Glycogen storage diseases(GSD) are inherited disorders affecting glycogen metabolism and type I GSD is due to the absence or deficiency of glucose-6-phosphatase(G6Pase) enzyme in the liver, kidney, and intestinal mucosa. The defect leads to inadequate hepatic conversion of G6P to glucose and thus make affected individuals susceptible to fasting hypoglycemia, and the accumulation of glycogen occurs in the liver and other organs. Type Ia is the most common form of GSD and clinically growth retardation may manifest of GSD itself rather than growth hormone deficiency(GHD), but we experienced a case of type I GSD with GHD in a 14-year-o1d male. The height was 125 cm, compatible with 50 th percentile of height of 8 years of age. He has doll-like face with fat cheek, relatively thin extremities, and metabolic acidosis, hyperuricemia, hypoglycemia, hyperlipidemia. GH stimulation test with clonidine and L-dopa revealed that the patient had decreased GH secretion. After laboratory work up including liver biopsy, he was diagnosed as type I GSD. Hypoglycemia was managed with frequent feeding with high starch diet(uncooked cornstarch). Metabolic acidosis and hyperuricemia were treated with sodium bicarbonate, allopurinol and probenecid. The patient is being followed at out-patient clinic with clinical improvement after of diet therapy and GH administration.
Acidosis ; Allopurinol ; Biopsy ; Cheek ; Clonidine ; Diet Therapy ; Extremities ; Glucose ; Glycogen Storage Disease* ; Glycogen* ; Growth Hormone* ; Humans ; Hyperlipidemias ; Hyperuricemia ; Hypoglycemia ; Intestinal Mucosa ; Kidney ; Levodopa ; Liver ; Male ; Metabolism ; Outpatients ; Probenecid ; Sodium Bicarbonate ; Starch

No abstract available.
Zygapophyseal Joint*

The increase in size of lymph node on Ct scan is the single most important finding of lymphadenopathy. The purpose of this study was to evaluate the size of mediastinal lymph nodes in patients with idiopathic pulmonary fibrosis with no evidence of malignancy or current infection. CT scans of 70 patients (16 with conventional CT and 54 with high-resolution CT) were assessed for lymph node size and locations. The duration of symptoms, and the extent and patterns of the parenchymal lung disease on CT scans were correlated with lymph node enlargement. In 54 of 70 patients, more than one lymph nodes were larger than 1-1.5cm. The prevalence of mnode enlargement increased significantly with a longer duration of symptom (p=0.001), larger extent of the disease (p=0.043), and with a greater proportion of honeycomb pattern (p=0.0344). Right paratracheal, subcarinal, right tracheobronchial, and paraesophageal nodes were the most common sites of nodes enlargement. In conclusion, mediastinal lymph node enlargement is common in patients with idiopathic pulmonary fibrosis and is more frequently seen in patients with a longer duration of clinical symptoms, greater extent of the disease, and with a larger proportion of honeycomb pattern.
Humans ; Idiopathic Pulmonary Fibrosis* ; Lung Diseases ; Lymph Nodes* ; Lymphatic Diseases ; Prevalence ; Tomography, X-Ray Computed*

PURPOSE: To analyse the macular thickness in normal and diabetic patient using OCT mapping protocol. METHODS: OCT using macular thickness map protocol was performed in 46 eyes of 25 healthy volunteers and 121 eyes of 85 diabetic patients with clinical diagnosis of no diabetic retinopathy (25 eyes), nonproliferative diabetic retinopathy (NPDR) without clinically significant macular edema (CSME; 35 eyes), proliferative diabetic retinopathy (PDR) without CSME (27 eyes), and diabetic retinopathy with CSME (34 eyes). RESULTS: The mean +/- standard deviation foveal thickness was 146 +/- 21 micrometer in normal eyes, 147 +/- 20 micrometer in diabetic eyes without retinopathy, 173 +/- 24 micrometer in NPDR without CSME, 174 +/- 27 micrometer in PDR without CSME, and 322 +/- 146 micrometer in diabetic retinopathy with CSME. There was significant difference in foveal and outer temporal retinal thickness between the normal eyes and the diabetic retinopathy without CSME group (p<0.01). Diabetic eyes with CSME had a statistically significant greater thickness in each of the areas compared with the other group and showed perifoveal focal macular edema in 5 eyes. CONCLUSIONS: OCT mapping protocol provide intuitive and objective information on focal diabetic macular edema.
Diabetic Retinopathy ; Diagnosis ; Healthy Volunteers ; Humans ; Macular Edema ; Retinaldehyde ; Tomography, Optical Coherence

We clinically reviewed four patients who were suffering from aseptic meningitis combined with polio-like paralysis and performed radiological. neurological and virological studies on them. Three patients were admitted to the department of Pediatrics. Asan Medical Center from March to August 1990, and one patient was admitted to the department of Pediatrics. Kangnam General Hospital. The results were as follows; 1) During the period of study, 18 patients (10.1%)out of 178 aseptic meningitis patients also suffered from hand, foot and mouth disease, and three patients (1.69%) out of 178 patients also suffered from polio-like paralysis. 2) The age of the patients who also suffered from polio-like paralysis were eight months, 22 months, 23 months and four years old respectively. Three patients were male and one patient was female. 3) Paralysis developed three to five days after aseptic meningitis infection. 4) In two cases, Magnetic Resonance Imaging (MR) showed two small cavitary lesions that means anterior horn cell necrosis in concordant with residual paralysis. Axial T1-weighted image showed decreased signal intensity and axial T2-weighted image showed increased signal intensity. In another case 20 days after onset of illness MR was performed, the transverse myelitis showed mild swelling of cornus medullaris. Follow up MR in 2 months revealed atropic change in lower thoracic spinal cord. 5) The electromyography and nerve conduction velocity test revealed acute polyradiculoneuropathy. 6) Serologic virus study were carried out in three cases for Coxsackievirus A16 and Enterovirus 71. The Enterovirus 71 antibody titer were 1:128 in two cases and 1:64 in one case. The Coxsackievirus A16 antibody titer were less than 1:4. This study showed that the causative agents for polio-like paralysis during this period seemed to be the Enterovirus 71 infection.
Anterior Horn Cells ; Chungcheongnam-do ; Cornus ; Electromyography ; Enterovirus ; Female ; Follow-Up Studies ; Hand, Foot and Mouth Disease ; Hospitals, General ; Humans ; Magnetic Resonance Imaging ; Male ; Meningitis, Aseptic* ; Myelitis, Transverse ; Necrosis ; Neural Conduction ; Paralysis* ; Pediatrics ; Polyradiculoneuropathy ; Spinal Cord

PURPOSE: To elucidate high-resolution CT(HRCT) findings and their pathologic basis in pulmonary oil embolism induced by LipiodoI-Adriamycin emulsion. MATERIALS AND METHODS: Pulmonary oil embolism was induced by infusing LipiodoI-Adriamycin emulsion through a peripheral vein in twelve Yorkshire pigs. Serial HRCT scans were performed on 2rid, 4th, 7th, 14th, and 28th day after the procedure. The pigs were sacrificed immediately after HRCT and histologic specimens were prepared in the same plane and level with HRCT. RESULTS: The basic pathology was reversible hemorrhagic edema of the lung. On HRCT, intraalveolar hemorrhage and edema in the acute stage manifested as ground-glass opacity or air-space consolidation of the whole secondary Iobule. The lesions were predominantly distributed over the dependent posterior lung fields because the specific gravity of Lipiodol is 1.28. Interlobular septal thickening due to edematous fluid collection was also associated. With the elapse of time, the extent and severity of the acute lesions resolved and, sometimes, changed into small nodular opacities. Pulmonary opacity was most severe on the post-embolization 2nd day and completely resolved within 2 weeks. CONCLUSION: Pulmonary embolization of LipiodoI-Adriamycin emulsion causes reversible hemorrhagic edema of the lung and Lipiodol toxicity seems to play a major role. HRCT findings of pulmonary oil embolism are quite different from those of pneumonia and pulmonary metastasis, which suggests the possibility of clincal application.
Edema ; Embolism* ; Ethiodized Oil ; Hemorrhage ; Lung ; Neoplasm Metastasis ; Pathology ; Pneumonia ; Specific Gravity ; Swine ; Veins

PURPOSE: To evaluate ethanolamine oleate (EAO)-microfibrillar collagen (MFC) mixture as a new scleroembolic material for the interventions requiring both permanent obliteration of vascular lumen and atrophy of mass, such as for the facial AVM and other hypervascular soft tissue masses. MATERIALS AND METHODS: Twenty-nine transcatheter transarterial embolizations of renal arteries were performed in six groups of rabbits classified by the EAO concentration and the addition of MFC. Postembolization angiography, gross morphological and microscopic examinations of embolized kidneys were performed immediately, 3 days, 2 weeks and 4 weeks after embotization. Analysing points were the usefulness as a scleroembolic material (endovascular retention, thrombogenic-sclerosing effect, perivascular fibrosis and inflammatory reaction), effects of the EAO concentration and the addition of MFC. RESULTS: EAO-MFC mixture satisfied all ideal conditions of scleroembolic agent;persistent endovascular retention, good thrombogenic-sclerosing effect with a mild inflammatory reaction and significant atrophy of kidney. The effect of increasing concentration of EAO was proximal embolization. The effects of MFC were promotion of proximal embolization, endovascular retention and sclerosing effect. CONCLUSION: EAO-MFC mixture can be used as a new effective scleroembolic material for the various hemodynamic situations in which embolic level can be controlled by EAO concentration and the addition of MFC.
Angiography ; Atrophy ; Collagen* ; Ethanolamine* ; Fibrosis ; Hemodynamics ; Kidney ; Oleic Acid* ; Rabbits* ; Renal Artery

OBJECTIVES: This study aimed to investigate North Korean refugees' knowledge of mental illnesses and treatments and analyze the factors affecting this knowledge. METHODS: Subjects were selected via a snowball sampling method, and the survey outcomes of 152 North Korean refugee participants were analyzed. The factors affecting knowledge of mental illnesses were analyzed via a regression analysis by constructing a multivariate model with mental illness knowledge score as the dependent variable. RESULTS: The North Korean refugees' mental illness scores ranged from 3 to 24 points, with an average score of 13.0. Regarding the factors that influence mental illness knowledge, the subjects with South Korean spouses and those who had spent more time in South Korea had higher knowledge scores. Furthermore, the subjects who considered the mental health of North Korean refugees to be a serious issue revealed lower knowledge scores than those who did not believe it was a serious issue. The subjects who visit psychiatric clinics showed higher knowledge scores than those who do not. The South Korean subjects who had at least a college education exhibited higher scores than did those without advanced education. The subjects who are satisfied with life in South Korea manifested a higher mental illness knowledge score than those who are not. CONCLUSIONS: This study is significant as being the first study to ever measure and evaluate the level of North Korean refugees' knowledge of mental illnesses. In addition, the evaluations of North Korean refugees' mental illness knowledge and influencing factors while residing in South Korea created basic data that formed the foundation of an effort to enhance mental health literacy and provide proper mental health services. The results of this study can be utilized to solve mental health problems that might frequently occur during the unification process of North and South Korea in the future.
Adult ; Aged ; Democratic People's Republic of Korea ; Female ; *Health Knowledge, Attitudes, Practice ; Humans ; Literacy ; Male ; *Mental Health ; Middle Aged ; Refugees/*psychology ; Republic of Korea ; Surveys and Questionnaires

In order to determine the factors affecting the length of stay by pay status, a total of 961 in-patients medical records with appendectomy, cholecystectomy and Cesarean section discharged from the January 1979 to December 1981 from the University hospital were reviewed. Average length of stay showed no statistically significant difference by year between the insured and the non-insured patients, however multiple diagnoses and surgical complication were significantly different from single diagnosis and non-complicated cases. Surgical complication explained the length of stay mostly, and physician in discharge, multiple diagnoses, and accommodation in order for insured patients. Surgical complication, admission route, physician in charge and age in order explained the length of stay for non-insured patients.
Appendectomy ; Cesarean Section ; Cholecystectomy ; Diagnosis ; Female ; Humans ; Length of Stay* ; Medical Records ; Pregnancy

Since nationwide compulsory health insurance started in 1977, shortage of hospital beds has become apparent and this is related in part to the length of hospital stay. To determine the factors which affect the length of stay a total of 1,003 in-patients medical records for appendectomy, cholecystectomy, and Cesarean section discharged in 1965, 1970, 1975 and 1980 from a university hospital were studied. The major findings are as follows; The average length of stay showed a statistically significant difference by year among the cholecystectomy and Cesarean section patients. For the appendectomies no statistically significant difference was found. Complications after surgical operation explained the length of stay mostly in all three procedures; the next factors were age, character of surgery, day of the week of surgery, and multiple diagnoses in this order for appendectomy; day of the week of surgery, age, type of accommodation, route of admission in this order for cholecystectomy; and day of the week of surgery, multiple diagnoses, residential area and type of accommodation in Cesarean section respectively.
Adolescent ; Adult ; Age Factors ; Child ; Hospitals, Teaching/utilization* ; Hospitals, University/utilization* ; Human ; Korea ; Length of Stay* ; Regression Analysis