We Reviewed 10 hypertensive children with pheochromocytoma retrospectively and the following results were obtained. 1) Out of 10 patients, 7 were male and 3 female. Age ranged from 5.5 years to 13.8 years and their median age was 9.9 years. 2) They complained of sweating, lethargy, headache. or chest pain and so on. Hypertension were noticed in all patients. Heart murmurs were detected in 7 patients and hypertensive retinopathy in 70%. 3) The three cases arised at extraadrenal gland and bilaterality was seen in 3 patients. In the view of diagnosis, abdominal sonography, computerized tomography and urine VMA test revealed the sensitivity of 100%. But MIBG scan showed 60% in sensitivity. 4) Waiting for operation, their hypertension were controlled by adrenergic blockers or calcium channel blockers. They received tumorectomy successfully except one who was in hypertensive state after operation and followed up through OPD. In conclusion high suspicion for the existence of pheochromocytoma from the clinical manifestations should be entertained in any pediatric patients and biochemical and imaging studies were mandatory. Furthermore, for the accurate localization of tumors, several imaging studies should be collaborated.
3-Iodobenzylguanidine ; Adrenergic Antagonists ; Calcium Channel Blockers ; Chest Pain ; Child* ; Diagnosis ; Female ; Headache ; Heart Murmurs ; Humans ; Hypertension ; Hypertensive Retinopathy ; Lethargy ; Male ; Pheochromocytoma* ; Retrospective Studies ; Sweat ; Sweating

A totally implanted venous access system was inserted in 19 children with cancer. The devices were utilized for the administration of antineoplastic drugs, parenteral fluids, antibiotics, and blood products. Total duration of implantation was 4,046 days for 23 implanted system (range 7-445 days). Complications included cather infection (0.247/100 catheter days), occlusion (0.692/100 catheter days), and dislodgement of needle (0.643/100 catheter days). There were major complications that necessitated removal of catheters, including systemic infections (0.09/100 catheter days) and complete occlusions (0.09/100 catheter days). The system was thought to be safe and convenient in chemotherapy, and permitted full physical activity.
Anti-Bacterial Agents ; Antineoplastic Agents ; Catheters ; Child* ; Drug Therapy ; Humans ; Motor Activity ; Needles ; Prospective Studies*

The corpus callosum consists of white fibers connecting the cerebral hemispheres. Agenesis of the corpus callosum is an uncommon congenital anomaly which is easily diagnosed in the postnatal period by ultrasound and computed tomographic scan or MRI, but its prenatal sonographic diagnosis is difficult because of fetal head positioning and limiting trans-axial scans. We experienced a case of agenesis of the corpus callusum with chromosomal anomaly. The prenatal sonographic findings are ventricular abnormalities that demonstrated dilatation of lateral ventricles and disproportionate enlargement of the occipital horns, which were suggestive findings for the corpus callosal agenesis. We could confim the diagnosis of the corpus callosal agenesis with chromosome anomaly by postnatal MRI and chromosome analysis.
Agenesis of Corpus Callosum* ; Animals ; Cerebrum ; Corpus Callosum ; Diagnosis ; Dilatation ; Head ; Horns ; Lateral Ventricles ; Magnetic Resonance Imaging ; Ultrasonography

BACKGROUND: Inhaled glucocorticoids are the medical treatment of choice in asthma patients. Fluticasone propionate is one of the most effective inhaled corticosteroids and has been reported to have minimal effect on the hypothalamic-pituitary-adrenal axis at the recommended dose. However, reports of long-term trials characterizing their systemic safety with chronic use are rare. This study was designed to evaluate the long-term safety of inhaled fluticasone propionate to the hypothalamic-pituitary-adrenal axis. METHOD: This study was conducted on 21 patients to evaluate the adrenal response to rapid ACTH stimulation test after 6 months of treatment with fluticasone propionate from 200 µg to 750 µg daily. The serum cortisol levels was measured to assess its effect on the hypothalamic-pituitary-adrenal axis just prior to the injection, at 30 minutes and 60 minutes after an intramuscular injection of synthetic ACTH. RESULT: The mean dose of inhaled fluticasone propionate was 355 µg per day(SD=174 µg, range=200 µg to 750 µg). The mean serum cortisol levels of the patients was 11.0 µg/dl(SD=6.4 µg/dl) prior to the injection, 20.0 µg/dl(SD=7.7 µg/dl) after 30 minutes, and 23.0 µg/dl(SD=6.3 µg/dl) after 60 minutes. Sixteen patients of the 21 patients had a normal response(>18 µg/dl), and 5 out of the 21 patients had serum cortisol levels below the normal range after the rapid ACTH stimulation test. CONCLUSION: Adrenal suppression occurred in 5 out of 21 patients with 6 months treatment with inhaled fluticasone propionate.
Adrenal Cortex Hormones ; Adrenocorticotropic Hormone ; Asthma* ; Axis, Cervical Vertebra* ; Cosyntropin ; Diethylpropion* ; Glucocorticoids ; Humans ; Hydrocortisone ; Injections, Intramuscular ; Reference Values ; Fluticasone

Nodular pulmonary amyloidosis is one of the rare manifestation of amyloid disease. It is known to be caused by anyloid L fibrils in the majority of case. We experienced an unusual case of a forty-one year-old woman who was presented with multiple nodular lesion on the chest X-ray. CT-guided core needle bilpsy, performed on the lesion, showed apple green birefringes, when stained Congo red and examined under polarized light. Ultrastructurally, there are randomly oriented, forming densed networks, and consists of fine, 7.5 to 10nm diameter, rigid, non-branching filaments of various lengths in electron-microscopic finding. We report a case of primary diffuse nodular pulmonary amyloidosis only localized in the lung, which was confirmed by CT guided core needle biopsy.
Amyloid ; Amyloidosis* ; Biopsy, Large-Core Needle ; Congo Red ; Female ; Humans ; Lung* ; Needles ; Thorax

The purpose of this study was to demonstrate survival rate changes after the introduction of inhaled nitric oxide (iNO) therapy, and to identify the factors that influence these changes in neonates with a congenital diaphragmatic hernia (CDH) at a single center. A total of 48 neonates were divided into two groups based on the time of admission, i.e., into period I (P1; n=17; before the introduction of iNO therapy) and period II (P2; n=31; after the introduction of iNO therapy). Survival rates of the 48 neonates showed a tendency to increase from 53% during P1 to 77% during P2, but without a statistical significance, but a significant difference was found between survival rates during the two periods after adjusting for initial clinical characteristics, when the postoperative survival rate increased significantly from 69% for P1 to 100% for P2. The mean duration of preoperative respiratory management was significantly longer for P2 than for P1. Seven of 12 patients who received preoperative iNO therapy due to persistent pulmonary hypertension or refractory preductal hypoxemia in P2 survived after operation. We speculate that a management strategy based on iNO therapy and delayed operation, rather than differences between the initial clinical characteristics of the two study groups, might partially contribute to the observed improvements in postoperative and overall survival rates in neonates with CDH.
Administration, Inhalation ; Female ; Hernia, Diaphragmatic/congenital/mortality/*therapy ; Humans ; Infant, Newborn ; Male ; Nitric Oxide/administration & dosage/*therapeutic use ; Postoperative Care/methods ; Preoperative Care/methods ; Respiratory Therapy/methods ; Survival Rate ; Time Factors ; Treatment Outcome

BACKGROUND: To study clinical characteristics and treatment outcomes of adenocarcinoma of unknown primary site (ACUPS). METHODS: A retrospective analysis of 81 patients who were diagnosed as ACUPS, seen at Samsung Medical Center from May, 1995 to July, 1999, was performed. RESULTS: The median age of the patients was 58 years. The common sites of metastases were the lymph node, liver, lung, bone. In 49 of 81 patients (60.5%), the dominant tumor location was below the diaphragm. The majority of patiens (76 of 81) were initially treated with systemic chemotherapy including cisplatin. Responses were evaluable in 70 of 76. Eighteen of 70 patients (25.7%) responded to chemotherapy and complete remission was observed in 6 patients. The overall median survival of 81 patients was 5.6 months. The median survival of the responding patients was 18.3 months but the median survial of the nonresponding patients was 4.6 months (p<0.01). In univariate and multivariate analysis, age, performance status and response to initial chemotherapy were significant prognostic factors for overall survial. CONCLUSION: Poor survival rate and treatment response were observed in ACUPS but complete response and long-term survival were observed in several patients.
Adenocarcinoma* ; Cisplatin ; Diaphragm ; Drug Therapy ; Humans ; Liver ; Lung ; Lymph Nodes ; Multivariate Analysis ; Neoplasm Metastasis ; Retrospective Studies ; Survival Rate

Autoimmune insulin syndrome is characterized by insulin autoantibody, hyperinsulinemia, and fasting hypoglycemia without previous insulin immunization. This syndrome shows discordant levels between immunoreactive insulin and C-peptide. Negative results of an anatomic study of the pancreas and an inability to reproduce hypoglycemia during a prolonged fast may be helpful in excluding insulinoma. Symptomatic hypoglycemia usually develops during an oral glucose tolerance test. This syndrome is a self-limited disorder. Recently, we experienced one case that developed symptomatic hypoglycemia during both the fasting & oral glucose tolerance test, and another that developed symptomatic hypoglycemia during the oral glucose tolerance test but not the fasting test. Hereby, we present these cases with a review of the literature.
C-Peptide ; Fasting ; Glucose Tolerance Test ; Hyperinsulinism ; Hypoglycemia* ; Immunization ; Insulin* ; Insulinoma ; Pancreas

OBJECTIVE:cDNA microarray technology was used to comprehensively analyze the gene expression in the placenta of term women with labor compared to without labor. METHODS:Placental tissue was obtained from patients in spontaneous labor (n=5) and those not in labor (n=5) during Cesarean section of full term pregnancy. mRNA levels were examined through cDNA microarray using Agilent GeneSpringGX 7.3 (Agilent technology, USA). SPSS 11.0 was used for statistical analysis. RESULTS:Among total 38,467 genes, 2,374 genes were detected to be up-regulated in labor samples, while 12 genes were down-regulated. 40 genes of them were identified as significantly up-regulated in levels of expression (up-regulated > or =5.0 fold, p<0.05). According to gene ontology analysis, they are associated with variable cell biologic functions including apoptosis, signal transduction, metabolic process, immune response, and transcription, etc. CONCLUSION:This study suggests that our results could provide interesting clues to understanding the initiation and the process of normal labor and might lead to further studies in a more targeted fashion.
Apoptosis ; Cesarean Section ; DNA, Complementary* ; Female ; Gene Expression Profiling ; Gene Expression* ; Gene Ontology ; Humans ; Metabolism ; Oligonucleotide Array Sequence Analysis* ; Placenta* ; Pregnancy ; Pregnant Women* ; RNA, Messenger ; Signal Transduction

BACKGROUND: It has been suggested that acromegalic patients are at increased risk of developing colonic neoplasia. We evaluated the prevalence of colonic neoplasia and the relationships of clinical parameters of acromegalic activity with presence of colonic neoplasia in Korean acromegalic patients. METHODS: In 30 patients with active acromegaly, colonoscopy was performed at the time of diagnosis. According to the endoscopic findings and histological classifications, the patients were grouped, and a number of clinical parameters of acromegalic activity were compared between those with and without colonic polyps. RESULTS: Colonic polyps were detected in 17 (56.7%) in the 30 acromegalic patients with colonoscopic examinations; 7 (23.3%) hyperplastic and 10 (33.3%) adenomatous polyps. Those with colonic polyps were significantly older (52.2+/-9.46 years vs. 42.2+/-12.97 years, p=0.02) and also had significantly longer disease duration (11.8+/-7.52 years vs. 5+/-2.20 years, p=0.004) than those without ones. In 21 patients who were more than 40 years old, significant differences were found in disease duration (12.7+/-7.54 years vs. 5.0+/-2.68 years, p=0.026) and serum IGF-1 level (1311.9+/-500.14 ng/mL vs. 715.9+/-330.58 ng/mL, p=0.015) between those with and without colonic polyps. CONCLUSION: These results suggest that screening colonoscopy should be recommended in acromegalic patients who are more than 40 year-old, have long disease duration or have high serum IGF-1 level.
Acromegaly ; Adenomatous Polyps ; Adult ; Classification ; Colon* ; Colonic Polyps* ; Colonoscopy ; Diagnosis ; Humans ; Insulin-Like Growth Factor I ; Mass Screening ; Prevalence*