Miller-Dieter syndrome consists of severe type I lissencephaly, abnormal facial appearance, and sometimes other birth defects. Lissencephaly is a brain malformation manifested by a smooth cerebral surface, thickened cortical mantle, and microscopic evidence of incomplete neuronal migration. It comprises the agyria-pachygyria spectrum of malformation, thus excluding polymicrogyria and other cortical dysplasia. Type I lissencephaly results from abnormal migration between about 10 and 14 weeks gestaion. The brain is often small, and the ventricle is enlarged posteriorly The corpus callosum may be small or absent. The structural pattern of the cerebral hemispheres and ventricles is distintly immature, reminiscent of fetal brain. The superficial cellular layer resembles an immature cortex, with some separation into zones similar to layers III, V, and VI of normal cortex, although the cell population is decreased. In 1963 Miller described a malformation syndrome in a brother and sister with postnatal growth deficiency, craniofacial defects, and serious abnormalities of neurologic function. Autopsy at 3 and 4month of age, respectively, revealed lissencephaly. Subsequently, Dieker reported four additional patients with this disorder and referred to it as the 'lissencephaly syndrome'. We have experienced a case with this syndrome. Then we report this rare case with brief review of literature.
Autopsy ; Brain ; Cerebrum ; Congenital Abnormalities ; Corpus Callosum ; Humans ; Lissencephaly ; Malformations of Cortical Development ; Neurons ; Siblings

BACKGROUND: Because the propofol TCI software commands the syringe pump to deliver a rapid infusion at a rate of 1200 ml/hr until the pharmacokinetic model predicts that the selected target concentration has been reached, the hemodynamic changes are predicted. To this change, several methods could be considered and the fentanyl injection is one of them. METHODS: Sixty adult patients scheduled for orthopedic surgery were randomly alldegrees Cated into four groups according to amount of fentanyl injected during induction period(group 1: no fentanyl, group 2: 0.75 microgram/kg, group 3: 1.5 microgram/kg, group 4: 3.0 microgram/kg). Target plasma concentration of propofol was selected as 4.0 microgram/ml and this concentration was achieved using modification of Prys-Roberts method. We evaluated the hemodynamic effect of various doses of fentanyl that injected at Vdpeak effect time and determined the optimal dose of fentanyl during propofol induction using TCI mode. RESULTS: Induction dose(range: 1.25~1.31 mg/kg) of propofol and induction time(range: 46~76 sec) showed no difference among groups. Use of fentanyl was effective for blood pressure stability immediately after intubation, but not effective before and 3 min following intubation. The higher the dosage of fentanyl, the more stable the heart rate. CONCLUSION: It suggest that use of fentanyl for the prevention of abrupt hemodynamic change during propofol induction using target controlled infusion mode is not necessary.
Adult ; Blood Pressure ; Fentanyl* ; Heart Rate ; Hemodynamics* ; Humans ; Intubation ; Orthopedics ; Plasma ; Propofol* ; Syringes

No abstract available.
Humans ; Microcomputers*

Papillary eccrine adenoma is a rare sweat gland tumor with a characteristic histopathological appearance and has a benign clinical course. Five cases were reported in Korea since a report by Song et al. in 1988. We report a case of papillary eccrine adenoma in a 38-year-old male who had a firm, dark brown nodule on the medial surface of the left thigh. Microscopically, the tumor was composed of multiple dilated ducts lined by two or more layers of epithelial cells and the luminal cells showed papillary projections into the lumen in some tubules. Immunoperoxidase staining was positive for CEA, S-100, and EMA. We excised the lesion completely and no evidence of recurrence was observed for 6 months.
Adenoma* ; Adult ; Epithelial Cells ; Humans ; Korea ; Male ; Music ; Phenobarbital ; Recurrence ; Sweat Glands ; Thigh

No abstract available.
Animals ; Rats* ; Urinary Bladder*

No abstract available.
Pregnancy*

No abstract available.
Estrogens* ; Humans ; Receptors, Estrogen* ; Thyroid Diseases* ; Thyroid Gland*

PURPOSE: To evaluate the temporal evolution of the ischemic area and trace ratio, and to define ischemic penumbra within the hyperacute experimental focal cerebral ischemia model. MATERIALS AND METHODS: A focal cerebral ischemia model of middle cerebral artery occlusion (MCAO) was constructed in twelve Korean cats weighing 2 -3 Kg. T2-weighted images (T2WI) and diffusion-weighted images(DWI) were obtained using a 1.5T MR imager. Trace images were reconstructed after post-image processing with IDL 5.0. The trace ratio (ipsilateral trace value/contralateral trace value) was calculated in the ischemic core and periphery, and MR images were obtained at 1, 3, 6, and 24 hrs after MCAO. The twelve cats were divided into three groups, and 4, 5, and 3 cats were sacrified after obtaining MR images at 3, 6, and 24 hrs after MCAO, respectively. After 2% triphenyl tetrazolium chloride (TTC) solution and formalin preparation, the infarction area of the brain slice and T2WI and DWI trace images of the same slice were compared. The trace ratio was calculated at the peripheral portion of the ischemic core, which was the presumed ischemic penumbra in images obtained 1hr after MCAO. Changes in trace ratio in the ischemic core and infarction territory were also evaluated according to time. RESULTS: The trace ratio in the peripheral portion of the ischemic core was 0.71 +/- 0.03. The region where the trace ratio was 0.83 +/- 0.06 in images obtained 1 hour after MCAO was presumed to be ischemic penumbra; the region progressed to infarction in images taken during the next time period. In all cases the abnormal area of trace images was larger than that seen on DWI. The trace ratio was lower, by 6.2 %, 3 -6hrs after MCAO than at any other time. In cat number 3, the trace radio decreased rapidly and progressively, by 21%, during the first six hours. For 3 -6hrs after MCAO, the area of infarction showed progressive enlargement. CONCLUSION: Within six hours of MCAO, ischemic penumbra is progressively incorporated into the ischemic core. In the experimental focal cerebral ischemia model, an area of trace ratio larger than 0.71 and less than 1may be defined as ischemic penumbra.
Animals ; Brain ; Brain Ischemia* ; Cats* ; Formaldehyde ; Infarction ; Infarction, Middle Cerebral Artery ; Magnetic Resonance Imaging* ; Stroke*

BACKGROUND: Target Controlled Infusion (TCI) is designed to achieve a predicted target blood concentration based on population pharmacokinetics and it provides the closest approximation for any individual patients. This study determined which target is appropriate for propofol induction using a TCI in korean adullt patients premedicated with midazolam. METHODS: Sixty six patients (ASA I or II, 18~55 years) premedicated with midazolam were allocated randomly to receive an infusion to achieve and maintain a target blood concentration of 3, 4, 5 and 6 microgram/ml using a TCI. Induction time was measured as the interval from the start of the infusion to loss of verbal contact and induction within 3 min was considered as successful. Calculated concentration, induction dose, context sensitive decrement time, vital signs, pain score and side effects were checked and compared each other during induction period. RESULTS: The success rate when the target was 3 microgram/ml was 25%, 58.8%, 77.8% and 100% when targets were 4 microgram/ml, 5 microgram/ml and 6 microgram/ml respectively. EC50 for induction was 3.87 microgram/ml and EC95 was 5.71 microgram/ml. Calculated concentration, induction dose, context sensitive decrement time in 3, 4, 5 microgram/ml group showed no differences among groups, but 6 microgram/ml group showed statistically significant differences compared with other groups. Vital signs, pain score and side effects showed no differences among groups. CONCLUSIONS: Target concentration of 5~6 microgram/ml would successfully induce anesthesia in the majority of patients premedicated with midazolam without major hemodynamic changes.
Anesthesia* ; Hemodynamics ; Humans ; Midazolam ; Pharmacokinetics ; Propofol* ; Vital Signs

OBJECTIVE: To assess the relationship between vaginal cytology and bone mineral density in postmenopausal women. METHODS: In 93 postmenopausal women, vaginal cytology smears were taken for maturation index, serum estradiol level and bone mineral density were also taken. the percentage of each cell type found By vaginal cytology was multiplied to its specific value:superficial cells, 1.0; intermediate cell, 0.6; parabasal cell, 0.2 and modified to maturation value. Bone mineral density was measured at lumbar spine using DEXA. RESULTS: The mean bone mineral density was 0.88+/-0.14g/cm2. Mean maturation value was 50.53+/-20.74 and it was related with age and bone mineral density. CONCLUSION: Maturation value of vaginal epithelial cell represents the influence of estrogen on bone mineral density.
Bone Density* ; Epithelial Cells ; Epithelium* ; Estradiol ; Estrogens ; Female ; Humans ; Spine