OBJECTIVE: The purpose of this study was to analyze the clinical study for patients with cervical cancer who had undergone radical hysterectomy. METHOD: The subjects of this study were one hundred and sixty two patients with cervical cancer who had undergone radical hysterectomy at Eulji Medical College Hospital, Taejon, Korea, from January 1983 to December 1992. We reviewed the medical record retrospectively and analyzed the data. RESULT: The distribution of patients by age was found in the order of 50 decade and 60 decade, 40 decade. Those by the clinical stages were as follows: Stage Ia, 12 cases(7.4%); Stage Ib, 84 cases(51.9%); Stage IIa, 39 cases(24.1%); Stage IIb 27 cases(16.7%). The results of histopathologic type were distributed as follows: squamous cell carcinoma was 91.9%, adenocarcinoma was 4.9% and adenosquamous cell carcinoma was 3.1%. The histologic subtypes of squamous cell carcinoma(149 cases) were as follows: Large cell non-keratinizing type was 75.9%, large cell keratinizing type was 14.8% and small cell type was 1.2%. The frequancy of lymph node metastasis was 22.9% in stage I and 31.8% in stage II. The overall incidence of lymph node metastasis was 26.4%. The frequency of external radiation therapy done after radical hysterectomy was 63.5% in stage I and 75.8% in stage II. The 5-year survival rate was as follows: The Ia was 100%; Stage Ib, 95.2%(4cases); Stage IIa, 87.2%(5cases); Stage IIb, 77.8%(6cases). The incidence of recurrence was 7.4% and recurrent sites were vaginal stump , rectum and pelvic wall. CONCLUSION: The highest incidence of cervix cancer in age distribution was 50 decade(30.9%) and 60 decade(30.9%). The most common clinical stage was Ib(51.9%) and most frequent pathologic type was squamous cell carcinoma(91.9%). The overall incidence of lymph node metastasis was 26.4% and The most common site of recurrence was vaginal stump. The 5-year survival rate was 100% in the stage Ia, 95.2% in the stage Ib, 87.2% in the stage IIa, 77.8% in the stage IIb.
Adenocarcinoma ; Age Distribution ; Carcinoma, Squamous Cell ; Daejeon ; Humans ; Hysterectomy* ; Incidence ; Korea ; Lymph Nodes ; Medical Records ; Neoplasm Metastasis ; Rectum ; Recurrence ; Retrospective Studies ; Survival Rate ; Uterine Cervical Neoplasms*

PURPOSE: Pompe disease is one of the glycogen storage diseases caused by a deficiency of acid alpha-glycosidase. This enzyme defect results in lysosomal glycogen accumulation in many tissues and shows a various spectrum of clinical features from early infantile hypotonia to mild weakness. For the investigation of the clinical characteristics of Pompe disease, we reviewed 6 cases of childhood Pompe disease diagnosed by muscle biopsy and acid alpha-glycosidase assay. METHODS: We reviewed the medical records of 6 childhood Pompe disease patients in Seoul National University Children's Hospital, retrospectively from January 2001 to October 2006. RESULTS: The age of the symptom onset was 1 month to 11 years(mean 2.2 years) and the diagnosis was made at the age of 8 months to 14 years(mean 5.3 years). The patients showed delayed motor development, motor weakness, hypotonia, cardiomegaly, hypertrophic cardiomyopathy, hepatomegaly, recurrent pulmonary infections but the severity was very diverse. Liver transaminase and CK levels were elevated in all of the patients. Their muscle biopsy showed the characteristic accumulation of purple colored glycogen granules and the degeneration of myofibers. CONCLUSION: Childhood Pompe disease had various clinical features and severities depending on the age of onset, organ involvement and the rate of progression. Enzyme replacement therapy may modify the disease courses, so we need to diagnose earlier for the treatment at an optimal time.
Age of Onset ; Biopsy ; Cardiomegaly ; Cardiomyopathy, Hypertrophic ; Diagnosis ; Early Diagnosis ; Enzyme Replacement Therapy ; Glycogen ; Glycogen Storage Disease ; Glycogen Storage Disease Type II* ; Hepatomegaly ; Humans ; Liver ; Medical Records ; Muscle Hypotonia ; Retrospective Studies ; Seoul

PURPOSE:Temporal lobe epilepsy(TLE) is now recognized as a distinct syndrome in adults. The seizure evolution in adult patients is well characterized, manifesting initially with an aura, behavioral arrest, automatism, and secondary generalized tonic-clonic seizures. In contrast, relatively few studies are available for the pediatric age group. In the present study, we investigated children undergoing temporal lobectomy for refractory seizures and correlated the pathologic findings with clinical presentations. METHODS:The records of the pediatric patients admitted at the Seoul National Children's Hospital for epilepsy surgery between January 1995 and December 2005 were reviewed. Then, eighteen patients were included in this study. The clinical records were reviewed in terms of the patient profiles imaging findings, surgical techniques, and pathologic findings. The seizure outcomes were described according to the Engel's classification. RESULTS:The postsurgical outcomes were favorable. Lateral temporal epilepsy was more common in childhood than in adulthood. Dual pathology was commonly found. Arm dystonia or tonic arm elevation have a lateralizing value. Head turning may have a lateralizing value based upon a time sequence. The brain MRI was less predictable for pathologic findings. The ictal EEG cannot always have a localizing value. Delta beginning in the ictal rhythm may suggest lateral lobe epilepsy. Anterior temporal beginning of the ictal location may suggest mesial temporal lobe epilepsy. Ganglioglioma tends to cause rhythmic beta activities at the beginning of the ictal event. CONCLUSION:TLE in childhood shows more complex and atypical clinical manifestations and have more variable etiologies. No single presurgical investigation can be a good predictable value to localization or lateralization.
Adult ; Arm ; Automatism ; Brain ; Child ; Classification ; Dystonia ; Electroencephalography ; Epilepsy ; Epilepsy, Temporal Lobe* ; Ganglioglioma ; Head ; Humans ; Magnetic Resonance Imaging ; Pathology ; Seizures ; Seoul ; Temporal Lobe*

Menkes disease is an X-linked recessive copper transport disorder characterized by neurological deterioration, connective-tissue damage, and abnormal hair growth. It is caused by the mutation of the ATP7A gene. This report describes a four-month-old boy with neurological symptoms typical of Menkes disease plus unusual liver involvement. He developed seizures at three months of age and exhibited hypotonia, cephalhematoma, a sagging face, redundant and hypopigmented skin, and abnormal hair growth. In addition, he had unexplained hepatomegaly and high hepatic transaminase. We confirmed the diagnosis of Menkes disease by mutation analysis of the ATP7A gene. To exclude other possible causes for the hepatic abnormalities, a liver biopsy was performed, revealing intracytoplasmic cholestasis, focal spotty necrosis, and minimal lobular activity. The patient's liver involvement may be an underestimated complication of Menkes disease.
Biopsy ; Cholestasis ; Copper ; Hair ; Hepatomegaly ; Infant ; Infant, Newborn ; Liver ; Menkes Kinky Hair Syndrome ; Muscle Hypotonia ; Necrosis ; Seizures ; Skin ; Spasms, Infantile

Although the prevalence of interracial marriages in Korea is increasing, little is known regarding the pregnancy outcomes of interracial couples. The aim of this study was to investigate the differences in pregnancy outcomes between Korean and interracial Korean-foreign couples. Data for infants born in 2011 and 2012 were obtained from the national birth registry of the Korean Statistical Office. The couples were subdivided into Korean father-Korean mother, Korean father-foreign mother, and foreign father-Korean mother groups. Pregnancy outcomes included neonates with low birth weight (< 2,500 g) and those with high birth weight (> 4,000 g). In 2010 and 2011, 888,447 Korean father-Korean mother, 36,024 Korean father-foreign mother, and 4,955 foreign father-Korean mother neonates were delivered in Korea. After adjustment for parental age, educational level, parity, gestational age at delivery, and neonatal sex, the birth weights were found to be different between groups, with the highest number of foreign father-Korean mother and lowest number of Korean father-foreign mother pregnancies. Based on multivariate logistic regression analysis, the risk of low and large birth weights was higher in the Korean father-foreign mother and foreign father-Korean mother groups, respectively, compared with that in the Korean father-Korean mother group. There are significant differences in pregnancy outcomes including birth weights between Korean and interracial Korean-foreign couples.
Birth Weight ; Family Characteristics* ; Female ; Gestational Age ; Humans ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Korea* ; Logistic Models ; Marriage ; Mothers ; Parents ; Parity ; Parturition ; Pregnancy ; Pregnancy Outcome* ; Pregnancy* ; Prevalence

Methylprednisolone (MP), a glucocorticoid steroid, has an anti-inflammatory action and seems to inhibit the formation of oxygen free radicals produced during lipid peroxidation in a spinal cord injury (SCI). However, the effects of MP on the functional recovery after a SCI is controversial. The present study was conducted to determine the effects of MP on the recovery of neural conduction following a SCI. A SCI was produced using the NYU spinal cord impactor. A behavioral test was conducted to measure neurological disorders, and motor evoked potentials (MEPs) were recorded. According to the behavioral test, using BBB locomotor scaling, MP-treated animals showed improved functional recoveries when compared to salinetreated animals. MEP latencies in the MP-treated group were shortened when compared to those in the control group. Peak amplitudes of MEPs were larger in the MP-treated group than those in the control group. The thresholds of MEPs tended to be lower in the MP-treated group than those in the control group. These results suggest that MP may improve functional recovery after a SCI.
Animals ; Disease Models, Animal ; Electrophysiology ; Evoked Potentials, Motor/*drug effects ; Free Radicals ; Glucocorticoids/metabolism ; Male ; Methylprednisolone/*pharmacology ; Neurons/*drug effects ; Oxygen/metabolism ; Rats ; Rats, Sprague-Dawley ; Receptors, Glucocorticoid/metabolism ; Research Support, Non-U.S. Gov't ; Sodium Chloride/pharmacology ; Spinal Cord/pathology ; Spinal Cord Injuries/*drug therapy ; Time Factors

OBJECTIVES: This report describes the development process of a drug dosing database for ethical drugs approved by the Korea Food & Drug Administration (KFDA). The goal of this study was to develop a computerized system that supports physicians' prescribing decisions, particularly in regards to medication dosing. METHODS: The advisory committee, comprised of doctors, pharmacists, and nurses from the Seoul National University Bundang Hospital, pharmacists familiar with drug databases, KFDA officials, and software developers from the BIT Computer Co. Ltd. analyzed approved KFDA drug dosing information, defined the fields and properties of the information structure, and designed a management program used to enter dosing information. The management program was developed using a web based system that allows multiple researchers to input drug dosing information in an organized manner. The whole process was improved by adding additional input fields and eliminating the unnecessary existing fields used when the dosing information was entered, resulting in an improved field structure. RESULTS: A total of 16,994 drugs sold in the Korean market in July 2009, excluding the exclusion criteria (e.g., radioactivity drugs, X-ray contrast medium), usage and dosing information were made into a database. CONCLUSIONS: The drug dosing database was successfully developed and the dosing information for new drugs can be continually maintained through the management mode. This database will be used to develop the drug utilization review standards and to provide appropriate dosing information.
Advisory Committees ; Databases, Pharmaceutical ; Drug Utilization Review ; Humans ; Isothiocyanates ; Korea ; Pharmacists ; Radioactivity