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

We report a case of a 47 years old woman who had secondary renal amyloidosis due to rheumatoid arthritis. She admitted our hospital due to generalized edema, weakness and polyarthralgia, and had been diagnosed as having rheumatoid atrthritis and treated irregularly with anti-rheumatic drugs for 3 years. She had nephrotic range proteinuria (24 hour urine protein was 3.8 gm/day), hypoallbuminemia and pitting edema on both lowers. In kidney biopsy, amyloid (AA type) was deposited in renal glomerular, tubule and vessel walls. We diagnosed secondary renal amyloidosis.
Amyloid ; Amyloidosis ; Antirheumatic Agents ; Arthralgia ; Arthritis, Rheumatoid* ; Biopsy ; Colitis, Ulcerative* ; Edema ; Female ; Humans ; Kidney ; Middle Aged ; Proteinuria ; Ulcer*

Although the most of mushroom poisoning have a clinical menifestation of mild to moderate gastroenteritis, some mushroom may cause a serious illness; acute renal failure, hepatic necrosis. We experienced two cases of acute renal failure complicated by the poisoning of amanita virosa. Amanita virosa have a amatoxin. Amatoxin deteriorate hepatocytes, renal tubular cells, intestinal mucosal cells, and pancreas. They were transferred from local hospital for renal failure management. On admission, blood urea nitrogen and serum creatinine were highly elevated. We diagnosed acute renal failure complicated by poisoning of amanita virosa. In one case, renal function was further deteriorated compared with initial laboratory findings after creatinine was normalized at fifth day. Thus, we did a kidney biopsy. Light microscopy and EM showed interstitial inflammation and moderate tubular atrophy. They were recovered with the supportive management. We report two cases of mushroom poisoning-induced acute renal failure with review of literature.
Acute Kidney Injury* ; Agaricales ; Amanita* ; Atrophy ; Biopsy ; Blood Urea Nitrogen ; Creatinine ; Gastroenteritis ; Hepatocytes ; Inflammation ; Kidney ; Microscopy ; Mushroom Poisoning ; Necrosis ; Pancreas ; Poisoning* ; Renal Insufficiency

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

Aminoglycoside has a broad-spectrum bactericidal activity and especially excellent effect on gram negative bacteria. But, its harmful effect is existed about kidney and ear. Among these, many studies were done about aminoglycoside nephrotoxicity. Until now, many authors have been reported its incidence was 4.45-45%. We needed parameters monitoring about nephrotoxicity. So, our study was done using 99mTc-DTPA renal scan to evaluate nephrotoxicity. We enrolled forty patients who admitted due to infection. 32 patients received Netilmicin and 8 patients Isepamicin. Mean duration and dosage were 8 days, 200mg daily. We accomplished 99mTc-DTPA renal scan, 24hr urine study and blood sampling before starting antibiotics. After mean 8 days, same procedure was repeated. Aminoglycoside nephrotoxicity(defined as 50% GFR reduction) was presented in only one patient. She didn't have risky factor, such as hepatic dysfunction, hypotension, previous renal problem. However, duration was longer than other patients(13 days). GFR was fallen by 25-50% in 6 patients. This finding represent nephrotoxic effects of aminoglycoside indirectly. Above 6 patients had not any characteristics compared to other 33 patients. In addition to renal scan, sodium excretion was increased compared to pre-antibiotics, and glomerular filtration rate using 99mTc-DTPA renal scan was positively correlated with 24hr urine creatinine clearance, GFR by Cockcroft & Gault equation. I conclude that aminoglycoside has nephrotoxic effect when used for longer period. Thus, I suggest that DTPA renal scan including other many renal monitoring method has usefulness in revealing aminoglycoside nephrotoxicity.
Anti-Bacterial Agents ; Creatinine ; Ear ; Glomerular Filtration Rate ; Gram-Negative Bacteria ; Humans ; Hypotension ; Incidence ; Kidney ; Netilmicin ; Pentetic Acid ; Sodium