Benign hemangioma of the mediastinum is rare. This slowly growing tumor is described as well circumscribed, cystic, hemorrhagic tumor. Histologically it can be differentiated into capillary or cavernous form. We present a case of mediastinal hemangioma. A 20-year-old-man was presented with a slowly growing posterior mediastinal mass of 6 years duration, 8x6 cm in size. The mass was relatively well defined but focally invasive. Microscopically, it was differentiated into vessels of capillary, cavernous, and venous patterns. A solid cellular proliferation with inconspicuous capillary lumens was focally seen. The stroma between variable-sized vessels showed marked myxoid change associated with some smooth muscle bundles and adipose tissue. Ultrastructurally, areas of solid cellular proliferation showed formation of lumens. These lumens were lined by active endothelial cells showing plasmalemmal vesicles and Weibel-Palade bodies on the abluminal surface.
Adipose Tissue ; Capillaries ; Cell Proliferation ; Endothelial Cells ; Hemangioma* ; Mediastinum ; Muscle, Smooth ; Weibel-Palade Bodies

BACKGROUND: Charcot-Marie-Tooth (CMT) disease is pathologically divided into the following two types: demyelinating type and axonal type. This study aimed to analyze the results of the electrophysiological studies of CMT1A and to reevaluate the clinical significance of nerve conduction studies (NCS). METHODS: The subjects of the study were 18 patients with genetically confirmed CMT1A during the period of 1995. 1.-2004. 8. The NCS data from 22 family members of the patients were also included. The nerve conduction velocities, conduction blocks and compound muscle action potentials were analyzed. RESULTS: The subjects were composed of 19 males and 21 females. The mean NCV was 21.70 m/s in the median nerve, and the conduction block was observed in 13 patients (32.5%). The NCV was uniformly slow. The intrafamilial variation of NCVs between parents and their children were analyzed in 30 patients from 11 families. The mean velocity was 24.44+/-3.67 m/s in parents and 19.53+/-5.37m/s in their children. CONCLUSIONS: The CMT1A showed the slowness in NCV, one of the characteristics of demyelinating neuropathy, and this slowing had a uniform pattern. Nerve conduction block was also frequently observed, the pattern of which was diffuse without dispersion, and non segmental. Because the NCV of the children tended to be slower than that of the parents, CMT1A may not be a simple progressive disease. The onset and progression of CMT1A may be determined by other genetic and environmental factors.
Action Potentials ; Axons ; Child ; Female ; Humans ; Male ; Median Nerve ; Neural Conduction ; Parents

PURPOSE: Neoplastic development is a multistep process that involves the accumulation of genetic alterations in proto- oncogenes, DNA repair genes, and tumor suppressor genes. Molecular studies in carcinoma have shown the high frequency of loss of heterozygosity (LOH) in some specific chromosome regions, but LOH on the HCC chromosome has not been thoroughly investigated in Korea. LOH is considered to be phenotypes of genomic instability. We investigated the genetic instability and microsatellite alterations of chromosome 5, 8, 13 and 17 in hepatocellular carcinoma (HCC). METHODS: Microsatellite alteration analysis was performed using polymerase chain reaction with 12 polymorphic microsatellite markers (BAT26, D5S123, D5S346, D8S254, D8S261, D8S262, D13S153, D13S159, D13S171, D17S250, D17S796, TP53) in 37 surgically resected HCCs and their respective non-tumorous counterparts. Pairs of tumorous part and normal tissue in the same patient were compared and then the size of microsatellite markers was measured. RESULTS: MSI was detected in 3 samples and LOH was detected in 51 samples of 37 cases. Fractional allelic loss (FAL) was above 0.2 in 10 cases and was correlate with high grade of HCC. we could detect only 1 case of LOH in D8S254 marker, which was advanced cancer. Markers D5S123 and D5S346 showed 2 and 3 cases of LOH, respectively. Markers D8S262, D17S250 and D17S796 had LOH and were significantly correlated with tumor grade. CONCLUSION: According to the results, our data revealed that specific LOH, rather than MSI, may be involved in hepatocarcinogenesis. LOH may be a useful tool for following HCC patients because the high frequency of LOH correlates with poor prognosis of HCC.
Carcinoma, Hepatocellular* ; Chromosomes, Human, Pair 5* ; DNA Repair ; Genes, Tumor Suppressor ; Genomic Instability ; Humans ; Korea ; Loss of Heterozygosity ; Microsatellite Instability ; Microsatellite Repeats* ; Oncogenes ; Phenotype ; Polymerase Chain Reaction ; Prognosis

Sjogren syndrome is a chronic autoimmune disease characterized by lymphocytic infiltration of exocrine glands resulting in dry mouth and eyes. Approximately one-third of patients present with systemic manifestations, but respiratory muscle involvements have been rarely reported. We report a case of acute respiratory failure complicated by primary Sjogren syndrome. Muscle biopsy revealed perivascular lymphocytic infiltrations. Corticosteroid therapy improved respiratory muscle weakness. Sjogren syndrome should be considered as one of the underlying diseases causing acute respiratory failure.
Autoimmune Diseases ; Biopsy ; Exocrine Glands ; Humans ; Mouth ; Muscular Diseases ; Respiratory Insufficiency ; Respiratory Muscles* ; Respiratory Paralysis ; Sjogren's Syndrome*

Pregnolone[5beta-pregnan-3alpha-ol-one(5beta3alpha)] and allopregnanolone [(5alpha-pregnan-3alpha-ol-20-one(5alpha3alpha))] are neuroactive steroids that are reduced metabolites of progesterone. It was reported that Neuroactive steroids may have anxiolytic and anticonvulsant action similar to benzodiazepines and barbiturates. Therefore, the present study was designed to assess the interaction of steroids with GABAA-benzodiazepine receptor complex. The effect of steroids on the ligands binding to GABAA receptor complex was investigated using rat cortices. 5beta3alpha and 5alpha3alpha enhanced the binding of [3H] flunitrazepam to GABAA receptor, but testosterone, progesterone and dexamethasone did not. GABA also showed the enhancement of [3H] flunitrazepam binding, but did not show the additive effect. Unlike to GABA, 5beta3alpha and 5alpha3alpha did not affect on the [3H] muscimol binding to rat cortices. From these findings, it can be concluded that Neuroactive steroids are potent positive modulators of the GABA A receptor, and do not act at GABA binding site.
Animals ; Barbiturates ; Benzodiazepines ; Binding Sites ; Dexamethasone ; Flunitrazepam ; gamma-Aminobutyric Acid ; Ligands ; Muscimol ; Pregnanolone ; Progesterone ; Rats ; Receptors, GABA-A ; Steroids* ; Testosterone

To determine applicability of the cryopreservation procedure for vessel grafts, the viability of endothelial cells (ECs) among the whole cells in three kinds of organs artery, vein, trachea in mongrel dogs was evaluated on the basis of histological analysis. The Griffonia simplicifolia agglutins-fluorescein isothiocyanate (GSA-FITC) and propidium iodide (PI) double staining methods were combined with flow cytometry (FCM), which was able to simultaneously determine the viability of whole cells and ECs from the same tissue, were performed after harvesting, after antibiotic solution treatment, and after cryopreservation and thawing. In most cases, the viability of ECs is lower than that of whole cells from veins and arteries. The viability of whole cells in veins was maintained until the antibiotic solution treatment and then decreased significantly after cryopreservation and thawing, while the ECs began to decrease significantly after the antibiotic solution treatment and more markedly decreased after thawing. The viability of ECs and whole cells from arteries was similar to that of the veins' conditions. The viability of whole cells from the trachea decreased with a similar pattern to that of the ECs from vessels. In consideration of maintaining cell viability among the three kinds of organs, the viability of arteries was better than that of the others. The cells in the trachea demonstrated a lower viability than the vessels. The effect of antibiotic solution treatment on the reduction of cell viability depends on the treatment time and temperature.
Animalt ; Arteries/transplantation ; Cell Survival ; Coronary Vessels*/transplantatione ; Cryopreservation* ; Dogs ; Endothelium, Vascular/physiology* ; Endothelium, Vascular/cytology ; Female ; Male ; Trachea*/transplantation ; Middle Age ; Veins/transplantation

BACKGROUND: Many studies about the skin elastic properties have been performed until now using diverse methods. Suction methods using Dermaflex and Cutometer are most prevalently used. OBJECTIVE: We performed this study to obtain the normal range of skin elastic properties using Cutometer and to know the correlation between the data of Cutometer and those of Dermaflex. METHODS: Eleven different anatomical sites were measured in 46 healthy young volunteers (M:=23:3) by Cutometer. The same anatomical sites were also measured by Dermaflex in sixteen volunteers(M:=8:). RESULTS: When measured by Cutometer, skin elasticity and distensibility were generally higher in the central part of the body than the distal portion, showing the distal gradient. There were differences according to sexes and a part of them were significant. When we compared Cutometer with Dermaflex, they were closely related to each other in the data about distensibility, there was a weak correlation in the data of elasticity. No correlation was detected between Cutometer and Dermaflex in the data about hysteresis. CONCLUSION: This study suggests the Korean standard values of elastic properties in their twenties by Cutometer. The values measured by Cutometer and Dermaflex were not interchangeable although they were the same in the aspect of theoretical concept. These features should be considered in the future study measuring skin elastic properties.
Elasticity ; Reference Values ; Skin* ; Suction ; Volunteers

No abstract available.
Protein-Losing Enteropathies

We report 3 cases of recurrent acute disseminated encephalomyelitis (ADEM) known as multiphasic disseminated encephalomyelitis (MDEM). The 3 cases presented with fever, headache, neck stiffness, abdominal pain, seizure and decreased mentality as well as focal neurologic deficits, which showed relatively good steroid responsiveness. Brain MRI revealed variable sized, multifocal, subcortical white matter lesions with gray matter involvement. The CSF study showed mild pleocytosis with negative oligoclonal band.
Abdominal Pain ; Brain ; Encephalomyelitis* ; Encephalomyelitis, Acute Disseminated ; Fever ; Headache ; Leukocytosis ; Magnetic Resonance Imaging ; Neck ; Neurologic Manifestations ; Seizures

A 74-year old woman presented with partial and secondarily generalized status epilepticus lasted for 11 days. Initially her seizures consisted of only unformed visual hallucination, which progressed to formed hallucinations, and then memory disturbance and GTCs. During the period of recurrent formed visual hallucinations, T2-weighted brain MRI revealed high signal intensities in the left occipital lobe. After intravenous phenytoin loading, she did not develop any further GTCs but visual hallucinations persisted. Follow-up MRI performed after complete recovery of seizures showed complete recovery of the previous focal lesions, however, 1H-MRS showed a significant decrease of NAA in the recovered area. These features suggested the neuronal loss in the area of seizure focus, despite the complete recovery of transient focal abnormalities in MRI. This case provides a supportive evidence of neuronal damage even in focal status epilepticus, which stress the importance of early treatment and EEG confirmation of the complete seizure control after the disappearance of clinically obvious seizures.
Aged ; Brain ; Electroencephalography ; Female ; Follow-Up Studies ; Hallucinations ; Humans ; Magnetic Resonance Imaging ; Memory ; Neurons* ; Occipital Lobe* ; Phenytoin ; Seizures ; Status Epilepticus*