PURPOSE: Among syndromes of idiopathic occipital lobe epilepsy there are Panayiotopoulos syndrome (PS) and idiopathic childhood occipital epilepsy of Gastaut (ICOE-G). However, the classification of epilepsy syndrome is difficult to diagnose at the first seizure because of varying characteristics. We analyzed the different clinical characteristics and prognostic factors of idiopathic occipital lobe epilepsy. METHODS: Forty-three patients who had at least two unprovoked seizures, no abnormalities on magnetic resonance imaging, no known etiology of seizure, and mainly occipital spikes on electroencephalography were retrospectively enrolled at the Korea University Medical Center. A good prognosis was defined as being seizure-free and taking two or less drugs for 1 year. RESULTS: Among all patients, the proportion of PS was 44.2%, ICOE-G was 7.0% and unclassified group was 48.8%. The age at seizure onset was 4.5±2.6 years (mean±SD) in the PS group, and 8.3±2.1 years in the ICOE-G group. The follow-up duration was 10.8±6.0 years. The percentage that the initial diagnosis had not been changed was 66.7% in the PS group and 100% in the ICOE-G group. Among other related symptoms only emesis (P<0.001) and visual symptoms (P=0.007) had varying characteristics between the PS and ICOE-G groups. Patients with PS had a better prognosis than those with ICOE-G or unclassified group (odds ratio [OR]=58.8). Patients with 1 more autonomic symptoms had a worse prognosis (OR=3.8). CONCLUSION: This study showed that only symptoms of emesis and visual symptoms can differentiate between PS and ICOE-G. More autonomic symptoms were associated with worse prognosis in childhood occipital lobe epilepsy.
Academic Medical Centers ; Classification ; Diagnosis ; Electroencephalography ; Epilepsies, Partial* ; Epilepsy ; Follow-Up Studies ; Humans ; Korea ; Magnetic Resonance Imaging ; Occipital Lobe* ; Prognosis ; Retrospective Studies ; Seizures ; Vomiting

CT and MR appearance of the brain in three children with ornithine transcarbamylase (OTC) deficiency are described. They showed clinical signs of vomiting and convulsion and were diagnosed by measurement of plasma ammonium, amino acids, acid-base balance, and urinary orotic acid levels. CT and MR were performed within one month from the onset of the symptom. CT and MRI demonstrated brain swelling with small ventricles and diffuse low density of white matter, which indicated cerebral hypoperfusion secondary to elevated intracranial pressure. With more prolonged survival hyperammonemia may cause cerebral atrophy. CT and MR appearance in these cases resembled a hypoxic brain damage and this finding should be included in the differential diagnosis.
Acid-Base Equilibrium ; Amino Acids ; Ammonium Compounds ; Atrophy ; Brain ; Brain Edema ; Child ; Diagnosis, Differential ; Humans ; Hyperammonemia* ; Hypoxia, Brain ; Intracranial Hypertension ; Magnetic Resonance Imaging* ; Ornithine Carbamoyltransferase Deficiency Disease* ; Ornithine Carbamoyltransferase* ; Ornithine* ; Orotic Acid ; Plasma ; Seizures ; Vomiting

Background: With increasing economic evaluation studies on the treatment of or screening tools for liver diseases that cause hepatocellular carcinoma (HCC), interest in the analysis of the medical utilization and costs of HCC treatment is increasing. Therefore, we aimed to estimate the medical utilization and costs of HCC patients, and calculate the cost of main procedures for HCC treatment, including liver transplant (LT), hepatic resection (HR), radiofrequency ablation (RFA), and transarterial chemoembolization (TACE). Methods: We analyzed claim data from January to December 2018 from the Health Insurance and Review and Assessment Service–National Patient Sample (HIRA-NPS-2018) dataset, including data of patients diagnosed with HCC (Korean Standard Classification of Diseases code C22.0) who had at least one inpatient claim for HCC. Results: A total of 715 HCC patients were identified. In 2018, the yearly average medical cost per HCC patient was ₩18,460K (thousand), of which ₩14,870K was attributed to HCC. Among the total medical costs of HCC patients, the inpatient cost accounted for the largest portion of both the total medical and HCC-related costs. The major procedures of HCC treatment occurred most frequently in the order of TACE, RFA, HR, and LT. The average medical cost per treatment episode was the highest for LT (₩87,280K), followed by HR (₩10,026K), TACE (₩4,047K), and RFA (₩2,927K). Conclusion By identifying the medical costs of HCC patients and the costs of the main procedures of HCC treatment, our results provide basic information that could be utilized for cost estimation in liver disease-related economic evaluation studies.

Background: With increasing economic evaluation studies on the treatment of or screening tools for liver diseases that cause hepatocellular carcinoma (HCC), interest in the analysis of the medical utilization and costs of HCC treatment is increasing. Therefore, we aimed to estimate the medical utilization and costs of HCC patients, and calculate the cost of main procedures for HCC treatment, including liver transplant (LT), hepatic resection (HR), radiofrequency ablation (RFA), and transarterial chemoembolization (TACE). Methods: We analyzed claim data from January to December 2018 from the Health Insurance and Review and Assessment Service–National Patient Sample (HIRA-NPS-2018) dataset, including data of patients diagnosed with HCC (Korean Standard Classification of Diseases code C22.0) who had at least one inpatient claim for HCC. Results: A total of 715 HCC patients were identified. In 2018, the yearly average medical cost per HCC patient was ₩18,460K (thousand), of which ₩14,870K was attributed to HCC. Among the total medical costs of HCC patients, the inpatient cost accounted for the largest portion of both the total medical and HCC-related costs. The major procedures of HCC treatment occurred most frequently in the order of TACE, RFA, HR, and LT. The average medical cost per treatment episode was the highest for LT (₩87,280K), followed by HR (₩10,026K), TACE (₩4,047K), and RFA (₩2,927K). Conclusion By identifying the medical costs of HCC patients and the costs of the main procedures of HCC treatment, our results provide basic information that could be utilized for cost estimation in liver disease-related economic evaluation studies.

Cheek ; Humans ; Incidence ; Middle Aged ; Mouth ; Pleura ; Solitary Fibrous Tumors

BACKGROUND: Hepatitis B Virus (HBV) is a major risk factor for hepatocellular carcinoma, and about five to six percents of people are infected with HBV in Korea. Lamivudine is a first-line drug having good control against HBV replication, but long-term treatment by lamivudine induces drug resistance. We analyzed the rate of HBV resistance mutation for lamivudine by direct sequencing and CLIP sequencing. METHODS: HBV DNA was isolated from 371 patients who were in treatment, or were planning to be treated with lamivudine. The direct sequencing for lamivudine resistance mutation was performed in 371 patients and CLIP sequencing in 138 patients. We analyzed the mutation rate and the type of mutations for lamivudine resistance. RESULTS: The mutation was detected in 203 patients (54.7%) and (CTG) L180M (ATG) was most common (36.1%) followed by (ATG) M204I (ATT) (29.9%) and (ATG) M204V (GTG) (18.6%). According to the duration of treatment, mutation rates were as follows: 45.3% for less than one year, 71.7% for one to two years, 66.7% for two to three years, and 87.9% for more than three years. The results of the direct sequencing and CLIP sequencing agreed in 134 out of 138 patients, in whom both tests were performed. CONCLUSIONS: We confirmed that HBV mutation rates for lamivudine resistance increased as the lamivudine treatment period increased. The lamivudine resistance mutations detected were similar to the previous studies. CLIP sequencing showed good correlation with the direct sequencing and gave additional mutation information. CLIP sequencing is a promising tool for the detection of lamivudine resistance mutation in HBV that can assist treatment plans.
Carcinoma, Hepatocellular ; DNA ; Drug Resistance ; Hepatitis B virus ; Hepatitis B, Chronic ; Hepatitis, Chronic ; Humans ; Korea ; Lamivudine ; Mutation Rate ; Risk Factors

Ael is a rare blood type which has the least amount of A antigen among A subgroups. It can be detected by special tests performed to resolve the discrepancy between red cell and serum typing in routine serological typing. The presence of A antigen on Ael red cell is demonstrable only by adsorption and elution tests. An Ael individual does not secret A substance in the saliva and may have anti-A antibody in the serum which is usually less reactive with the reagent red cells than anti-B antibody. In Korea, Ael02 has been reported more frequently than other Ael alleles. We report a case of Ael02/O04 who presented as typical phenotype O with strong anti-A and anti-B antibodies and no A antigen detected even by adsorption and elution tests. The case has been proved to be Ael02/O04 by direct sequencing analysis. In individuals with history of discrepancies in the results of ABO phenotyping, ABO genotyping is needed for an accurate evaluation of their blood type.
ABO Blood-Group System/classification/*genetics ; Alleles ; Child ; Genotype ; Heterozygote ; Humans ; Male ; Pedigree ; Phenotype ; Sequence Analysis, DNA

Various diseases can diffusely involve central airways, including the trachea and main stem bronchi. Central airway abnormalities are frequently not apparent or are overlooked on chest radiographs, even though the patient may have significant symptoms. Recent advances in spiral and multi-detector computed tomography (CT) with multi-planar reconstruction and three-dimensional demonstration, including virtual bronchoscopy, allow for excellent display of central airway anatomy and abnormalities with visualization of accurate locations of lesions. Early detection and proper diagnosis of airway diseases based on various radiographic findings will help determine appropriate treatment, including surgical planning and evaluation of treatment response. Herein we describe and illustrate the imaging findings of a wide spectra of diffuse central airway diseases.
Bronchi ; Bronchoscopy ; Humans ; Imaging, Three-Dimensional ; Thorax ; Tomography, X-Ray Computed ; Trachea

Various diseases can diffusely involve central airways, including the trachea and main stem bronchi. Central airway abnormalities are frequently not apparent or are overlooked on chest radiographs, even though the patient may have significant symptoms. Recent advances in spiral and multi-detector computed tomography (CT) with multi-planar reconstruction and three-dimensional demonstration, including virtual bronchoscopy, allow for excellent display of central airway anatomy and abnormalities with visualization of accurate locations of lesions. Early detection and proper diagnosis of airway diseases based on various radiographic findings will help determine appropriate treatment, including surgical planning and evaluation of treatment response. Herein we describe and illustrate the imaging findings of a wide spectra of diffuse central airway diseases.
Bronchi ; Bronchoscopy ; Humans ; Imaging, Three-Dimensional ; Thorax ; Tomography, X-Ray Computed ; Trachea

Eating ; Female ; Humans ; Male ; Salivary Ducts ; Salivary Gland Calculi ; Salivary Glands ; Submandibular Gland