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

The positive binding activity of lectin, peanut agglutinin (PNA), against the mucinous malignancies of the ovary was studied in order to clarify biologic differences among those lesions using immunoperoxidase method (ABC). A total of 23 cases were included in this study and they were classified as 10 cases of mucinous cystadenocarcinoma, 9 mucinous tumors of borderline malignancy and 4 pseudomyxoma peritonei, histologically. Nine of 10 cystadenocarcinomas and all cases of pseuomyxoma peritonei showed more than moderate degree of positive binding activity (>2+) with PNA in the neoplastic epithelial cells. In the cases of borderline malignancy, only 3 of 9 revealed as much similar binding pattern with PNA as cystadenocarcinoma group, in contrast, minimal degree of positivity (1+) was noted in the remainder. These findings may suggest heterogeneity in the biochemical characteristics among the cases of borderline lesion. And it is proposed that the higher PNA binding cases in ovarian mucinous borderline malignancy require extensive sampling by multiple sections and further careful follow-up study.
Follow-Up Studies

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

We report an autopsy of a male fetus that showed multiple congenital anomalies that could best be designated as Meckel-Gruber syndrome. The fetus was born dead at the gestational age of 38 weeks. His parents denied any history of congenital malformation. And the parity of the mother was 0-0-0-0, but she had the past history of receiving herb medication for common cold. The congenital anomalies found in this case consited of occipital meningoencephalocele, midline cleft palate, bifid epiglottis, hepatic fibrosis, choledochal cyst, bilateral polycystic kidneys, postaxial polydactyly of both hands and feet, aplasia of the left testis, secundum type atrial septal defect and patent ductus arterious. This malformation syndrome is rare and lethal. The prenatal diagnosis should be made by ultrasound study or analysis of the amniotic fluid for alpha-feto protein during intrauterine period. The kidneys showed Potter type III cystic change and there was a characteristic hepatic fibrosis.
Male ; Humans

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

No abstract available.
Gastric Mucosa* ; Meckel Diverticulum* ; Ulcer*

Menetrier's disease is characterized by enlarged gastric folds with foveolar hyperplasia and cystic dilatation of gastric glands. The additional biochemical features of hypoproteinemia, hypochlorhydria, and increased gastric mucus are often encountered. The pathogenesis and etiologic factors have not been clearly defined. In this report, we present two cases of Menetrier's disease in the stomach, one occurring in a 38-year-old male, associated with massive hematemesis, and the other in a 39-year-old male. Grossly, both cases showed marked giant gastric rugal folds resembling cerebral convolutions, sparing the antral portion. Microscopically, the giant gastric rugal folds consisted of the striking foveolar hyperplasia accompanied by an occasional presence of the smooth muscle fibers from the muscularis mucosa. The immunohistochemical stain revealed an intense positive reaction for transforming growth factor-alpha (TGF-alpha) and epidermal growth factor receptor (EGF-R) in the majority of mucous cells throughout the gastric mucosa and parietal cells, but did not reveal for epidermal growth factor (EGF). We suggested that TGF-alpha and EGF-R might be involved in the pathogenesis of Menetrier's disese.
Achlorhydria ; Adult ; Dilatation ; Epidermal Growth Factor ; Gastric Mucosa ; Gastritis, Hypertrophic* ; Hematemesis ; Humans ; Hyperplasia ; Hypoproteinemia ; Immunohistochemistry ; Male ; Mucous Membrane ; Mucus ; Muscle, Smooth ; Rabeprazole ; Receptor, Epidermal Growth Factor ; Stomach ; Strikes, Employee ; Transforming Growth Factor alpha

PURPOSE: To evaluate the radiologic findings of alimentary anthrax. MATERIALS AND METHODS: 19 patients with alimentary anthrax, which was caused by ingestion of contaminated beef, were included in this study. The diagnosis was made .b.y demonstration of Bacillus anthracis in smear and culture of the contaminated meat. We evaluated the clinical manifestations and the findings of thoracic, abdominal radiographs, cervical, abdominal ultrasonograms and abdominal CT scans. RESULTS: Out of the 19 patients with the alimentary infection, 9 had oropharyngeal form, 18 had abdominal form and 8 had combination of oropharyngeal and abdominal form. The patients had general symptoms and signs such as fever, chill, myalgia. Clinical symptoms and signs were sore throat, throat injection, throat ulcer and patch in oropharyngeal form, and nausea, vomiting, abdominal pain, diarrhea, and gross GI bleeding in abdominal form. Radiologic findings included enlarged cervical lymph nodes(36%) in oropharyngeal form, and paralytic ileus(26%), ascites(26%), hepatomegaly(21%), enlarged mesenteric lymph nodes(26%), small bowel wall thickening(5%) in abdominal form. In two patients, late complications occurred as intestinal obstruction due to ileal stricture with perforation, and inflammatory changes of pelvic cavity due to ileovesical fistula. CONCLUSION: Radiologic findings of alimentary anthrax are difficult in differentiation from those of other inflammatory bowel disease, but those radiologic findings with clinical manifestations may be helpful in diagnosis and evaluation of disease process in patients with alimentary anthrax.
Abdominal Pain ; Anthrax* ; Bacillus anthracis ; Constriction, Pathologic ; Diagnosis ; Diarrhea ; Eating ; Fever ; Fistula ; Hemorrhage ; Humans ; Inflammatory Bowel Diseases ; Intestinal Obstruction ; Meat ; Myalgia ; Nausea ; Pharyngitis ; Pharynx ; Tomography, X-Ray Computed ; Ulcer ; Ultrasonography ; Vomiting

PURPOSE: The purpose of this study was to evaluate the patency, procedure related complications and effectiveness of Wallstent application to the malignant biliary obstruction as a palliative treatment. MATERIALS AND METHODS: We retrospectively reviewed the clinical results, duration of survival, patency rate and complication of the Wallstent application on 33 patients who had had obstructive jaundice by the malignant lesion in recent 3 years. One or two step procedures. were mainly taken with 10mm diameter Wallstents. Grouping according to place the stent at the hilum or not, and grouping according to place the stent through the ampulla or not were done to evaluate the difference of the patency and survival rate between the groups. RESULTS: Biliary endoprosthesis with Wallstent were successfully placed in all patients without difficulty. Procedure related short-term complication rate was about 18.1% (n=6/33). Complications were fever(n=4), cholecystitis(n=1) and sepsis(n=l). Long-term complications were mainly obstruction(n=9/31) of the Wallstent during the follow-up period. Also cholecystitis occurred in one patient 3 months later. Mean survival duration was 139.72 (46-237)days ormong those who expired. Mean patency duration of stents was 139.67 (26-310) days. Survival rates were 93.5% at the second month, 68.8% at the third month, 61.2% at the 4th month, 53.5% at the 5th month, 49.1% at the 7th month and 35.7% at the 9th month. Patency rates were 93.7% at the second month, 84.2% at the 4th month, 66.9% at the 5th month, 59.5% at the 7th month and 39.6% at the 10th month. The application was repeated in the 6 patients with stent occlusion. Significant statistical difference could not be found between the groups according to placing the stent at the hilum and according to placing the stent through the ampulla. Patency rates were higher than survival rates in the follow-up period. CONCLUSION: Wallstent application provides good palliation with little discomfort and few complications in the patients with malignant obstructive jaundice.
Cholecystitis ; Follow-Up Studies ; Humans ; Jaundice, Obstructive ; Palliative Care ; Retrospective Studies ; Stents ; Survival Rate

PURPOSE: To evaluate CT findings of internal hernia. MATERIALS AND METHODS: Three patients with internal hernia had abdominal CT scans and two of them had small bowel follow through examinations. The CT features of two patients with retroanastomotic and one patient with left paraduodenal hernia were evaluated and correlated with small bowel follow through examinations, and surgical findings. Two patients with retroanastomotic hernia had a history of subtotal gastrectomy with antecolic gastrojejunostomy. The diagnosis of internal hernia was made on the basis of surgical find ings in two patients of retroanastomotic hernia, and characteristic barium study findings in one patient of left paraduodenal hernia. RESULTS: The small bowel follow through examination showed small bowel loops gathered in a circumscribed mass in the left mid abdomen, and delay in passage through these loops. In case of left paraduodenal hernia, abrupt narrowing of the distal duodenum could be identified. The characteristic CT findings of all three patients were whorling of the mesentery and mesenteric vessels in the left mid-abdomen. In the patient with left paraduodenal hernia, after resolution of the gastrointestinal symptoms, a follow-up CT scan showed spontaneous disappearance of whorling of the mesentery and mesenteric vessels suggesting spontaneous resolution of the hernia. In the other two cases, upon exploration of the abdomen, dilatation of afferent duodenum caused by adhesion between ligament of Treitz and adjacent bowel walls, and retro- anastomotic herniation of the efferent loops were found. CONCLUSION: The usual diagnosis of internal hernia is based on the appearance of the small bowel follow through examination. However, we consider that the whorling appearance(we call it whorling sign) of the mesentery and mesenteric vessels on CT scan is also suggestive of internal hernia in patients under clinical suspicion.
Abdomen ; Barium ; Diagnosis ; Dilatation ; Duodenum ; Follow-Up Studies ; Gastrectomy ; Gastric Bypass ; Hernia ; Humans ; Ligaments ; Mesentery* ; Tomography, X-Ray Computed