PURPOSE: While treating 14 phenylketonuria(PKU) patients, we evaluated bone density, changes in bone age, andbony changes such as spiculation or metaphyseal widening. MATERIALS AND METHODS: A total of 14 PKU patients agedbetween 1 month and 14 years(mean, 6.4 years) were under dietary treatment. Eight and eleven patients underwentradiography of the left hand and wrist and bone densitometry(BMD) of the lumbar spine, respectively. The resultswere reviewed with regard to abnormal bony changes, delayed bone age, and osteopenia. Patients were assigned toeither the early or late treatment group, depending on whether or not dietary therapy was started before 3 monthsof age. Those in whom a blood phenylalanine level of under 10 mg/dl was maintained were assigned to the 'goodcontrol' group; others were classified as 'variable control'. The findings of radiographs of the left hand andlumbar BMD were evaluated in relation to the time of dietary therapy, and adequacy of treatment. RESULTS: Onlumbar BMD, four of 11 patients (36%) showed reduced bone density of more than 1 S.D. None of the 11 who underwentradiography of the left hand showed bony abnormalities such as spiculation or metaphyseal widening. In four of the11, bone age was less than chronological age by at least one year. According to Fisher's exact test there was norelation between delayed bone age , osteoporosis and the time and adequacy of dietary therapy (p >0.05). CONCLUSION: None of the 14 PKU patients who underwent dietary therapy had bony abnormalities such as spiculationor metaphyseal widening. In four of the 11, bone age was at least one year less than chronological age, and onlumbar BMD, osteoporosis was seen. For the evaluation of bone change in PKU patients, plain radiography and BMDare thus complementary.
Bone Density ; Bone Diseases, Metabolic ; Hand ; Humans ; Metabolism ; Osteoporosis ; Phenylalanine ; Phenylketonurias* ; Radiography ; Spine ; Wrist

We evalusted the magnetic resonance imaging (MRI) findings of the femoral heads in 20 normal and 45 abnormal patients. The bone marrow in the healthy adults consisted of a combination of hematopoietic and fatty marrow, which showed age-related dirtribation, that is the component of fatty marrow was increased and the marrow vascularity was dereased with age. Avascular necrosis (AVN) showed a decreased bone marrow signal within an normal appearing femoral head on T1 and T2-weighted images. In addition, we could see inhomogenous low signal intensity (31 cases), a ring of low intensity with central normal signal intensity (25cases), focal low signal intensity (12 cases), or a band of low signal intensity (4 cases). MRI findings were abnormal in 10 cases with normal radiographic findings as well as in all the cases with abnormal ones. In conclusion, MRI should be the choice of the imaging modality for the evaluation of early bone marrow chages of AVN.
Adult ; Bone Marrow ; Head* ; Humans ; Magnetic Resonance Imaging* ; Necrosis*

The study was conducted to assess school dietitians' preferences for traditional foods, to determine the association with their perception and usage status, and to develop the strategies to increase its utilization of traditional foods in school lunches. The information was obtained by the self-administered questionnaire from the subjects, which were 198 dietitians in schools located in Seoul and Gyeonggi-do. According to the preference for traditional foods, subjects were distributed into three groups (High = 54, Medium = 128, Low = 16). The significances of differences between groups were determined using SPSS 12.0 at p < 0.05. Compared to the dietitians with lower (medium and low) preferences, those with high preferences answered that they had better knowledge (p < 0.001) for traditional foods, and served them more in school lunches (p < 0.01). Also, more dieticians in the high preference group not only felt that they need to train school administrators (p < 0.01) as well as themselves (p < 0.05), but also were currently providing students with the nutrition education on traditional foods (p < 0.05). The results presented the evidence suggesting that having dietitians with higher preference for traditional foods can offer an effective strategy to increase the opportunities for schoolchildren to be exposed to traditional foods. Development of the standard recipes for use in school lunches by the government or the association was answered as the best strategy to increase the use of traditional foods, regardless of the dietitians' preferences.
Administrative Personnel ; Food Preferences ; Humans ; Lunch ; Surveys and Questionnaires

CT and MR appearance of the brain in three children with ornithine transcarbamylase (OTC) deficiency are described. They showed clinical signs of vomiting and convulsion and were diagnosed by measurement of plasma ammonium, amino acids, acid-base balance, and urinary orotic acid levels. CT and MR were performed within one month from the onset of the symptom. CT and MRI demonstrated brain swelling with small ventricles and diffuse low density of white matter, which indicated cerebral hypoperfusion secondary to elevated intracranial pressure. With more prolonged survival hyperammonemia may cause cerebral atrophy. CT and MR appearance in these cases resembled a hypoxic brain damage and this finding should be included in the differential diagnosis.
Acid-Base Equilibrium ; Amino Acids ; Ammonium Compounds ; Atrophy ; Brain ; Brain Edema ; Child ; Diagnosis, Differential ; Humans ; Hyperammonemia* ; Hypoxia, Brain ; Intracranial Hypertension ; Magnetic Resonance Imaging* ; Ornithine Carbamoyltransferase Deficiency Disease* ; Ornithine Carbamoyltransferase* ; Ornithine* ; Orotic Acid ; Plasma ; Seizures ; Vomiting

Colorectal cancer is the third most common malignancy in Korea. In contrast, pericolic or mesenteric lymphoma is relatively rare. We experienced an extremely rare case of synchronous primary colon cancer in the ascending colon with T-cell lymphoma in the pericolic lymph node. A 79-year-old woman presented with complaints of epigastric and right lower abdominal pain combined with anorexia and nausea. Colonoscopic evaluation and biopsy were performed, and the diagnosis was cecal adenocarcinoma. She underwent right hemicolectomy with lymph node dissection. The pathology report revealed adenocarcinoma in cecum with metastasis to 1 regional lymph node out of 37 lymph nodes. In addition, there was malignant angioimmunoblastic T-cell lymphoma in 1 pericolic lymph node. There was no evidence of lymphoma in ileum, cecum and ascending colon, so the possibility of early phase of lymphoma was suggested.
Abdominal Pain ; Adenocarcinoma ; Aged ; Anorexia ; Biopsy ; Cecum ; Colon ; Colon, Ascending ; Colonic Neoplasms ; Colorectal Neoplasms ; Female ; Humans ; Ileum ; Korea ; Lymph Node Excision ; Lymph Nodes ; Lymphoma ; Lymphoma, T-Cell ; Nausea ; Neoplasm Metastasis ; T-Lymphocytes

PURPOSE: The purpose of this study was to evaluate the HRCT findings of bronchial asthma during maintenance bronchodilator therapy and to determine whether there were irreversible bronchial changes occurred in pediatric patients with this condition. MATERIALS AND METHODS: HRCT findings of the lung in 21 asthmatic children [14 boys and 7 girls aged between 3.5 and 13.8 (mean : 7.7) years] who were receiving maintenance bronchodilator therapy were retrospectively studied. At the time of CT examination, 16 were receiving nonsteroid bronchodilator therapy only, and five were receiving both bronchodilator and steroid therapy. Thirteen patients were defined as allergic and eight were nonallergic. The clinical severity of chronic asthma was graded as severe in seven cases, and moderate in 14. The duration of the disease ranged from 4 months to 6 years (mean 3.2 years). HRCT was performed in 19 cases for evalvation of the atelectasis, hyperinflation, and prominent bronchovascular bundles seen on plain radiographs, and in two cases for evaluation following acute exacerbation. A CT W-2000 scanner (Hitachi Medical Co. Tokyo, Japan) was used during the end inspiratory phase, and in addition, ten patients were scanned during the expiratory phase. Scans were reviewed for evidence of bronchial thickening, bronchiectasis, emphysema, abnormal density, mucus plugs, and other morphological abnormalities. The presence of bronchial wall thickening or air trapping was evaluated according to the duration, severity and type of asthma. RESULTS: Among the 21 patients, 7(33.3%) had normal HRCT findings, while in 14 (66.7%), bronchial wall thickening was demonstrated. Eleven of the 14 patients with bronchial wall thickening (78.6%) also had air trapping. No patient was suffering from bronchiectasis or emphysema. There were no statistically significant correlations between the presence of bronchial wall thickening or air trapping and the duration of the disease, its severity, or type of asthma. There was, however, a statistically significant correlation between bronchial wall thickening and air trapping (p < . 0 5 ). CONCLUSION: In asthmatic children who were under maintenance therapy, the most frequent HRCT findings were bronchial wall thickening and air trapping, with significant correlation between the presence of these two phenomena. No destructive lesion such as bronchiectasis or emphysema was found in these asthmatic children, however, and this is probably due to the short duration of the disease, and different disease processes.
Asthma ; Bronchiectasis ; Child* ; Emphysema ; Female ; Humans ; Lung ; Mucus ; Pulmonary Atelectasis ; Retrospective Studies

Individual identification procedure is one of the most improtant part in medicolegal fields. Recently, radiolegal investigation methods have been widely applicated to the medicolegal field for the purpose of individual identification. So authors attempted to determine sex and calculate stature by using roentgenographic findings of long bones of 248 subjects the living materials. In orthoscanographic study for long bones, we measured total length, midshaft width, epiphyseal width, cortical width, head diameter of each bones The total length, midshaft width, cortical width, condylar breath, horizontal & vertical head diameter of femur show statistically significant differentiation between two sexes, in tibia, total length, midshaft, cortical width, proximal and distal epiphyseal width show statistically significant. In fibula, Humerus, radius and ulna, total length is only statistically significant. And other wresults are statistically insignificant. Using femoral and fibial lengths (mm) with "Regression Analysis method" in SAS program, we derived the following fomulae. Height (cm)=95.62±0.148×Total length of Femur. (mm) Height(cm)=82.07±0.22×Total length of Tibia. (mm). In conclusion, radiologic measurement of long bone might be one of the useful methods in individual identification of unknown subject in Korea.
Femur ; Fibula ; Head ; Humerus ; Korea ; Radius ; Tibia ; Ulna

Multiple large bowel polyps are the hallmark of familial adenomatous polyposis (FAP), and many progress to colorectal cancer. Desmoid tumors are more common in patients with FAP than in other people, occurring, particularly, in those who have previously undergone prophylatic total colectomy. In such patients, desmoid tumors are a common cause of death. In an FAP patient without extracolic manifestation, who has undergone prophylatic surgery, multifocal desmoid tumors occur periodically. We report the serial radiologic findings of progressive desmoid tumors in FAP, drawing attention to the related findings of previous research.
Adenomatous Polyposis Coli ; Cause of Death ; Colectomy ; Colorectal Neoplasms ; Fibromatosis, Aggressive* ; Humans ; Polyps

The cause responsible for the lack of an efficient cell-mediated immunity or a delayed type hypersensitivity to M. leprae in lepromatous patients is poorly understood. But the resistance to M. leprae infection in humans is likely mediated by the activated macrophages to present M. leprae antigen to T cells for cell-mediated immunity. Phenolic glycolipid-I (PGL-I) is a M. leprae-specific antigen and is supposed to play a significant role in the long lasting unresponsiveness in lepromatous leprosy. In this study, IL-1 activities were tested among leprosy patients to evaluate monocyte function and the role of IL-1 in the immunosuppression in leprosy. We found that peripheral blood mononuclear cells (PBMCs) from tuberculoid patients were strongly reactive to M. leprae (mean cpm; 28,853 +/- 28,916), but the proliferative responses of PBMCs from lepromatous patients (mean cpm; 6,051 +/- 803) were significantly lower. IL-1 concentration in culture supernatant of monocytes from lepromatous patients was similar to that from tuberculoid patients with stimulation of M. leprae (lepromatous: 1,014 +/- 637 pg/ml, tuberculoid: 1,012 +/- 167 pg/ml) or lipopolysaccharides (IPS) (lepromatous: 3,479 +/- 2,188 pg/ml, tuberculoid: 4,246 +/- 2,432 pg/ml). The IL-1 concentration is sera from lepromatous patients (42 +/- 30 pg/ml) tended to be higher than those from tuberculoid patients (28 +/- 69 pg/ml). And there was no significant difference in IL-1 production between peritoneal macrophages from mice sensitized with PGL-1 and those from nonsensitized mice. In conclusion, this study suggests that the immunosuppression in lepromatous patients may not be due to the decreased production of IL-1. And the increased IL-1 activity in sera may affect the inflammatory response of lepromatous patients.
Glycolipids/pharmacology ; Human ; Immunity, Cellular ; Interleukin-1/*biosynthesis ; Leprosy, Lepromatous/blood/*metabolism ; Lymphocyte Activation ; Monocytes/*metabolism ; Mycobacterium leprae/metabolism ; Support, Non-U.S. Gov't

PURPOSE: To evaluate the radioloic findings of malignant retroperitoneal fibrosis. MATERIALS AND METHODS: Post-contrast CT (n=9) and urographic (n=7) findings of nine patients with malignant retroperitoneal fibrosis were retrospectively analyzed. Primary tumors were found to be advanced gastric cancer (n=6), early gastric cancer (n=1), breast cancer (n=1), and cervical cancer (n=1). We analyzed CT findings with regard to the site of soft tissue lesion, ureteral involvement, the presence or absence of hydronephrosis, and distant metastasis. The level and length of ureteral involvement, presence or abscence of ureteral stenosis, and ureteral displacement as seen on urography, were analyzed. RESULTS: On CT scans, enhanced soft tissue lesions (mass, 5 cases; plaque, 4 cases) encircling the abdominal aorta and IVC were noted in all cases. Thickening of the ureteral wall (n=8), hydronephrosis (n=9), and enlarged lymph node (n=5) were also seen. On urography, irregular stenosis and medial displacement of ureters from level L2 to S2 were noted in all cases. The length of ureteral involvement was 4-6.5cm. CONCLUSION: The common CT findings of malignant retroperitoneal fibrosis were enhanced soft tissue lesion encircling the abdominal aorta and IVC, hydronephrosis, and thickening of the ureteral wall. On urography, ureteral stenosis and medial displacement were seen.
Aorta, Abdominal ; Breast Neoplasms ; Constriction, Pathologic ; Humans ; Hydronephrosis ; Lymph Nodes ; Neoplasm Metastasis ; Retroperitoneal Fibrosis* ; Retrospective Studies ; Stomach Neoplasms ; Tomography, X-Ray Computed ; Ureter ; Urography ; Uterine Cervical Neoplasms