Cerebral gigantism(Sotos syndrome) is a growth disorder that consists of large size at birth, rapid early growth rate with accompanying advanced bone age, acromegalic features, and developmental delay. Clumsiness in the absence of other abnormal neurologic findings is common. The cause is unknown. We report here a case of 238/12-year-old Sotos syndrome with final adult height above 97 percentile, abnormal brain MRI findings(large ventricles, prominent trigone, prominent occipital horn & thining of corpus callosum), clumsiness, and some behavioral problems.
Adult ; Animals ; Brain ; Growth Disorders ; Horns ; Humans ; Magnetic Resonance Imaging ; Neurologic Manifestations ; Parturition ; Sotos Syndrome

STATEMENT OF PROBLEM: Standard type of ITI solid implant model in the 6.2mm thick jaw bone was axisymmetrically modelled for finite element stress analyses. PURPOSE: Primary objective was to investigate the influences of the characteristic design configuration of the ITI solid implant model on the bone stress with the course of osseointegration process at the bone/implant interfaces. To simulate the characteristics of the osseointegration process, five different stages of the bone/implant interface model were implemented. As load conditions, vertical load of 50N was taken into consideration. Bone at the cervical region of implant was the areas of concern where the higher level of stress were likely to take place. RESULTS: The results indicated that rather slightly different stress level could be obtained as a function of the osseointegration conditions. CONCLUSION: Under vertical load, the lower level of stress was observed at the cervical cortical bone in the initial and final stages of osseointegration. Relatively higher stress level, however, was observed during the transitional stages where the osseointegration at the cancellous bone interface were yet to fully develop.
Jaw ; Osseointegration*

PURPOSE: Early postoperative arrhythmias are a major cause of mortality and morbidity after open heart surgery in the pediatric population. We evaluated the incidence and risk factors of early postoperative arrhythmias after surgery of congenital heart disease. METHODS: From January 2002 to December 2008, we retrospectively reviewed the medical records of the 561 patients who underwent cardiac surgery in Kyungpook National University Hospital. We analyzed patients' age and weight, occurrence and type of arrhythmia, cardiopulmonary bypass (CPB) time, aortic cross clamp (ACC) time, and postoperative electrolyte levels. RESULTS: Arrhythmias occurred in 42 of 578 (7.3%) cases of the pediatric cardiac surgery. The most common types of arrhythmia were junctional ectopic tachycardia (JET) and accelerated idioventricular rhythm (AIVR), which occurred in 17 and 13 cases, respectively. The arterial switch operation (ASO) of transposition of the great arteries (TGA) had the highest incidence of arrhythmia (36.4%). Most cases of cardiac arrhythmia showed good response to management. Patients with early postoperative arrhythmias had significantly lower body weight, younger age, and prolonged CPB and ACC times (P<0.05) than patients without arrhythmia. Although the mean duration of ventilator care and intensive care unit stay were significantly longer (P<0.05), the mortality rate was not significantly different among the 2 groups. CONCLUSION: Early postoperative arrhythmias are a major complication after pediatric cardiac surgery; however, aggressive and immediate management can reduce mortality and morbidity.
Accelerated Idioventricular Rhythm ; Arrhythmias, Cardiac ; Arteries ; Body Weight ; Cardiopulmonary Bypass ; Child ; Heart ; Heart Diseases ; Humans ; Incidence ; Intensive Care Units ; Medical Records ; Retrospective Studies ; Risk Factors ; Tachycardia, Ectopic Junctional ; Thoracic Surgery ; Ventilators, Mechanical

BACKGROUND: Reduced lung compliance and increased lung resistance are the primary lung mechanical abnomalities in acute respiratory distress syndrome (ARDS). Although there is little information regarding the mecha nisms responsible for the increases in the respiratory resistance of ARDS, bronchodilators have been frequently administered in mechanically ventilated ARDS patient. To determine the effect of a bronchodilator on the respiratory mechanics depending on the level of applied positive end-expiratory pressure (PEEP), the change in the respiratory mechanics by salbutamol ingalation was measured under the variable PEEP level in patients with ARDS. METHODS: Fifteen mechanically ventilated paralyzed ARDS patientss (14 of male, mean age 57 years) were enrolled in this study. The respiratory system compiance, and the maximum and minimun inspiratory occlusion method during constant flow inflaction using the CP-100 pulmonary monitor (Bicore, Irvine, CA, USA). The measurements were performed at randomly applied 8, 10 and 12 cm H2O PEEP before and 30 mins after administrating salbutamol using a meter-dose-inhaler (100 micro gram X 6). RESULTS: 1) The maximum inspiratory resistance of the lung was higher than the reported normol values due to an increase in the minimal inspiratory resistance and additional resistance. 2) The maximum inspiratory resistance and peak airway pressure were significantly higher at 12 cm H2O of PEEP compared with those at 10cm H2O of PEEP. 3) Salbutamol induced a significant decrease in the maximum and the minimum inspiratory resistance but no significant change in the additional resistance only was observed at 12 cm H2O of PEEP (from 15.66+/-1.99 to 13.54+/-2.41, from 10.24+/-2.98 to 8.04+/-2.34, and from 5.42+/-3.41 to 5.50+/-3.58 cm H2O/l/sec, respectively). 4) The lung compliance did not change at the applied PEEP and salbutamol inhalation levels. CONCLUSIONS: The bronchodiator response would be different depending on the level of applied PEEP despite the increased respiratory resistance in patients with ARDS.
Albuterol ; Bronchodilator Agents ; Humans ; Inhalation* ; Lung ; Lung Compliance ; Male ; Positive-Pressure Respiration ; Respiratory Distress Syndrome, Adult* ; Respiratory Mechanics* ; Respiratory System

The original article contained an error in Figure 1.

The original article contained an error in Figure 1.

BACKGROUND: Techniques for identification of the epidural space have advantages and disadvantages. We made an electronic apparatus to ensure an epidural space by detecting the reduction of pressure. We investgated the instrument to see if this could be adequately used for identification of the epidural space. METHODS: Thirty adult patients scheduled to receive an epidural injection for surgery were selected for the investigation. We connected the apparatus with an epidural Tuohy needle of which the tip was in the ligamentum flavum. We injected air to increase the pressure inside the epidural needle to 50 mmHg or 100 mmHg, and then the epidural needle was advanced slowly until we heard the alarm from the epidural detector when pressure inside the epidural needle suddenly decreased under the set point, suggesting the epidural needle reached the epidural space. After ensuring the epidural space we injected the anesthetics to induce epidural anesthesia. RESULTS: In both groups, we obtained successful anesthesia results and there were no complications. The volume of air injected into the epidural space was 0.64 +/- 0.1 in 50 mmHg group and 0.95 +/- 0.2 in 100 mmHg group. CONCLUSIONS: The use of the epidural detector in 30 patients demonstrated that this instrument could be a safe and effective means of identifying the epidural space. It allows the reduction of air injected into the epidural space, reducing the chance of complications due to an overinjection of air.
Adult ; Anesthesia ; Anesthesia, Epidural ; Anesthetics ; Epidural Space* ; Humans ; Injections, Epidural ; Ligamentum Flavum ; Needles

BACKGROUND: Hepatitis B virus (HBV) DNA quantification is important for the management of HBV infection and identification of the development of resistance. The susceptibility to contamination and more variable reproducibility of results with the conventional HBV DNA quantification method have raised the need of a more simple and accurate method for HBV DNA quantification. Real-time quantitative PCR assays recently introduced in the laboratory can meet these needs. In this study, we evaluated the performance of the Real-Q HBV Quantification kit developed in Korea. METHODS: We evaluated the recovery of DNA extraction, the interference of internal control, an analytical sensitivity, specificity, and reproducibility, a clinical specificity, and a reportable range of the Real-Q HBV Quantification kit. The quantification result was also compared to that obtained by the Digene Hybrid-Capture II. RESULTS: The mean percent recovery was 108.6% and there was no interference with the internal control on DNA extraction. None of HIV, hepatitis C virus, or cytomegalovirus showed a cross-reactivity with HBV. This assay detected HBV DNA in a linear range from 10(2) to 10(10) copies/mL, with the detection limit of 56 copies/mL. The assay exhibited a low within-run CV (coefficient of variation) (8.7-11.9%), between-run CV (10.5-14.7%), and between-day CV (13.2-21.4%). No HBV DNA was detected in any of 100 samples without HBV, resulting in a clinical specificity of 100%. The levels of HBV DNA showed a good correlation with those determined with Digene Hybrid-Capture II (R2=0.9827). CONCLUSIONS: The Real-Q HBV Quantification kit showed a good analytical sensitivity, specificity, and high reliability with a broad reportable range. This assay should be clinically useful in managing patients with HBV infection.
Cytomegalovirus ; DNA* ; Hepacivirus ; Hepatitis B virus ; HIV ; Humans ; Korea ; Limit of Detection ; Polymerase Chain Reaction ; Real-Time Polymerase Chain Reaction* ; Reproducibility of Results ; Sensitivity and Specificity

BACKGROUND: Carcinoma of the ampulla of Vater is rare and its pathogenesis is unclear. The role of epigenetic changes in the APC or CDH1, in the Wnt pathway, has not been reported in ampullary carcinomas. METHODS: We performed immunohistochemistry on 73 sporadic ampullary carcinomas to identify Wnt-related molecules (APC, beta-catenin, E-cadherin, c-erbB2, cyclin D1) and examined mutations in the CTNNB1, loss of heterozygosity of 5q21, and the methylation status of the CpG island of APC and CDH1. RESULTS: Thirteen tumors (17.8%) showed abnormal nuclear localization of beta-catenin; this was more prominent in the intestinal type than in the pancreaticobiliary type (p=0.01). The loss of APC correlated with the loss of beta-catenin or c-erb B2 (p<0.01). The prognosis was worse in the group with APC loss than when APC was maintained (p<0.05). There was no mutation identified in CTNNB1. Six (24%) out of 25 informative cases had 5q21 allelic loss. CpG island methylation in APC and CDH1 was detected in 33 (45.2%) and 29 (31.5%) cases, respectively. CONCLUSIONS: The absence of mutations in CTNNB1 and the epigenetic alteration of APC and CDH1, might be characteristic changes in the Wnt/beta-catenin signaling pathway during the carcinogenesis of ampullary carcinomas.
Ampulla of Vater* ; beta Catenin ; Cadherins ; Carcinogenesis ; CpG Islands ; Cyclins ; Epigenomics* ; Immunohistochemistry ; Loss of Heterozygosity ; Methylation ; Prognosis ; Wnt Signaling Pathway

BACKGROUND/AIMS: Assessment of fluid status in hemodialysis patents is very important. Overhydration in hemodialysis is associated with generalized edema, cardiovascular complications, and hypertension. The aim of this study was to determine the factors correlated with mortality of hemodialysis patients, assessing body muscle mass and fluid status using bioelectrical impedance analysis (BIA). METHODS: This study enrolled 93 patients who underwent hemodialysis between January 2010 and May 2015 at CHA Bundang Medical Center. Medical records of enrollees up to June 2017 were reviewed retrospectively. These included laboratory results (serum albumin, C-reactive protein [CRP], lipid profile, etc.) and BIA data (extracellular water, intracellular water, total body water, soft lean mass, fat free mass, skeletal muscle mass, etc.). RESULTS: Eleven of 93 patients had expired by May 2017. Among the surviving subjects, mean age was younger, CRP levels were lower, albumin levels were higher, and extracellular water/total body water (ECW/TBW) ratios were lower than in the expired patient group. Kaplan-Meier survival analysis revealed that overhydration (ECW/TBW > 0.4) was associated with higher mortality. CONCLUSIONS: In hemodialysis patients, overhydration is an important factor in mortality, and BIA could be a reliable modality in its assessment. We suggest that, for hemodialysis patients, overhydration is more of a risk factor for mortality than is muscle wasting.
Body Water ; C-Reactive Protein ; Edema ; Electric Impedance* ; Humans ; Hypertension ; Medical Records ; Mortality* ; Muscle, Skeletal ; Renal Dialysis* ; Retrospective Studies ; Risk Factors ; Water