Multiple endocrine neoplasia (MEN) 2B is charaterized by tumors of endocrine glands, consisting of medullary thyroid carcinoma (MTC), pheochromocytoma and mucosal neuromas of the tongue, lips and other sites. Especially, MTC is the main cause of death in patients who have not received early prophylactic treatment, and MTC in MEN 2B represents more aggressive progress than that of MEN 2A. We encountered two cases of multiple endocrine neoplasia type 2B. One was a 13 month old boy who had familial history of MEN 2B without any symptoms, and the other was a 6-year old boy who manifested multiple mucosal neuromas of the tongue which had been aggravated in four months. Their genetic analysis revealed a point mutation 918th cordon in the RET proto-oncogene. Both of them underwent an operation for prophylactic total thyroidectomy and the 6 year old boy's specimen turned out to be thyroid medullary carcinoma. We encountered two cases of MEN 2B with prophylactic thyroidectomy by early diagnosis of RET proto-oncogene, and report the cases with review of literature.

Ovarian myoma is a rare mesenchymal tumor, accounting for only 1% of benign ovarian neoplasm. These tumors only originate from smooth muscle on the walls of blood vessels in the cortical stroma, in the corpus luteum and in the ovarian ligaments at the point of attachment to the ovary. Most patients are asymptomatic and the tumors are usually found incidently during operations or at autopsies. Two cases of ovarian myoma experienced in our hospital recently are reported with a brief review of the literatures."
Autopsy ; Blood Vessels ; Corpus Luteum ; Female ; Humans ; Leiomyoma* ; Ligaments ; Muscle, Smooth ; Myoma ; Ovarian Neoplasms ; Ovary

BACKGROUND: Diffusion-weighted MR imaging (DWI) was designed to detect the random molecular otion of water in tissue. DWI has gained increasing interest recently because it can demonstrate yperacute stroke earlier than any other imaging methods so far. The purpose of our study was to etermine the clinical usefulness and significance of DWI compared with those of Fast Spin Echo T2-weighted image (SE T2WI) and Fluid Attenuated Inversion Recovery (FLAIR) image in acute and subacute ischemic stroke. METHOD: Twenty-five adult patients with clinical diagnoses of acute or subacute cerebral infarctions were imaged with fast SE T2WI, FLAIR and DWI using a 1.5-T image. RESULTS:All the patients had areas of high signal intensity indicating acute or subacute lesions on DWI and these lesions provide an excellent clinicoanatomic correlation. CONCLUSIONS: DWI is most useful in the diagnosis and localization of acute or subacute lacuna or subcortical infarction in patients with preexisting chronic infarctions or white matter hyperintensity showing high signal intensity similar to acute or subacute infarctions on T2WI or FLAIR image.
Adult ; Brain ; Cerebral Infarction ; Diagnosis ; Diffusion* ; Humans ; Infarction ; Magnetic Resonance Imaging ; Stroke*

PURPOSE: With a duration of action of approximately 24 hours and peakless levels, Lantus is a more physiologic basal insulin analogue compared with NPH. The aim of this study was to compare the glycemic control of Lantus plus Humalog with that of premixed insulin in children and adolescents with type 1 diabetes mellitus. METHODS: The subjects consisted of 25 patients with type 1 diabetes mellitus, aged 12-19 years, who changed their insulin regimen from premixed insulin to Lantus plus Humalog. Daily insulin doses, frequency of hypoglycemia, fasting blood glucose, C-peptide concentration and HbA1c before and 6 months after Lantus treatment were compared. 24 hour blood glucose of 11 patients among Lantus treatment group (n=25) and premixed insulin treatment group (n=10) were self-monitored and compared. RESULTS: 6 months after Lantus treatment, the episodes of hypoglycemia were reduced by 50%(15.1 vs. 7.6 events/month), especially nocturnal hypoglycemia by 67%(6.7 vs. 2.5 events/month). HbA1c was reduced from 9.3% to 8.7% after Lantus treatment. Self-monitored blood glucose of Lantus treatment group at postbreakfast 30, 60, 90 and 120 minutes were 171.1, 169.5, 171.0 and 154.1 mg/dL respectively and lower than those of premixed insulin treatment group (259.7, 282.7, 280.0 and 250.9 mg/dL respectively). CONCLUSION: Compared with premixed insulin, Lantus plus Humalog is more effective in glycemic control and reduction in nocturnal hypoglycemia in children and adolescents with type 1 diabetes mellitus.
Adolescent* ; Blood Glucose ; C-Peptide ; Child* ; Diabetes Mellitus, Type 1* ; Humans ; Hypoglycemia ; Insulin ; Insulin Glargine ; Skin

No abstract available.
Female ; Humans ; Marriage*

No abstract available.

PURPOSE: The aims of this study were to find the risk factor and outcomes of patients with an early recurrence (ER) of a hepatocellular carcinoma (HCC) after liver transplantation (LT) and the actual impact of transarterial chemoembolization (TACE) before LT for a HCC on patient survival and HCC recurrence. METHODS: Ninety-eight cases of adult LT, performed between September 1995 and January 2006, were evaluated. The risk factors and prognosis of patient with a HCC after transplantation for an ER, defined as a recurrence within 6 months of transplantation, and the effects of Pre-LT TACE on the disease-free and overall survival rates, as well as the patterns of recurrence after LT, were studied. RESULTS: A total of 18 patients (18.4%) experienced a HCC recurrence after LT; 10 and 8 patients had early and late recurrences, respectively. From a univariate analysis, the serum alpha-fetoprotein (P=0.003), tumor size (P=0.003), serosa invasion (P=0.000), tumor grade (P=0.011) and vascular invasion (P=0.014) were statistically significant risk factors for an ER. From a multivariate analysis, the presence of serosa invasion of a HCC was the only independent risk factor for an ER (P=0.009; OR=9.407: 95% CI, 1.764~50.164). There was no difference in the disease free sur-vival and overall survival rates between the TACE and without TACE groups, but the extrahepatic recurrence rate was higher in the TACE than without TACE group. CONCLUSION: Serosa invasion by a HCC is independently associated with an ER of HCC after LT. Pre-LT TACE does not influence the disease-free and overall survivals after LT for a HCC.
Adult ; alpha-Fetoproteins ; Carcinoma, Hepatocellular* ; Humans ; Liver Transplantation* ; Liver* ; Multivariate Analysis ; Prognosis ; Recurrence* ; Risk Factors* ; Serous Membrane ; Survival Rate

Lymphocytic infundibuloneurohypphysitis(LINH) is a newly classified disorder and proposed as a common cause of idiopathic central diabetes insipidus. It is characterized by thickening of the infundibulum and/or pituitary stalk and absence of high signal in the neurohypophysis in sellar MRI and the microscopic findings of diffuse lymphocytic infiltration within the neurohypophysis. A 12year-old boy presented polyuria and headache. The result of water deprivation test was compatible with central diabetes insipidus. Brain MRI showed thickening of the pituitary stalk and loss of high signal in the neurohypophysis. Craniotomy and excisional bi6opsy was done under the impression of pituitary tumor. Microscopically, neurohypophysis showed dense lymphocytic infiltration and no malignant cells. Six months after the operation, the previously thickened pituitary stalk and neurohypophysis appeared normal in follow-up MRI.
Brain ; Craniotomy ; Diabetes Insipidus, Neurogenic ; Follow-Up Studies ; Headache ; Humans ; Magnetic Resonance Imaging ; Male ; Pituitary Gland ; Pituitary Gland, Posterior ; Pituitary Neoplasms ; Polyuria ; Water Deprivation

OBJECTIVE: Heart rate variability(HRV) is helpful to diagnosis autonomic disturbance and sympathetic-parasympathetic imbalance in patients with myocardial infarction and diabetes mellitus. Patients with liver cirrhosis demonstrate reduced blood pressure despite increased heart rate and increased cardiac output, indicating a fall in peripheral vascular resistance. Autonomic disturbance may contribute to this phenomenon. The aim of the present study is to evaluate the degree of autonomic disturbance and the circadian rhythm of autonomic nervous system by estimating HRV with 24 hour-Holter recorder, METHODS: 24 hour-HRV with Del Mar Avionics 563 Holter recorder and cardiovascular reflex tests were carried out on 32 patients with liver cirrhosis and 20 control subjects. We evaluated the presence of autonomic disturbance, and assessed quantitatively the autonomic disturbance. RESULTS: 1) Among cardiovascular reflex tests, Valsalva test, standing test and deep breathing test were showed a significantly decreased response in liver cirrhosis compared with control groups. 2) The standard deviation of 24hours average R-R intervals were showed a significantly decrease in liver cirrhosis than control groups(P<0.0001). The HRV of low frequency(LF. P<.D.001), high frequency(HF, P<0.0001) and total power spectral density (P<0.0001) in liver cirrhosis were statistically lower than control. 3) The LF/HF ratio of patients with liver cirrhosis was showed higher than control at night CONCLUSION: Non-invasive assessment of 24 hour-HRV has a few advantages in the diagnosis and degree of autonomic disturbance, evaluation of diurnal variation of autonomic tone.
Autonomic Nervous System ; Blood Pressure ; Cardiac Output ; Circadian Rhythm ; Diabetes Mellitus ; Diagnosis ; Heart Rate* ; Heart* ; Humans ; Liver Cirrhosis* ; Liver* ; Myocardial Infarction ; Reflex ; Respiration ; Vascular Resistance

PURPOSE: Adenosine myocardial perfusion SPECT has proven to be useful in the detection of coronary artery disease, in the follow up the success of various therapeutic regimens and in assessing the prognosis of coronary artery disease. The purpose of this study is to define the reproducibility of myocardial perfusion SPECT using adenosine stress testing between two consecutive Tc-99m sestaMIBI (MIBI) SPECT studies in the same subjects. METHODS: Thirty patients suspected of coronary artery disease in stable condition underwent sequential Tc-99m MIBI SPECT studies using intravenous adenosine. Gamma camera, acquisition and processing protocols used for the two tests were identical and no invasive procedures were performed between two tests. Mean interval between two tests were 4.1 days (range: 2-11 days). The left ventricular wall was divided into 18 segments and the degree of myocardial tracer uptake was graded with four-point scoring system by visual analysis. Images were interpretated by two independent nuclear medicine physicians and consensus was taken for final decision, if segmental score was not agreeable. RESULTS: Hemodynamic responses to adenosine were not different between two consecutive studies. There were no serious side effects to stop infusion of adenosine and side effects profile was not different. When myocardial uptake was divided into normal and abnormal uptake, 481 of 540 segments were concordant (agreement rate 89%, Kappa index 0.74). With four-grade scoring system, exact agreement was 81.3% (439 of 540 segments, tau b=0.73). One and two-grade differences were observed in 97 segments (18%) and 4 segments (0.7%) respectively, but three-grade difference was not observed in any segment. Extent and severity scores were not different between two studies. The extent and severity scores of the perfusion defect revealed excellent positive correlation between two test (r value for percentage extent and severity score is 0.982 and 0.965, p< 0.001) CONCLUSION: Hemodynamic responses and side effects profile were not different between two consecutive adenosine stress tests in the same subjects. Adenosine Tc-99m sestaMIBI SPECT is highly reproducible, and could be used to assess temporal changes in myocardial perfusion in individual patients.
Adenosine* ; Consensus ; Coronary Artery Disease* ; Coronary Vessels* ; Diagnosis* ; Exercise Test ; Follow-Up Studies ; Gamma Cameras ; Hemodynamics ; Humans ; Nuclear Medicine ; Perfusion ; Prognosis ; Tomography, Emission-Computed, Single-Photon*