Relapsing polychondritis is a rare disease characterized by widespread destructive inflammatory lesions, involving cartilaginous tissue throughout the body. Commonly involved organs include the external ear, nose, joints, eyes, tracheobronchial tree, cardiovascular system and cutaneous tissues. Erythema nodosum or mesenteric panniculitis have sometimes been described in association with relapsing polychondritis, but cutaneous panniculitis is rarely reported in relapsing polychondritis. We report here a relapsing polychondritis patient who developed cutaneous panniculitis, which was resolved by corticosteroid therapy.
Cardiovascular System ; Ear, External ; Erythema Nodosum ; Humans ; Joints ; Nose ; Panniculitis* ; Panniculitis, Peritoneal ; Polychondritis, Relapsing* ; Rare Diseases

Myasthenia gravis is a chronic neuromuscular disease with the chief complaints of muscle weakness and generalized fatigue. Many difficult problems may be encountered in the anesthetic management and the postoperative respiratory management. The authors anesthetized 2 cases of myasthenia gravis for thymectomy with success and these experiences are presented in this report with a brief review of the literature relevant to anesthetic management in patients with myasthenia gravis.
Fatigue ; Humans ; Muscle Weakness ; Myasthenia Gravis* ; Neuromuscular Diseases ; Thymectomy*

OBJECTIVES: Gallium 67(Ga-67) scintigraphy has been used to diagnose inflammatory and neoplastic diseases. We undertook a study to determine the clinical value of Ga- 67 renal scan in patients with various glomerular diseases. METHODS: Ga-67 scintigraphy was performed in 48 patients with various biopsy proven forms of renal diseases. Renal uptake in 48 patients images was graded as follow: Grade 0 = not visualization at 48 hours: 1 = faintly visualize: 2 = equal to uptake in spine: 3 = greater than over the spine: 4 = greater than activity over the liver. RESULTS: 1) Of the 48 patients, 31 were male, and mean age was 32 years. 11 patients had hypertension and 29 patients had hematuria. 2) Positive scintigram were seen in 40 of 48(83%) cases. In results of renal biopsy, IgA nephropathy(IgAN) was 15 patients, minimal change disease(MCD) was 14, focal segmental glomerulosclerosis (FSGS) was 8, membranoproliferative glomerulonephritis (MPGN) was 3, lupus nephritis(LN) was 3, poststreptococcal glomerulonephritis(PSGN) was 3 and membranous glomerulonephritis(MGN) was 2. 3) In 26 patients (54%) with nephrotic-range proteinuria, Grade 2 or higher renal uptake was observed in 9 (75%) of MCD, 5(100%) of FSGS, 2(100%) of LN and 3(75%) of IgAN. 4) In comparision nephrotics with non-nephrotics at biopsy, renal Ga-67 uptake in who patients had nephrotic-range proteinuria was correlated with clinical severity determined by serum albumin, serum total cholesterol and 24 hours urine protein excretion. CONCLUSIONS: Renal Ga-67 scintigraphy may be able to be a predictor in the assessment for severity of nephrotic syndrome.
Biopsy ; Cholesterol ; Gallium ; Glomerulonephritis ; Glomerulonephritis, Membranoproliferative ; Glomerulosclerosis, Focal Segmental ; Hematuria ; Humans ; Hypertension ; Immunoglobulin A ; Liver ; Male ; Nephrotic Syndrome ; Proteinuria ; Radionuclide Imaging* ; Serum Albumin ; Spine

OBJECTIVE: This study was performed to investigate the prognostic value of multi-sensory evoked potentials (MSEPs) in neonatal period for the early diagnosis of delayed motor development, especially cerebral palsy. METHOD: The MSEPs studies composed of auditory brainstem evoked potentials (AEPs), visual evoked potentials (VEPs) and somatosensory evoked potentials were taken on 237 neonates, 136 boys and 101 girls, using Viking IV machine. Follow up MSEPs were repeated in every 4 or more weeks for those who showed abnormal responses in any of the MSEPs. Each neonate was also evaluated for motor development as an outpatient or by telephone interview. RESULTS: Among 237 neonates, 6.4% showed delayed development, and 4.6% were cerebral palsy: 3.8%, spastic type; 0.8%, athetoid type, and the others revealed normal motor development. AEP was useful method to predict motor development when this was done at 39.7 0.4 weeks of postmenstrual age (PMA). VEPs failed to show the validity, but there was the typical waveform change in accordance with increase of the postmenstrual age. Median nerve SEPs were valuable for prediction of motor development which were taken at PMA 40.7 0.6 weeks. After 45.3 1.5 weeks of PMA, median nerve SEPs did not reflect motor development outcome significantly. However, posterior tibial SEPs significantly reflect motor outcome regardless of the time of examination. CONCLUSION: Median and posterior tibial SEPs done before 40weeks of PMA are useful tool to predict motor development outcome. When any of these tests showed abnormal findings, follow up study is recommended and posterior tibial SEP study is thought to be the most useful for its predictability. It is necessary to correlate the AEPs and VEPs with hearing and vision whenever abnormal findings are found.
Brain Stem ; Cerebral Palsy ; Early Diagnosis ; Evoked Potentials* ; Evoked Potentials, Auditory ; Evoked Potentials, Somatosensory ; Evoked Potentials, Visual ; Female ; Follow-Up Studies ; Hearing ; Humans ; Infant, Newborn* ; Interviews as Topic ; Median Nerve ; Muscle Spasticity ; Outpatients

The purpose of this study was to determine whether esterification of dehydroepiandrosterone with aspartate (DHEA-aspartate) could reduce peroxisomal proliferation induced by DHEA itself, without loss of antiosteoporotic activity. Female Sprague-Dawley rats were ovariectomized, then DHEA or DHEA-aspartate was administered intraperitoneally at 0.34 mmol/kg BW 3 times a week for 8 weeks. DHEA-aspartate treatment in ovariectomized rats significantly increased trabeculae area in tibia as much as DHEA treatment. Urinary Ca excretion was not significantly increased by DHEA or DHEA-aspartate treatment in ovariectomized rats, while it was significantly increased by ovariectomy. Osteocalcin concentration and alkaline phosphatase activity in serum and cross linked N-telopeptide type I collagen level in urine were not significantly different between DHEA-aspartate and DHEA treated groups. DHEA-aspartate treatment significantly reduced liver weight and hepatic palmitoyl-coA oxidase activity compared to DHEA treatment. DHEA-aspartate treatment maintained a nearly normal morphology of peroxisomes, while DHEA treatment increased the number and size of peroxisomes in the liver. According to these results, it is concluded that DHEA-aspartate ester has an inhibitory effect on bone loss in ovariectomized rats with a marked reduction of hepatomegaly and peroxisomal proliferation compared to DHEA.
Adjuvants, Immunologic/pharmacology* ; Adjuvants, Immunologic/metabolism ; Adjuvants, Immunologic/chemistry ; Animal ; Aspartic Acid/pharmacology* ; Aspartic Acid/metabolism ; Aspartic Acid/chemistry ; Biological Markers ; Calcium/urine ; Calcium/blood ; Disease Models, Animal ; Esterification ; Fatty Acid Desaturases/metabolism ; Female ; Injections, Intraperitoneal ; Lipoproteins, HDL Cholesterol/blood ; Lipoproteins, LDL Cholesterol/blood ; Liver/enzymology ; Liver/drug effects ; Organ Weight ; Osteoporosis/pathology ; Osteoporosis/metabolism* ; Osteoporosis/drug therapy* ; Ovariectomy* ; Peroxisomes/metabolism* ; Prasterone/pharmacology* ; Prasterone/metabolism ; Prasterone/chemistry ; Rats ; Rats, Sprague-Dawley ; Tibia/pathology ; Tibia/metabolism ; Triglycerides/blood

Visual evoked potentials(VEPs) are the cerebral electrical activities recorded from the occipital scalp following a flash or pattern stimulation and can detect the lesions of sensory visual pathways. Although the VEPs change with the maturation of CNS in children, a few studies have documented the maturational changes in premature infants. Using the light- emitting diode goggles, VEPs were studied in 131 neurologically intact infants of 28~41 weeks gestational age or 34~59 weeks postmenstrual age. The VEPs were analysed by three waveforms; normal, abnormal, and flat, and four patterns; N300, P200-N300, P100-N155-P200-N300, and P100 pattern. Normal waveforms were 63 of 131 VEPs(48.1%). Of the normal waveforms, N300 patterns were 38.1%, P200-N300 36.5%, P100-N155-P200-N300 19.0%, and P100 6.4%. Each pattern correlated with the postmenstrual age. These findings support the hypothesis of VEP pattern changes according to the maturation of the visual system with age.
Child ; Evoked Potentials, Visual* ; Eye Protective Devices ; Gestational Age ; Humans ; Infant ; Infant, Newborn ; Infant, Premature* ; Scalp ; Visual Pathways

It has been considered that the nutrition affects the mortality of chronic renal failure patients. Thus, several studies reported the method of examination about nutritional status in chronic renal failure patients. The purpose of the present study was to evaluate the nutritional status of 32 chronic renal failure patients, and recommend DEXA as a objective method. Thirty two chronic renal failure patients and 24 disease-free persons on kidney(control group) were included in this syudy. There were no difference in mean age, sex, weight, height, body mass index between two groups. We measured % total body fat, fat-free mass, limb fat, trunl fat, limb fat/trunk fat ratio with Hologic QDR 4500. The results were as follows. 1)% Total body fat of chronic renal failure patients was 20.7+/-7.9%. That was lower than 26.3+/-7.9% of control group(P<0.05). 2)Fat-free mass of chronic renal failure patients revealed lower result compared with control group. Each result was 40.2+/-12.2kg, 46.2+/-9.1kg(P<0.05). 3)Despite of no significant difference between two groups in ratio of limb fat and trunk fat, limb fat and trunk fat were statistically different(Limb fat:CRF-5.6+/-2.4kg, Control-7.1+/-1.9kg, Trunk fat:CRF- 5.2+/-3.0kg, Control-7.7+/-2.7kg)(P<0.05). 4)Triceps skinfold thockness was positively correlated with % total body fat, limb fat, trunk fat, and negatively correlated with fat-free mass. Mid- arm muscle area circumference had inverse relationship above(P<0.05). 6)Severe malnurished patients(SGA-C group) had lower result compared to mild-moderate malnurished patients(SGA-B group) about %TBF, LF,TF, total lymphocyte count. In addition to DEXA, we examined triceps skinfold thickness and mid-arm muscle area circumference. The result showed significant comparison between two groups, too. But albumin, cholesterol, potassium level was no statistical significant difference, except lymphcyte count. In conclusion, as suggested by above results, chronic renal failure patients has protein-calorie malnutrition. This nutritional status affects long-term survival of chronic renal failure patients. DEXA can give objective data accompanied with simple anthropometric measurement in nutriti onal status. Thus, we recommend DEXA as objective method of nutritional examination. In future, more precise method will be discovered, and that contribute more long-term survival of chronic renal failure patients.
Adipose Tissue ; Arm ; Body Height ; Cholesterol ; Equidae* ; Extremities ; Humans ; Kidney Failure, Chronic* ; Lymphocyte Count ; Malnutrition ; Mortality ; Nutritional Status ; Potassium ; Protein-Energy Malnutrition ; Skinfold Thickness

Uncontrolled infection quite often 1eads to systemic inflammatory response syndrome and multi-organ dysfunction syndrome. Despite advances in medical knowledge and technology, the mortality of patient with sepsis is still 35-60%, and even reach up to 50-90% in septic patients having acute renal failure. The purpose of this study was to examine the characteristics and predictive factors of progression to acute renal failure(ARF) in sepsis. We analyzed the bacteriologic and laboratory data of 54 admitted patients with SIRS (systemic inflammatory response syndrome) at Pusan National University Hospital from July 1997 to July 1999 (ARF 23 vs non-ARF 31). Multiple factor which may influence mortality and progression to AEK in sepsis, were evaluated and measured on admission day. The following of results, 1) Of the 54 patients, 23 were ARF group and 31 were non-ARF group. Mean age were, 52 years and 51 years. The mortality of ARF group and non-ARF group were 78% and 23%, Urine output, albumin, cholesterol, mean arterial blood pressure and evidence of underlying disease were not statistically different in each group. 2) Although the sources of sepsis could not identified in 9% (ARF), 23% (non-ARF), the others had the primary site of infections: gastrointestinal tract (35% vs 29%), lung (30% vs 19%), genitourinary tract(9% vs 13%), skin (17% vs 16%). 3) Although statistically not different, gram-positive bacterial infection was more common in ARF group (mainly staphylococcus aureus). Culture negative results were 4 patients (ARF), 1 patient (non-ARF). 4) APACHE III score in ARF group was higher than non-ARF group (48.1+/-16.5 vs 30.2+/-15.6). Liafio score in ARF group was higher than non-ARF group (39.1+/-13.0 vs 28.9+/-8.3). 5) APACHE III score and Liailo score in non-survivors were higher than survivors(APACHE III score: 48.6+/-15.3 vs 28.1+/-14.0, Liaho score:37.9+/-12.0 vs 29.4+/-9.2) 6) APACHE lII system was positively correlated with Liaho system (r=0.512, p=0.001). In conclusion, APACHE III system and Liaho system were significant predictors of progression to ARF and mortality in sepsis. In the future, prospective and multicenter studies are required to improve the method of treatment and the prognosis in sepsis.
Acute Kidney Injury* ; APACHE* ; Arterial Pressure ; Busan ; Cholesterol ; Gastrointestinal Tract ; Gram-Positive Bacterial Infections ; Humans ; Lung ; Mortality ; Prognosis ; Sepsis* ; Skin ; Staphylococcus ; Systemic Inflammatory Response Syndrome

PURPOSE: The protein kinase Chk1 is required for cell cycle arrest in response to DNA damage and is shown to play an important role in the G2/M checkpoint. The aim of this study was to investigate the relationship between microsatellite instability and frameshift mutation of the Chk1 gene in gastric cancers. MATERIALS AND METHODS: The microsatellite instability was analyzed in 95 primary gastric carcinomas by using microdissection and 6 microsatellite markers. We also performed single strand conformational polymorphism and sequencing to detect frameshift mutation of the Chk1 gene. RESULTS: We found positive microsatellite instability in 19 (20%) of the 95 gastric cancers, 13 high- and 6 low-frequency microsatellite instability cases. The frameshift mutation of Chk1, which resulted in a truncated Chk1 protein, was detected in two high-frequency microsatellite instability cases. CONCLUSION: These data suggest that the microsatellite instability may contribute to the development of gastric carcinomas through inactivation of Chk1.
Cell Cycle ; Cell Cycle Checkpoints ; DNA Damage ; Frameshift Mutation ; Microdissection ; Microsatellite Instability* ; Microsatellite Repeats* ; Protein Kinases ; Stomach Neoplasms*

BACKGROUND: Massive hemoptysis is one of the major medical emergency with high risk of mortality. Though the best predictor of mortality associated with hemoptysis appears to be the amount of bleeding within the first 24 hours, catastrophic hemorrhage could be occurred to the patients who were apparently in a stable condition with scanty hemoptysis at the time of admission. We evaluated APACHE III score system to find if it could be a prognostic index that can predict the mortality of the patients with hemoptysis. METHODS: We identified all the patients who had admitted with hemoptysis in the Medical Intensive Care Unit of Asan Medical Center between May 25, 1989, and July 31, 1995. A retrospective analysis was done in 66 patients with hemoptysis on APA- CHE III score. RESULTS: The overall mortality rate was 17.4% (12/69). In univariate analysis of possible prognostic factors, independent predictors of mortality were age(P=0.016), amount of hemoptysis(P=0.012), AaDO2 (P=0.017), requirement of transfusion(P=0.036), mechanical ventilatory care(P<0.05) and APACHE III score(P=0.02), In multivariate analysis with sex, age, amount of hemoptysis, AaDO2, requirement of transfusion and APACHE III score, APACHE III score was the only independent predictor of mortality(P=0.015, odd ratio=19.3, 95% confidence interval, 3.4 to 249.7) CONCLUSION: APACHE IU score may be a clinically significantly important independent predictor of outcome in the patients with hemoptysis. In addition, invasive procedure, such as bronchial artery embolizaticn or operation, could be considered in advance in the patients with more than 30 points of APACHE III.
APACHE* ; Bronchial Arteries ; Chungcheongnam-do ; Emergencies ; Hemoptysis* ; Hemorrhage ; Humans ; Intensive Care Units* ; Critical Care* ; Mortality ; Multivariate Analysis ; Prognosis ; Retrospective Studies