Mucopolysaccharidoses (MPSs) are a group of inherited heterogenous metabolic disorders, caused by deficiency of an enzyme involved in the degradation of mucopolysaccharides, and thus deposition of mucopolysaccharides in all connective tissue. MPS type III is heparan sulphate storage disorder, especially marked central nervous system involvement. As with all the MPS disorders, this patient with MPS III presents coarse outlook features, such as macrocephaly, macroglossia, prominent mandible, short neck, and unstable atlantoaxial joints, as well as tracheal abnormality. These clinical findings give significant challenges to the anesthesiologist for adequate airway management and tracheal intubation. Recently, a number of new devices have been developed to facilitate intubation. We report an experience of facilitated tracheal intubation using video styletscope (OptiScope PM201(R), Pacific Medical, Seoul, Korea) in a 16 year old male patient with MPS III, who was undergoing inguinal hernia repair.
Airway Management ; Atlanto-Axial Joint ; Central Nervous System ; Connective Tissue ; Glycosaminoglycans ; Hernia, Inguinal ; Humans ; Intubation ; Intubation, Intratracheal ; Macrocephaly ; Macroglossia ; Male ; Mandible ; Mucopolysaccharidoses ; Neck

PURPOSE: Hyperhomocysteinemia is known as an independent risk factor for cardiovascular diseases such as atherosclesosis and myocardiac infarct. A common mutation in 5, 10-methylenetetrahydrofolate reductase(MTHFR) gene results in a valine for alanine substitution, which makes enzyme thermolabile and reduces enzymal activity. We examined the relation of MTHFR genetic mutation and Kawasaki disease. METHODS: We extracted DNA from the peripheral blood of Kawasaki disease patients who were treated in Ewha University Mokdong Hospital between January 2000 and June 2003, and normal children. We genotyped for the polymorphism by using PCR of known alleic varients and digested them with Hinfl restriction enzyme. Products were visualized after electrophoresis in 2.5% agarose gel. RESULTS:The respective prevalence of the homozygous and heterozygous genotypes(CC genotype, CT genotype, TT genotype) was 33.3%, 38.9%, and 27.8% in the control group and 31.3%, 47.9%, and 20.8% in the Kawasaki disease group(P>0.05). CONCLUSION:The mutation of MTHFR gene shows no relation to development of coronary artery complications in Kawasaki disease.
Alanine ; Cardiovascular Diseases ; Child ; Coronary Vessels ; DNA ; Electrophoresis ; Gene Expression* ; Genotype ; Humans ; Hyperhomocysteinemia ; Methylenetetrahydrofolate Reductase (NADPH2) ; Mucocutaneous Lymph Node Syndrome* ; Polymerase Chain Reaction ; Prevalence ; Risk Factors ; Sepharose ; Valine

PURPOSE: We evaluated the diagnostic implications and therapeutic efficacy of antithrombin III(AT III) in the disseminated intravascular coagulation(DIC) of premature infants. METHODS: Ninety-two premature infants diagnosed with DIC and treated with AT III from March, 2000 to May, 2003 were retrospectively reviewed. Clinical manifestations, complete blood counts, coagulation tests, and AT III levels were compared between the two groups:definite DIC if clinical signs of DIC and AT III <70% with more than two abnormal laboratory parameters were present, and suspected DIC if not more than two abnormal laboratory parameters were present. RESULTS: AT III was given for an average of 3.2 days and no side effects related to the treatment were reported. The AT III levels increased significantly more than four fold with treatment in both groups. The clinical signs and laboratory values improved significantly after treatment. CONCLUSION: AT III level is a sensitive parameter in the diagnosis of DIC in premature infants, and it is useful as a treatment modality since it improves the clinical symptoms and the laboratory parameters without significant side effects.
Antithrombin III* ; Blood Cell Count ; Dacarbazine ; Diagnosis* ; Disseminated Intravascular Coagulation* ; Humans ; Infant, Newborn ; Infant, Premature* ; Retrospective Studies

BACKGROUND AND PURPOSE: White-matter (WM) lesions are known to potentiate cognitive impairment in poststroke patients. The present study was designed to assess whether Ginkgo biloba extract (GB) and cilostazol, which were evaluated alone and in a combination formula (Renexin), can attenuate the WM lesions and cognitive decline caused by chronic hypoperfusion in the rat. METHODS: Animals were divided into five treatment groups: cilostazol (25 mg/kg/day), GB (20 mg/kg/day), Renexin (25 mg/kg/day cilostazol + 20 mg/kg/day GB), vehicle, and sham. The animals received the treatments orally 1 day after bilateral common carotid artery occlusion [two-vessel occlusion (2VO); except for the sham group, which underwent the surgery but the arteries were not occluded], and then the same dose every day for 21 days thereafter. Prior to sacrificing the rats, repetitive eight-arm radial maze testing was performed to examine their cognitive abilities. After drug administration and cognitive testing, brain tissues were isolated for Kluver-Barrera and terminal deoxynucleotidyl transferase-mediated biotin-dUTP nick end-labeling (TUNEL) staining, immunohistochemical assessment of glial fibrillary acidic protein (GFAP) and CD11b (OX-42), and to assay free-radical scavenging activity. RESULTS: We found that the significant WM lesions induced by 2VO was ameliorated significantly by treatment with cilostazol, GB, and Renexin, in association with increased TUNEL-positive cells. In addition, chronic cerebral hypoperfusion caused a large increase in the degree of GFAP and OX-42 immunoreactivity and free-radical activity in the optic tract. These abnormalities were significantly reversed by the three drugs, but most prominently by Renexin, suggesting a markedly enhanced or supra-additive effect of cilostazol and GB when administered together. CONCLUSIONS: Significant attenuation of cytoarchitectural damage and apoptotic cell death was found with GB and cilostazol, but a markedly enhanced effect was seen for treatment with their combination in the WM of rat brains after bilateral occlusion of the common carotid arteries. We suggest that combination therapy with GB and cilostazol provides enhanced neuroprotective effects and induces subsequent cognitive improvement in patients with chronic ischemic conditions.
Animals ; Arteries ; Brain ; Carotid Artery, Common ; Cell Death ; Ginkgo biloba ; Glial Fibrillary Acidic Protein ; Humans ; Neuroprotective Agents ; Rats ; Salicylamides ; Tetrazoles ; Visual Pathways

PURPOSE: The aim of this study was to assess the diagnostic value of the (99m)Tc-DMSA pinhole imaging by comparing the detection rate of cortical defects identified from planar and pinhole imaging. METHODS: (99m)Tc-DMSA planar and pinhole imaging, voiding cystoureth-rogram(VCUG), renal ultrasonography were performed in 67 patients diagnosed with urinary tract infection at the Department of Pediatrics, Sung-Ae General Hospital, from September, 1999 to Feburary, 2001. The follow-up of (99m)Tc-DMSA pinhole imaging was performed on 25 patients. RESULTS: Overall detection rate of (99m)Tc-DMSA planar imaging was 41.8%(28 patients/67 patients), but (99m)Tc-DMSA pinhole imaging showed cortical defects in 62.7%(42 patients/67 patients) and the patients with renal cortical defects in planar imaging showed all definite or more additional cortical defects in pinhole imaging. The detection rate of cortical defect by (99m)Tc- DMSA pinhole imaging in patients with vesicoureteral reflux(VUR) was 80%(24 kidneys/30 kidneys). Those without VUR, however, also presented high detection rate as 25.9%(27 kidneys/ 104 kidneys). Out of 25 patients, improvement of cortical defect was presented in 19, no change in 4, and aggravation in 2 from the follow-up studies by (99m)Tc-DMSA pinhole imaging. CONCLUSION: This study indicates that (99m)Tc-DMSA pinhole imaging is superior to planar imaging and, hence, that pinhole imaging is a useful initial diagnostic tool for children suspected of urinary tract infection, irrespective of VUR, and to identify patients who need more extensive radiological studies and early treatment.
Child ; Follow-Up Studies ; Hospitals, General ; Humans ; Pediatrics ; Succimer ; Ultrasonography ; Urinary Tract Infections* ; Urinary Tract*

PURPOSE: Treatment options for patients with advanced gastric cancer remain limited. Few studies have investigated the efficacy and tolerability of the combination regimen of oxaliplatin and 5-fluorouracil with leucovorin for patients with advanced gastric cancer. The goal of this study was to examine the efficacy and toxicity of a modified FOLFOX-6 (mFOLFOX-6) regimen as a first-line chemotherapy regimen for patients with advanced gastric cancer. MATERIALS AND METHODS: From March, 2006, to December, 2007, 82 patients with advanced gastric cancer received 100 mg/m2 oxaliplatin and 100 mg/m2 leucovorin on the first day of treatment, followed by 2,400 mg/m2 of 5-fluorouracil on the first and second days of treatment every 2 weeks as a first-line treatment. RESULTS: The median age of the enrolled patients was 62 years (range; 30~75). Out of 82 patients, 34 cases (41.5%) were recurrent cases after curative resection, and the other 48 cases were unresectable or non-curative resectable cases. Their response was evaluated every 6 weeks. The overall response rate was 40.2%, with 2 (2.4%) complete response and 31 (37.8%) partial responses. The median time to progression (TTP) and overall survival (OS) time were 6.0 months (95% confidence interval [CI]: 4.69~7.31) and 13.0 months (7.99~18.0), respectively. The grade 3~4 hematologic toxicities observed included neutropenia (34.1%), thrombocytopenia (7.3%), and anemia (1.2%). The gastrointestinal toxicities observed included grade 3~4 nausea (9.8%) and vomiting (7.3%). Six patients (7.3%) experienced grade 3 neuropathy. No treatment-related deaths were recorded. CONCLUSION: The modified FOLFOX-6 regimen is effective and well tolerated as a first-line chemotherapy regimen for patients with advanced gastric cancer.
Anemia ; Antineoplastic Combined Chemotherapy Protocols ; Chemotherapy, Adjuvant ; Fluorouracil ; Humans ; Leucovorin ; Nausea ; Neutropenia ; Organoplatinum Compounds ; Retrospective Studies ; Stomach Neoplasms ; Thrombocytopenia ; Vomiting

PURPOSE: Total knee replacement is one of the most painful orthopedic procedures, and effective pain relief is essential for early mobility and discharge from hospital. The aim of this study was to evaluate whether addition of single-injection femoral nerve block to epidural analgesia would provide better postoperative pain control, compared to epidural analgesia alone, after total knee replacement. MATERIALS AND METHODS: Thirty-eight patients received a single-injection femoral nerve block with 0.25% levobupivacaine (30 mL) combined with epidural analgesia (femoral nerve block group) and 40 patients received epidural analgesia alone (control group). Pain intensity and volume of patient-controlled epidural analgesia medication and rescue analgesic requirements were measured in the first 48 hours after surgery at three time periods; 0-6 hours, 6-24 hours, and 24-48 hours. Also, side effects such as nausea, vomiting, and pruritus were evaluated. RESULTS: Median visual analog scale at rest and movement was significantly lower until 48 hours in the femoral nerve block group. Patient-controlled epidural analgesia volume was significantly lower throughout the study period, however, rescue analgesia requirements were significantly lower only up to 6 hours in the femoral nerve block group. The incidences of nausea and vomiting and rescue antiemetic requirement were significantly lower in the femoral nerve block group up to 6 hours. CONCLUSION: The combination of femoral nerve block with epidural analgesia is an effective pain management regimen in patients undergoing unilateral total knee replacement.
Aged ; Analgesia, Epidural/*methods ; Analgesia, Patient-Controlled/*methods ; Anesthetics, Local/administration & dosage/therapeutic use ; Arthroplasty, Replacement, Knee/*methods ; Bupivacaine/administration & dosage/analogs & derivatives/therapeutic use ; Female ; Femoral Nerve/*drug effects ; Humans ; Injections ; Male ; Middle Aged ; Nerve Block/*methods

Intrathecal baclofen (ITB) therapy has been proven to reduce severe spasticity in cerebral palsy (CP). However, few results reported the objective gait pattern change after ITB bolus injection in adult ambulatory CP. We therefore evaluated observational and kinematic gait patterns at different ITB bolus injection doses. We performed a test trial of 3-day ITB bolus injections at doses of 12.5 microg, 25 microg, and 50 microg in ambulatory CP. We evaluated modified Ashworth scale, visual analogue scale, observational gait scale, and kinematic gait analysis after ITB bolus injection. Intrathecal administration of low-dose baclofen 25 microg was successfully used not only for the treatment of spasticity but also for the treatment of gait disturbance, whereas the higher dose baclofen 50 microg induced foot drop and deteriorated gait pattern. We experienced dose-dependent changes in gait pattern confirmed by the observational and kinematic gait assessments after ITB bolus injection in adult ambulatory CP.
Adult* ; Baclofen* ; Cerebral Palsy* ; Foot ; Gait* ; Humans ; Muscle Spasticity

OBJECTIVE: To evaluate the effect of hospital-based cardiac rehabilitation (CR) on quality of life (QOL) and physical ability in patients with myocardial infarction (MI). METHODS: Patients with MI who were referred to the Cardiac Health and Rehabilitation Center 2 weeks after percutaneous coronary intervention were divided into CR and non-CR groups. The CR group performed supervised exercises 3 times a week for 2 months. QOL assessment, using the 36-item Short-Form Health Survey (SF-36) and physical ability evaluation were performed at the beginning and end of CR. RESULTS: The CR group demonstrated statistically significant improvements in physical functioning (PF), physical role functioning (RP), bodily pain (BP), general health perceptions (GH), vitality (VT), social role functioning (SF), emotional role functioning (RE), mental health (MH), physical component summary (PCS), and mental component summary (MCS). The non-CR group showed improvement in RP. Secondary outcomes, including resting heart rate (RHR), maximal oxygen consumption (VO(2max)), metabolic equivalent of task (MET), maximal exercise time (ET(max)), stage 3 Borg rating of perceived exertion (3RPE), maximal Borg rating of perceived exertion (RPEmax), and stage 3 rate pressure product (3RPP), improved in the CR group. The non-CR group showed improvements in VO(2max), MET, ET(max), and 3RPE. There were significant differences in improvements in PF, RP, BP, VT, SF, MH, MCS, RHR, VO(2max), MET, ET(max), 3RPE, and 3RPP between the two groups. CONCLUSION: Male patients with MI demonstrated improvements in QOL and physical ability following hospital-based CR; the impact on the mental component was greater than that on the physical component.
Exercise ; Health Surveys ; Heart Rate ; Home Care Services, Hospital-Based ; Humans ; Male ; Mental Health ; Metabolic Equivalent ; Myocardial Infarction* ; Oxygen Consumption ; Percutaneous Coronary Intervention ; Physical Fitness ; Quality of Life* ; Rehabilitation Centers ; Rehabilitation*

No abstract available.
Dermatitis, Atopic ; Stem Cells