Syringomyelia was described by Etienne et al in 1954 and was named by Ollivier in 1827 by reason of the pathologic cavitation of cord. Also it is considered a very chronic and irregulary progressive disease of the spinal cord of unknown cause which has predilection for the cervical and lumbar enlargements. The patient of 19 years old male had been suffered from the painless, severe swelling and instability of right elbow after trauma on right elbow, aud had complained of the sensory and temperature dissociation, muscle weakness with clawing hand in right upper extremity and scoliosis of mild degree. These symptoms and findings were same as those of the classic syringomyelia. Therefore the syningomyelia which is relatively rare in incidence will be reviewed by this report with the references.
Animals ; Elbow ; Hand ; Hoof and Claw ; Humans ; Incidence ; Male ; Muscle Weakness ; Scoliosis ; Spinal Cord ; Syringomyelia ; Upper Extremity

No abstract available.
Evoked Potentials, Motor*

PURPOSE: An atrophic renal scar(RS) is one of the underlying causes for childhood hypertension and chronic renal failure. The risk factors for atrophic renal scar were evaluated. METHODS: 41 children, who presented with first febrile urinary tract infection at the Ewha Womans University Hospital between 1995 and 2003 and had generalized atrophic RS on 99mTc-DMSA renal scan, were retrospectively studied. Atrophic RS was divided into severe atrophic RS(n=14) if relative uptake on renal scan was below 10%, or mild atrophic RS(n= 27) if relative uptake on renal scan was between 10-35%. RS was defined as congenital if the scar was detected on the first renal scan, and as acquired if the scar developed on the follow-up renal scan from acute pyelonephritis of the first renal scan. The control group was consisted of randomly selected 41 children with segmental RS. The risk factors for atrophic RS such as the generation time, VUR, gender and ACE gene polymorphism were evaluated. RESULTS: The age distribution of atrophic RS and segmental RS did not differ significantly (P>0.05). The rate of congenital RS in atrophic RS was 61.0%(25/41), which was significantly higher than 9.8%(4/41) of segmental RS(P<0.01). Atrophic RS developed more frequently in male children(M:F 68.3%:31.7%) than segmental RS(M:F 41.4%:58.6%)(P<0.05). Vesicoureteral reflux(VUR) was found in 92.7%(38/41) of the atrophic RS, which was significantly higher than 53.7%(22/41) of segmental RS(P<0.05). In children without VUR, the male to female ratio did not differ between atrophic RS and segmental RS(P>0.05). But in children with VUR, there was a higher proportion of males with severe atrophic RS than segmental RS(85.7%:45.5%). ACE gene polymorphism did not differ between the atrophic and segmental RS groups, irrespective of the presence of VUR(P>0.05). CONCLUSION: Most atrophic RSs were congenital which could not be preventable postnatally and the major risk factors were VUR and the male gender. ACE gene polymorphism was not the significant risk factor for an atrophic RS.
Age Distribution ; Child ; Cicatrix* ; Female ; Follow-Up Studies ; Humans ; Hypertension ; Kidney Failure, Chronic ; Male ; Pyelonephritis ; Retrospective Studies ; Risk Factors* ; Technetium Tc 99m Dimercaptosuccinic Acid ; Urinary Tract Infections ; Vesico-Ureteral Reflux

BACKGROUND: Intravenous immunoglobulin (IVIg) has been widely used in the management of patients with various autoimmune neurological diseases, however, its action mechanisms have not fully been elucidated yet. This study focused on the effects of IVIg on the production of interleukin-6 (IL-6), one of major proinflammatory cytokine, using a human skeletal muscle cell line (HM4). METHODS: After HM4 cells were cultured in Dulbecco's modified eagle's medium (DMEM) containing 5% fetal bovine serum for 24 h, the culture medium was changed with serum-free media. TNF-alpha (tumor necrosis factor-alpha, 100 ng/mL) and IVIg (5 mg/mL) were treated alone or in combination and cultured for various time. RT-PCR and ELISA kit were employed for mRNA expression and secretion of IL-6, respectively. RESULTS: Treatment with TNF-alpha or/and IVIg significantly induced IL-6 mRNA expression (p<0.001). Although IL-6 production was markedly increased by TNF-alpha (p<0.001), IVIg treatment alone or in combination with TNF-alpha had no effect on the production of IL-6 except at 6 h after the treatment. CONCLUSIONS: IVIg seems not to have a significant effect on IL-6 production as an action mechanism of its immunomodulatory capabilities, at least in the HM4 cell line.
Cell Line* ; Culture Media, Serum-Free ; Enzyme-Linked Immunosorbent Assay ; Humans* ; Immunoglobulins ; Immunoglobulins, Intravenous ; Interleukin-6* ; Muscle, Skeletal* ; Necrosis ; RNA, Messenger ; Tumor Necrosis Factor-alpha

PURPOSE: To evaluate the usefulness of three-dimensional(3D) Fourier transform(FT) gradient refocused acquisition in steady state(GRASS) technique for MR imaging of the knee. MATERIALS AND METHODS : Sixty-three kneesin 61 patients were imaged on the 1.5T MR system. We compared 3DFT GRASS technique with 2D spin echo(SE) techniquein terms of conspicuousness of the lesions of internal knee structures based on the results of arthroscopy or open surgery. As a SE technique, sagittal T1-and T2-weighted, and coronal fat-suppressed T2-weighted sequences were performed. Sixty contiguous axial scans with 0.7 or 1mm section thickness were performed using 3D GRASS technique, and we also evaluated arbitrarily reformatted images produced from the original axial voxel images. RESULTS: For the depiction of the tear, 3DFT GRASS was superior to 2D SE in three cases of medial meniscus, one of later almeniscus, and two of anterior cruciate ligament. Specificity of 3D GRASS was also higher than that af 2D SE inevaluation of lateral meniscus and anterior cruiciate ligament. There was no significant difference in MR diagnosis for tears of the posterior cruciate, medial collateral, and lateral collateral ligaments. 3D GRASS was superior in evaluating the extent and morphology of the torn menisci. CONCLUSION: The 3DFT GRASS technique was comparable or even superior to the 2D SE technique in the evaluation of the internal structure of the knee, andcan be expected to supplement standard MR knee techniques, especially in complicated cases of meniscal orligamentous tears.
Anterior Cruciate Ligament ; Arthroscopy ; Fourier Analysis* ; Humans ; Knee* ; Lateral Ligament, Ankle ; Ligaments ; Magnetic Resonance Imaging* ; Menisci, Tibial ; Poaceae* ; Sensitivity and Specificity

Studies describing the effects on blood pressure control by hemodialysis(HD) or continuous amulatory peritoneal dialysis(CAPD) have yielded conflicting results with respect to 24 hour blood pressure control, diurnal variation and blood pressure loads. The aim of the study was to investigate the effect of beginning with HD or CAPD on blood pressure control, diurnal variation and blood pressure loads using ambulatory blood pressure monitoring(ABPM). Twen-ty-seven end-stage renal disease(ESRD) patients(12 on HD and 15 on CAPD) were enrolled into the study. Patients with cardiovascular diseases, erythro-poietin therapy, or severe edema were excluded. ABPM were performed two times before and after the initiation of dialysis. Mean duration of interval between pre- and post-dialysis ABPM were 17+/-4 days on HD and 13+/-3 days on CAPD. Daytime and nighttime were defined as the time from 6:00 AM to 10:00 PM and from 10:00 PM to 6:00 AM of the next day. Systolic and diastolic loads were defined as the percentage of the incidence of systolic and diastolic blood pressure over l% and 90mmHg. Dipper meaning the presence of normal diurnal difference were defined as the differences of daytime- nighttime mean arterial pressure more than 5mmHg. In HD patients, mean systolic and diastolic blood pressure and mean arterial pressure were significantly decreased after dialysis during 24 hour, day- time and nighttime. In CAPD patients, those were also significantly decreased after dialysis during 24 hour, daytime and nighttime(p<0.05). Diurnal differences were increased after CAPD(3.3+/-9.4 vs 5.4+/-6.8mmHg) but decreased after HD(4.3+/-6.2 vs 2.4+/-10.8mmHg) and the differences of diurnal difference between two groups were significantly different(+2.1+/-9.0 vs 1.9+/-8.4mmHg, p<0.05). Proportions of dipper among patients were increased from 16.7 to 66.7% in HD and from 33.3% to 60% in CAPD without statistical significance between two groups. Systolic and diastolic loads were significantly decreased after HD(from 75.0+/-38.0 to 37.5+/-43.8%, from 45.2+/-29.7 to 12.5+/-12.8%, respectively, p<0.05) and after CAPD(from 63.1+/-30,1 to 32.3+/-27.1%, from 43.4+/-36.2% to 12.2+/-16.9%, respectively, p<0.05). Systolic and diastolic loads of daytime and nighttime were significantly decreased after each dialysis modality except nighttime diastolic pressure load in HD. In conclusions, both HD and CAPD improve BP control in ESRD patients. CAPD has more benefit to control of diurnal variations in ESRD patients.
Arterial Pressure ; Blood Pressure* ; Cardiovascular Diseases ; Dialysis ; Edema ; Humans ; Incidence ; Kidney Failure, Chronic ; Peritoneal Dialysis, Continuous Ambulatory* ; Renal Dialysis*

The objective of aneurysm surgery is to exclude the aneurysm from the circulation while preserving blood flow distal to the lesion. In certain situations, the aneurysm neck cannot be clipped safely or the parent vessel reconstructed, primarily in large or giant size with incorporation of parent vessels or perforating arteries, calcification at the aneurysm base, and fusiform or dissecting aneurysms. In such cases, occlusion of the parent vessel is a treatment of option. In many patients, however, sacrifice of the parent artery has an associated risk of ischemic stroke. Therefore, sacrifice of the parent vessel can be supplimented with distal revascularization to provide the necessary distal blood flow while allowing the aneurysm to be trapped. The indications, options, and surgical approaches are described with review of literatures. Finally the authors' experiences of revascularization in 7 patients with unclippable aneurysms are reported.
Aneurysm ; Aneurysm, Dissecting ; Arteries ; Humans ; Intracranial Aneurysm* ; Neck ; Parents ; Stroke

Microscopic polyangiitis is a primary systemic vasculitis which involve small arterioles and capillaries. Clinical manifestations show generalized weakness, fever, myalgia, weight loss. oliguria, proteinuria, skin rash, neuromuscular symtoms, and pulmonary, gastrointestinal symtoms. Prednisolone and cyclophosphamide therapy is effectve, more than 80% of patients with microscopic polyangiitis survive for longer than 5years. However, about 50% of patients will have at least one relapse in the 4-5years after initial treatment. There is a very strong correlation between the presence of ANCA(especially p-type) and microscopic polyangiitis. We herein report a case of microscopic polyandiitis with the review of literature. A 65 years-old woman admitted to our hospital due to general weakness, fever, generalized edema, and dyspnea. Laboratory result showed high serum creatinine and CRP, moderate proteinuria, and microscopic hematuria. P-ANCA was 1:1280 positive in indirect immunofluorescence method, and kidney biopsy showed focal segmental necrotizing glomerulonephritis, crescent formation, fibrin clots, PMN and lymphocytes infiltrations in small arterioles and capillries. Immunofluorescence study was negative. Her clinical feature, laboratory findings, and the kidney biopsy result were compatible with microscopic polyangiitis. The patient had been treated with prednisolone and cyclophosphamide. P-ANCA titration was decreased 1:320 at the 19th day of treatment and was negative about 2months later.
Aged ; Antibodies, Antineutrophil Cytoplasmic ; Arterioles ; Biopsy ; Capillaries ; Creatinine ; Cyclophosphamide ; Dyspnea ; Edema ; Exanthema ; Female ; Fever ; Fibrin ; Fluorescent Antibody Technique ; Fluorescent Antibody Technique, Indirect ; Glomerulonephritis ; Hematuria ; Humans ; Kidney ; Lymphocytes ; Microscopic Polyangiitis* ; Myalgia ; Oliguria ; Prednisolone ; Proteinuria ; Recurrence ; Systemic Vasculitis ; Weight Loss

Methemoglobin (MetHb) is an oxidation product of hemoglobin in which the sixth coordination position of ferric iron is bound to a water molecule or to a hydroxyl group. The most common cause of acquired MetHb-emia is accidental poisoning which usually is the result of ingestion of water containing nitrates or food containing nitrite, and sometimes the inhalation or ingestion of butyl or amyl nitrite used as an aphrodisiac. We herein report a case of MetHb-emia after ingestion of an aphrodisiac, later identified as dapsone by gas chromatograph/mass selective detector (GC/MSD). A 24-year old male was admitted due to cyanosis after ingestion of a drug purchased as an aphrodisiac. On arterial blood gas analysis, pH was 7.32, PaCO2 26.8 mmHg, PaO2 75.6 mmHg, and bicarbonate 13.9 mmol/L. Initial pulse oxymetry was 89%. With 3 liter of nasal oxygen supplement, oxygen saturation was increased to 90-92%, but cyanosis did not disappear. Despite continuous supplement of oxygen, cyanosis was not improved. On the fifth hospital day, MetHb was 24.9%. Methylene blue was administered (2 mg/kg intravenously) and the patient rapidly improved. We proved the composition of aphrodisiac as dapsone by the method of GC/MSD.
Administration, Oral ; Adult ; Antidotes/therapeutic use ; Aphrodisiacs/adverse effects* ; Case Report ; Cyanosis/drug therapy ; Cyanosis/chemically induced ; Cyanosis/blood ; Dapsone/adverse effects* ; Human ; Male ; Methemoglobinemia/drug therapy ; Methemoglobinemia/chemically induced* ; Methylene Blue/therapeutic use

Sodium concentration in the hemodialysis solution has been increased to prevent intradialytic hypotension after highly effective and shortened time hemodialysis(HD) was introduced in the late 70's. Many authors have pointed out that the high concentration in the dialysate sodium HD may be one of causes of increasing difficulty in the management of hypertension in HD patients. Sodium profiling hemodialysis (SPHD) is a modified form of high sodium dialysate HD. Even though sodium concentration is decreased progressively to the conventional level during the HD session, the time-averaged sodium concentration is usually higher compared to that of conventional HD. To evaluate the effect of dialysate sodium concentration on interdialytic blood pressure(BP) control, we conducted a cross over study. Eleven patients showing more than four episodes of intradialytic hypertension per month were studied(5 male, 6 female; 52+-13 years). All subjects underwent 8-week conventional HD(CHD)(dialysate Na+ 138mEq/L X 4 hour) and 8-week step-down SPHD(Na+ 150mEq/L X 2 hours> OR =140 X 1> OR =138 X 1) on the order of random assignment. At the end of each peiords, interdialytic 24-hour BP were measured by 24-hour Ambulatory BP monitor(ABPM : 90207, Space Labs, USA). 1) Time-averaged sodium concentration in dialy sate were 138mEq/L during SPHD and 144.5mEq/L during CHD. Pre HD serum sodium were not significantly different between two periods but post HD serum sodium and intradialytic increase of serum sodium significantly higher during SPHD period 138.1+/-0.5 v 141.1+/-0.6mEq/L, 0.5+/-0.6 v 2.6+/-0.4mEq/L, p<0.05). Dry weight was determined before the start of study and not changed throughout the study periods. Interdialytic weight gain and the amount of ulfrafiltration required to maintain the determined dry weight were significantly higher during SPHD period compared to those during CHD period(2.5+/-0.5 v 3.6+/-0.6 kg, 2.6+/-0.8 v 3.6+/-0.8kg, p<0.01). 2) The frequency of interdialytic hypotension was significantly reduced during SPHD period(23.9 v 15 %, p<0.01). But the frequency of symptoms requiring intervetion such as ultrafiltration adjustment or saline infusion was not different between two periods. Thirst during interdialytic period was significantly frequent during SPHD(37.8 vs 30% 138.1+/-0.5 v 141.1+/-0.6mEq/L, 0.5+/-0.6 v 2.6+/-0.4mEq/L, p<0.05). 3) Day-time, night-time and 24 hour mean systolic BP measured by 24 hour ABPM were significantly higher during SPHD period(149.2+/-4.8, 144.3+/-3.6, 146.6+/-4.1mmHg) than during CHD period(140.1+/-4.8, 133.0+/-4.1, 136.4+/-4.6mmHg, p<0.01). Day-time, night-time and 24 hour mean diastolic BP were also significantly higher during SPHD period(82.6+/-1.5, 84.1+/-1.4, 86.1+/-1.4mmHg) than during CHD period (78.7+/-2.2, 79.6+/-2.3, 81.8+/-2.2mmHg, p<0.05). 4) Systolic load and diastolic load by the criteria of higher than 150/90mmHg throughout the day increased significantly from 21.1+/-7.0 and 18.2+/-6.3% during CHD period to 41.7+/-9.9 and 28.4+/-4.7% during SPHD period. Diurnal difference was not different between the two periods but a significant number of dippers(36.4%) converted to nondipper during SPHD period. Our results shows SPHD increases interdialytic BP and its load. It also adversely alter diurnal variation and dipping status. The additional sodium load and an consequent excessive interdialytic weight gain aassociates with SPHD might contribute to this findings.
Blood Pressure* ; Female ; Humans ; Hypertension ; Hypotension ; Male ; Renal Dialysis* ; Sodium* ; Thirst ; Ultrafiltration ; Weight Gain