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

No abstract available.

Subperiosteal Cortical Defect is a symptomless rarefaction of cortical bone which begins most often on the external surface of the medial and posterior cortical walls of the lower end of the femur in children between the ages of 3 and 6 years. The name of Subperiosteal Cortical Defect was first used by Aegerter who differentiated it from non-osteogenic fibroma. The authors found out 5 positive cases of incidental findings among 62 cases of both knee joint X-ray of which 3 was male and 2 was female. Also we experienced 3 cases which necessiat d a correct diagnosis and differential diagnosis. Because this is a common lesion, it frequently accompanies more serious bone conditions, and because this is self-limited disease, treatment is not necessary. It was emphasized that its nature should be understood by all those dealing with bone disease lest it be overdiagnosed and overtreated.
Bone Diseases ; Child ; Diagnosis ; Diagnosis, Differential ; Female ; Femur ; Fibroma ; Humans ; Incidental Findings ; Knee Joint ; Male

Rhabdomyosarcoma which was first described by Weber has generally been considered an uncommon tumor. Recently it was classified as four types such as pleomorphic, embryonal, botryoid and alveolar type by Stout, Stobbe et al, Horn et al. and Riopelle et al. Five cases of rhabdomyosarcoma treated recently in Seoul National University Hospital were repored in this paper. 1. There were three pleomorphic types, one alveolar type and one undifferentiated malignant mesenchymal tumor. 2. Four cases were between 18-23 years old and one was 37 years old. 3. Three were male and two were female. 4. Time to be consulted to us was average 8 months. 5. Three were in lower extremity (two in thigh, one in calf) and two were in upper extremity (one in elbow, the other in forearm). 6. Follow-up study was done for three cases. One was exoired at,three months after hindquarter amputation, the other was expired at two years after supracondylar amputation and no recurrence and metastases were developed one year after local resection in another one case.
Amputation ; Animals ; Elbow ; Female ; Follow-Up Studies ; Horns ; Humans ; Lower Extremity ; Male ; Neoplasm Metastasis ; Recurrence ; Rhabdomyosarcoma ; Seoul ; Thigh ; Upper Extremity

Spontaneous coronary artery dissection(SCD) is a rare entity that is infrequently diasnosed antemortem. The majority of patients have presented with acute myocardial infaction or sudden death. the etiology, prognosis and treatment of this entity remain ill-defined. We report two cases of SCD which were diagnosed by coronary agniogram and intracoronary ultrasound.
Coronary Vessels* ; Death, Sudden ; Humans ; Prognosis ; Ultrasonography

Hemodialysis is a major mode of renal replacement therapy in children as well as CAPD. PermCath , a dual lumen silicone atrial catheter with a dacron cuff, has been used as vascular access for hemodialysis. Between Aug. 1, 1994 and Mar. 31, 1996, 29 children had been received PermCath catheter implantation for hemodialysis in the Department of Pediatrics, Seoul National University Children's Hospital. Their medical records were analyzed retrospectively. The mean age and weight of the patients were 9.8+/-4.6years (6months-17years) and 25.7+/-11.0kg (6.2kg-40kg), respectively. For short-term use (60days) users, mean life span of the catheter was 230.8 +/-158.6days (61-591days) and the actuarial catheter survival rate at 1 year was 44 %. The mean recirculation rate checked in 5 patients was 9.6+/-4.0%. The rate of thrombotic occlusion was 1.5/1,000 catheter days. Most of the clots except one could be lysed with local urokinase therapy. The rate of catheter associated fever was 0.6/1,000 catheter days. One catheter needed revision due to malposition and local bleeding developed during the procedure. PermCath catheterization was immediate, durable and relatively safe vascular access in childhood hemodialysis.
Arteriovenous Fistula ; Catheterization* ; Catheters* ; Child ; Fever ; Hemorrhage ; Humans ; Kidney Transplantation ; Medical Records ; Pediatrics ; Peritoneal Dialysis, Continuous Ambulatory ; Polyethylene Terephthalates ; Renal Dialysis* ; Renal Replacement Therapy ; Retrospective Studies ; Seoul ; Silicones ; Survival Rate ; Thrombosis ; Urokinase-Type Plasminogen Activator

Objective: This study aimed to determine whether there are regional differences in the blood-brain barrier (BBB) permeability of cognitively normal elderly participants and to identify factors influencing BBB permeability with a clinically feasible, 10-minute dynamic contrast-enhanced (DCE) MRI protocol. Materials and Methods: This IRB-approved prospective study recruited 35 cognitively normal adults (26 women; mean age, 64.5 ± 5.6 years) who underwent DCE T1-weighted imaging. Permeability maps (Ktrans) were coregistered with masks to calculate the mean regional values. The paired t test and Friedman test were used to compare Ktrans between different regions. The relationships between Ktrans and the factors of age, sex, education, cognition score, vascular risk burden, vascular factors on imaging, and medial temporal lobar atrophy were assessed using Pearson correlation and the Spearman rank test. Results: The mean permeability rates of the right and left hippocampi, as assessed with automatic segmentation, were 0.529 ± 0.472 and 0.585 ± 0.515 (Ktrans, x 10-3 min-1 ), respectively. Concerning the deep gray matter, the Ktrans of the thalamus was significantly greater than those of the putamen and hippocampus (p = 0.007, p = 0.041). Regarding the white matter, the Ktrans value of the occipital white matter was significantly greater than those of the frontal, cingulate, and temporal white matter (p < 0.0001, p = 0.0007, p = 0.0002). The variations in Ktrans across brain regions were not related to age, cognitive score, vascular risk burden, vascular risk factors on imaging, or medial temporal lobar atrophy in the study group. Conclusion Our study demonstrated regional differences in BBB permeability (Ktrans) in cognitively normal elderly adults using a clinically acceptable 10-minutes DCE imaging protocol. The regional differences suggest that the integrity of the BBB varies across the brains of cognitively normal elderly adults. We recommend considering regional differences in Ktrans values when evaluating BBB permeability in patients with neurodegenerative diseases.

Objective: This study aimed to determine whether there are regional differences in the blood-brain barrier (BBB) permeability of cognitively normal elderly participants and to identify factors influencing BBB permeability with a clinically feasible, 10-minute dynamic contrast-enhanced (DCE) MRI protocol. Materials and Methods: This IRB-approved prospective study recruited 35 cognitively normal adults (26 women; mean age, 64.5 ± 5.6 years) who underwent DCE T1-weighted imaging. Permeability maps (Ktrans) were coregistered with masks to calculate the mean regional values. The paired t test and Friedman test were used to compare Ktrans between different regions. The relationships between Ktrans and the factors of age, sex, education, cognition score, vascular risk burden, vascular factors on imaging, and medial temporal lobar atrophy were assessed using Pearson correlation and the Spearman rank test. Results: The mean permeability rates of the right and left hippocampi, as assessed with automatic segmentation, were 0.529 ± 0.472 and 0.585 ± 0.515 (Ktrans, x 10-3 min-1 ), respectively. Concerning the deep gray matter, the Ktrans of the thalamus was significantly greater than those of the putamen and hippocampus (p = 0.007, p = 0.041). Regarding the white matter, the Ktrans value of the occipital white matter was significantly greater than those of the frontal, cingulate, and temporal white matter (p < 0.0001, p = 0.0007, p = 0.0002). The variations in Ktrans across brain regions were not related to age, cognitive score, vascular risk burden, vascular risk factors on imaging, or medial temporal lobar atrophy in the study group. Conclusion Our study demonstrated regional differences in BBB permeability (Ktrans) in cognitively normal elderly adults using a clinically acceptable 10-minutes DCE imaging protocol. The regional differences suggest that the integrity of the BBB varies across the brains of cognitively normal elderly adults. We recommend considering regional differences in Ktrans values when evaluating BBB permeability in patients with neurodegenerative diseases.

Amyloidosis is a rare disease that results from the deposition of extracellular protein in various body tissues, causing progressive organ dysfunction. Secondary renal amyloidosis is a rare but serious complication of chronic inflammatory bowel disease, particularly in patients with Crohn's disease or ulcerative colitis. We report a case of secondary renal amyloidosis in a pediatric patient who reported a 16-year history of “very early onset inflammatory bowel disease”. Intensive treatment including repeated infliximab infusions improved clinical parameters of inflammatory bowel disease, although renal dysfunction showed progression. Amyloidosis should be considered in patients with IBD, particularly if they suffered disease progression.
Amyloidosis ; Colitis, Ulcerative ; Crohn Disease ; Disease Progression ; Humans ; Inflammatory Bowel Diseases ; Infliximab ; Rare Diseases

OBJECTIVES: We sought to examine the diverse factors associated with aneurysms of the posterior circulation. In addition, the results of conventional craniotomy were compared with those of endovascular treatment. METHODS: One hundred and one patients with posterior circulation aneurysms were selected for study inclusion. The factors that might affect the clinical outcomes were studied , such as the initial Hunt-Hess (H-H) grade, aneurysm location, size of the aneurysm, and therapeutic modalities. In addition, the morbidity and mortality rates were analyzed. The treatment outcomes were evaluated using the Glasgow Outcome Scale (GOS) 6 months after the initial insult. RESULTS: The patient population consisted of 67 women and 34 men, with a mean age of 52 (range 28-81 years). The overall morbidity and mortality rates at 6 months were 13.9% (14/101) and 17.8% (18/101), respectively. Sixty-one operations (60.3%) were performed, and 32 patients were treated with endovascular therapy. Forty-two (85.7%) of the 49 patients that had initial H-H grades of I and II had a better prognosis (GOS more than 4) than those with poor H-H grades (P<0.001). Patients that underwent endovascular treatment had better outcomes than those that had clipping (P=0.032). There was no significant difference in outcome according to the size of the aneurysm, location of the aneurysm, or the age of the patients. CONCLUSIONS: The results of this study showed that the factors affecting the prognosis were the initial HH grade and treatment modality. Considering the very high mortality rate in patients with rebleeding, early management may help improve the prognosis of patients with posterior circulation aneurysms. Endovascular therapy should be considered the primary treatment modality in patients with posterior circulation aneurysms.
Aneurysm ; Craniotomy ; Female ; Glasgow Outcome Scale ; Humans ; Intracranial Aneurysm ; Male ; Prognosis