No abstract available.
Hyperplasia* ; Islets of Langerhans* ; Nesidioblastosis*

Diurnally fluctuating hereditary progessive dystonia is a rare movement disorder characterized by marked diurnal fluctuation of symptoms and dramatic response to levodopa. We report two advanced female patients of two families who presented with progressive dystonia and gait disturbance. Both patients have a family history and showed excellent response to levodopa.
Dystonia* ; Female ; Gait ; Humans ; Levodopa ; Movement Disorders

BACKGROUND: Herpes simplex virus thymidine kinase (HSVtk) phosphorylates the prodrug ganciclovir to a nucleoside analog that inhibits DNA synthesis, causing cell death. Neighbouring nontransfected cells may be affected through a 'bystander effect', thereby amplifying the antiproliferative actions. This study was carried out to determine whether retrovirus-mediated HSVtk gene therapy could reduce intimal hyperplasia and prevent stenosis following balloon injury of the rat carotid artery. METHODS: A replication-defective recombinant retroviral vector containing HSVtk cDNA (LtkSN) was constructed. Cultured primary rat smooth muscle cells (SMCs) infected with this vector (SMC/LtkSN) were transplanted to the balloon injured rat right carotid artery. One week after transplantation, HSVtk gene therapy group was administered a 2-week treatment of ganciclovir (30 mg/kg/d). Three weeks after balloon injury and SMC/LtkSN transplantation, carotid arteriography was performed and carotid arteries were perfusion-fixed for histologic examination. RESULTS: Carotid arteriographic evaluation comparing with the uninjured left carotid artery showed that the mean luminal diameter of HSVtk gene therapy group (n=5, 85+/-3%) was significantly larger than that of balloon injury only group (n=5, 65+/-5%). The neointimal mass of HSVtk gene therapy group was less than that of balloon injury only group. SMC/LtkSN transplantation without ganciclovir treatment group (n=3) showed asymmetric intimal proliferation probably because of gravitational pooling of seeding. There were inflammatory cell infiltrations at the gravity dependent portion of HSVtk gene therapy group. CONCLUSION: Retrovirus-mediated HSVtk gene therapy following balloon injury of the rat carotid artery reduced neointimal expansion and arteriographic stenosis.
Angiography ; Animals ; Carotid Arteries* ; Carotid Artery Injuries* ; Cell Death ; Constriction, Pathologic* ; DNA ; DNA, Complementary ; Ganciclovir ; Genetic Therapy* ; Gravitation ; Herpes Simplex* ; Hyperplasia ; Myocytes, Smooth Muscle ; Phenobarbital ; Phosphotransferases* ; Rats* ; Simplexvirus* ; Thymidine Kinase ; Zidovudine

We present the case of a 33-year-old man who experienced a 10,000-V electrical shock when working with electrical wiring. He suffered third-degree burns on his scalp at the right occiput (entry wound) and on his left arm (exit would), and a second-degree burn on his left foot (exit wound). He presented with severe spasticity of both lower extremities, motor weakness with a Medical Research Council grade of 3, and sensory impairments below thoracic level 11 that included an inability to sense light touch and defects in proprioception. Initial magnetic resonance imaging (MRI) scans of his spine and brain showed no definite abnormalities. However, tractography obtained by diffusion tensor imaging of the brain showed absence of the right medial lemniscus tract. A cervical MRI scan 1 month later showed spinal cord swelling from cervical 1-5 levels, and signal changes in the lateral and posterior white matter in the axial view. After 6 months of rehabilitation, he recovered almost normal degree of motor function in his lower extremities and disappearance of spasticity. However, since the sensory impairments persisted, especially defects in proprioception, he was unable to walk independently.
Adult ; Arm ; Brain ; Burns ; Diffusion Tensor Imaging ; Electric Injuries ; Electric Wiring ; Foot ; Humans ; Lower Extremity ; Magnetic Resonance Imaging ; Muscle Spasticity ; Proprioception ; Rehabilitation ; Scalp ; Shock ; Spinal Cord ; Spine ; White Matter

No abstract available.
Humans* ; Retroviridae*

No abstract available.
Child* ; Emergencies* ; Emergency Service, Hospital* ; Humans ; Infant* ; Poisoning*

PURPOSE: The residual intrahepatic stones with biliary strictures are difficult to remove percutaneously via T-tube tract after surgery in patients with recurrent pyogenic cholangitis. We evaluated the effectiveness of percutaneous balloon dilatation of benign biliary strictures. MATERIALS AND METHODS: The balloon dilatations with 6-12mm angioplasty balloon catheter and stone extractions were performed via a T-tube tract after surgical treatment in 15 patients with recurrent pyogenic cholangitis. The balloons were inflated for 3-4minutes under 5 atm. until disappearance of waist of the stricture site, from one to seven session. After balloon dilatation, residual stones were extracted with saline irrigation or stone basket. RESULTS: Among total 42 strictures, the balloon dilatation was succesful in 27 strictures(64.3%), partially successful in 12 strictures(28.6%), and failed in three strictures(7.1%). Single or central biliary strictures were dilated easily rather than multiple ductal strictures. Of 26 sites with residual intrahepatic stones, stone extraction was complete in 17 sites(65.4%), incomplete in seven sites(26.9%) due to impacted or large stone and acute ductal angulation, and failed in two sites(7.7%). CONCLUSION: Percutaneous balloon dilatation of benign biliary strictures is an effective procedure for extraction of residual intrahepatic stones associated with recurrent pyogenic cholangitis.
Angioplasty ; Catheters ; Cholangitis ; Constriction, Pathologic* ; Dilatation* ; Humans

PURPOSE: It may be difficult to differentiate renal cell carcinoma involving collecting system from renal urothelial tumor invading into renal parenchyma. The purpose of this study was to assess the differences of CT findings between two conditions. MATERIALS AND METHODS: CT findings of 5 cases of renal cell carcinoma involving the renal collecting systems and 10 cases of renal urothelial tumors invading the renal parenchyma were compared, and analyzed about the presence or absence of hydronephrosis, normal or abnormal CT nephrogram, renal contour changes due to mass and tentative diagnosis. The diagnoses were confirmed at surgery. RESULTS: Renal cell carcinoma showed hydronephrosis in only 20% and normal CT nephrogram and outward contour bulging in all cases. In contrast, renal urothelial tumor showed hydronephrosis(70%), abnormal CT nephrogram(60%), and preservation of reniform shape(100%). CONCLUSION: Renal contour changes and CT nephrogram may be useful in distinguishing both disease entities.
Carcinoma, Renal Cell* ; Diagnosis ; Hydronephrosis

In Korean, there has been an increasing concern on rickettsiosis as a possile common cause of unknown febrile illness since Tsutsugamushi fever among koreans was reported first in 1986. We experienced 10 cases of Tsutsugamushi fever ocurring in the Mokpo area during the period of 3 months (Oct, to Dcc.) in 1990, which were diagnosed clinically and serologically by indirect immunofluorescent antibody test. The following results were obtained. The most frequent symptoms were fever (100%), headache (90%), chill (60%), conjunctival injection, and lymphadenopathy. Common laboratory findings were leukopenia (WBC<5000/mm3 30%), elevated ESR (>20mm/hr, 30%), positive CRP (60%), and elevated Alt/AST. Antibody titers against R. tsutsugamushi ranged from 1:80 to 1:1,280, but they showed no antibody reaction to Hantaan virus and leptospira. All patients showed good response to antibiotic therapy with chloramphenicol.
Chloramphenicol ; Fever ; Hantaan virus ; Headache ; Humans ; Jeollanam-do ; Leptospira ; Leukopenia ; Lymphatic Diseases ; Scrub Typhus*

PURPOSE: This study was performed to detect the diseases of congenital hypothyroidism by the neonatal mass screening test early and to demonstrate the possible role of topical iodinated antiseptics(povidone-iodine, PVP-I) on transient hyperthyrotropinemia in newborn infants. METHODS: We performed neonatal screening tests for inborn errors of metabolism since 1985 by Guthrie test for PKU, maple syrup urine disease, histidinemia, and homocystinuria and enzyme-linked immunosorbent test for TSH. And for our prospective study, 366 newborns with a appropriate gestational age (between 37 and 42 weeks) were submitted to TSH screening test. We divided the study subjects into 4 groups; the group used PVP-I for mother and alcohol for cord care was categorized as Group A, the group used PVP-I for both mother and cord care as Group B, the group used triple dye for mother and PVP-I for cord care as Group C, and the group used triple dye for mother and alcohol for cord as Group D. RESULTS: 1) By neonatal screening test for inborn errors of metabolism during the last 10 years, we detected one case of phenylketonuria (PKU), one case of histidinemia and 28 cases of congenital hypothyroidism. The incidence of congenital hypothyroidism was 1 in 3,918 live births. 2) The mean TSH levels were 6.25+/-5.09 microU/ml(male 5.85+/-4.77 and female 6.6+/-5.41 microU/ml) in Group A, 8.30+/-7.68 microU/ml(male 6.30+/-7.15 and female 9.98+/-7.77U/ml) in Group B, 7.42+/-5.35 microU/ml(male 7.40+/-6.11 and female 7.44+/-4.63 microU/ml) in Group C, and 6.33+/-1.95 microU/ml(male 6.19+/-1.90 and female 6.42+/-2.03 microU/ml) in Group D, There were statistically significance between Group A and Group B, and Group D, but there was no statistically significance between gender. 3) The number of the newborns showed high levels of TSH over 15 muU/ml were 4.2%, 26.7% and 7.7% in Group A, B, and C, respectively. But nobody showed high TSH level over 15 microU/ml in Group D without use of PVP-I. CONCLUSIONS: In conclusion, we might be concerned for neonatal mass screening test on a national scale to prevent mental retardation and it is necessary to switch the povidone-iodine to other preparations as a perinatal antiseptics because of the possibility of the effect on neonatal screening test for congenital hypothyroidism.
Anti-Infective Agents, Local ; Congenital Hypothyroidism* ; Female ; Gestational Age ; Homocystinuria ; Humans ; Incidence ; Infant, Newborn ; Intellectual Disability ; Iodine* ; Live Birth ; Maple Syrup Urine Disease ; Mass Screening* ; Metabolism, Inborn Errors ; Mothers ; Neonatal Screening ; Phenylketonurias ; Povidone-Iodine ; Prospective Studies ; Umbilical Cord