Pheochromocytomas might be sporadic or genetic. Genetic pheochromocytoma is associated with multiple endocrine neoplasia (MEN) type 2A, MEN type 2B, and von Hippel-Lindau (VHL) disease. RET mutations are identified in more than 90% of index cases of MEN2 and familial medullary thyroid cancer and in about 4–12% of apparent sporadic cases. Here, we report a 54-year-old man presenting with pheochromocytoma and renal cell carcinoma, who was identified as having a novel missense RET mutation.
Carcinoma, Renal Cell ; Humans ; Male ; Middle Aged ; Multiple Endocrine Neoplasia ; Pheochromocytoma ; Thyroid Neoplasms

Adrenal myelolipoma (AML) is a rare, usually benign, and nonfunctioning tumor. About 7-15% of adrenal incidentalomas are AMLs, composed of normal hematopoietic elements and mature adipose tissue. AML is usually unilateral and < 4 cm. It is often discovered incidentally on abdominal computed tomography or magnetic resonance imaging. It is related to a chronic increase in adrenocorticotropic hormone, such as that observed in patients with congenital adrenal hyperplasia, Cushing disease, Conn's syndrome, and pheochromocytoma. Here, we report a 28-year-old man diagnosed with non-salt-losing congenital adrenal hyperplasia with huge bilateral AMLs and a literature review.
Adipose Tissue ; Adrenal Hyperplasia, Congenital* ; Adrenocorticotropic Hormone ; Adult ; Humans ; Hyperaldosteronism ; Magnetic Resonance Imaging ; Myelolipoma* ; Pheochromocytoma ; Pituitary ACTH Hypersecretion

Osteochondromadevelop most commonly at distal femur, proximal humerus and proximal tibia, but the rib osteochondroma was reported less commonly. In this report, scapular snapping syndrome complicated by adventitious bursa and soft tissue pseudotumor surrounding the osteochondroma of the 6th rib body was treated successfully by surgical excision of them. We report this rare case with reviewing the relevant literature.
Femur ; Humerus ; Osteochondroma* ; Ribs* ; Shoulder* ; Tibia

BACKGROUND: To assess the preoperative nutritional status of patients with various disorders and to provide data for pre- and postoperative patient management plans, particularly in the elderly. There is no published information on age-matched and disease-matched preoperative nutritional/immunologic status for orthopedic patients, especially in the elderly, in Jeju. METHODS: In total, 331 patients with four categories of orthopedic conditions were assessed: 92 elective surgery patients, 59 arthroplasty patients, 145 patients with fractures, and 35 infection patients. Malnutrition was defined as body mass index (BMI) below 18 kg/m2 of expected body weight (below 20% of normal), serum albumin/globulin ratio below 1.5 (normal range, 1.5 to 2.3), albumin level below 3.5 g/dL, total lymphocyte count below 1,500 cells/mm3, and lymphocyte/monocyte ratio below 5 versus 1. RESULTS: In 92 elective surgery patients, the average BMI was 23 kg/m2, hemoglobin was 15 g/dL, lymphocytes (2,486 cells)/monocytes (465 cells) ratio was 6.1, and the albumin (4.4 g/dL)/globulin (2.5 g/dL) ratio as a protein quotient was 1.7. Among the 59 hip and knee arthroplasty patients, the average BMI was 25 kg/m2, hemoglobin was 12 g/dL, lymphocytes (2,038 cells)/monocytes (391 cells) ratio was 6.6, and albumin (4.1 g/dL)/globulin (2.4 g/dL) ratio was 1.6. No subject showed malnutrition. Among the 145 fracture patients, the average BMI was 23 kg/m2. The hemoglobin level was 13 g/dL, monocytes (495 cells)/lymphocytes (1,905 cells) ratio was 1 versus 4.6, and albumin (4.1 d/gL)/globulin (2.5 d/gL) ratio was 1.6. However, both ratios decreased after 70 years of age. Among the 17 of 35 infection patients, albumin levels were below 3.5 g/dL, the average BMI was 22 kg/m2, lymphocytes (1,532 cells)/monocytes (545 cells) ratio was 2.4 versus 1, and albumin (3.0 g/dL)/globulin (3.3 g/dL) ratio was 0.9, while in 18 patients albumin levels were over 3.5 g/dL, the average BMI was 22 kg/m2, hemoglobin was 12 g/dL, lymphocytes (1,998 cells)/monocytes (583 cells) ratio was 3 versus 1, and albumin/globulin ratio was 1.4. Thus, in the infection group, approximately 50% of the patients showed poor nutrition and immunosuppression. CONCLUSIONS: It was found that nutritional and immune condition deteriorated gradually to some degree in elderly patients over 60 years of age.
Adolescent ; Adult ; Age Factors ; Aged ; Aged, 80 and over ; Body Mass Index ; Body Size ; Child ; Humans ; Malnutrition/blood/complications/*diagnosis ; Middle Aged ; Musculoskeletal Diseases/complications/*surgery ; *Nutritional Status ; Preoperative Period ; Republic of Korea ; Young Adult

BACKGROUND/AIMS: In order to determine the malignant potential of gallbladder adenoma for progression to carcinoma, we evaluated the histopathologic features of adenoma and adenoma-related lesions on cholecystectomized specimens. METHODS: Among 1,847 cholecystectomized specimens, 63 specimens from 26 benign adenomas, 9 carcinomas in situ (CIS), and 28 invasive carcinomas were selected. A pathologist reviewed all specimens and selected benign adenomas, CIS in the adenoma, and adenoma residue in invasive carcinomas. Adenomas and adenoma-related lesions were classified according to morphology (tubular, tubulopapillary, and papillary) and the consisting epithelium (biliary, pyloric metaplasia, and intestinal metaplasia). The age and the size of the benign adenomas and carcinomas in the adenoma were also compared. RESULTS: Adenoma and adenoma-related lesions were found in 34 out (1.8%) of all resected gallbladder. Among 9 CIS and 28 invasive carcinomas, adenoma-related lesions were detected in 7 and 1 case, respectively. All eight carcinomas arising in the adenoma were well-differentiated solitary tumors. The diameters of the carcinomas in the adenoma were, on average, larger than that of the benign adenomas (1.8 cm vs. 0.9 cm, p=0.01). The patients with carcinomas in the adenoma were, on average, older than those with benign adenomas, although the difference was insignificant (57 years vs. 47 years, p=0.09). The morphology and consisting epithelium did not differ between the benign adenomas and carcinomas in the adenoma. The malignant transformation occurred in 23.5% of adenomas. CONCLUSIONS: Gallbladder adenoma is a rare disease, although malignant transformation occurs frequently. Adenoma is a precancerous lesion and the adenoma-carcinoma sequence is one of the gallbladder cancer carcinogenesis.
Adenoma/epidemiology/*pathology/surgery ; Adult ; Age Factors ; Aged ; Carcinoma/epidemiology/pathology/surgery ; Cell Transformation, Neoplastic ; Cholecystectomy ; Cystadenoma/epidemiology/pathology/surgery ; Female ; Gallbladder Neoplasms/epidemiology/*pathology/surgery ; Gallstones/complications ; Humans ; Male ; Middle Aged ; Neoplasm Invasiveness

Isolated left main coronary ostial stenosis is a very rare condition. In the majority of cases there are coexisting diseases in multiple coronary vessels. Here, a case of isolated left main coronary ostial stenosis due to an acute angle take-off, with clockwise rotation of the coronary sinus, confirmed by cardiac MRI is presented. A 44-year old female patient presented with an exertional and stabbing anterior chest pain. The patient had no premedical history. A coronary angiogram showed an isolated left main coronary ostial stenosis due to an acute take-off of the left main coronary artery. A cardiac MRI showed an acute angle take-off of the left main coronary artery, with clockwise rotation of the coronary sinus. The patient underwent surgical angioplasty of the coronary ostia, with a patch of autologous pericardium. This acute angle take-off may be due to rotation of the coronary sinus.
Adult ; Angioplasty ; Chest Pain ; Constriction, Pathologic* ; Coronary Sinus* ; Coronary Stenosis ; Coronary Vessels ; Female ; Humans ; Magnetic Resonance Imaging* ; Pericardium

There are a few case reports describing prolonged myoclonus following propofol. A previously fit 26 year old woman presented for a left knee lateral impingement under general anesthesia as a day case. She had no personal or family history of epilepsy. Induction was smooth and anesthesia was maintained with propofol TCI, nitrous oxide 65% and oxygen 35%. 30 minutes after stopping of the propofol infusion, the patient developed involuntary movement and shivering that recurred intermittently. After drug therapy and psychotic therapy the patient was progressively stabilized. However 4 hours later the patient developed involuntary movement and myoclonus. She was admitted to the department of neurology under the diagnosis of propriospinal myoclonus. Forty days later she was discharged without long term sequelae.
Adult ; Anesthesia* ; Anesthesia, General ; Diagnosis ; Drug Therapy ; Dyskinesias ; Epilepsy ; Female ; Humans ; Knee ; Myoclonus* ; Neurology ; Nitrous Oxide ; Oxygen ; Propofol* ; Shivering

Atrial septal aneurysm (ASA) was reported as a cause of unknown origin of central or peripheral thromboembolism with patent foramen ovale, aortic debris and spontaneous echo contrast. Especially the paradoxical right to left shunt via micro-fenestration on this sac may be the important cause of this embolism. We report a case of 39 year-old female patient who had atrial septal aneurysm with multiple renal infarction. Symptom was improved with aneurysmectomy and patch closure, then long term anticoagulation has been continued.
Adult ; Aneurysm* ; Embolism ; Female ; Foramen Ovale, Patent ; Humans ; Infarction* ; Thromboembolism

BACKGROUND: Several studies on the frequency and distribution of unexpected antibodies of Koreans have been done by some investgators. But more studies are necessory to grasp the real frequency and distribution. We hoped to investigate the frequency and distribution of unexpected antiboddis of Koreans. METHODS: We performed 188 unexpected antibody identification tests to evaluate the distribution of unexpected antibodies from May 1997 to June 1998. The sera we tested are antibody screening positive ones sent from 11 hospitals and 1 blood center. We also analized the frequecny of unexpected antibodues from the data of Dongsan Medical Center's antibody screening tests. RESULTS: The frequency of unexpected antibody of this study (using tube method) was 1.0%. Both anti-Lea and anti-E (with anti-c) antibodies were the most frequent antibodies up to 31 cases (33.0%) each. Among the identified antibodies from Dongsan Medical Center which used tube method, anti-Lea antibody was the most frequent antibody (41.3%). Otherwise among the identified antibodies from other two hospitals which used gel card method, anti-E (with anti-c) antibody was the most frequent antibody (45.5%). The cause of all four cases of hemolytic disease of newborn was anti-E antibody. About half of the specimens' antibodies were not identified due to the problemes of identification cells, tested sera, test methods. CONCLUSION: Anti-Lea and anti-E (with anti-c) antibodies were the most frequent ones in Korean people. But the prequency was varied according to antibody screenig test methods. Anti-E antibody is the most important unexpected antibidy for hemolytic disease of newborn in Korea. To decrease the missed cases of antibody identification tests both Korean and foreign identification cells, enzyme method should be used.
Antibodies* ; Erythroblastosis, Fetal ; Hand Strength ; Hope ; Infant, Newborn ; Korea ; Mass Screening

BACKGROUND: Platelets can be stored for 3days at 22degrees C in conventional plastic bags plasticizer with di-(2-ethylhexyl)phthalate(DEHP). However, with such a short interval for storage, platelets could not be made easily available for thrombocytopenic patients. In vitro platelet function during 5 days of storage at 22degrees C was studied in a new plastic bag (second generation bag) which contained as plasticizer a tri (2-ethylhexyl)trimellitate and developed in Korea (Boin Medica Co.). METHODS: In vitro function (mean platelet volume, platelet distribution width, pH, platelet aggregation, platelet morphology and swirling phenomenon) was evaluated in 20 units of platelet concentrate at day 0, 2, 5 while mixing in 60 rpm platelet rotator at 22degrees C. RESULTS: At day 5, platelet count, mean platelet volume, platelet distribution width, pH, platelet aggregation, platelet morphology and swirling phenomenon were well maintained. pO2, pCO2 and HCO3- were 65.6mmHg, 43.4mmHg and 12.3mmol/L at day 5, respectively. CONCLUSION: The data indicate that the use of the new platelet storage container will permit satisfactory storage for at least 5 days at 22degrees C.
Blood Platelets* ; Humans ; Hydrogen-Ion Concentration ; Korea ; Mean Platelet Volume ; Plastics ; Platelet Aggregation ; Platelet Count