PURPOSE: The in vitro dissolution of intrahepatic stones was evaluated using the various solvent mixtures. MATERIALS AND METHODS: Sixty four intrahepatic stones from 16 patients were used. Four kinds of solvent mixtures(No. 1 = basic buffer + EDTA, No. 2=1 + Sulfobetain-12, No. 3=2 + N-acetylcysteine, No. 4=3 + urea) were used. Dissolution rates were determined by measuring the weight loss of stones after 6, 12, 24, 48 hours incubation periods, respectively. RESULTS: The highest dissolution rates in dissolving intrahepatic stones were achieved with No. 4 solvent mixture(1% W/V EDTA/80mM, Sulfobetain-12/1 M, urea, pH 9.5). CONCLUSION: lntrahepatic stones could be largely dissolved up to about 70% of their initial weight after 48 hours incubation period in vitro.
Acetylcysteine ; Edetic Acid ; Humans ; Hydrogen-Ion Concentration ; Urea ; Weight Loss

OBJECTIVE: Treatment with cyclosporine(CsA) for a long-term period may induce renal glomerulosclersosis and interstitial fibrosis. Reactive oxygen species(ROS) seems to be involved in the process of glomerulosclersosis and interstitial fibrosis. We investigated the effect of CsA on the generation of ROS and extracellular matrix accumulation in cultured human mesangial cells. We also studied the relationship between ROS formation and extracellular matrix and the effect of antioxidant on ROS formation and/or extracellular matrix degradation. METHODS: Mesangial cells were treated with varying dose of Cyclosporine(0, 2.5, 5, 7.5 and 10microgram/ mL) and also with cyclosporine(5microgram/mL) plus N- acetylcysteine(1mM). ROS was measured by flow cytometric analysis. mRNA expression of MMP-2, TIMP-2, MT1-MMP and collagen III was assessed by RT-PCR method. MMP-2 activity was measured by gelatin zymography. RESULTS: No significant difference was noted in cell viability with each CsA concentration. CsA inhibited the cell proliferation in a dose dependent manner and induced the expression of ROS. Antioxidant NAC reversed the effect of cyclosporine. CsA had no effect on the mRNA expression of collagen III, MMP-2, TIMP-2, MT1-MMP. However CsA decreased the MMP-2 activity in a dose dependent manner, which was also reversed by NAC. CONCLUSION: Cyclosporine-induced ROS may be associated with the extracellular matrix accumulation, that is glomerulosclersosis and interstitial fibrosis by inhibiting the cell proliferation and by decreasing the degradation of extracellular matrix. Antioxidant, at least in vitro, may prevent some of the adverse effects of CsA on renal function.
Cell Proliferation ; Cell Survival ; Collagen ; Cyclosporine* ; Extracellular Matrix* ; Fibrosis ; Gelatin ; Humans* ; Matrix Metalloproteinase 14 ; Mesangial Cells* ; Oxygen ; RNA, Messenger ; Tissue Inhibitor of Metalloproteinase-2

Transient cortical blindness is a known but rare complication occurring in 0.3~1% of patients undergoing cerebral or vertebral angiography. It is an extremely rare complication following cardiac catheterizaton and coronary arteriography. Cortical blindness is characterized by complete loss of visual perception and optokinetic nystagmus with preservation of ocular motility, pupillary responses, and normal fundoscopic examination. It has been suggested that its occurrence is due to an adverse reaction to the contrast agent, which causes an osmotic disruption of the blood-brain barrier. Patients outcome appears to be generally favorable with return of vision within 24-48 hr. We report a case of 48 year-old male patient with transient bilateral cortical blindness after coronary angiography.
Angiography ; Blindness, Cortical* ; Blood-Brain Barrier ; Coronary Angiography* ; Humans ; Male ; Middle Aged ; Nystagmus, Optokinetic ; Visual Perception

OBJECTIVE: The present study was undertaken to determine the value of developmental assessment, multimodality evoked potentials, brain magnetic resonance image (MRI) and electroencephalography (EEG) and to identify correlations between each evaluation. METHOD: Developmental assessments such as Bayley scales of infant development and Vineland social maturity scale, brain MRI, EEG and evoked potentials findings were evaluated in 45 children with spastic cerebral palsy to assess the developmental level and abnormalities of the anatomical structure of the brain and to elucidate the relationship between the test methods. RESULTS: 1) Mean mental developmental index (MDI) and psychomotor developmental index (PDI) were 69.6 and 68.6, respectively and mean Vineland social maturity quotient (SQ) was 76.1 and there was a significant correlation between the MDI, PDI, and SQ in cerebral palsied children. 2) Abnormal findings of brain MRI and EEG were found in 73.3% and 44.4% of the cases, respectively. 3) There was significant correlation between findings of brain MRI, auditory evoked potentials, visual evoked potentials, median somatosensory evoked potentials and social quotient. CONCLUSION: Developmental assessment, multimodality evoked potentials, EEG, and brain MRI would be a useful method to evaluate the maturity of brain and estimate the level of development.
Brain* ; Cerebral Palsy* ; Child Development ; Child* ; Electroencephalography* ; Evoked Potentials* ; Evoked Potentials, Auditory ; Evoked Potentials, Somatosensory ; Evoked Potentials, Visual ; Humans ; Magnetic Resonance Imaging* ; Weights and Measures

OBJECTIVE: To investigate the causes of amenorrhea in Korean women. METHODS: Medical records from 1,212 women with amenorrhea who visited the Department of Obstetrics and Gynecology, Asan Medical Center, between January 1989 and December 2011 were retrospectively reviewed. Amenorrhea was categorized as either primary or secondary. RESULTS: Primary amenorrhea was identified in 132 of the patients (10.9%) and secondary amenorrhea in 1,080 (89.1%). The most frequent causes of primary amenorrhea were gonadal dysgenesis (28.0%, 37/132); Mayer-Rokitansky-Kuster-Hauser syndrome (20.0%, 27/132); and constitutional delay and androgen insensitivity syndrome (8.3%, 11/132; 8.3%, 11/132, respectively). Secondary amenorrhea was due to polycystic ovary syndrome (48.4%, 523/1,080); premature ovarian insufficiency (14.0%, 151/1,080); and nutrition-related hypogonadotropic hypogonadism (8.3%, 90/1,080). CONCLUSION: In this retrospective study, gonadal dysgenesis was the most common cause of primary amenorrhea and polycystic ovary syndrome was the most common cause of secondary amenorrhea in Korean women.
Amenorrhea* ; Androgen-Insensitivity Syndrome ; Chungcheongnam-do ; Female ; Gonadal Dysgenesis ; Gynecology ; Humans ; Hypogonadism ; Korea* ; Male ; Medical Records ; Obstetrics ; Polycystic Ovary Syndrome ; Retrospective Studies

BACKGROUND: Many recent studies based on non-linearity have been performed to quantify the complex behavior of the brain in order to understand the pathophysiology of Parkinson's disease (PD). METHODS: We calculated the Fractal dimension (FD) and Lyapunov exponent (L1), the non-linear biologic signals, by digital EEG using 'CHASIM' program, non-linear times series signal simulator and then compared the UPDRS score with the degree of atrophy in the substantia nigra upon brain MRI and EEG data respectively. All subjects (N=20) showed definite hemiparkinsonism. RESULTS: Upon EEG analysis, a strong positive correlation was noted between FD of the left hemispheric electrodes (FP1,F3,T3,T5) and the UPDRS scores in left-sided symptomatic patients. Additionally, positive correlations were noted between the ipsilateral MRI index ratio in the right and left-sided symptomatic patients and respective UPDRS scores. CONCLUSIONS: These results suggested that thalamocortical drive is reduced in the contralateral hemisphere to parkinsonian symptoms and thalamocortical or corticothalamic glutaminergic projection in the ipsilateral hemisphere is increased in the early stage of Parkinson's disease. Additionally, hemiparkinsonim may primarilly cause anatomic and functional changes in the contralateral hemisphere and a compensatory effect in the ipsilateral hemisphere at the same time. We suggest that disease duration may be a compensating factor and of which require further investigation. We hope that our results will aid the understanding of the specific patterns of dysfunction and treatment effects by non-linear EEG measures and anatomic changes of the substantia nigra through continuous follow up of the patients. (J Korean Neurol Assoc 19(3):232~238, 2001)
Atrophy ; Brain ; Electrodes ; Electroencephalography* ; Fractals ; Hope ; Humans ; Magnetic Resonance Imaging ; Parkinson Disease* ; Substantia Nigra

PURPOSE: Duchenne/Becker muscular dystrophy(DMD/BMD) is an X-linked recessive disorder caused by mutations of dystrophin genes. The purpose of the present study is to determine the frequency and the patterns of dystrophin gene deletions and to investigate the correlation of genotypes and phenotypes. METHODS: There were included a total of 89 children(88 boys and 1 girl) diagnosed as DMD/BMD by immunohistochemistry and/or genetic analysis from 1999 to 2003 at Seoul National University Children's Hospital. We analyzed the genomic DNA by multiplex PCR using a 26 dystrophin exon primer set. Direct sequencing was performed on 23 exons(in which point mutations were detected in other previous reports) in 22 patients without deletions. Phenotype and genotype relationship analysis was performed on the basis of retrospective clinical reviews. RESULTS: The frequency of dysmorphin gene deletions was 54%(32/59), which is lower than that of European and American data. Exon deletions were detected in 59 cases and the deletion "hot spots" were exon 44-54 constituting 80% of all deletions. In 6 cases without detectable deletions, 6 point mutaions(3 nonsense mutations and 3 nucleotide variants) were detected. The patients whose deletions were in the central parts or the patients with multiple exon deletions tended to show earlier symptom onsets and more rapid progressions of weakness but there were no statistical significances. CONCLUSION: Since deletions in dystrophin genes were detected in about 50% of the patients, studies on dystrophin protein expressions using muscle biopsy samples must be done for correct diagnosis.
Biopsy ; Codon, Nonsense ; Diagnosis ; DNA ; Dystrophin* ; Exons ; Gene Deletion ; Genotype ; Humans ; Immunohistochemistry ; Molecular Biology* ; Multiplex Polymerase Chain Reaction ; Muscular Dystrophies* ; Phenotype ; Point Mutation ; Retrospective Studies ; Seoul

PURPOSE: Duchenne/Becker muscular dystrophy(DMD/BMD) is an X-linked recessive disorder caused by mutations of dystrophin genes. The purpose of the present study is to determine the frequency and the patterns of dystrophin gene deletions and to investigate the correlation of genotypes and phenotypes. METHODS: There were included a total of 89 children(88 boys and 1 girl) diagnosed as DMD/BMD by immunohistochemistry and/or genetic analysis from 1999 to 2003 at Seoul National University Children's Hospital. We analyzed the genomic DNA by multiplex PCR using a 26 dystrophin exon primer set. Direct sequencing was performed on 23 exons(in which point mutations were detected in other previous reports) in 22 patients without deletions. Phenotype and genotype relationship analysis was performed on the basis of retrospective clinical reviews. RESULTS: The frequency of dysmorphin gene deletions was 54%(32/59), which is lower than that of European and American data. Exon deletions were detected in 59 cases and the deletion "hot spots" were exon 44-54 constituting 80% of all deletions. In 6 cases without detectable deletions, 6 point mutaions(3 nonsense mutations and 3 nucleotide variants) were detected. The patients whose deletions were in the central parts or the patients with multiple exon deletions tended to show earlier symptom onsets and more rapid progressions of weakness but there were no statistical significances. CONCLUSION: Since deletions in dystrophin genes were detected in about 50% of the patients, studies on dystrophin protein expressions using muscle biopsy samples must be done for correct diagnosis.
Biopsy ; Codon, Nonsense ; Diagnosis ; DNA ; Dystrophin* ; Exons ; Gene Deletion ; Genotype ; Humans ; Immunohistochemistry ; Molecular Biology* ; Multiplex Polymerase Chain Reaction ; Muscular Dystrophies* ; Phenotype ; Point Mutation ; Retrospective Studies ; Seoul

BACKGROUND: Some researchers have questioned the necessity of adjusting glomerular filtration rate (GFR) by body surface area (BSA). We compared the relationship between estimated GFR (eGFR) and radionuclide GFR (rGFR) with or without BSA adjustment by comparing the results obtained using various formulae with those obtained using 2 new proposed formulae. METHODS: A retrospective study was performed using 204 Korean individuals whose GFR had been estimated by the (99m)Tc-diethylenetriaminepentaacetic acid method between March 2004 and July 2008. We used the modification of diet in renal disease (MDRD) II formula, Mayo clinic quadratic (MCQ) formula, Cockcroft-Gault (CG) formula, and lean body mass-adjusted CG formula. Two new formulae, skeletal muscle mass index (SMI)-adjusted CG formula and SMIx3.4/SCr, were proposed by us. We analyzed each parameter with Pearson's correlation coefficient and also obtained the bias values. RESULTS: BSA did not satisfy the fundamental prerequisites of an adjustment factor for rGFR. MDRD II and MCQ GFR estimates demonstrated higher Pearson's correlation coefficient with BSA-unadjusted rGFR than they did with BSA-adjusted rGFR. The other GFR formulae estimates showed better correlation with rGFR and more favorable bias (P<0.001) when both GFR estimates and rGFR values were BSA-unadjusted. SMI-adjusted CG and SMIx3.4/SCr GFR estimates demonstrated correlation with rGFR and bias values similar to those of the MDRD II and CG GFR estimates. CONCLUSIONS: We suggest that absolute, non-corrected GFR and GFR estimate be preferred in daily practice. The absolute, non-corrected GFR and GFR estimate are considered helpful for patients with eGFR< or =60 mL/min/1.73 m2. We also recommend the clinical use of the new formulae, SMI-adjusted CG and SMIx3.4/SCr (BSA-unadjusted).
Adult ; Aged ; Aged, 80 and over ; Algorithms ; Body Surface Area ; Creatinine/blood ; Female ; *Glomerular Filtration Rate ; Humans ; Male ; Middle Aged ; Organotechnetium Compounds/chemistry ; Pentetic Acid/analogs & derivatives/chemistry ; Republic of Korea/ethnology ; Retrospective Studies

Limitations due to lack of appropriate available donors for liver transplantation necessitates the use of ABO-mismatched donors. Transplantation of ABO-mismatched solid organs is sometimes associated with the development of immune hemolytic anemia, which is caused by production of antibodies by the donor B lymphocytes in a primary or secondary immune response against the recipient's red blood cell antigens. This condition is referred to as Passenger Lymphocyte Syndrome (PLS). PLS is more frequent in heart and lung transplants than in liver and kidney transplants with incidence of PLS in liver transplantation at 30~40%. When present, PLS typically manifests 1~3 weeks after transplantation, and subsides within 3 months after symptoms are first detected. In most patients, PLS is self-limiting and exhibits mild symptoms, but in some cases PLS can be life-threatening. We report a case of immune hemolytic anemia after an ABO-mismatched liver transplantation involving a blood group O donor and a blood group A recipient, and successful treatment of the resulting PLS symptoms by transfusion of gamma-irradiated group O Red Blood Cells (RBCs) accompanied by administration of 60 mg/day of methylprednisolone for 1 week.
Anemia, Hemolytic ; Antibodies ; B-Lymphocytes ; Erythrocytes ; Heart ; Humans ; Incidence ; Kidney ; Liver ; Liver Transplantation ; Lung ; Lymphocytes ; Methylprednisolone ; Tissue Donors ; Transplants