No abstract available.
Adrenal Hyperplasia, Congenital*

Idiopathic renal hypouricemia is a hereditary disease characterized by abnormally high renal uric acid clearance. A defect in the SLC22A12 genes, which encodes the renal uric acid transporter, URAT1, is the known major causes of this disorder. Most patients are clinically silent, but exercise-induced acute kidney injury, urolithiasis or hematuria may develop. The patient presented with azotemia, decreased urine output and abdominal pain without vigorous exercise past history. He was diagnosed with rapidly progressive glomerulonephritis at admission, but low serum uric acid level was persisted. Since the diagnosis of the patient was familial renal hypouricemia, we performed sequence analysis of the SLC22A12 gene in all family members. We report a case of 17-year-old boy with severe acute kidney injury with familial renal hypouricemia confirmed by genotyping of SLC22A12 .

BACKGROUND: During the last three decades, the resistance of S. pneumoniae to penicillin has been rapidly increasing in many parts of the world, especially in Korea. To characterize the clinical features and epidemiology of penicillin-resistant S. pneumoniae (PRSP) infections in the community and hospital, as well as to investigate the possible spread of resistant clone, we performed the antimicrobial susceptibility tests, pulsed filed gel electrophoresis (PFGE) and penicillin-binding protein (PBP) profile analysis of PRSP isolates. METHODS: A total 48 PRSP isolates from patients who visited or were admitted to Korea University Guro hospital during the period form July 1998 to June 1999 were studied. Anitimicrobial susceptibility tests for 48 isolates were performed with microbroth dilution method to determine the minimal inhibitory concentration of 11 antibiotics. 39 isolates and 35 isolates were subjected to PFGE and PBP profile analysis, respectively to investigate the genetic relatedness between PRSP isolates. RESULTS: Pneumonia was most common site of infection in the community and the hospital as 50%. There were no significant differences of clinical features and prognosis between community and hospital isolates. But, patients with serious underlying diseases had poor prognosis despite of acquisition site. High level penicillin resistance were observed in 69%, multi-drug resistance were 64.6% of isolates. PFGE showed that 13 of 29 community acquired infection were identical PFGE pattern but not that of 23F Spanish clone. There were various PFGE patterns were observed from community and hospital acquired infection isolates. Some of them were existed in both. PBP profiles showed more diverse, even if in isoaltes of the same PFGE pattern. CONCLUSOIN: In our study, high level penicillin resistance and multi-drug resistance were observed in PRSP clinical isolates. No clinical and prognostic differeces were observed between community and hospital acquired infections. Molecular epidemiology study were suggest the there were various genotypes of PRSP within our society. Some of them were observed in the hospital and community. Therefore, there was an evidence of communication of PRSP clones between the community and hospital.
Anti-Bacterial Agents ; Clone Cells ; Drug Resistance, Multiple ; Electrophoresis ; Epidemiology ; Genotype ; Humans ; Korea ; Molecular Epidemiology ; Penicillin Resistance ; Penicillin-Binding Proteins ; Penicillins ; Pneumonia ; Prognosis ; Streptococcus pneumoniae* ; Streptococcus*

Abutment teeth supporting removable partial denture could be faced a number of problems including development of dental caries. If the existing removable partial denture is in clinically acceptable state and the patient does not want to replace the existing removable partial denture, then a new prosthesis for abutment teeth need to be made. The procedure of fabricating a new prosthesis of abutment teeth for existing removable partial denture is complicate and technically challenging. To fabricate the abutment crown, the original cast of patient obtained before any complication to the abutment teeth is required. The original cast should also contain teeth other than the abutment teeth as a reference point. Once the cast is prepared, CAD/CAM could be used to produce retrofitting prosthesis effortlessly and efficiently. This clinical report presents fabricating a crown to fit existing removable partial denture using CAD/CAM for a patient with post and core failure and dislodged prosthesis. The prosthesis had high stability with minimum adjustment yielding satisfying result.
Crowns ; Dental Caries ; Denture, Partial, Removable ; Humans ; Prostheses and Implants ; Tooth

BACKGROUND: Cerebellar hemorrhage may present with a wide spectrum of clinical manifestations, from a benign course with little to no neurological deficit to a rapidly fatal course with hydrocephalus and brainstem compression. However, controversy remains concerning the management of patients with cerebellar hemorrhage. The aim of this study was to set the criteria for conservative or surgical treatment and predictive factors of poor outcomes. METHODS: During the 5-year period from July 1997 through July 2002, a series of 64 consecutive patients with spontaneous cerebellar hemorrhage was evaluated. On admission, all patients underwent a standard neurological examination, and a computed tomography (CT) scan. The location and size of hematoma, hypertension, hydrocephalus, intraventricular hemorrhage and compression of quadrigeminal cistern on a CT scan were compared with the Glasgow coma scale (GCS) and Glasgow Outcome Scale (GOS). RESULTS: Patients with GCS scores of 13 or more at admission and with a hematoma of less than 15 mL showed good outcomes. Patients with GCS scores of 10 or less at admission or with a hematoma measuring 15 mL or more had poor outcomes. Patients with intraventricular hemorrhage (p<0.05), compression of quadrigeminal cistern (p<0.05), and hydrocephalus (p<0.05) in the brain CT had poor outcomes. CONCLUSIONS: In treating patients with spontaneous cerebellar hemorrhage, an initial GCS and CT scan were quite helpful in determining treatment strategies. For patients whose CT scan show intraventricular hemorrhages, compression of the quadrigeminal cistern, and hydrocephalus, intensive therapy should be deemed necessary.
Brain ; Brain Stem ; Glasgow Coma Scale ; Glasgow Outcome Scale ; Hematoma ; Hemorrhage* ; Humans ; Hydrocephalus ; Hypertension ; Neurologic Examination ; Prognosis ; Tomography, X-Ray Computed

OBJECTIVE: The introduction and development of the embolic protecting device (EPD) has resulted in a decreased rate of stroke after carotid artery stenting (CAS). The authors performed a retrospective study to investigate the risk factors for developing large emboli after CAS which can lead to ischemic events. METHODS: A total of 35 consecutive patients who underwent CAS between January 2009 and March 2012 were included in this study. Patients were divided into two groups including those with small emboli (group A; grade 1, 2) and those with large emboli (group B; grade 3, 4). The size and number of emboli were assigned one of four grades (1=no clots, 2=1 or 2 small clots, 3=more than 3 small clots, 4=large clots) by microscopic observation of the EPD after CAS. We compared demographic characteristics, medical history, and angiographic findings of each group. RESULTS: Thirty-five patients underwent CAS, and technical success was achieved in all cases. Twenty-three patients were included in group A and 12 patients in group B. Our results demonstrated that advanced age [odds ratio (OR) 1.24; 95% confidence interval (CI) 1.01-1.52; p=0.044] and smoking (OR 42.06; CI 2.828-625.65, p=0.006) were independent risk factors for developing large emboli after CAS. CONCLUSION: In patients with carotid artery stenosis treated with CAS, advanced age and smoking increased the number and size of emboli. Although use of an EPD is controversial, it may be useful in CAS in patients with risk factors for large emboli in order to reduce the risk of ischemic events.
Carotid Arteries ; Carotid Stenosis ; Embolic Protection Devices ; Humans ; Retrospective Studies ; Risk Factors ; Smoke ; Smoking ; Stents ; Stroke

FISH is suggested as a possible method to detect the numerical and structural abnormalities of chromosomes in interphase nucleus. We performed this study to discuss the clinical usefulness of FISH in uncultured amniocytes and to set up the cut-off value for further study. We collected amniotic fluid samples from patients whose chromosome studies were recommended due to screen positive for Down and Edword syndrome in triple marker test using maternal serum. The centromeric probe for chromosome 18 and the locus-specific probe for chromosome 21 were used and the results were compared to their karyotypes. We could find 2 cases of trisony 21 and 2 cases of trisony 18 and the other cases represented normal karyotypes. The accuracies were 91% for disomy 18, 89% for trisomy 18, 92% for disomy 21 and 88% for trisomy 21. Therefore FISH technique is a possible method to detect the chromosomal abnormalities in uncultured amniocytes and the use of locus-specific probe for chromosome 21 would be more useful for detecting the aneuploidy of chromosome 21 than 13/21 centromeric probe.
Amniotic Fluid ; Aneuploidy ; Chromosome Aberrations ; Chromosomes, Human, Pair 18 ; Chromosomes, Human, Pair 21 ; Down Syndrome* ; Female ; Fluorescence* ; Humans ; In Situ Hybridization* ; In Situ Hybridization, Fluorescence ; Interphase ; Karyotype ; Trisomy

BACKGROUND: Measles is an acute highly contagious infection caused by measles virus. Since the introduction of measles vaccines in 1963, measles has been dramatically decreased in Korea. However, measles outbreaks have occurred periodically every 4 to 6 years during the 1990s. During 2000, measles outbreak of large scale occurred throughout the country and the total of 32,088 cases were reported. The majority of affected population was infants and school-aged children, but measles was reported also among adolescent and adult population. There was no report about the epidemiology of adult measles cases during the last decade. In this study, we retrospectively analyzed the epidemiologic and clinical features of adult measles patients in Korea. METHODS: From July of 2000 to January of 2001, 50 adult cases of measles had been admitted to Korea University Hospital. Medical records of these 50 patients, who were clinically diagnosed as measles, were reviewed. Demographic data, clinical features, history of measles vaccination and laboratory findings were evaluated. RESULTS: The median age of patients was 23 years : 28 (56%) were male. Most cases (49/50, 98%) occurred between November of 2000 to January of 2001 and 8 (16%) patients were associated with outbreaks. The occupation of patients was diverse but, students (28%) and office workers (20%) were the most common. 44 (88%) of 50 patients had no underlying diseases. Adult measles patients demonstrated similar clinical symptoms, which were shown in children with measles. In laboratory findings, mean WBC count was 4,933/micro L and 18 (36%) patients had thrombocytopenia. 21 (42 %) patients had complication of measles, and hepatitis was the most common episodes among them. Measles vaccination history revealed that 5 (10%) had two-dose and 7 (14%) had one-dose measled vaccine. In 35 patients, measles IgM antibody test was done. It appears that IgM positive rate among one-dose measle vaccination group, two-dose measle vaccination group, and group with unknown vaccination history were 86% (6/ 7), 40% (2/5), and 78% (18/23), respectively. Most (94%) of patients were hospitalized and more than two-third of patients were isolated. All the patients were recovered without sequelae. CONCLUSION: In 2000 measles outbreak, adult measles had the typical clinical features similar to those in the children and the severe complication was rare. Adult measles patients demonstrated low measles vaccination rate and occurred mainly among the students and office workers.
Adolescent ; Adult* ; Child ; Disease Outbreaks ; Epidemiology ; Hepatitis ; Humans ; Immunoglobulin M ; Infant ; Korea ; Male ; Measles virus ; Measles* ; Medical Records ; Occupations ; Retrospective Studies ; Thrombocytopenia ; Vaccination ; Vaccines

BACKGROUND: Epidural pressure is reported to change in accordance with intracranial pressure (ICP). As ICP changes during general anesthesia, it is also possible that epidural pressure may change during general anesthesia. The aim of this study was to obtain trends of epidural pressure change during general anesthesia. METHODS: Eighteen patients scheduled for gastrectomy were allocated for this study after obtaining informed consent. Epidural catheter was inserted at T7-8, T8-9 interspace before induction. Catheter was connected to a pressure transducer after calibration. General anesthesia was induced with thiopental sodium (5 mg/kg), succinylcholine (1 mg/kg), followed by 3% enflurane. Anesthesia was maintained with 50% N2O in oxygen and 1-2% enflurane with vecuronium (0.1 mg/kg). Each patients was mechanically ventilated with tidal volume of 10 ml/kg at a rate of 10 bpm. Epidural pressure was measured before induction, at the time of injection of thiopental sodium, succinylcholine, laryngoscopy, intubation, surgical incision, and 30 minutes after surgical incision. Stastical analysis was done using repeated measures of ANOVA with Helmert option (p<0.05). RESULTS: Epidural pressure significantly changed dynamically during general anesthesia. Epidural pressures increased at intubation and at 30 minutes after surgical incision when compared with those at the time of laryngoscopy and incision, respectively (p<0.05). CONCLUSION: Our study indicates that epidural pressures changes dynamically during induction period of general anesthesia and also showed possibility that epidural pressure monitoring could be used instead of more invasive direct ICP monitoring.
Anesthesia* ; Anesthesia, General ; Calibration ; Catheters ; Enflurane ; Gastrectomy ; Humans ; Informed Consent ; Intracranial Pressure ; Intubation ; Laryngoscopy ; Oxygen ; Succinylcholine ; Thiopental ; Tidal Volume ; Transducers, Pressure ; Vecuronium Bromide

The brachial plexus block is a valuable method of providing anesthesia for the surgery of forearm and hand. Common technique for brachial plexus block include axillary, supraclaviculer, and interscalene approaches. The supraclavicular brachial plexus block offers adventages, ie, high success rate, rapid onset of action and relatively complete block over axillary and interscalene approach, but may be associated with pneumothorax. We marked the P point which was placed on the upper margin of clavicle that crossed the first rib on the chest PA film and marked on the skin of the patient and inserted 23G scalp needle at that point to the first rib. We performed this modified technique of supraclavicular brachial plexus block using chest PA film in 20 patients and there was no incidence of pneumothorax after this block but general anesthesia was sdministered in one case because of incomplete block. We recommend that this modified technique guided by chest PA film for supraclavicular block is simple, reliable and may reduce the incidence of pneumothorax and to increase the success rate in difficult case such as patient with extremely obesity.
Anesthesia ; Anesthesia, General ; Brachial Plexus* ; Clavicle ; Forearm ; Hand ; Humans ; Incidence ; Needles ; Obesity ; Pneumothorax ; Ribs ; Scalp ; Skin ; Thorax*