Lipid storage myopathy （LSM） is a lipid metabolic disorder characterized by excessive lipid droplet accumulation in muscle fibers. Classic multiple Acyl-CoA dehydrogenase deficiency （MADD）， also known as glutaric aciduria type Ⅱ， is a disease with various clinical manifestations caused by mutations in electron transfer flavoprotein （ETF） and ETF-ubiquinone oxidoreductase. In recent years， a large amount of evidence has shown that classic late-onset MADD caused by mutations in the electron transfer flavoprotein dehydrogenase gene is the main cause of LSM. Besides classic MADD， many other diseases with similar changes in blood acylcarnitines and urinary organic acids can also cause LSM， and such diseases are call MADD-like disorders or MADD spectrum. This article reviews the clinical， pathological， biochemical， and molecular features of LSM with various etiologies and the latest advances in treatment， with a focus on the latest findings associated with MADD spectrum.
Riboflavin

Preliminary application of fluorescerin spectrum to analyze riboflavine in Aphavita filmed tablet. Aphavita was a combination of vitamins B1, B6, B5, PP and adjuvants. Basing on the fluorescein property of ribofleavine, which depends on pH of tested media, the product was quantified. The qualitification showed the line criterion, the repeatation and the accuracy
Riboflavin ; Pharmaceutical Preparations ; Tablets

No abstract available.
Humans ; Infant, Newborn* ; Phototherapy* ; Riboflavin*

Cornea ; Epithelium, Corneal ; Iontophoresis ; Riboflavin

This study was carried out to evaluate nutritional quality of the dosirak severed by 3 Dosirak manufacturing establishment in Kangnung. The subjects of this study came from 3 different male high schools in Kangnung. The portion amounts of Dosirak were weighed and the food intake was measured by substracting the leftover from the averaged portion amount. The leftover was measured by a modified aggregate selection plate waste measurement technique. The nutrient intakes were analyzed by a computer aided nutritional analysis program for professionals(CAN pro). The menu of the dosirak was too simple. The total amount of Dosirak was 714g and the percent of plate waste was 714g and the percent of plate waste was 7.2%. The male high school students were taking adequate energy, but the nutrients which did not meet 75% of the 1/3RDA(Recommended Dietary Allowances) were Ca, Fe, and vitamin B2.
Adolescent* ; Eating ; Humans ; Male* ; Nutritive Value* ; Riboflavin

BACKGROUND/OBJECTIVES: Physical exercise promotes energy producing pathways requiring thiamin and riboflavin as a coenzyme. Therefore, this study investigated the effects of regular exercise training on urinary excretion of thiamin and riboflavin. MATERIALS/METHODS: Fifty rats were randomly assigned to one of two groups: non-exercise training (NT, n = 25) and regular exercise training (ET, n = 25) for 5 weeks. The rats performed moderate exercise on a treadmill (0.5-0.8 km/hour) for 30 min/day, 5 days/week. Twenty-four hour urine samples were collected at the end of the 0 week, 3rd week, and 5th week of training and thiamin and riboflavin were analyzed. RESULTS: No significant differences in thiamin and riboflavin intakes for each week were observed between the NT and ET groups. Urinary thiamin excretion of each group was the highest at the 5th week compared to the levels at 0 and 3rd week. Urinary thiamin at the 5th week was significantly lower in the ET group than in the NT group. Urinary riboflavin excretion was increased by training duration, however, no difference was observed between NT and ET for each week. At 0 and 3rd week, no significant relationships were observed between dietary intake and urinary excretion of thiamin and riboflavin, however, at the 5th week, urinary excretion was significantly increased by dietary intake only in the NT group (P < 0.05). Thiamin excretion of both NT and ET groups was significantly increased with riboflavin excretion at the 5th week (P < 0.01). CONCLUSION: Regular moderate exercise training increased urinary excretion of thiamin. Dietary intakes and urinary excretions of thiamin and riboflavin showed positive correlation in both the exercise training and non-exercise training groups as the exercise training period went by, while the correlations in the exercise training group were weaker than those in the non-exercise training group. Therefore, regular exercise training can alter the urinary excretion of thiamin and riboflavin in rats.
Animals ; Exercise ; Rats ; Riboflavin* ; Vitamin B Complex

this study was undertaken to compare the riboflavin status of rural women with different physical activity intensity and to determine factors influencing biochemical fiboflavin status. The study was carried out over three different farming seasons : planting (June), harvest(October) and interim(February) in two rural regions of Korea. One was a traditional farming region, the other a commercial farming region with heavier work intensity. Twenty women in the traditional region and eighteen women in the commercial region were involved. The intensity of physical activity was determined by a daily activity record. Body composition was assessed by bioelectrical impedence. Dietary riboflavin intake was measured by the food frequency method. Riboflavin biochemical status was assessed by erythrocyte glutathione reductase activity coefficient (EGR AC) and ruinary riboflavin excretion. The results from the EGR AC and urinary riboflavin excretion during the period showed the overall riboflavin status of the commercial farming women was significantly worse than that of the traditional farming women(EGR AC p<0.0001, urinary riboflavin excretion p<0.05). The traditional farming group had about 40% with risk of riboflavin deficiency, whereas the commercial farming group had about 70%. Overall mean nutrient intake was not significantly different between the two groups, however, overall mean percent lean body mass representing long term physical activity was significantly higher in the commercial farming group (<0.005). It appears that the biochemical riboflavin status of traditional farming women was significantly influenced by riboflavin intake and crude nitrogen balance while the biochemical riboflavin status of the commercial farming women was significantly influenced by riboflavin intake and percent of lean body mass over the three seasons.
Body Composition ; Erythrocytes ; Female ; Glutathione Reductase ; Humans ; Korea* ; Motor Activity ; Nitrogen ; Plants ; Riboflavin Deficiency ; Riboflavin* ; Seasons

The purpose of this study was to investigate the effect of frying methods of chickens ; deep-fat frying with raw chickens(A), with pre-cooked chickens(B) on the physicochemical properties of frying oil and fried chickens in the school foodservice. Acid, peroxide and TBA value of frying oil were significantly increased by increasing the number of frying times and those of method A were higher than those of method B. On the contrary, Iodine value was decreased significantly with increasing the number of frying times but there is no significant difference between method A and B. In fatty acids, the content of saturated fatty acid increased while that of unsaturated fatty acid decreased as the frying times increased. Also viscosity, yellowness and redness increased significantly by increasing the number of frying times. The content of vitamin B, of fried chicken by the frying methods was not significantly different, but vitamin B2 by the method A was higher than by the method B. Ca, P, Na, K and Fe contents of fried chickens by the method A were higher than those by the method B.
Chickens* ; Fatty Acids ; Humans ; Iodine ; Riboflavin ; Viscosity ; Vitamins

For the purpose of estimating the maximum absorption of riboflavin and thiamine from the intestine, large test doses of riboflavin and thiamine were administered orally to apparently normal male subjects, receiving normal Korean diets, and their maximum absorption were estimated by determining the excretion in urine. The maximum absorption of riboflavin and thiamine were estimated 10.700 and 3.800 mg. respectively.
Human ; Intestinal Absorption* ; Male ; Riboflavin/metabolism* ; Thiamine/metabolism*

The aim to this study was to investigated the effects of family type on the health-related behaviors, food behaviors, and nutrient adequacy ratio of the elderly. Studies were performed on 109 home-bound elderly in a rural area of Asan city, in 1996. Subjects were divided into two groups by their family type, one was single-elderly family(n=58) and the other was extended family(n=51). The results obtained by questionaires and personal interviews as follows. 1) The average age 68.6. They served in primary industry, and 89.1% of responders received less than a primary school education. There was no significant difference by family type. 2) Single-elderly family members themselves felt more negative about their health than extended family members. 3)Each nutrient adequacy ratio of single-elderly family/extended family members was 0.72/0.76 of energy, 0.73/0.76 of protein, 0.59/0.66 of Ca, 0.98/0.99 of Fe, 0.62/0.74 of vitamin A, 0.86/0.87 of thiamin, 0.72/0.73 of riboflavin, 0.71/0.77 of niacin, 0.90/0.91 of ascorbic acid, and 0.76/0.80 of Mar. The NAR of vitamin A of the single-elderly family members was significantly lower than for extended family members(p<0.05). Energy, protein, Ca, vitamin A, riboflavin showed insufficient intake for both groups. The percentage of INQ<1 of the single-elderly family/extended family members was 45.6/51.0 of protein, 66.7/66.7 of Ca, 64.9/56.9 of vitamin A. By NAR and INQ, the most insufficient nutrient to the elderly in this rural area was Ca. We there for suggest that it is needed for elderly in rural areas to receive of food that is higher ING of Ca.
Aged* ; Ascorbic Acid ; Chungcheongnam-do ; Education ; Humans ; Niacin ; Riboflavin ; Vitamin A