Two Cases of Adult Onset Still's Disease with Concomitant Hemophagocytic Syndrome / 대한류마티스학회지

Adult onset Still's disease is an rare inflammatory disease with the characteristic of fever, skin rash, arthralgia or arthritis, lymphadenopathy, leukocytosis and multiple systemic organ involvement. Its accurate pathogenesis has not been elucidated yet. Its clinical manifestation is also very diverse, from relatively mild symptoms to severe complications such as concomitant infection, liver failure, disseminated intravascular coagulation, myocarditis, adult respiratory distress syndrome, which may lead to death in some cases. Particularly, concomitant hemophagocytic syndrome is rare complication that could induce a fatal outcome. Thus it is important to diagnose early and start treatments. Until now, it has been reported in only one case of adult onset Still's disease in Korea. Here, we report two female cases of adult onset Still's disease with concomitant hemophagocytic syndrome.

Remission of Refractory Intestinal Behcet's Disease with Infliximab / 대한류마티스학회지

Behcet's disease is a chronic, relapsing vasculitis of unknown origin that affects nearly all organs. The most frequent extra-oral sites of gastrointestinal involvement are the ileocecal lesion and the colon. The lesions are typically resistant to medical treatment and frequently recur with surgical treatment. We describe a patient who had intestinal Behcet's disease complicated perforation and enterocutanous fistula with a good response to infliximab.

A Case of Overlap Syndrome of Dermatomyositis and Sjogren's Syndrome associated with IgA Nephropathy / 대한류마티스학회지

An "overlap syndrome" is used to describe patients who have two or more well-defined connective tissue diseases such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and polymyositis. Their coexistence is defined clinically and often by specific serologic tests. We report a case of dermatomyositis associated with IgA nephropathy that had overlapping features of Sjogren's syndrome. This dermatomyositis and Sjogren's overlap is a rarely reported overlap syndrome worldwide.

A Case of Primary Aldosteronism with Aortic Dissection / 대한내분비학회지

Hypertension and atherosclerosis are the most important factors contributing to the development of aortic dissection. Primary aldosteronism is a rare cause of hypertension. The concurrence of aortic dissection is very rare in primary aldosteronism. However, when aortic dissection is found as a life-threatening complication of primary aldosteronism, then the diagnosis of primary aldosteronism is important because the therapeutic intervention can be curative. Only 3 cases of primary aldosteronism with aortic dissection have been reported in the literature. We report here on a case of primary aldosteronism with aortic dissection, which was treated by laparoscopic adrenalectomy. We lowered the blood pressure with antihypertensive drugs and potassium replacement was done to treat the aortic dissection. After stabilization of aortic dissection, we removed his left adrenal mass by laparoscopic adrenalectomy. Postoperatively, the patient's blood pressure has been within normal limits and the serum potassium increased to a normal level without supplementation. The aortic dissection has remained in a stable state

A Case of Primary Aldosteronism with Aortic Dissection / 대한내분비학회지

Hypertension and atherosclerosis are the most important factors contributing to the development of aortic dissection. Primary aldosteronism is a rare cause of hypertension. The concurrence of aortic dissection is very rare in primary aldosteronism. However, when aortic dissection is found as a life-threatening complication of primary aldosteronism, then the diagnosis of primary aldosteronism is important because the therapeutic intervention can be curative. Only 3 cases of primary aldosteronism with aortic dissection have been reported in the literature. We report here on a case of primary aldosteronism with aortic dissection, which was treated by laparoscopic adrenalectomy. We lowered the blood pressure with antihypertensive drugs and potassium replacement was done to treat the aortic dissection. After stabilization of aortic dissection, we removed his left adrenal mass by laparoscopic adrenalectomy. Postoperatively, the patient's blood pressure has been within normal limits and the serum potassium increased to a normal level without supplementation. The aortic dissection has remained in a stable state

A Case of Primary Cardiac Non-Hodgkin's Lymphoma

Although 25 to 36% of systemic lymphoma patients develop cardiac involvement, a primary lymphoma involving only the heart or pericardium is much less common. We detected an intracavitary mass in the right atrium and right ventricle in a 73-year-old man with dyspnea on exertion and generalized edema using transthoracic and transesophageal echocardiography. A thoracotomy was performed due to a possible cardiac tamponade, and a myocardial biopsy showed a malignant non-Hodgkin's lymphoma of the diffuse large B cell type. We report a rare case of a primary cardiac lymphoma detected using a transthoracic and transesophageal echocardiography in patient presenting with a massive pericardial effusion.

Oxaliplatin and UFT, leucovorin combination chemotherapy in patients with advanced colorectal cancer / 대한내과학회지

BACKGROUND: UFT/oral leucovorin (LV) provided a safer, more convenient oral alternative to bolus i.v. 5-Fluorouracil/LV regimen for advanced colorectal cancer while producing equivalent survival. We evaluated the efficacy and safety of a combination of oxaliplatin and UFT/LV in patients with advanced colorectal cancer. METHODS: From January 1999 to December 2001, a total 28 patient with metastatic or relapsed colorectal cancer were enrolled in this study. Treatment was consisted of oxaliplatin 130 mg/m2 i.v. for 2 hours on day 1, and UFT 300 mg/m2 p.o. and LV 30 mg p.o. on day 1-21. Chemotherapy repeated every three weeks until disease progression. RESULTS: Of the 28 patients, 1 complete response and 10 partial responses were observed. The overall response rate was 39.3%. The estimated median time to progression and survival were 6.0 months and 18.2 months, respectively. Peripheral neuropathy was the most common adverse effect. But, peripheral neuropathy was mild (grade 1, 2) and reversible. From the 129 cycles analyzed, grade 3, 4 adverse effects were observed only 3% included neutropenia (1.5%), and thrombocytopenia (1.5%). There were no treatment-related deaths. CONCLUSION: This combination of oxaliplatin and UFT/oral leucovorin is active and feasible in patients with advanced colorectal cancer. The regimen deserve further evaluation in a phase III prospective study.

The Usefulness of Endoscopic Ultrasonography in the Diagnosis of Diseases of Liver, Biliary Tree, Gallbladder and around Distal Common Bile Duct / 대한소화기내시경학회지

BACKGROUND/AIMS: Endoscopic ultrasonography (EUS) is now accepted as an essential tool for the diagnosis and the therapy of various gastrointestinal diseases. With regard to the biliary system, its high diagnostic sensitivity and specificity were reported recently. We assessed the clinical usefulness of EUS for the diagnosis of diseases of the liver, biliary tree, gallbladder and distal common bile duct. METHODS: We sent questionnaires about the clinical usefulness of EUS to the doctors who requested EUS examination for 32 patients with suspected diseases of the liver, gallbladder and around extrahepatic biliary tree from Aug. 2001 to Aug. 2002. We evaluated the answers and clinical characteristics of the patients. RESULTS: Patients were 17 males and 15 females with mean age of 61.8 years old. All received abdominal computed tomography (CT) before EUS and some had abdominal ultrasonography (USG) or endoscopic retrograde cholangiopancreatography (ERCP). EUS was more accurate and informative than other imaging modalities and gave definite final diagnosis for 31.2%. In 40.6%, EUS was helpful as an additional diagnostic tool. CONCLUSIONS: EUS was useful in 71.8% of the cases for the diagnosis of diseases of the liver, biliary tree, gallbladder and around distal common bile duct. EUS can be used as an important adjunct to USG, CT and ERCP.