodium 140 mmol/L, potassium 4.0mmol/L, chloride 113mmol/L, total cholesterol 338mg/dL, triglyceride 203mg/dL; 24-hour protein excretion 3.6g, creatinine clearance 52.8 mL/min ; serologic tests were unremarkable. A renal biopsy revealed no abnormality except for focal mild interstitial infiltration of chronic inflammatory cells with a few atrophic tubules on light microscopy. Immunofluorescence studies showed diffuse trace mesangial deposits of IgM, and electromicroscopy revealed diffuse obliteration of the epithelial foot process and villous transformation of the epitherial cell cytoplasms without electrondense deposits. These findings were consistent with minimal-change disease. Diclofenac was discontinued on admission because of the likelihood the renal disease was drugrelated and she treated with low-dose(40-80mg/d) of furosemide. Fourteen days after stopping diclofenac, her massive edema and weight gain resolved and laboratory studies showed a 24-hour urine protein excretion of 80mg and serum albumin of 2.7g/dL. There has been no relapse for five months since then.
Anti-Inflammatory Agents, Non-Steroidal ; Biopsy ; Cholesterol ; Creatinine ; Cytoplasm ; Diclofenac* ; Edema ; Eosinophils ; Female ; Filtration ; Fluorescent Antibody Technique ; Foot ; Furosemide ; Humans ; Hyperkalemia ; Immunoglobulin M ; Inflammation ; Knee ; Microscopy ; Middle Aged ; Necrosis ; Nephritis, Interstitial ; Nephrosis, Lipoid ; Potassium ; Recurrence ; Serologic Tests ; Serum Albumin ; Sodium ; Triglycerides ; Weight Gain

Alendronate sodium (Fosamax(R)) is an aminophosphonate used in treatment of postmenopausal osteoporosis by inhibition of osteoclastic activity. Esophageal ulcers related to alendronate use have been reported at the rate of 2~6%. However, there have been no reports of esophagitis or esophageal ulcer by alendronate in Korea. We present a case of esophageal ulcer caused by alendronate. The patient presented with retrosternal pain and severe odynophagia for a few days. Esophagogastroduodenoscopy revealed multiple deep ulcers at the esophagus. Her symptoms resolved completely after stopping alendronate. A follow-up esophagogastroduodenoscopy 6 weeks later revealed complete healing of the ulcers.
Alendronate* ; Endoscopy, Digestive System ; Esophagitis ; Esophagus ; Female ; Follow-Up Studies ; Humans ; Korea ; Osteoclasts ; Osteoporosis, Postmenopausal ; Ulcer*

Behcet's disease is a complex multisystem disease that features recurrent aphthous stomatitis, recurrent genital ulcerations, and eye lesions(uveitis or chorioretinitis). Among the systemic manifestat ions, pulmonary involvement is known to be rare and only a few cases have been documented. The most important features of pulmonary lesions in Behcet's disease are recurrent hemoptysis, which is often massive, and fatal pleuritic chest pain and recurrent high fever and fleeting nature of the pulmonary infiltrates. We report a case of Behcet's disease manifestated as high fever and pleural effusions which was complicated by pulmonary infarction ans a result of pulmonary arteritis.
Arteritis ; Chest Pain ; Fever ; Hemoptysis ; Ions ; Pleural Effusion* ; Pulmonary Infarction ; Stomatitis, Aphthous ; Ulcer

Myasthenia gravis is an autoimmune disease that results from an antibody-mediated reaction and occurs with thymoma in 15+ACU- of patients. It is very rarely associated with autoimmune hepatitis. Four cases of myasthenia gravis with autoimmune hepatitis have been reported in the world. We recently experienced a case of 30-year-old man with myasthenia gravis associated with thymoma and autoimmune hepatitis. This condition is the first case that has not been reported previously in Korea. We report this rare condition along with a brief review of the literature.
Adult ; Case Report ; HLA-DR Antigens/genetics ; Hepatitis, Autoimmune/etiology+ACo- ; Human ; Male ; Myasthenia Gravis/complications+ACo- ; Thymoma/etiology+ACo- ; Thymus Neoplasms/etiology+ACo-

Lung cancer begins insidiously, metastasizes early, and is frequently nonresectable at the time of diagnosis. Gastrointestinal metastases are considered the most unusual. A 60 year-old man was admitted for the evaluation of dizziness. For two weeks prior to admission, he had mild blood-tinged sputum and melena. He had right cervical lymphadenopathy, measured by 2 2 cm. Laboratory studies demonstrated hemoglobin of 3.7 g/dL. Posteroanterior and lateral chest radiographs revealed mass-like lesion of right upper lobe. An umblicated mass was found in the sigmoid colon by colonoscopic examination and biopsy was done. He developed abdominal pain, tenderness and rebound tenderness at admission 10 days. Abdominal CT showed that focal thickened wall and suspicious perforated lesion at the ileum. Operation was done. In recent time, we experienced a case in which perforation of gastrointestinal metastases developed with malignant tumors of the lung.
Abdominal Pain ; Biopsy ; Colon, Sigmoid* ; Diagnosis ; Dizziness ; Humans ; Ileum ; Lung Neoplasms* ; Lung* ; Lymphatic Diseases ; Melena ; Middle Aged ; Neoplasm Metastasis* ; Radiography, Thoracic ; Sputum ; Tomography, X-Ray Computed

BACKGROUND/AIMS: Percutaneous endoscopic gastrostomy (PEG) is a method widely used for long-term enteral nutrition in dysphagia. Mostly, it is preceded by nasogastric intubation (NI) for short-term enteral nutrition; endoscopic findings associated with NI are encountered during PEG. The purpose of this study was to discuss such findings and to delineate a relationship between these findings, especially esophageal lesions and the duration of NI. METHODS: This study involved 185 individuals who had undergone PEG at Kyung Hee Medical Center from January 1999 to May 2002. The medical records were examined retrospectively. RESULTS: The dysfunction of the CNS comprised 98.4% of the causes of dysphagia. The duration of NI was 15.2 weeks on average, with median value of 8.7 weeks, indicating that PEG was performed relatively soon. Endoscopic findings revealed esophagitis in 63 cases, esophageal ulcers in 27 and active bleedings in another 10. The incidence of esophageal lesions was shown to be higher in subjects with duration of NI under 12 weeks than in those with duration over 12 weeks (p=0.032). CONCLUSIONS: PEG was carried out in many cases during the early stages of dysphagia, and NI-associated esophageal lesions appeared to be more prevalent within 12 weeks of NI duration. These results may be of help in deciding the timing of PEG.
Adult ; Aged ; Deglutition Disorders/therapy ; *Endoscopy, Gastrointestinal ; Enteral Nutrition ; Esophageal Diseases/*diagnosis/etiology ; Esophagus/*pathology ; Female ; *Gastrostomy ; Humans ; *Intubation, Gastrointestinal/adverse effects ; Male ; Middle Aged

BACKGROUND/AIMS: Increasing evidence suggests that manganese deposition in globus pallidus is responsible for MRI signal hyperintensity and for extrapyramidal symptoms in cirrhotics. However, the relationships between blood manganese, the severity of liver dysfunction, the pallidal signal intensity, and neurological signs have not been well established. METHODS: Blood manganese concentrations were measured together with brain MRI and neurological evaluation in six controls, six patients with Parkinson's disease with normal liver function, and fourteen cirrhotic patients with hepatic encephalopathy including six cirrhotics with extrapyramidal symptoms. The neurological state was evaluated using the Columbia scale and the pallidal index (PI). The ratio of globus pallidus to frontal subcortical white-matter signal intensity was measured-multiplied by 100. RESULTS: Pallidal signal hyperintensity was observed in 85.7% of cirrhotics, and the PI was higher in cirrhotics with extrapyramidal signs or high grade varices than those without them, but there was no increase in the Parkinson's disease patients. No correlations were demonstrated between the blood manganese level and PI as well as the Child-Pugh score. The blood manganese level was not significantly different between cirrhotics and other groups. However, there was an overt increase only in two cirrhotic patients with extrapyramidal signs. The Columbia scale did not reveal any correlations with the blood manganese level and the Child-Pugh score. CONCLUSIONS: Cirrhotics with extrapyramidal signs showed a significant increase in PI, but there was no increase in the patients with Parkinson's disease. The PI was not significantly correlated with the blood manganese level. These findings suggest that extrapyramidal signs in cirrhotics might be caused by a different mechanism than those in Parkinson's disease, which could possibly be related with manganese.
Brain ; Globus Pallidus ; Hepatic Encephalopathy ; Humans ; Liver ; Liver Diseases ; Magnetic Resonance Imaging ; Manganese* ; Parkinson Disease ; Varicose Veins

BACKGROUND/AIMS: It is well known that Crohn's disease (CD) can affect all gastrointestinal tract. However, there was no report that evaluated upper gastrointestinal CD in Korea. Therefore, we assessed the endoscopic and histological characteristics of gastrointestinal CD and the prevalence of Helicobacter pylori infection in Korea. METHODS: We examined forty-six patients who were diagnosed as having CD in Kyung Hee University Hospital. We analyzed the endoscopic and histological characteristics by endoscopic examination with biopsy and the prevalence of H. pylori infection. We also investigated the immunohistochemical characteristics of the biopsy specimen and assessed the effects of medications on both histologic and bacteriologic status. RESULTS: High proportion (69.6%) of CD patients showed upper gastrointestinal mucosal alterations. Erosive endoscopic alteration was the most common finding with major involvement in the antrum and duodenum. In H. pylori negative CD patients, granuloma and focal gastritis were found in 30.6% and 44.4% of cases, respectively. There were no significant correlations between histologic features and clinical characeteristics of CD. CONCLUSIONS: We conclude that histologic demonstration of focal gastritis and/or granuloma on endoscopic biopsy specimens in oligosymptomatic patients, especially in H. pylori negative patients, might be considered as patients with possible CD.
Biopsy ; Crohn Disease* ; Duodenum ; Gastritis ; Gastrointestinal Tract ; Granuloma ; Helicobacter pylori* ; Helicobacter* ; Humans ; Korea ; Prevalence* ; Upper Gastrointestinal Tract*