Hyperinsulinemic hypoglycemia is a relatively rare disease in childhood period except neonate, but hypoglycemia due to delicate imbalance between glucose production & consumption is evoked easily and left permanent damage to brain at these period. The definition is that serum insulin level is above 10microU/ml when blood sugar level is below the 40mg/dl and so I/G ratio is higher than 0.4. The clinical manifestations are irrtability, frequent feeding and seizures etc. and there is no specific pancreatic pathology in most cases. We experienced 2 cases of hyperinsulinemic hypoglycemia with pancreatic hyperplasia and pancreatic adenoma each other. The diagnosis was made on clinical manifestations, laboratory results, radiologic and pathologic findings. We reported these cases with brief review of literature.
Adenoma ; Blood Glucose ; Brain ; Diagnosis ; Glucose ; Humans ; Hyperplasia ; Hypoglycemia* ; Infant, Newborn ; Insulin ; Pathology ; Rare Diseases ; Seizures

No abstract available.
Cleft Lip*

40 cases of bile duct carcinoma gathered over a 6-year period at Kosin Medical College were reviewed and theirclinical and cholangiographic findings were as follows: 1. There were 29 males and 11 females (the ratio of men towomen, 2.6:1) ranging from 37 to 74 years of age. The majority (70% of cases) were in 4th & 5th decades. 2.Clinical symptoms and signs: jaundice in 95%, RUQ or epigastric pain in 75%, pruritus in 52.5%, dark urine in 35%,weight loss in 32.5%, fever and chills in 22.5%, clay colored stool in 12.5%, and palpable mass in 12.5%. 3.Lab,findings: elevated serum total bilirubin(above 20.0mg% in 45%, 10.0-19.0mg% in 22.5%, 5.0-9.9mg% in 20%,1.3-4,9mg% in 5%), elevated alkaline phosphatase in 95%. Clonorchiasis were noted in 17.5%. 4. Histologic findingswere adenocarcinoma in most cases. 5. The location of bile duct carcinoma were common hepatic duct in 35%, commonbile duct in 32.5%, porta hepatis in 12.5%, junction with cystic duct in 10% and diffuse form in 10%. 6. In 33cases, PTC or post-operative cholangiographic examination were done. And the most frequent findings weredilatation of the proximal bile duct and abrupt narrowing or complete obstruction of distal lumen. In 27cases(82%), complete obstruction of bile duct was noted. Attempts were made to analyze the type of obstruction:Constricted type in 39%, Nipple type in 18%, round or flat type (smooth or slightly irregular) in 15%, andserratd type in 9%. Incomplete obstruction were noted in 6 cases(18%). Among them, abrupt narrowing of lumen wasnoted in 9% and diffuse narrowing in 9%. 7. ERCP was done in 7 cases. Findings were constricted type in 42.6%,constricted and slightly irregular type in 14.3%, downward convexity in 14.3%, diffuse irregular narrowing in14.3% and intraluminal filling defect in 14.3%.
Adenocarcinoma ; Alkaline Phosphatase ; Bile Ducts ; Bile ; Chills ; Cholangiopancreatography, Endoscopic Retrograde ; Clonorchiasis ; Cystic Duct ; Female ; Fever ; Hepatic Duct, Common ; Humans ; Jaundice ; Male ; Nipples ; Pruritus

Alzheimer's disease (AD) is the most common etiology of dementia, that has not been previously reported in Korea. We have experienced two cases of Alzheimer's disease, one occurred in a 53-year-old man with dementia and the other occurred in a 36-year-old woman with a family history of early onset dementia. A neocortical biopsy was done to rule out the cause of dementia and showed diffusely scattered numerous senile plaques and neurofibrillary tangles in cortex of both cases. Interestingly, GFAP, an immunohistochemical stain showed strong positivity in neuritic plaques and the surrounding fibrillary gathering.
Adult ; Alzheimer Disease* ; Biopsy ; Dementia ; Female ; Humans ; Korea ; Middle Aged ; Neurofibrillary Tangles ; Plaque, Amyloid

Alzheimer's disease (AD) is the most common etiology of dementia, that has not been previously reported in Korea. We have experienced two cases of Alzheimer's disease, one occurred in a 53-year-old man with dementia and the other occurred in a 36-year-old woman with a family history of early onset dementia. A neocortical biopsy was done to rule out the cause of dementia and showed diffusely scattered numerous senile plaques and neurofibrillary tangles in cortex of both cases. Interestingly, GFAP, an immunohistochemical stain showed strong positivity in neuritic plaques and the surrounding fibrillary gathering.
Adult ; Alzheimer Disease* ; Biopsy ; Dementia ; Female ; Humans ; Korea ; Middle Aged ; Neurofibrillary Tangles ; Plaque, Amyloid

PURPOSE: To elucidate the mechanism of neoangiogenesis of human retinal pigment epithelium (HRPE) under hypoxia. METHODS: HRPE cells were cultured for 2 and 24 hours in a hypoxic chamber. Expression and production of the angiogenic factor, vascular endothelial growth factor (VEGF) and the anti-angiogenic factor, pigment epithelium-derived factor (PEDF) were analyzed by RT-PCR and Western blot, respectively. Neoangiogenesis was induced by adding culture supernatant harvested from cells exposed to hypoxic conditions. Neoangeogenesis was measured with a tube formation assay that uses ECV 304 cells and with a migration assay that uses human dermal microvascular endothelial cells. RESULTS: Competitive RT-PCR showed that the expression of the PEDF gene in HRPE cells under hypoxic state decreased compared to normoxic state (p<0.01) but the expression of the VEGF gene increased (p<0.01) when exposed to hypoxic conditions. These results corresponded to those of the Western blot analysis which revealed a significant increase of VEGF production (p<0.01) and a decrease of PEDF production (p<0.01). Moreover, the tube formation and migration assays demonstrated that angiogenesis was increased by exposure to hypoxic stress. Taken together, HRPE cells under hypoxic stress produce more VEGF and less PEDF, which lead to neoangiogenesis. CONCLUSIONS: These results suggest that the subretinal neovascularization that occurs under hypoxic stress might be caused by an imbalance of angiogenesis-related factors in HRPE cells.
Angiogenesis Inducing Agents ; Anoxia* ; Blotting, Western ; Endothelial Cells ; Epithelial Cells* ; Humans ; Retinal Pigment Epithelium ; Retinaldehyde* ; Vascular Endothelial Growth Factor A

McCune-Albright syndrome, which consists of cafe-au lait spots, multiple fibrous dysplasia, sexual precocity and endocrinal dysfunction such as pituitary, thyroid and adrenal aberrations, has been increasingly recognized recently. The etiology is now suggested that activating mutations on the gene that encodes the alpha subunit of stimulatory G protein cause McCune-Albright syndrome. The child presented with sexual precocity and elevated estrogen level was still persisted after left oophorectomy and right partial oophorectomy due to misdiagnosed as ovarian granulosa-theca cell tumor. Whole body bone scan revealed abnormal radiotracer uptake on left facial bone and left proximal femur. Facial bone MRI showed marked thickening of facial bone from fibrous dysplasia. And the patient had cafe-au-lait pigmentations on neck and buttock area. Other endocrine function was normal. Pathologic finding of ovary was finally confirmed with cystic follicles. Skull biopsy revealed fibrous dysplasia. And she was finally diagnosed McCune-Albright syndrome.
Biopsy ; Buttocks ; Cafe-au-Lait Spots ; Child ; Estrogens ; Facial Bones ; Female ; Femur ; Fibrous Dysplasia, Polyostotic* ; GTP-Binding Proteins ; Humans ; Magnetic Resonance Imaging ; Neck ; Ovariectomy ; Ovary ; Pigmentation ; Skull ; Thyroid Gland

No abstract available.
Pulmonary Atelectasis*

The adrenocartical carcinoma is a rare tumor with an estimated incidence of 1 case per 1,700,000 population. Despite its rarity, a large number of investigators have studied this neoplasm for the following two reasons. The First is the occasional difficulty of differentiation between careinoma and adenoma at the time of initial surgery, even by histopathologic examination. The other is its unique feature of corticosteroidogenesis. Steroid metabolism of adrenocortieal carcinoma is characterized by its low efficiency of steroid production due to deficiency of steroidogenic enzyme. The deficieney of 11 B-hydroxylase has been indieated in case of adrenoeortical carcinoma by several investigators. In this study, the serum level of cortisol was within normal range, but the serum level of 11-deoxycortisol was 50 times higher than normal. After the removal of tumor, the serum level of ll-deoxycortisol was markedly decm. In conclusion, the results from the this case suggest that measurement of serum 11-deoxy- cortisol may be a useful tool in the diagnosis and follow-up of adrenocortical carcinoma.
Adenoma ; Adrenocortical Carcinoma* ; Cortodoxone ; Diagnosis ; Follow-Up Studies ; Humans ; Hydrocortisone ; Incidence ; Metabolism ; Reference Values ; Research Personnel

PURPOSE: To study the effects of growth hormone(GH) treatment on glucose metabolism and insulin resistance in children with idiopathic short stature(ISS). METHODS: Glucose and insulin concentrations were measured during oral glucose tolerance test (OGTT) before and after GH treatment(0.6-0.7 IU/kg/week) in 20 patients with ISS. Insulin resistance was assessed by homeostasis model assessment(HOMA). RESULTS: During OGTT, the mean blood glucose level did not show any significant changes after GH treatment. However, mean blood insulin levels of fasting and 30 minutes of OGTT showed significant increases after GH treatment, accompanying significant increases of insulin resistance. There was no difference in change of glucose, insulin levels and insulin resistance before and after GH treatment between two groups of body mass indices(BMI) of 25< and >25. There also was no significant difference between two groups of with and without family histories of diabetes mellitus (DM). There was no case of newly developed impaired glucose tolerance, fasting glucose tolerance, nor newly developed DM. CONCLUSION: GH treatment with doses of 0.6-0.7 IU/kg/week for mean 9.6 months in patients with ISS did not show any significant changes in blood glucose levels during OGTT. However, GH treatments induced considerably higher fasting insulin levels compared to pretreatment, resulting in statistically higher insulin resistance. Higher BMI and family history of DM did not induce any significant changes in glucose, insulin level and insulin resistance after GH treatment than the other groups.
Blood Glucose ; Child ; Diabetes Mellitus ; Fasting ; Glucose Tolerance Test ; Glucose* ; Growth Hormone* ; Homeostasis ; Humans ; Insulin ; Insulin Resistance ; Metabolism*