Marfan syndrome is an inheritable connective tissue with protean clinical manifestations involving the ocular, skeletal and cardiovascular system. It is well estabalished that a clinical hall marker and the major cause of morbidity in Marfan syndrome is aortic root dilatation and associated aortic dissection which begins just above the coronary ostia in ascending aorta. We report a rare case of Marfan syndrome with aortic dissection which began just below left subclavian artery in descending aorta. A 20-years old woman was admitted to Soonchunhyang hospital because of sudden onset of back pain. On phsical examination, she had characteristic Marfanoid feature. Chest X-ray and Echocardiography showed cardiomegaly and severe dilatation of ascending aorta. Chest CT and aortography showed severe aneurysm of ascending aorta and aortic dissection of descending aorta with intimal flap. she was treated with medical treatment because of poor general condition.
Aneurysm ; Aorta ; Aorta, Abdominal* ; Aorta, Thoracic ; Aortic Aneurysm* ; Aortography ; Back Pain ; Cardiomegaly ; Cardiovascular System ; Connective Tissue ; Dilatation ; Echocardiography ; Female ; Humans ; Marfan Syndrome* ; Subclavian Artery ; Thorax ; Tomography, X-Ray Computed ; Young Adult

Marfan syndrome is an inheritable connective tissue with protean clinical manifestations involving the ocular, skeletal and cardiovascular system. It is well estabalished that a clinical hall marker and the major cause of morbidity in Marfan syndrome is aortic root dilatation and associated aortic dissection which begins just above the coronary ostia in ascending aorta. We report a rare case of Marfan syndrome with aortic dissection which began just below left subclavian artery in descending aorta. A 20-years old woman was admitted to Soonchunhyang hospital because of sudden onset of back pain. On phsical examination, she had characteristic Marfanoid feature. Chest X-ray and Echocardiography showed cardiomegaly and severe dilatation of ascending aorta. Chest CT and aortography showed severe aneurysm of ascending aorta and aortic dissection of descending aorta with intimal flap. she was treated with medical treatment because of poor general condition.
Aneurysm ; Aorta ; Aorta, Abdominal* ; Aorta, Thoracic ; Aortic Aneurysm* ; Aortography ; Back Pain ; Cardiomegaly ; Cardiovascular System ; Connective Tissue ; Dilatation ; Echocardiography ; Female ; Humans ; Marfan Syndrome* ; Subclavian Artery ; Thorax ; Tomography, X-Ray Computed ; Young Adult

BACKGROUND: Benign pathologic findings are shown in 800% of thyroid nodules by fine needle aspiration cytology (FNAC) or needle biopsy. About half of these benign nodules are follicular lesions which are presented only as thyroid follicles or thyroid cell clumps. Differential diagnosis of follicular adenoma, follicular carcinoma and adenomatous goiter is impossible by FNAC or needle biopsy. Thyroxine suppression therapy has been performed traditionally in order to discriminate malignant nodules, but few studies are available which confirmed the efficacy of thyroxine suppression therapy in thyroid nodules of those the initial pathologic findings were follicular lesions. So we tried to evaluate the efficacy of thyroxine suppression therapy in benign thyroid nodules and also the incidence of thyroid cancer of the thyroid nosules which were not decreased on thyroxine suppression therapy after surgical resection. METHODS: Total 1027 patients with thyroid nodules were evaluated by FNAC or needle biopsy at Soonchunhyang university hospital from 1990 to 1996. Among 1027 patients, 507 patients showed follicular lesions in FNAC or needle biopsy and they received thyroxine suppression therapy. Thyroid nodule volume was measured before and after thyroxine suppression therapy using ultrasonography. We studied 184 patients who were followed up for more than 1 year. Serial changes of thyroid function tests, thyroid nodule volume, serum thyroglubulin (Tg) level before and after therapy were analyzed. RESULTS: l. In 80 (43.5%) of the 184 patients, nodule volumes decreased more than 50 percent after thyroxine suppression therapy. 2. There was no significant difference in serum T3, T4, TSH levels before and after thyroxine suppression therapy between group I (nodule volume decreased less than 50%) and group II (nodule volume decreased more than 50%). 3. In group II patients, thyroid nodule volumes were decreased continuously at 12 month, 18 month and 30 month after thyroxine suppression (p<0.05). 4. There was no significant difference between the group I and group II in the frequency of multiple thyroid nodules on ultrasonography. 5. Among 37 patients who underwent thyroidectomy, 19 cases (51.4%) were revealed as malignant thyroid nodules (papillary cancer 4 cases, follicular cancer 15 cases). Eighteen cases (48.6%) were revealed as benign thyroid nodules (follicular adenoma 10 cases, adenomatous goiter 8 cases). 6. There was no significant difference in the frequency of multiple nodules on ultrasonography between benign and malignant nodules. CONCLUSION: Our data suggested thyroxine suppression therapy was effective in discriminating malignant thyroid nodules from benign nodules, especially in selecting follicular carcinoma from follicular lesion by FNAC or biopsy.
Adenoma ; Biopsy ; Biopsy, Fine-Needle ; Biopsy, Needle ; Diagnosis, Differential ; Goiter ; Humans ; Incidence ; Thyroid Function Tests ; Thyroid Gland* ; Thyroid Neoplasms ; Thyroid Nodule* ; Thyroidectomy ; Thyroxine* ; Ultrasonography

A substantial proportion of adrenal incidentalomas demonstrates subtle hormonal hypersecretion; however, adenomas that cosecrete aldosterone and cortisol are rare. We here report a case of an adrenal mass that was incidentally detected on a computed tomography scan in a 57-year-old man. The patient had a 10-year history of diabetes mellitus and a 5-year history of hypertension. Evaluation revealed hyperaldosteronemia with an elevated plasma aldosterone-to-renin ratio, hypokalemia, unsuppressed cortisol after dexamethasone administration, and elevated urinary free cortisol concentration. The appearance of the right adrenalectomy specimen indicated adrenal adenoma. Postoperatively, the blood glucose and blood pressure control improved and the urinary cortisol and aldosterone-to-renin ratio normalized. A complete endocrine evaluation in patients with incidentally discovered adrenal masses should be performed, even if the patient has a long-standing history of hypertension and diabetes, to avoid any postoperative adrenal crises.
Adenoma ; Adrenalectomy ; Adrenocortical Adenoma* ; Aldosterone* ; Blood Glucose ; Blood Pressure ; Dexamethasone ; Diabetes Mellitus ; Humans ; Hydrocortisone* ; Hyperaldosteronism ; Hypertension ; Hypokalemia ; Middle Aged ; Plasma

Nesidioblastosis is a term that describes multifocal hyperplasia of all panereatic cell components and is characterized primarily by their disorganization and proliferation throughout the entire panaeas. Adult onset nesidioblastosis is an extremely rare entity associated with hypersecretion of insulin. The authors have recently experieneed a case of nesidioblastosis in an adult. A 41-year old man was admitted due to interrnittenr hypoglycemic symptoms, that had been relieved by carbohydrate ingestion. Hyperinsulinemic hypoglycemia was documented during prolonged fast. Under the presumptive diagnosis of insulinoma, abdominal CT, celiac angiogram and percutaneous transhepatic portal venous sampling were done but we could not find any definitive mass. Eight-five percent of the panacas was removed. Pathologic examination of the resected pancreas revealed irregularly sized islets and scattering of small endocrine cell clusters throughout the acinar tissue and ductuloinsular complex.
Adult ; Cellular Structures ; Congenital Hyperinsulinism ; Diagnosis ; Eating ; Endocrine Cells ; Humans ; Hyperplasia ; Hypoglycemia ; Insulin ; Insulinoma ; Nesidioblastosis ; Pancreas ; Tomography, X-Ray Computed

Gastrointestinal stroma tumors (GISTs) are CD117- positive primary mesenchymal tumors of the gastrointestinal tract and are noted to have a possible non-random association with neurofibromatosis-1 (NF-1, Von Recklinghausen disease). We report a case of a duodenal GIST presenting with gastrointestinal bleeding in a 74-year-old female, and this condition was accompanied with NF-1. A upper gastrointestinal endoscopy and abdominal computed tomography scan revealed several submucosal tumors in the duodenum, jejunum and ileum. Histological and immunohistochemical studies on the surgical resection specimen revealed gastrointestinal stromal tumors of an uncommitted type. The patient was treated with local excision of the tumors and is now in a favorable state.
Aged ; Duodenum ; Endoscopy, Gastrointestinal ; Female ; Gastrointestinal Stromal Tumors ; Gastrointestinal Tract ; Hemorrhage* ; Humans ; Ileum ; Jejunum

Rectal involvement by systemic lupus erythematosus (SLE) is quite rare. Approximately 14 cases have been reported worldwide, but only one with ischemic colitis has been reported in Korea. A 17-year-old female patient was hospitalized with abdominal pain and hematochezia. Sigmoidoscopy revealed only a simple rectal ulcer without ischemic colitis. cytomegalovirus and bacterial infections were excluded. A sigmoidoscopic rectal biopsy indicated a rectal invasion by SLE, but the patient showed an acute worsening conditions that did not respond to treatment. This paper reports a case of rectal ulcer that developed in SLE without ischemic colitis with a review of the relevant literature.
Abdominal Pain ; Adolescent ; Bacterial Infections ; Biopsy ; Colitis, Ischemic ; Cytomegalovirus ; Female ; Gastrointestinal Hemorrhage ; Humans ; Korea ; Lupus Erythematosus, Systemic ; Sigmoidoscopy ; Ulcer

Rectal involvement by systemic lupus erythematosus (SLE) is quite rare. Approximately 14 cases have been reported worldwide, but only one with ischemic colitis has been reported in Korea. A 17-year-old female patient was hospitalized with abdominal pain and hematochezia. Sigmoidoscopy revealed only a simple rectal ulcer without ischemic colitis. cytomegalovirus and bacterial infections were excluded. A sigmoidoscopic rectal biopsy indicated a rectal invasion by SLE, but the patient showed an acute worsening conditions that did not respond to treatment. This paper reports a case of rectal ulcer that developed in SLE without ischemic colitis with a review of the relevant literature.
Abdominal Pain ; Adolescent ; Bacterial Infections ; Biopsy ; Colitis, Ischemic ; Cytomegalovirus ; Female ; Gastrointestinal Hemorrhage ; Humans ; Korea ; Lupus Erythematosus, Systemic ; Sigmoidoscopy ; Ulcer

BACKGROUND: Two representative groups of family physicians and oncologists were surveyed concerning their knowledge and attitudes toward cancer pain management (CPM). METHODS: A questionnaire, designed to assess knowledge (13 items) and attitudes (6 items) toward CPM, was sent to 2,200 members of Korean Association of Family Medicine and 800 members of Korean Cancer Association. RESULTS: Three hundreds and eighty eight family physicians (17.6%) and one hundred and forty seven oncologists (18.4%) responded and completed the questionnaire. They had inadequate knowledge toward CPM, especially, chance of respiratory depression, tolerance to adverse effect, ceiling effect, risk of addiction, and use of adjuvants. Most of the physicians had positive attitude toward importance of CPM, best judge of pain severity, and priority of CPM but had negative attitude toward concerns about potential problem of addiction and earlier prescription of maximum dose of opioid. Family physicians displayed more lack of knowledge about CPM in 9 of 13 items and more negative attitudes toward 4 of 6 items. Adjustment for demographic characteristics and experiences in cancer patients with pain showed that while family physicians had poor knowledge of tolerance to confusion, they had more adequate knowledge on the effect of antidepressant and had positive attitude towards best judge of pain severity and priority of CPM. Poor knowledge about CPM was rated by 66.3% of physicians as the most important barrier to adequate CPM. CONCLUSION: These findings that most of the physicians had poor knowledge and negative view toward CPM suggest a continuous need for comprehensive education program focused on CPM for physicians.
Education ; Humans ; Korea* ; Pain Management* ; Physicians, Family* ; Prescriptions ; Respiratory Insufficiency ; Surveys and Questionnaires

Interferons play critical roles in tumor pathogenesis by controlling apoptosis and through cellular anti-proliferative and differentiation activities. Interferon inducible transmembrane protein (IFITM) family genes have been implicated in several cellular processes such as the homotypic cell adhesion functions of IFN and cellular anti-proliferative activities. Expression levels of IFITM genes have been found to be up-regulated in gastric cancer cells and colorectal tumors. IFITM3 (also known as 1-8U) is a member of the IFITM family, and has been described as a key player in specification of germ cell fate. IFITM3 was first isolated from a genetic screen aimed at identifying genes involved in acquisition of germ cell competence. It has been proposed that epiblast cells have the highest expression of IFITM3 initiated germ cell specification and that homotypic association can discriminate germ cells from their somatic neighbors. In an attempt to better understand the genetic influences of IFITM3 on ulcerative colitis, we have identified possible variation sites and single nucleotide polymorphisms (SNPs) through two exons and their boundary IFITM3 intron sequences including the ~2.1 kb promoter regions. To determine whether or not these IFITM3 SNPs are associated with susceptibility to ulcerative colitis, frequencies of the genotype and allele of IFITM3 polymorphisms were analyzed on genomic DNAs isolated from patients with ulcerative colitis and from healthy controls. We also investigated the haplotype frequencies constructed by these SNPs in both groups. In this study, we also showed that expression level of IFITM3 mRNA was significantly higher in tissues of the ileum and cecum of the digestive system. We identified a total of seven SNPs and multiple variation regions in the IFITM3 gene. The genotype frequency of the g.-204T>G polymorphism in patients with ulcerative colitis was significantly different from that of the control group. Our results strongly suggest that polymorphisms of the IFITM3 gene may be associated with susceptibility to ulcerative colitis.
Cecum/*metabolism ; Colitis, Ulcerative/epidemiology/*genetics/immunology ; Gene Expression Profiling ; Gene Frequency ; Genetic Association Studies ; Genetic Predisposition to Disease ; Haplotypes ; Ileum/*metabolism ; Korea ; Membrane Proteins/*genetics/immunology/metabolism ; Organ Specificity ; Polymorphism, Single Nucleotide ; RNA-Binding Proteins/*genetics/immunology/metabolism