Bartter syndrome is a renal tubular defect in electrolyte transport characterized by hypokalemia, metabolic alkalosis, hyperreninemia, hyperaldosteronism, normal blood pressure, and other clinical symptoms. As a clinical and genetical heterogeneous disorder, this syndrome can be classified into two clinical variants, antenatal Bartter syndrome and classic Bartter syndrome according to the onset age. Nephrocalcinosis is common in antenatal Bartter syndrome, but is rare in classic Bartter syndrome. It can also be classified into five genetic subtypes by the underlying mutant gene, all of which are expressed in the tubular epithelial cells of the thick ascending limb of the loop of Henle. Patients with Bartter syndrome type 1, 2 and 4 present at a younger age than classic Bartter syndrome type 3. We have experienced a case of Bartter syndrome with nephrocalcinosis in a 42-year-old woman diagnosed by biochemical and radiologic studies. We had successful response with potassium chloride and spironolactone.
Adult* ; Age of Onset ; Alkalosis ; Bartter Syndrome* ; Blood Pressure ; Epithelial Cells ; Extremities ; Female ; Humans ; Hyperaldosteronism ; Hypokalemia ; Loop of Henle ; Nephrocalcinosis* ; Potassium Chloride ; Spironolactone

PURPOSE: Hollow fiber assays offer an early in vivo method of anticancer drug screening. The assays have been optimized for human cancers originating from the lung, breast, colon, ovary, and brain, but not from the stomach and liver. The current study focused on optimization of hollow fiber assays for gastric and hepatocellular carcinoma cell lines. MATERIALS AND METHODS: Gastric (SNU-16, SNU-484, SNU-668) and hepatocellular (HepG2, SK-Hep-1, Hep3B) carcinoma cell lines in hollow fibers were transplanted subcutaneously and intraperitoneally into mice, which were subsequently treated with a standard anticancer agent, paclitaxel. The hollow fiber activity of paclitaxel in each cell line was compared with the xenograft activity. RESULTS: Using optimized inoculation densities and schedules, treatment with paclitaxel was effective in gastric carcinoma cell lines, SNU-16 and SNU-484, but not in SNU-668. In the hollow fiber assays, paclitaxel was effective in hepatocellular carcinoma cell lines, HepG2 and SK-Hep-1, but not in Hep3B. Consistent with the results of the hollow fiber assay, SNU-16 and SNU-484, but not SNU-668, showed tumor regression, and HepG2 and SK-Hep-1, but not Hep3B, showed effective tumor responses following treatment with paclitaxel in xenograft models. When EW7197, a novel compound, and flavopiridol were tested in SNU-16 cells under optimized conditions, the hollow fiber activity showed good correlation with the xenograft activity of each compound. CONCLUSION: Our protocols may be useful for screening candidate small molecules that may exhibit activity against stomach and liver cancers, both of which are common in Korea.
Animals ; Appointments and Schedules ; Brain ; Breast ; Carcinoma, Hepatocellular ; Cell Line ; Colon ; Drug Evaluation, Preclinical ; Female ; Heterografts ; Humans ; Korea ; Liver ; Liver Neoplasms* ; Lung ; Mass Screening ; Mice ; Ovary ; Paclitaxel ; Stomach Neoplasms ; Stomach*

Stress-induced cardiomyopathy, which is also known as takotsubo cardiomyopathy, is a cardiac syndrome of a transient, reversible left ventricular dysfunction that is caused by emotional and/or physical stress and surgery. Its clinical manifestations are similar to those of myocardial ischemia without a coronary artery lesion. Stress-induced cardiomyopathy is more common in middle-aged women, and the prognosis is favorable. We report the case of a 50-year-old female patient who underwent a total gastrectomy and developed stress-induced cardiomyopathy after surgery.
Anesthesia ; Anesthesia, General ; Cardiomyopathies ; Coronary Vessels ; Female ; Gastrectomy ; Humans ; Middle Aged ; Myocardial Ischemia ; Prognosis ; Takotsubo Cardiomyopathy ; Ventricular Dysfunction, Left

Acute renal failure in Immunoglobulin A nephropathy (IgAN), a rare event, is associated with acute tubular necrosis mainly induced by intratubular erythrocytic cast and crescentic glomerulonephropathy (rapidly progressive glomerulonephritis) and the severity paralleled to the degree of glomerular damage. The changes are regarded as those of secondary atrophic response to the glomerular lesions. In that case, renal progression correlates more closely with the severity of tubulointerstitial lesions than with the degree of glomerular lesions in IgAN. Rarely, acute tubulointerstitial nephritis (TIN) could develop independently in primary glomerulonephritis. In this case, the severity of tubulointerstitial lesion was out of proportion with damage of glomerular lesion. To the best of our knowledge, we report the first case of a patient with independently developed severe acute TIN complicating IgAN in Korea. A 38-year-old man was admitted with recurrent hematuria. Proteinuria (<1 g) and severe renal failure were noted and hemodialysis was started. In renal biopsy, IgAN associated with acute TIN was diagnosed. He showed good response to steroid therapy and maintained normal renal function after discontinuation of medication.
Acute Kidney Injury ; Adult ; Biopsy ; Glomerulonephritis ; Glomerulonephritis, IGA ; Hematuria ; Humans ; Immunoglobulin A ; Korea ; Necrosis ; Nephritis, Interstitial ; Proteinuria ; Renal Dialysis ; Renal Insufficiency ; Tin

Chimerism in humans is a rare phenomenon often initially identified in the resolution of an ABO blood type discrepancy. We report a dispermic chimera who presented with mixed field in his B antigen typing that might have been mistaken for the B3 subtype. The propositus is a healthy Korean male blood donor. Neither his clinical history nor initial molecular investigation of his ABO gene explained his mixed field agglutination with murine anti-B. Chimerism was suspected, and 9 short tandem repeat (STR) loci were analyzed on DNA extracted from blood, buccal swabs, and hair from this donor and on DNA isolated from peripheral blood lymphocytes from his parents. The propositus' red blood cells demonstrated mixed field agglutination with anti-B. Exon 6 and 7 and flanking intronic regions of his ABO gene were sequenced and revealed an O01/O02 genotype. B allele haplotype-specific PCR, along with exon 6 and 7 cloning and sequencing demonstrated a third ABO allele, B101. Four STR loci demonstrated a pattern consistent with a double paternal chromosome contribution in the propositus, thus confirming chimerism. His karyotype revealed a mosaic pattern: 32/50 metaphases were 46,XY and 18/50 metaphases demonstrated 47,XYY.
ABO Blood-Group System ; Adult ; Alleles ; Blood Grouping and Crossmatching ; Chimera ; Chimerism ; Chromosome Disorders/*diagnosis/*genetics ; Genotype ; Humans ; Karyotyping ; Korea ; Male ; Phenotype ; Sequence Analysis, DNA ; *XYY Karyotype

Silicone fluid is a biomaterial widely used in modern cosmetic procedures because there are few side effects, considerable chemical stability and predictable physical properties. However, many local and systemic adverse reactions have reported. In particular some serious pulmonary complications have been reported such as pulmonary thromboembolism, acute respiratory distress syndrome with some cases leading to mortality. Most of the serious complicated cases were induced by an illegal silicone fluid injection. We experienced two cases of acute respiratory distress syndrome with pulmonary hemorrhage induced by an illegal silicone fluid injection. The patients were 41 & 51 year old women, who complained of dyspnea. The chest X-ray and HRCT scan findings showed a bilateral ground glass attenuation on the bilateral dependent portion of the upper and middle lung zone. The patients clinical symptoms and the radiologic and other laboratory findings were compatible with acute respiratory distress syndrome induced by the silicon fluid injection. Here we report two cases of acute respiratory distress syndrome with pulmonary hemorrhage induced by an illegal silicone injection with a review of the relevant literature.
Dyspnea ; Female ; Glass ; Hemorrhage* ; Humans ; Lung ; Mortality ; Perineum* ; Pulmonary Embolism ; Respiratory Distress Syndrome, Adult* ; Silicon* ; Silicones* ; Thorax

Theophylline has been widely used in the treatment of asthma and chronic obstructive lung disease. To date, there have been very few reports on hepatotoxicity due to theophylline. We diagnosed, through biochemical testing and a liver biopsy, a case of acute cholestatic hepatitis developed after oral consumption of theophylline. A 43 year-old man was admitted to the department of internal medicine due to jaundice and pruritus which developed after ten days administration of oral theophylline (Etheophyl ). Liver function tests showed elevated serum bilirubin at 13.2 mg/dL with AST and ALT of 71 U/L and 194 U/L. Alkaline phosphatase and gamma-GTP were also elevated at 175 U/L and 301 U/L. There was no evidence of viral or autoimmune hepatitis in laboratory tests. The patient's symptoms and liver function tests were improved after conservative treatment. After 9 months oral theophylline was readministered for the control of relapsed asthma. Then, jaundice and pruritus again developed again. A liver biopsy showed a few lymphocytes and eosinophilic inflammatory cell infiltration in portal tract and cholestasis in the lobule. Drug-induced hepatitis was diagnosed with a typical clinical course; the exclusion of all possible causes of acute hepatic dysfunction; and a positive response to accidental readministration of drug. We report this case with a review of the literature.
Adult ; Alkaline Phosphatase ; Asthma ; Bilirubin ; Biopsy ; Cholestasis ; Drug-Induced Liver Injury ; Eosinophils ; Hepatitis* ; Hepatitis, Autoimmune ; Humans ; Internal Medicine ; Jaundice ; Liver ; Liver Function Tests ; Lymphocytes ; Pruritus ; Pulmonary Disease, Chronic Obstructive ; Theophylline

BACKGROUND: As one of the etiologies of acute respiratory distress syndrome (ARDS), sepsis is one of the morbid causes of this cryptogenic malady. Even though many documents on the role of endotoxin (ETX) in the pathogenesis of ARDS have been issued, still the underlying mechanism associated with oxidative stress and activation of PLA2 has been a controversy controversial . In the present study, the role of phospholipase A2 (PLA2) in the neutrophilic respiratory burst(,) which is presumed to cause acute lung injury during sepsis(,) was probed. METHOD: In glutathione (-)depleted Sprague-Dawley rats, lung leak, infiltration of neutrophils, PLA2 activity and lipid peroxidation in the lung were measured after intratracheal instillation of endotoxin intratracheally (delete). In addition, gamma glutamyl transferase (GGT) activity and the amount of pulmonary surfactant were measured. Morphologically, changes of the changes in ultrastructure and cytochemical demonstration of oxidants were presented to confirm the neutrophilic oxidative stress and to elucidate the effects of the activation of PLA2 activation on the (delete) oxidative stress. RESULTS: Instillation of ETX to glutathione (-) depleted rats intensified lung leak and lipid peroxidation when compared with non-glutathione depleted rats treated with the endotoxin. Moreover, oxidative stress was confirmed by the assay of GGT and malondialdehyde. Functionally, the depletion of glutathione altered the secretion of pulmonary surfactant from alveolar type II cells. Ultrastructurally and cytochemically, oxidative stress was also confirmed after treatment of with ETX and diethylmaleate (DEM). CONCLUSION: The endotoxin-induced acute lung injury was mediated by oxidative stress(,) which in turn was provoked by the neutrophilic respiratory burst. The activation of PLA2 in the lung seems to play the a pivotal role in the oxidative stress of the lung.
Acute Lung Injury ; Animals ; Glutathione* ; Lipid Peroxidation ; Lung* ; Malondialdehyde ; Neutrophils ; Oxidants ; Oxidative Stress* ; Phospholipases A2 ; Pulmonary Surfactants ; Rats* ; Rats, Sprague-Dawley ; Respiratory Burst ; Respiratory Distress Syndrome, Adult ; Sepsis ; Transferases

Widespread brain-derived neurotrophic factor (BDNF) mRNA and protein expression has been detected in the brain. Despite substantial overlap between BDNF mRNA and protein expression, there is general anatomical regions, where there is discordance of these expression. We performed, therefore, immunohistochemistry after colchicine treatment into the ventricle to evaluate the possible presence of BDNF-immunoreactive (IR) in the regions where BDNF mRNA was expressed, but not BDNF-IR. The results obtained were as follows; There was substantial increase in the number of BDNF-IR neurons in the anterior olfactory nucleus, the piriform cortex, the cerebral cortex, the claustrum, the stratum pyramidale of the CA2 and the CA3, the granule cell layer of the dentate gyrus, the basolateral amygdaloid nucleus, the lateral geniculate nucleus, the anteromedial thalamic nucleus, the anterodorsal thalamic nucleus, the paraventricular thalamic nucleus, the paraventricular hypothalamic nucleus and the ventromedial hypothalamus nucleus, compared to the same brain area of non-colchicine treated rat. We detected many new BDNF-IR neurons in the stratum pyramidale of the CA1, A1, A2, A4-A10 cell groups, C1-C3 cell groups, the raphe magnus nucleus, the lateral paragigantocellular nucleus and the spinal vestibular nucleus. The results show that the localization of BDNF-IR neurons after colchicine treatment is consistant with that of BDNF mRNA containing neurons in the brain.
Animals ; Anterior Thalamic Nuclei ; Basal Ganglia ; Brain* ; Brain-Derived Neurotrophic Factor ; Cerebral Cortex ; Colchicine* ; Dentate Gyrus ; Hypothalamus ; Immunohistochemistry ; Midline Thalamic Nuclei ; Neurons* ; Paraventricular Hypothalamic Nucleus ; Rats* ; RNA, Messenger

In order to understand the pathogenetic mechanism of adult respiratory distress syndrome (ARDS), the role of phospholipase A2 (PLA2) in association with oxidative stress was investigated in rats. Interleukin-1alpha (IL-1, 50 mug/rat) was used to induce acute lung injury by neutrophilic respiratory burst. Five hours after IL-1 insufflation into trachea, microvascular integrity was disrupted, and protein leakage into the alveolar lumen was followed. An infiltration of neutrophils was clearly observed after IL-1 treatment. It was the origin of the generation of oxygen radicals causing oxidative stress in the lung. IL-1 increased tumor necrosis factor (TNF) and cytokine-induced neutrophil chemoattractant (CINC) in the bronchoalveolar lavage fluid, but mepacrine, a PLA2 inhibitor, did not change the levels of these cytokines. Although IL-1 increased PLA2 activity time-dependently, mepacrine inhibited the activity almost completely. Activation of PLA2 elevated leukotriene C4 and B4 (LTC4 and LTB4), and 6-keto-prostaglandin F2alpha (6-keto-PGF2alpha) was consumed completely by respiratory burst induced by IL-1. Mepacrine did not alter these changes in the contents of lipid mediators. To estimate the functional changes of alveolar barrier during the oxidative stress, quantitative changes of pulmonary surfactant, activity of gamma glutamyltransferase (GGT), and ultrastructural changes were examined. IL-1 increased the level of phospholipid in the bronchoalveolar lavage (BAL) fluid, which seemed to be caused by abnormal, pathological release of lamellar bodies into the alveolar lumen. Mepacrine recovered the amount of surfactant up to control level. IL-1 decreased GGT activity, while mepacrine restored it. In ultrastructural study, when treated with IL-1, marked necroses of endothelial cells and type II pneumocytes were observed, while mepacrine inhibited these pathological changes. In histochemical electron microscopy, increased generation of oxidants was identified around neutrophils and in the cytoplasm of type II pneumocytes. Mepacrine reduced the generation of oxidants in the tissue produced by neutrophilic respiratory burst. In immunoelectron microscopic study, PLA2 was identified in the cytoplasm of the type II pneumocytes after IL-1 treatment, but mepacrine diminished PLA2 particles in the cytoplasm of the type II pneumocyte. Based on these experimental results, it is suggested that PLA2 plays a pivotal role in inducing acute lung injury mediated by IL-1 through the oxidative stress by neutrophils. By causing endothelial damage, functional changes of pulmonary surfactant and alveolar type I pneumocyte, oxidative stress disrupts microvascular integrity and alveolar barrier.
Acute Lung Injury ; Animals ; Bronchoalveolar Lavage ; Bronchoalveolar Lavage Fluid ; Cytokines ; Cytoplasm ; Endothelial Cells ; gamma-Glutamyltransferase ; Insufflation ; Interleukin-1 ; Interleukin-1alpha* ; Leukotriene C4 ; Lung Injury* ; Lung* ; Microscopy, Electron ; Necrosis ; Neutrophils ; Oxidants ; Oxidative Stress ; Phospholipases A2 ; Pneumocytes ; Pulmonary Surfactants ; Quinacrine ; Rats ; Reactive Oxygen Species ; Respiratory Burst ; Respiratory Distress Syndrome, Adult ; Trachea ; Tumor Necrosis Factor-alpha