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*

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

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

Recessive mutations of the SLC26A4 (PDS) gene on chromosome 7q31 can cause sensorineural deafness with goiter (Pendred syndrome, OMIM 274600) or NSRD with goiter (at the DFNB4 locus, OMIM 600791). H723R (2168A>G) is the most commonly reported SLC26A4 mutations in Korean and Japanese and known as founder mutation. We recently experienced one patient with enlarged vestibular aqueduct syndrome. The genetic study showed H723R homozygous in the proband and H723R heterozygous mutation in his family members. The identification of a disease-causing mutation can be used to establish a genotypic diagnosis and provide important information to both families and their physicians.
Asian Continental Ancestry Group ; Databases, Genetic ; Deafness ; Goiter ; Goiter, Nodular ; Hearing Loss, Sensorineural ; Humans ; Vestibular Aqueduct

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

Acute renal failure secondary to acute pyelonephritis is developed rarely. But acute pyelonephritis is considered in differential diagnosis of acute renal failure, particularly in elderly patient. Elderly patient showed subtle symptoms or signs of infections and can be missed easily. We experienced two cases of acute renal failure secondary to acute pyelonephritis. In first case, one patient complained fever, chilling and right flank pain for 10 days. Three repeated blood and urine cultures showed E. coli, respectively. At admission serum creatinine showed 2.4 mg/dL and thereafter increased to 4.5 mg/dL, and then decreased to 1.7 mg/dL with antibiotic therapy and hydration at 14 days of admission. In second case, patient complained right flank pain, costovertebral tenderness and urinary difficulty at admission. Two repeated blood culture showed no growth, two repeated urine culture showed > 105 ml/dL of E. coli. At admission serum creatinine level was 2.69 mg/dL and then decreased to 1.7 mg/dL with antibiotic therapy and hydration at 14 days of admission.Acute pyelonephritis should be considered in differential diagnosis of acute renal failure in the elder ages, although this developed rarely. Early recognition and appropriate antibiotic treatment helps recover acute renal failure secondary to acute pyelonephritis.
Acute Kidney Injury* ; Aged ; Creatinine ; Diagnosis, Differential ; Fever ; Flank Pain ; Humans ; Pyelonephritis*

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*