Gaucher's disease is an uncommon metabolic disorder, which was first described by Gaucher in 1882, characterized by accumulation of distinctive Gaucher's cells in the reticuloendothelial system such as spleen, liver, and bone marrow. The great majority of cases have been reported in Jews, and others in negros and orientals. We are presenting two cases in homozygous twins in Korea, whose clinical manifestations are hepatosplenomegaly and bone lesions due to expansion of involved bones.
African Continental Ancestry Group ; Bone Marrow ; Gaucher Disease ; Humans ; Jews ; Korea ; Liver ; Mononuclear Phagocyte System ; Spleen ; Twins

Intensive insulin therapy effectively delays the onset and slows the progression of nephropathy in patients with IDDM. TGF- 0 has recently been implicated in the pathogenesis of diabetic nephropathy. We evaluated the effects of different level of glucose control with insulin therapy on the progression of diabetic nephropathy in age-matched control rats(C) and 3 groups of streptozotocininduced diabetic rats', high blood glucose diabetic rats without insulin therapy(HG), rnoderate glucose diabetic rats with insulin therapy(MG), and normal glucose diabetic rats with intensive insulin treatment (NG). Glomerular volume(VG) was measured using Image-Pro morphometric software, glomerular TGF- Bl mRNA expression by in situ hybridization, and glomerular expression of TGF-8 and type IV collagen proteins by immunohistochemical staining. VG was significantly higher in HG than in other groups in 12 weeks. Kidney weight(KW) was the highest while the body weight the lowest in HG of all groups in 12 weeks. Daily urine albumin excretion (UAE) increased with time in all groups but was significantly larger in HG than in all other groups in 12 weeks. MG also had significantly larger UAE than C in 12 weeks. There was no difference in VG, KW, and UAE between NG and C. Glomerular TGF-Bl mRNA expression was significantly higher in HG than in all the rest of the groups in 4 and 12 weeks. Glomerular expression of TGF-B and type IV collagen proteins was proportional to the levels of blood glucose, being the highest in HG in 12 weeks. There was little or no expression of TGF-0 1 mRNA and protein or type IV collagen protein in NG. Thus these results support the view that high blood glucose is the prerequisite for glomerular injury in diabetes mellitus and that the glomerular injury in diabetes mellitus is mediated, in part, by TGF-01 and suppressed by glucose control.
Animals ; Blood Glucose* ; Body Weight ; Collagen Type IV ; Diabetes Mellitus ; Diabetes Mellitus, Type 1 ; Diabetic Nephropathies* ; Glucose ; Humans ; In Situ Hybridization ; Insulin* ; Kidney ; Rats ; RNA, Messenger

Purpose: To retrospectively compare the fixation methods for reattaching a trochanteric fragment in a pertrochanteric fracture treated with bipolar hemiarthroplasty. Materials and methods: Forty cases of an unstable femur pertrochanteric fracture were analyzed and followed up for average of 19(6~40) months. There were 18 cases fixed with tension band wiring (group 1), 7 cases treated using modified tension band wiring with K-wires (group 2) and 15 cases treated with the GTRD (Greater Trochanteric Reattachment Device) (group 3). Result: Group 1 was treated with simple a surgical procedure and the results were good. The second group had firm fixation postoperatively but required additional surgery in two cases to remove the K-wires because of wire migration after ambulation. Group 3 had relatively good results but required more dissection and a longer operating time. Conclusion: Tension band wiring or GTRD are good fixation methods for reattaching trochanteric fragments in pertrochanteric fractures. Moreover, tension band wiring is recommended for old osteoporotic patients due to a simple procedure and firm fixation. The use of modified tension band wiring using K-wire or a Steinmann-pin should not be used due to the possibility of distant migration.
Femur* ; Hemiarthroplasty* ; Humans ; Retrospective Studies ; Walking

Appendicitis is one of the most common disease in emergency department(ED), but produces perplexing diagnostic problems. A complete understanding of the anatomy, pathophysiology, and presenting signs and symptoms of appendicitis, combined with thorough history and physical examination, will be the most important factors in allowing emergency physician to make the correct diagnosis of appendicitis. Situs inversus totalis is a rare congenital anomaly occurring in every 10,000 to 50,000 people. Its etiology is obscure but apparently dose not influence normal health or life expectancy. We present a case of perforates appendicitis with situs inversus totalis in a 24-year-old woman whose chief complaint was pain on the left lower quadrant of the abdomen. Chest and abdominal X-ray showed situs inversus totalis, but these typical findings of situs inversus totalis were missed before performing abdominal ultrasonography in ED. We must carefully consider whether congenital anomaly is or not by physical examination and X-ray finding. For patients whose diagnosis is less clear, additional diagnostic tool, for instance ultrasonography is recommended in ED.
Abdomen ; Appendicitis* ; Diagnosis ; Emergencies ; Female ; Humans ; Life Expectancy ; Physical Examination ; Situs Inversus* ; Thorax ; Ultrasonography ; Young Adult

No abstract available.
Animals ; Ankle ; Blood Pressure ; Peripheral Arterial Disease

The object of this study was to investigate the influences of hypoglycemia and hypothermia on the direct current(DC) pontetial changes during cortical spreading depression(CSD) in rats. The induction of CSD was achieved by the application of KCI solution on the cortex of the frontal lobe. Hypoglycemia and hypothermia were induced respectively by insulin injection and the application of an ice pack. The DC potential changes during progressive hypoglycemia and hypothermia were measured with microelectrodes from the cortex of the parietal lobe of rats. Under contril condition, the rate of CSD was one per 5-10 min and the negative shift of DC potential was about 30 mV. The recovery time from negative shift to base line of DC potential was about 40 sec. In rats treated with insulin, the amplitude of DC potential shift was unaffected by hypoglycemia. The recovery time of DC shift was 40+/-2.26 sec at normoglycemia and it was delayed progressively as the blood glucose level lowered. The mean of it was 63+/-8.02 sec at 30 mg/dl and 77.1+/-22.0 sec with the blood glucose falling below 20 mg/dl. The same delay in the recovery time as seen in the hypogylcemia group was observed in rats treated with hypothermia. The recovery time of DC shift was 39.4+/-3.02 sec in normothermia(36.5degrees C), but it was delayed to 61.15+/-4.15 sec at 30degrees C and 96.67+/-14.92 sec at 26degrees C body temperature. This study suggested that each condition of profound hypoglycemia below 30 mg/dl and hypothermia below 30degrees C was to be harmful to the ion homeostasis and the integrity of the cell membrane and it may lead neurons to death.
Animals ; Blood Glucose ; Body Temperature ; Cell Membrane ; Cortical Spreading Depression* ; Frontal Lobe ; Homeostasis ; Hypoglycemia* ; Hypothermia* ; Ice ; Insulin ; Microelectrodes ; Neurons ; Parietal Lobe ; Rats*

Carboplatin intra-arterial chemotherapy(IAC) has an advantage of increased uptake during the first passage of the drugs through tumor capillaries. Although not common, this type of therapy is known to cause neurological complications, myelosuppression, and ototoxicity. However, the incidence of ocular toxicity is reported to be rare. Eleven of our patients with glioma(Grade II Astrocytoma: 3, Grade III Astrocytoma: 1, Grade IV Astrocytoma: 5, Gliofibroma: 1, Oligodendroglioma: 1) underwent IAC regimen with carboplatin(300mg/m2) which were administrated after blood-brain barrier disruption. Of there, 3 patients had ocular complications after supra-ophthalmic IAC injection of carboplatin but fully recovered following steroid therapy. Although our results from IAC seem to be favorable for these patients, we suggest that its complications, such as ocular toxicity, need to be carefully considered prior to treatment.
Astrocytoma ; Blood-Brain Barrier ; Capillaries ; Carboplatin* ; Glioblastoma ; Glioma* ; Humans ; Incidence ; Oligodendroglioma

Mitral valvular heart diseases are known to be one of the easily detectable and the hemodynamic chane to the severity and duration of diseases can directly be described by echocardiography. The objective of this study was to determine the echocardiographic characteristics in 3 groups of mitral valvular diseases, that is mitral stenosis(112 cases), mitral stenoinsufficiency (66 cases). and mitral insufficiency(20 cases). 1) In mitral valve study, closing velocity of anterior leaflet reduced in mitral stenosis than other two groups and DE amplitude was greater in mitral insufficiency but there were no significant differences in 3 groups of mitral valvular diseases. DE and AC slope were slightly more rapid in mitral stenosis group and left ventricular outflow tract was much increased in mitral insufficiency group. 2) In aorta and left atrium study, aortic cusps separation was much increased in mitral insufficiency and left atrium was slightly more dilated in mitral stenoinsufficiency than other two groups. But left atrial dimension in mitral insufficiency was more diminished than that in mitral, stenosis, which is probably due to the short durationn of diseases and small range of materials in mitral insufficiency group. 3) In left ventricle study, thickness of interventricular septem, LSa, Ena, left ventricular dimension, LVPW, LV volume. and stroke volume were more increased in mitral insufficiency than mitral stenosis, because of the left ventricular volume overloading. Vcf and PEP/LVET were higher in mitral insufficiency than other two groups. Ejection fraction and fractional shortening were more diminished in mitral stenoinsufficiency but no significant difference was found. 4) In right ventricle study, there were no remarkable changes in right ventricular dimension and right ventricular internal dimension index in all 3 groups of mitral valvular diseases. 5) Calcification of mitral leaflets was observed in 85.7% of mitral stenosis, 90.9% of mitral stenoinsufficiency and 10% of mitral insufficiency, but heavy calcification was more remarkable in mitral stenosis group(25%). 6) Atrial fibrillation was observed in 47% of total mitral valvular diseases, mitral stenosis being 43.8%, mitral stenoinsufficiency 60.6% and mitral insufficiency 20%. In cases of atrial fibrillation, left atrial dimension was significantly enlarged compared with the group without atrial fibrillation.
Aorta ; Atrial Fibrillation ; Constriction, Pathologic ; Echocardiography* ; Heart Atria ; Heart Valve Diseases* ; Heart Ventricles ; Hemodynamics ; Mitral Valve ; Mitral Valve Insufficiency ; Mitral Valve Stenosis ; Stroke Volume

PURPOSE: Transient global amnesia (TGA) patients were retrospectively reviewed to determine the usefulness of high-field strength MRI in detecting probable ischemic lesions in TGA. MATERIALS AND METHODS: We investigated the lesion detection rate in patients with TGA using 1.5T and 3.0T MRI. Acute probable ischemic lesions were defined as regions of high-signal intensity in diffusion weighted image with corresponding low-signal intensity in apparent diffusion coefficient map. RESULTS: 3.0T MRI showed 11 out of 32 patients with probable ischemic lesions in the hippocampus with mean lesion size of 2.8 +/- 0.6 mm, whereas 1.5T MRI detected no lesion in any of 11 patients. There were no significant differences in clinical characteristics between the groups of 1.5 and 3.0T MRI. CONCLUSION: High-field strength MRI has a higher detection rate of probable ischemic lesions than low-field strength MRI in patients with TGA.
Adult ; Aged ; Aged, 80 and over ; Amnesia, Transient Global/*diagnosis/pathology ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Retrospective Studies ; Young Adult

PURPOSE: To evaluate the size of the olfactory bulb using MRI in normal volunteers and anosmic or hyposmicpatients without nasal diseases. MATERIALS AND METHODS: MRI was performed in 20 normal volunteers with a normalsense of smell, and in 15 anosmic or hyposmic patients without nasal disease but with abnormality in the olfactoryfunction test. Coronal T1-weighted MRI was performed, with a section thickness of 3mm. The cross sectional area,width and height of the olfactory bulb were measured in multiple sequential images and the largest values of thesewere analysed. The difference in the size of the olfactory bulb between normal volunteers and anosmic or hyposmicpatients was evaluated and Student's t test was used for statistical analysis. RESULTS: In most cases, theolfactory bulb was demonstrated in three sequential coronal images; in normal volunteers, the largest crosssectional area, width and height were not significantly different between the right and left olfactory bulb. In 40 olfactory bulbs(right, left) in 20 normal volunteers and 30 olfactory bulbs in 15 anosmic or hyposmic patients,the respective means of various measurements were as follows: 7.5mm2 and 6.0mm2; greatest width, 4.6mm and 3.8mm;greatest height, 2.7mm and 2.0mm. For the largest cross-sectional area and greatest height, the difference inolfactory bulb size between normal volunteers and patients was statistically significant (P<0.01) but for thegreatest width, the difference was not significant (P>0.05). CONCLUSION: The size of the olfactory bulb issignificantly less in anosmic or hyposmic patients without nasal disease than in normal volunteers; in suchpatients, olfactory MRI could be a useful evaluative modality.
Cranial Nerves ; Healthy Volunteers* ; Humans ; Magnetic Resonance Imaging* ; Nose Diseases ; Olfactory Bulb* ; Smell