No abstract available.
Drug Therapy* ; Humans ; Neutropenia*

PURPOSE: This study was performed to analyse urine gamma-hydroxybutyric acid(GHB) in children with seizures, and to investigate the pattern of seizures and neurologic abnormalities in children related with gamma-hydroxybutyric aciduria. METHODS: We reviewed retrospectively medical records of children who admitted to our hospital with seizures between August 1. 2001 and February 28. 2003. We compared urine GHB levels with controls, and also analyzed the clinical features of patients who showed increased urine GHB. RESULTS: The mean urine GHB was 1.7+/-1.6 mmol/mol cr in febrile seizures, 1.8+/-2.5 mmol/mol cr in non-febrile seizures, and 1.8+/-2.0 mmol/mol cr in controls. Compared with control group, there was no significant difference in urine GHB levels(P>0.05). In 8 of 64 children with seizures, GHB levels increased above 2 standard deviation of normal controls. The types of seizure in children who showed increased urine GHB were generalized tonic clonic seizure in 3 patients, complex partial seizure in 2 patients, febrile seizure in 2 patients, and benign Rolandic epilepsy in 1 patient. 3 patients showed neurologic abnormalities, 4 patients showed electroencephalographic abnormalities, and 2 patients of 6 patients who performed brain imaging study showed brain imaging abnormalities. CONCLUSION: Children with gamma-hydroxybutyric aciduria should be suspected succinic semialdehyde dehydrogenase deficiency as a cause of underlying disease.
Child* ; Epilepsy* ; Epilepsy, Rolandic ; Humans ; Medical Records ; Neuroimaging ; Retrospective Studies ; Seizures* ; Seizures, Febrile ; Succinate-Semialdehyde Dehydrogenase

PURPOSE: In this retrospective study, we analyzed the outcomes of prostate cancer patients treated with the CyberKnife radiotherapy system (Accuray). MATERIALS AND METHODS: Between 2007 and 2010, 31 patients were treated for prostate cancer by use of the CyberKnife radiotherapy system. After excluding six patients who were lost to follow-up, data for the remaining 25 patients were analyzed. Patients were divided into the CyberKnife monotherapy group and a postexternal beam radiotherapy boost group. Clinicopathologic features and treatment outcomes were compared between the groups. The primary endpoint was biochemical recurrence-free survival period based on the Phoenix definition. Toxicities were evaluated by using the Radiation Therapy Oncology Group scoring criteria. RESULTS: Of 25 patients, 17 (68%) and 8 (32%) were classified in the monotherapy and boost groups, respectively. With a median follow-up of 29.3 months, most of the toxicities were grade 1 or 2 except for one patient in the boost group who experienced late grade 3 gastrointestinal toxicity. The overall biochemical recurrence rate was 20% (5/25) and the median time to biochemical recurrence was 51.9 months. None of the patients with low or intermediate risk had experienced biochemical recurrence during follow-up. Among D'Amico high-risk populations, 16.7% (1/6) in the monotherapy group and 50.0% (4/8) in the boost group experienced biochemical recurrence. CONCLUSIONS: Our data support that prostate cancer treatment by use of the CyberKnife radiotherapy system is feasible. The procedure can be a viable option for managing prostate cancer either in a monotherapy setting or as a boost after conventional radiotherapy regardless of the patient's risk stratification.
Follow-Up Studies ; Humans ; Lost to Follow-Up ; Prostate* ; Prostatic Neoplasms* ; Radiosurgery ; Radiotherapy* ; Recurrence ; Retrospective Studies

PURPOSE: Antiepileptic drugs may alter serum lipid status in epileptic patients. We conducted this study to assess the effect of valproate on serum levels of total cholesterol(TC), triglycerides(TG), low-density lipoprotein(LDL), high-density lipoprotein(HDL), and TC/HDL ratio, and to investigate the factors affecting serum lipid status in children with epilepsy who had been receiving valproate therapy. METHODS: Thirty epileptic children(16 males, 14 females, mean age 7.4+/-3.3 years) were evaluated for serum lipid status at the onset and the 6, 12 and 24 months of valproate therapy, and were analysed changes and potential factors of affecting changes such as sex, body mass index, valproate concentration, in serum lipid levels during valproate therapy. RESULTS: TC were significantly lowered during first 12 months of valproate theraphy (P<0.05). LDL were lowered during first 12 months. HDL and TC/HDL ratio were not changed and TG were increased during valproate theraphy but not reach to statistical significance. TC, TG, LDL, and HDL return to pretreatment levels after 24 months of valproate theraphy. TC, LDL, HDL, and TC/HDL ratio changes were not significantly different by sex and initial body mass index, but TG were significantly increased in group of BMI below 20(P<0.05). LDL levels were significantly decreased correlation to serum valproate concentration(r=-0.2915. P<0.05). CONCLUSION: Our results suggest that valproate therapy would not increase a risk for atherosclerotic disorders in adulthood, but weight gain with a metabolic consequence of obesity would increase risk for atherosclerotic disorders in adulthood.
Anticonvulsants ; Body Mass Index ; Child* ; Epilepsy* ; Female ; Humans ; Male ; Obesity ; Valproic Acid* ; Weight Gain

A series of 119 Mycobacterium avium complex isolates were subjected to clarithromycin susceptibility testing using microplates containing 2,3-diphenyl-5-thienyl-(2)-tetrazolium chloride (STC). Among 119 isolates, 114 (95.8%) were susceptible to clarithromycin and 5 were resistant according to the new and the standard method. STC counts the low cost and reduces the number of procedures needed for susceptibility testing.
Clarithromycin/*pharmacology ; Culture Media ; Humans ; Microbial Sensitivity Tests/*methods ; Mycobacterium avium Complex/*drug effects/isolation & purification ; Tetrazolium Salts/*chemistry

A series of 119 Mycobacterium avium complex isolates were subjected to clarithromycin susceptibility testing using microplates containing 2,3-diphenyl-5-thienyl-(2)-tetrazolium chloride (STC). Among 119 isolates, 114 (95.8%) were susceptible to clarithromycin and 5 were resistant according to the new and the standard method. STC counts the low cost and reduces the number of procedures needed for susceptibility testing.
Clarithromycin/*pharmacology ; Culture Media ; Humans ; Microbial Sensitivity Tests/*methods ; Mycobacterium avium Complex/*drug effects/isolation & purification ; Tetrazolium Salts/*chemistry

No abstract available.
Factor VIII* ; Polymorphism, Restriction Fragment Length*

Total parotidectomy is indicated when the tumor is originated from a deep lobe of the parotid gland. Because of the facial nerve, the usual sequence of total parotidectomy of a deep lobe tumor is to first perform superficial parotidectomy separately and then to remove the deep lobe. However, it is desirable to remove the parotid gland en-bloc while preserving the facial nerve. We designed a simple procedure that could remove a deep lobe tumor without separating the superficial portion of the parotid gland. This surgical technique is discussed with the present cases.
Facial Nerve* ; Parotid Gland*

Umbilical cord blood (UCB)-derived mesenchymal stem cells (MSCs) have been introduced as a possible therapy in acute lung injury and acute respiratory distress syndrome (ARDS). This case history is reported of a 59-yr-old man who was treated with MSCs in the course of ARDS and subsequent pulmonary fibrosis. He received a long period of mechanical ventilation and weaning proved difficult. On hospital day 114, he underwent the intratracheal administration of UCB-derived MSCs at a dose of 1 x 10(6)/kg. After cell infusion, an immediate improvement was shown in his mental status, his lung compliance (from 22.7 mL/cmH2O to 27.9 mL/cmH2O), PaO2/FiO2 ratio (from 191 mmHg to 334 mmHg) and his chest radiography over the course of three days. Even though he finally died of repeated pulmonary infection, our current findings suggest the possibility of using MSCs therapy in an ARDS patient. It is the first clinical case of UCB-derived MSCs therapy ever reported.
Bacterial Infections/diagnosis ; Drug Resistance, Multiple, Bacterial ; Fetal Blood/*cytology ; Humans ; Male ; *Mesenchymal Stem Cell Transplantation ; Mesenchymal Stromal Cells/*cytology ; Middle Aged ; Respiratory Distress Syndrome, Adult/complications/radiography/*surgery ; Seizures/etiology ; Shock, Septic/diagnosis ; Tomography, X-Ray Computed ; Treatment Outcome

PURPOSE: Since 1997, the quantification of organic acids in urine has become possible in Korea. This helped to diagnose a great variety of inborn errors of metabolism. However, we still don't know the normal value of organic acids in amniotic fluid, therefore it is impossible for doctors to make a correct diagnosis of inborn errors of metabolism in prenatal care. We tried to confirm the normal value of organic acid in amniotic fluid. METHODS: From Jan. 1998 to Dec. 2001, we carried out amniocentesis and were able to obtain 43 samples of amniotic fluid from between 16 and 20 weeks of gestation, and quantified 82 organic acids to come up with a normal value. Organic acid concentrations were quantified with gas chromatography, and the individual acids were identified with mass spectrometry. To isolate organic acids from amniotic fluid, we used a solvent extraction method with ethylacetate. Derivatization was done with MSTFA(N-methy-N-trimethylsilylfluoroacetamide). RESULTS: The results of this study showed that when organic acid concentrations in amniotic fluid were compared with those in urine, TCA cycle intermediates(lactate, pyruvate, malate, 2-ketoglutarate, citrate etc) and ketone body(3-hydroxybutyric acid, acetoacetate etc) were found at significantly higher levels. CONCLUSION: Because TCA cycle intermediate in amniotic fluid is found at high concentrations, we could expect that diagnosis of mitochondria disorder is difficult. Organic acids other than TCA cycle intermediates were undetectable in amniotic fluid. Therefore, prenatal diagnosis of organic acidemias is possible. In our study, the prenatal diagnosis of methylmalonic acidemia could be made by using the measurement of methylmalonic acid in the amniotic fluid taken at high risk pregnancy with a family history of methylmalonic acidemia.
Amniocentesis ; Amniotic Fluid* ; Chromatography, Gas ; Citric Acid ; Diagnosis ; Female ; Humans ; Korea ; Mass Spectrometry ; Metabolism, Inborn Errors ; Methylmalonic Acid ; Mitochondria ; Pregnancy ; Pregnancy, High-Risk ; Prenatal Care ; Prenatal Diagnosis* ; Pyruvic Acid ; Reference Values