PURPOSE: Anaplastic thyroid cancer (ATC) is a rare but highly aggressive neoplasm with a dismal prognosis. However, a few patients survive for a long time after treatment. We tried to identify prognostic factors of this disease and analyzed treatment outcomes in patients with ATC. METHODS: We reviewed the medical records of 15 patients diagnosed with ATC in our institution between 1988 and 2003. The survival was compared by the Kaplan-Meier logrank test using SPSS program. RESULTS: The female-to-male ratio was 1.5:1 (9 women and 6 men), and the mean age at diagnosis was 63.9 years (range, 44~91). The mean tumor size was 6.3 cm (range, 4~10 cm). Extrathyroidal invasion was present in 12 cases and distant metastasis at diagnosis was present in 6 cases. Surgery was performed in 8 cases. Radiotherapy was used for 10 cases and chemotherapy for 5 cases. The mean overall survival time of the 15 patients was 237 days (range, 28~717 days). The 6~, 12~, 18~ and 24~ month survival rates were 33%, 26%, 13% and 0%. No association was found between survival and presenting symptoms, age, gender, tumor size, previous goiter history, extrathyroidal invasion, distant metastasis, surgery, radiotherapy or chemotherapy. A significantly better outcome was observed in patients received triple modality treatment (surgery, radiotherapy and chemotherapy) than in those received single or dual modality treatment (P=0.05). CONCLUSION: Although most patients with ATC had a poor prognosis, a multimodal approach including surgery, radiotherapy and chemotherapy, might improve survival.
Diagnosis ; Drug Therapy ; Female ; Goiter ; Humans ; Medical Records ; Neoplasm Metastasis ; Prognosis ; Radiotherapy ; Survival Rate ; Thyroid Carcinoma, Anaplastic*

There are considerable in vitro and in vivo evidences for remineralization and demineralization occurring simultaneously in incipient enamel caries. In order to "heal"the incipient dental caries, many experiments have been carried out to determine the optimal conditions for remineralization. It was shown that remineralization is affected by different pH, lactic acid concentrations, chemical composition of the enamel, fluoride concentrations, etc. Eighty specimens from sound permanent teeth without demineralization or cracks, 0.15 mm in thickness, were immersed in lactic acid buffered demineralization solutions for 3 days. Dental caries with a surface zone and subsurface lesion were artificially produced. Groups of 10 specimens were immersed for 10 or 12 days in lactic acid buffered remineralization solutions consisting of pH 4.3 or pH 6.0, and 100, 50, 25, or 10 mM lactic acid. After demineralization and remineralization, images were taken by polarizing microscopy (x100) and micro-computed tomography. The results were obtained by observing images of the specimens and the density of the caries lesions was determined. 1. As the lactic acid concentration of the remineralization solutions with pH 4.3 was higher, the surface zone of the carious enamel increased and an isotropic zone of the subsurface lesion was found. However, the total decalcification depth increased at the same time. 2. In the remineralization solutions with pH 6.0, only the surface zone increased slightly but there was no significant change in the total decalcification depth and subsurface zone. In the lactic acid buffer solutions with the lower pH and higher lactic acid concentration, there were dynamic changes at the deep area of the dental carious lesion.
Dental Caries ; Dental Enamel ; Fluorides ; Hydrogen-Ion Concentration ; Lactic Acid ; Microscopy ; Tooth

Chylous ascites is extravasation of lymphatic fluid and retention in the peritoneal cavity due to traurna or obstruction of the lymphatic system. Chylous ascites is very rare complication of Continuous Ambulatory Peritoneal Dialysis (CAPD) and is associated with trauma to the lymphatics during catheter insertion in the early stage of CAPD and repeated mild trauma to the lyrnphatics during longterm dialysis. Chylous ascites in the CAPD is suspected when the drained peritoneal fluid is milky white and confirmed by demonstration of the specific components of chyle, such as elevated triglyceride and low cholesterol than plasma and should be differentiated from pseudochyle and bacterial peritonitis. We report a case of chylous ascites in a patient undergoing CAPD at 2 months later of initiation of CAPD, which was not improved by conservative management. So CAPD catheter was removed and renal replacement therapy was changed to hemodialysis.
Ascitic Fluid ; Catheters ; Cholesterol ; Chyle ; Chylous Ascites* ; Dialysis ; Humans ; Lymphatic System ; Peritoneal Cavity ; Peritoneal Dialysis, Continuous Ambulatory* ; Peritonitis ; Plasma ; Renal Dialysis ; Renal Replacement Therapy ; Triglycerides

Water transport is mediated by two distinct pathways, diffusional and channel-mediated water transport. The first molecular water channel was identified from human erythrocytes in 1992. Genetically-related proteins from other mammalian tissues have subsequently been identified to transport water, and the group is referred to as the "Aquaporins". Aquaporin-4 (AQP4) is most abundant in the brain, which may be involved in CSF reabsorption and osmoregulation. However, ontogeny and regulatory mechanisms of AQP4 channels have not been reported. Northern blot analysis showed that AQP4 mRNA began to be expressed in the brain just before birth and that its expression gradually increased by PN7 and then decreased at adult level. AQP4 was expressed predominantly in the ependymal cells of ventricles in newborn rats. And then its expression decreased in ependymal cells and increased gradually in other regions including supraoptic and paraventricular nuclei. AQP4 is also expressed in the subfornical organ, in which the expression level is not changed after birth. Cryogenic brain injury did not affect expression of AQP4 mRNA, while ischemic brain injury decreased it. Osmotic water permeability of AQP4 channel expressed in Xenopus oocytes was inhibited by the pretreatment of BAPTA/AM and calmidazolium, a Ca2+/ Calmodulin kinase inhibitor, in a dose-dependent manner. These results indicate that the expression and the function of AQP4 channel are regulated by developmental processes and various pathophysiological conditions. These results will contribute to the understanding of fluid balance in the central nervous system and the osrmoregulatory mechanisms of the body.
Adult ; Animals ; Blotting, Northern ; Brain Injuries ; Brain* ; Calcium-Calmodulin-Dependent Protein Kinases ; Central Nervous System ; Diffusion ; Erythrocytes ; Humans ; Infant, Newborn ; Ischemia* ; Oocytes ; Osmoregulation ; Parturition ; Permeability ; Rats ; RNA, Messenger ; Subfornical Organ ; Water* ; Water-Electrolyte Balance ; Xenopus

BACKGROUND: Mitofusin 2 (MFN2) is a membrane protein and is an essential component of mitochondrial fusion machinery. Mitochondrial fusion is essential for various biological functions in mammalian cells. Thus mutations in MFN2 are the underlying cause of Charcot-Marie-Tooth neuropathy type 2A (CMT2A). However, there has been no reports investigating the MFN2 genes in Korean CMT patients. Therefore, we investigated to find the clinical and genetic characteristics in Korean patients with the MFN2 gene mutation. METHODS: We examined the mutations of the MFN2 gene in 137 Korean CMT families. According to criteria from the European CMT consortium, CMT2 was 45 families. Mutations were confirmed by both strands sequencing. Nerve conduction studies were carried out in CMT patients having each mutation. RESULTS: Eight pathogenic mutations were found in 10 families. Six mutations (Leu92Pro, Gly127Asp, His165Arg, Ser263Pro, Arg364Trp, Met376Thr) were determined to be novel, and those were not detected in the 100 healthy controls. A de novo missense mutation was found in three CMT families (30%). The frequency of the MFN2 mutation was 22.2%, which was higher than those found in the Cx32 mutation. In CMT2A, the frequencies with early age at onset (<10 years) and flat feet were 46.2%. CONCLUSIONS: We found MFN2 mutations in patients with sporadic or dominantly inherited CMT. In the majority of cases with CMT type 2, the axonal neuropathy, may be due to MFN2 mutations.
Axons ; Charcot-Marie-Tooth Disease ; Flatfoot ; Humans ; Membrane Proteins ; Mitochondrial Dynamics ; Mutation, Missense ; Neural Conduction

The publisher wishes to apologize for incorrectly displaying the author (Seok Beom Gwon) name. We correct his name from Seok Beom Gwon to Seok Beom Kwon.

BACKGROUND AND PURPOSE: Charcot-Marie-Tooth disease (CMT) type 1A (CMT1A) is the demyelinating form of CMT that is significantly associated with PMP22 duplication. Some studies have found that the disease-related disabilities of these patients are correlated with their compound muscle action potentials (CMAPs), while others have suggested that they are related to the nerve conduction velocities. In the present study, we investigated the correlations between the disease-related disabilities and the electrophysiological values in a large cohort of Korean CMT1A patients. METHODS: We analyzed 167 CMT1A patients of Korean origin with PMP22 duplication using clinical and electrophysiological assessments, including the CMT neuropathy score and the functional disability scale. RESULTS: Clinical motor disabilities were significantly correlated with the CMAPs but not the motor nerve conduction velocities (MNCVs). Moreover, the observed sensory impairments matched the corresponding reductions in the sensory nerve action potentials (SNAPs) but not with slowing of the sensory nerve conduction velocities (SNCVs). In addition, CMAPs were strongly correlated with the disease duration but not with the age at onset. The terminal latency index did not differ between CMT1A patients and healthy controls. CONCLUSIONS: In CMT1A patients, disease-related disabilities such as muscle wasting and sensory impairment were strongly correlated with CMAPs and SNAPs but not with the MNCVs or SNCVs. Therefore, we suggest that the clinical disabilities of CMT patients are determined by the extent of axonal dysfunction.
Action Potentials ; Axons ; Charcot-Marie-Tooth Disease ; Cohort Studies ; Humans ; Muscles ; Neural Conduction