• KSBB Journal
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• v.23 no.5
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• pp.381-386
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• 2008

Lipophilic ammonia is toxic gas and can easily diffuse across cell membranes. Excess ammonia is implicated in the pathogenesis of several metabolic disorders including hepatic encephalopathy and may result in the death. The purpose of this study was to clarify the inhibition effect of metronidazole on liver cell damage due to ammonia in human Hep G2 cell and rat hepatocytes. The effects of metronidazole were studied in ammonium chloride treated human Hep G2 cell (75 mM) and rat hepatocyte (100 mM) following $0.1{\mu}M$ metronidazole treatment. In MTZ+AC group, cell viabilities increased prominently and LDH activities decreased over 25% than AC group. Furthermore, ammonia level according to ammonium chloride treatment reduced over 30% and lipid peroxidation as an index of cell membrane damage decreased more than twice. By comparison with control, catalase activity showed more than 30% reduction in AC group while less than 10% reduction in MTZ+AC group, respectively. In addition, MTZ+AC group showed the similar cell structure as control in cell morphology study by using light microscope, and represented fluorescent intensity decrement compared with AC group in fluorescent microscopic study with avidin-TRITC fluorescent dye. And cleaved PARP expression due to ammonia reduced twofold or more in MTZ+AC group. As the results suggest, metronidazole may protect the liver cell by inhibiting cell damages due to ammonia and be used for an effective antagonist of ammonia in hyperammonemia.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.1
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• pp.18-22
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• 2018

Carbamoyl phosphate synthetase I (CPS1) deficiency is a rare autosomal recessive urea cycle disorder that causes hyperammonemic crisis. CPS1 is the first enzyme encoded by the CPS1 gene, which catalyzes the first step of the urea cycle. In CPS1 deficiency, ammonia, the toxic metabolite produced by the interruption of the urea cycle, is accumulated in the blood and brain, leading to hyperammonemic encephalopathy and irreversible brain damage. Here, we report a fatal case of neonatal-onset CPS1 deficiency in a 4-day-old girl presenting with recurrent seizures, who was revealed to be homozygous for c.1529delG ($p.Gly510Alafs^*5$).

• Journal of The Korean Society of Clinical Toxicology
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• v.17 no.1
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• pp.42-45
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• 2019

Dioscorea tokoro has long been used in Korean traditional medicine as a pain killer and anti-inflammatory agent. A 53-year-old male who consumed water that had been boiled with raw tubers of D. tokoro as tea presented with numbness and spasm of both hands and feet. Laboratory results showed hypocalcemia, hypoparathyroidism, and vitamin D insufficiency. During his hospital stay, colitis, acute kidney injury, and toxic encephalopathy developed. The patient received calcium gluconate intravenous infusion and oral calcium carbonate with alfacalcidol. His symptoms improved gradually, but hypocalcemia persisted despite the calcium supplementation. We suggest that ingestion of inappropriately prepared D. tokoro can cause symptomatic hypocalcemia in patients with unbalanced calcium homeostasis.

• Clinical and Experimental Pediatrics
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• v.50 no.11
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• pp.1097-1103
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• 2007

Purpose : Acute symptomatic seizure is defined as a temporary seizure together with acute systemic, metabolic, or toxic insult in association with an acute central nervous system insult. And unprovoked seizure is defined as seizure without provocating factors. We studied the risk factors of unprovoked seizures after acute symptomatic seizure in children. Methods : We retrospectively reviewed the records of one hundred and ten children with acute symptomatic seizures who were admitted to the pediatric department of Chungbuk National University Hospital between January, 1998 and December, 2003. We analyzed overall risk factors of unprovoked seizures after acute symptomatic seizures involving etiology, incidence, type of seizure, duration and neuroimaging. Results : We analyzed records of 110 children with acute symptomatic seizures aged from 1 month to 17 years. 24 children had unprovoked seizures (21.8%) after acute symptomatic seizures. Causes in order of frequency were encephalopathy, central nervous system infection, brain tumor, cerebrovascular disease. The risk of unprovoked seizure was significantly greater for those with status epilepticus (68.4%) than without status epilepticus, with partial seizure (64.7%) than generalized seizure. And the risk of unprovoked seizure was strongly associated with abnormal finding of electroencephalogram (79.1%) and neuroimaging (41.6%). Conclusion : In conclusion, the leading cause of subsequent unprovoked seizure in children with acute symptomatic seizure was encephalopathy and age specific incidence was high in the group aged 24-72 months. The risk for subsequent unprovoked seizure was greater for those with partial seizure, status epilepticus, abnormal finding of neuroimaging and electroencephalography.

• Investigative Magnetic Resonance Imaging
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• v.6 no.1
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• pp.64-72
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• 2002

Purpose : To introduce and demonstrate the advantages of the new hybrid two-dimensional (2D) proton spectroscopic imaging (SI) over the single voxel spectroscopy (SVS) and conventional 2D SI in the clinical application of spectroscopy for pediatric cerebral disease. Materials and Methods : Eighty-one hybrid 2D proton spectroscopic imaging was performed in 79 children (36 normal infants and children, 10 with hypoxic-ischemic injury, 20 with toxic-metabolic encephalopathy, seven with brain tumor, three with meningoencephalitis, one with neurofibromatosis, one with Sturge-Weber syndrome and one with lissencephaly) ranging in age from the third day of life to 15 years. In adult volunteers (n=5), all three techniques including hybrid 2D proton SI, SVS using PRESS sequence, and conventional 2D proton SI were performed. Both hybrid 2D proton SI and SVS using PRESS sequence were performed in clinical cases (n=). All measurements were performed with a 1.5-T scanner using standard head quadrature coil. The 16$\times$16 phase encoding steps were set on variable field of view (FOV) depending on the size of the brain. The hybrid volume of interest inside FOV was set as $75{\times}75{\times}15{\;}\textrm{mm}^3$ or smaller to get rid of unwanted fat signal. Point-resolved spectroscopy (TR/TE=1,500 msec/135 or 270msec) was employed with standard chemical shift selective saturation (CHESSI pulses for water suppression. The acquisition time and spectral quality of hybrid 2D proton SI were compared with those of SVS and conventional 2D proton SI. Results : The hybrid 2D proton SI was successfully conducted upon all patients.

• Pediatric Infection and Vaccine
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• v.21 no.1
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• pp.9-21
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• 2014

Purpose: To determine the clinical significance of voriconazole therapeutic drug monitoring (TDM) in the pediatric population. Methods: Twenty-eight patients with invasive fungal infections administered with voriconazole from July 2010 to June 2012 were investigated retrospectively. Fourteen received TDM, and 143 trough concentrations were analyzed. All 28 patients were assessed for adverse events and treatment response six weeks into treatment, and at the end. Results: Out of 143 samples, 53.1% were within therapeutic range (1.0-5.5 mg/L). Patients administered with the same loading (6 mg/kg/dose) and maintenance (4 mg/kg/dose) dosages prior to initial TDM showed highly variable drug levels. Adverse events occurred in 9 of 14 patients (64.3%) in both the TDM and non-TDM group. In the TDM group, voriconazole-related encephalopathy (n=2, 14.3%) and aspartate aminotransferase (AST) or alanine aminotransferase (ALT) elevation (n=8, 57.1 %) occurred with serum levels in the toxic range (>5.5 mg/L), whereas blurred-vision (n=2, 14.3%) occurred within the therapeutic range (1.18 mg/L and 3.9 mg/L). The frequency of voriconazole discontinuation due to adverse events was lower in the TDM group (0.0% vs. 18.2%, P =0.481). Overall, 57.2% of the patients in the TDM group versus 14.3% in the non-TDM group showed clinical response after 6 weeks (P =0.055), whereas 21.4% in the TDM group versus 14.3% in the non-TDM group showed response at final outcome (P =0.664). In the TDM group, >67.0% of the serum levels were within therapeutic range for the first 6 weeks; however 45.5% were within therapeutic range for the entire duration. Conclusion: Routine TDM is recommended for optimizing the therapeutic effects of voriconazole.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.1
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• pp.16-23
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• 2004

Purpose: In this study, we tried to evaluate the clinical characteristics or circumstances that lead to unintentionally the delay in the diagnosis of intussusception or to the wrong direction that prevent the proper management early. Methods: All the patients of intussusception with delayed diagnosis in the department of pediatrics or emergency room at Gyeongsang National University Hospital from 1990 to 2003 were enrolled and reviewed retrospectively. Results: There were 8 boys and 6 girls and their median age was 8 months (range 2 months to 10 years). Their initial symptoms and signs were vomiting, seizure, diarrhea, lethargy, irritability, bloody stool, palpable abdominal mass, foul odor of urine and tachycardia. Clinical diagnosis or impressions at admission consisted of acute gastroenteritis, shigellosis and toxic encephalopathy, convulsive disorders, urinary tract infections, sepsis, abdominal mass and intestinal obstruction. Eight patients were luckily diagnosed due to the delayed manifestations of cyclic irritability or currant jelly stool. Six patients were not paid attentions for the possibilities of intussusception and diagnosed serendipitiously by the abdominal sonography or CT during the evaluation of the abdominal mass or distension. Only five of 14 cases (35.7%) were successfully managed by barium or air reductions. The other 9 cases needed surgical operations. Conclusion: Delayed diagnosis of intussusception arise when doctors initially diagnose the patients incorrectly due to the unusual presentations or when they overlook the newly arising symptoms or signs suggestive intussusception after the admission because they are ardently attached to the first impressions or initial clinical diagnosis.