To know the efficacy of intravenous gammaglobulin(IVGG) treatment and the resulting outcome at 18 months for acute Guillain-Barr syndrome (GBS) in children, clinical courses of 27 GBS patients who had been treated with IVGG in a dose of 1g/kg/day oyer 2 consecutive days were investigated. One died with respiratory failure and 8 patients received assisted ventilatory care with improvement. All consecutively treated patients responded well to IVGG. The mean plateau period in 26 recovered patients was 8.5 days. At the 6th month evaluation, only one failed to reach grade 2 on GBSSG scales. At the 18th month evaluation, minor motor symptoms such as easy fatigability or mild tremor on writing were remained in 9 patients. 9 patients showed mild to moderate febrile response to IVGG infusion, but no significant side effects were noted. IVGG can be chosen as an initial treatment for acute GBS in children because of its rapid effect, relative safety even in unstable patients and convenience of administration.
Child* ; Humans ; Respiratory Insufficiency ; Tremor ; Weights and Measures ; Writing

Duplication of the urethra within a single penile shaft is a rare anomaly. These can be divided into those that are in the sagittal plane, which is most common, and those that occur side by side. In some cases, the accessory channel is complete, having a separate bladder opening and no communication with the more normal ventral urethra. In other cases, the accessory urethra is incomplete, either ending blindly or communicating with the urethra distal to the bladder neck. We report on a 20-year-old male with incomplete double urethra.
Humans ; Male ; Neck ; Urethra* ; Urinary Bladder ; Young Adult

The Currarino triad is a unique complex of congenital anomalies including anorectal malformation, scral bonyabnormality, and presacral mass. The usual symptomatology is constipation due to anorectal stenosis. Threepatients with this triad confirmed by surgical operation during recent two yeas in Seoul National Universitychildren's Hospital were presented with a brief review of clinical features, unique radiologic appearance andpostulated pathophysiology. The presacral mass was a mature teratoma in the first patient, alipomyelomeningocele-epidermoid inclusion cyst in the second patient, and myelomeningocele in the third patient. Acorrect diagnosis of the Currarino triad by radiologists is important, and may help the phsicians to investigatedetailed family history and associated anomalies and to establish adequate surgical plans.
Constipation ; Constriction, Pathologic ; Diagnosis ; Humans ; Meningomyelocele ; Seoul ; Teratoma

PURPOSE: The incidence of lung cancer in patients with idiopathic pulmonary fibrosis(IPF) is higher than that of general population. To evaluate the frequency and CT findings of lung cancer associated with idiopathic pulmonary fibrosis, we analyzed 19 patients with lung cancer associated with idiopathic pulmonary fibrosis. MATERIALS AND METHODS: We analyzed retrospectively 19 patients with histologically confirmed lung cancer out of 208 patients diagnosed as IPF either by CT and clinical findings(n=188) or histologically(n=20). All 19 patients were male, aged 40--85 years (mean 66 years). Scanning techniques were conventional CT in 12 patients, HRCT in 1 patient and both conventional CT and HRCT in 6 patients. We analyzed the CT patterns of lung cancer and IPF, locations of the tumor and histologic types of lung cancer. RESULTS: The incidence of lung cancer in patients with idiopathic pulmonary fibrosis was 9.1%(19/208). In 11 of 19 patients, CT findings of lung cancer were ill-defined consolidation-like mass. Lung cancer was located mainly in lower lobes(right lower Iobe;10/19, left lower Iobe;5/19) and at the periphery(12/19). Histologically, squamous cell carcinoma was the most common cell type (11/19). CONCLUSION: The incidence of lung cancer in patients with idiopathic pulmonary fibrosis was much higher than that of general population. Typical CT findings of lung cancer were predominantly ill-defined consolidation like mass at the peripheral lung portion which is the [ocatiaon where the most advanced fibrosis occur.
Carcinoma, Squamous Cell ; Fibrosis ; Humans ; Idiopathic Pulmonary Fibrosis* ; Incidence ; Lung Neoplasms* ; Lung* ; Male ; Retrospective Studies

Keratosis punctata of the palmar creases (KPPC) is rare skin condition characterized by punctiform hyperkeratotic pits confined to the palmar and digital creases. Although this condition has been regarded as a variant of classical punctate keratoses, there are some differences between classical punctate keratosis and KPPC. We herein report a case of KPPC in a 22-year-old man who had numerous, tiny, hyperkeratotic pits limited to the palmar creases of both hands with typical histologic findings.
Hand ; Humans ; Keratosis* ; Skin ; Young Adult

Hypertensive encephalopathy is characterized by an acute, severe rise in blood pressure associated with headache, nausea, vomiting, altered mental status, and focal neurologic deficits, and rapid improvement after control of blood pressure. An eight-year old boy had been admitted with symptoms of red urine, fever, headache, convulsion, and visual blurring of vision. Blood pressure was 140/90mmHg, and CSF exam revealed WBC 8/mm3, glucose 83mg/dL, protein 106mg/dL. Serum C3 was 8mg/dL, C4 17mg/dL, ASO 1,024 Todd units. Brain CT showed non specific findings, however, MRI revealed symmetric high signal intensity lesions on T2WI over the parieto-occipital areas bilaterally. These lesions had been completely resolved on the following MRI 4 weeks later.
Blood Pressure ; Brain* ; Fever ; Glomerulonephritis* ; Glucose ; Headache ; Humans ; Hypertensive Encephalopathy* ; Magnetic Resonance Imaging* ; Male ; Nausea ; Neurologic Manifestations ; Seizures ; Vomiting

Vasectomy has become a popular method for male sterilization in Korea and this, in turn, has been followed by an increase in the number of patients requesting vasectomy reversal. Recently, many authors have reported a high success rate of vasovasostomy using microsurgical techniques. However, a significant discrepancy persist between the patency and pregnancy rate despite improvements in surgical techniques. A total of 200 patients undergoing vasovasostomy from May 1983 to April 1990 were reviewed. Of these 200 patients, only 130 patients (98 patients by two-layer, 32 patients by modified two-layer technique) could be followed up and analyzed as to pregnancy rate related to age, vasal obstructive interval, gross appearance and sperm quality of the vas fluid, operative method, and presence or absence of sperm granuloma. Success rates were 90.3% for patency and 65.4% for pregnancy. With regard to factors influencing to operation, it seemed that the results were better when there was a shorter vasal obstructive interval, watery vas fluid, presence of normal morphologic sperm quality, presence of sperm granuloma, and when the operation was done using the two-layer technique. However, no statistically significant difference was found, except between the bilateral presence and absence of sperm granuloma at the vasectomy site. Thus it is suggested that the most important single factor influencing the success rate of vasovasostomy is a meticulous and skillful operative technique itself, althought other factors may affect the outcome. More effort and research are needed to find the factors affecting the success rate of vasovasostomy.
Granuloma ; Humans ; Infertility* ; Korea ; Pregnancy Rate ; Pregnancy* ; Spermatozoa ; Sterilization, Reproductive ; Vasectomy ; Vasovasostomy*

PURPOSE: Cerebral cortical dysplasias are one of the important causes for epileptic seizures and developmental disabilities in children, particularly in diffuse or bilateral cases. These developmental malformations are generally regarded as a group of neuronal migration disorders, however, the classification system and pathogenetic mechanisms of cortical dysplasias are not yet entirely clear. Even a novel entity, congenital bilateral perisylvian syndrome, characterized by speech delay, pseudobulbar Palsy, intractable seizures, and bilateral perisylvian abnormalities on imaging studies, have rather diverse figures on morphology and symptomatology than initially considered. We have studied the clinical features and correlations of clinical outcomes 3nd magnetic resonance imaging(MRI) findings of bilaterally involved cortical dysplasias. METHODS: 20 cases of bilateral cortical dyspalsias were grouped into three categories on the basis of MRI findings; centroparietal dysplasias(6 cases), diffuse dysplasias with (5) or without white matter lesions(4), and schizencephaly(5). EEGs, motor and language development, epilepsy, and outcomes were reviewed in each groups. RESULTS: Language delay(100%), motor developmental delay(94.7%), motor deficit(65%), epilepsy(40%) were the main reasons for their initial hospital visits. A meaningful word expression was possible at the mean age of 2 years and 2 months(1 to 4 years of age). Hypotonia and spastic motor paralysis were evident in all the cases of diffuse dysplasia with white matter lesions and the schizencephaly groups. All but one case of centroparietal dysplasia showed motor developmental delay. Epilepsies were developed in 8 cases at the mean age of 5 years and 5 months(2 months to 12 years of age) and the seizures were relatively well controlled with anticonvulsants. EEG findings were variable; normal, focal or diffuse abnormalities. High amplitude diffuse fast activities were only noted in the diffuse dysplasia group. CONCLUSION: The severity of neurological deficits and developmental delay had correlated to the size of cortical abnormalities. The epilepsies were relatively well controlled during childhood and the EEG finding of high amplitude diffuse fast activities was specific for the diffuse cortical dysplasias. Bilateral centroparietal dysplasias should be included on consideration of the causes for developmental aphasia.
Anticonvulsants ; Aphasia ; Child ; Classification ; Developmental Disabilities ; Electroencephalography ; Epilepsy ; Humans ; Language Development ; Language Development Disorders ; Magnetic Resonance Imaging* ; Malformations of Cortical Development* ; Muscle Hypotonia ; Muscle Spasticity ; Neuronal Migration Disorders ; Paralysis ; Pseudobulbar Palsy ; Seizures

The total 223 cases of tetralogy of Fallot is analysed angiographically, in which selective biplanecinecardioangiography with axial projection has been done at the Departement of Radiology, Seoul National University Hopital, in recent 2 year and 4 months, with specific refrence to the location of pulmonic stenosis,and associated cardiac anomalies. The summary of the analysis is as follow; 1. Left ventriculogram (long axialoblique view) was necessary to detect the ventricular septal defects including anterior marginal VSD, and washelpful to identify the coronary artery anatomy and presence of PDA and evaluate the size of left ventricle. 2.Right ventriculogram(elongated RAO view) was the best projection for the demonstration of pulmonic infundibulumand was essential to differenciate and localize the ventricular septal defects. 3.In the right ventriculogram(4chamber view), well demonstrated the right ventricular outflow tract, pulmonary valve, and pulmonaryarteries(main, right, left and peripheral), and also perimitted the detection of ASD in levophase. 4. The positionof ventricular septal defects was perimembranous in 199 cases(89.2%), subarterial in 15 cases(6.8%) andinfundibular (intracristal) in 9 cases(4%). Multiple ventricular septal defects (perimembranous& marginal ) werefound in 11 cases(4.5%). 5. In 35 cases(15.5%), pulmonary stenosis is found only in pulmonary infundibulum. Thecombination of its components was most commonly anterior wall and conus septum hypertrophy in 24 cases(68.6%) 6.Combined pulmonary stenosis was identified in 188 cases(84.3%). The most common combination was infundibular andpulmonary valvular stenosis in 56 cases(29.8%) 7. Associated cardiac anomalies were right aortic arch in 53 cases(23.8%), persistent left SVC in 17 cases (7.7%), ASD in 13 cases (5.9%), PDA in 9 cases(4.1%), aberrant subclavianartery in 5 cases(2.2%), IVC interruption with azygos continuation in 2 cases, single coronary artery in 2 cases,and anterior descending artery from right coronary artery in 1 case, etc. 8. Selective biplanecinecardioangiography with axial projection is essential to evaluate the size of left ventricle and to localizethe ventricular septal defects and pulmonary stenosis, and to detect associated cardiac anomalies in T.O.F. Sometimes aortography is necessary when anomaly of coronary arteries is suspected.
Aorta, Thoracic ; Aortography ; Arteries ; Constriction, Pathologic ; Conus Snail ; Coronary Vessels ; Heart Septal Defects, Ventricular ; Heart Ventricles ; Hypertrophy ; Pituitary Gland ; Pulmonary Valve ; Pulmonary Valve Stenosis ; Seoul ; Tetralogy of Fallot

We report two cases of type V hyperlipoproteinemia and eruptive xanthomas associated with diabetes mellitus in a 44-year-old woman and a 25-year-old woman. They had asymptomatic, diffuse, erythematous yellowish papules on the extremities and buttocks. The laboratory studies showed increased serum blood glucose, total cholesterol, triglyceride, increasing pre-β and chylomicron bands on electrophoresis of lipoprotein. Histopathologic findings of erythematous yellowish papules on the thigh and buttock revealed aggregation of foam cells in the dermis. They have been treated with diet control, hypoglycemic agent (gliclazide) and hypolipidemic drug (gemfibrozil), and are under continued supervision.
Adult ; Blood Glucose ; Buttocks ; Cholesterol ; Dermis ; Diabetes Mellitus* ; Diet ; Electrophoresis ; Extremities ; Female ; Foam Cells ; Humans ; Hyperlipoproteinemia Type V* ; Lipoproteins ; Organization and Administration ; Thigh ; Triglycerides ; Xanthomatosis*