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

Riboflavin in unstable in light and has a maximum absorption at 450nm. A study was designed to determine whether riboflavin deficiency developed in neonate who received phototherapy with eight day light bulbs for hyperbilirubinemia and to get normal value of activity coefficient of erythrocyte glutathione reductase in neonate. Twenty-seven infants who received phototherapy were investigated. Thirth-eight normal infants without phototherapy served as controls. All the infants of the two groups were fed artificially and then dialy riboflavin intake in each was more than 0.3mg. Riboflavin was determined from the degree of flavin adenine dinucleotide saturation of erythrocyte glutathione reductase, a method shown to reflect riboflavin nutritional status on the neonates. This is expressed as the activity coefficient. The mean activity coefficient of erythrocyte glutathione reductase in control group was 1.15+/-0.16. In phototherapy group, mean activity coefficient of just prior to the initiation and just after cessation of phototherapy were similar with the mean activity coefficient of control group and there was no significant increment of activity coefficient after phototherapy. There was no significant difference between the changes of activity coefficient according to light intensity. And also there was no significnat difference between the changes of activity coefficient according to duration of phototherapy and according to total phototherapeuti cdose.
Absorption ; Erythrocytes ; Flavin-Adenine Dinucleotide ; Glutathione Reductase ; Humans ; Hyperbilirubinemia ; Hyperbilirubinemia, Neonatal* ; Infant ; Infant, Newborn ; Nutritional Status ; Phototherapy* ; Reference Values ; Riboflavin Deficiency ; Riboflavin*

Riboflavin in unstable in light and has a maximum absorption at 450nm. A study was designed to determine whether riboflavin deficiency developed in neonate who received phototherapy with eight day light bulbs for hyperbilirubinemia and to get normal value of activity coefficient of erythrocyte glutathione reductase in neonate. Twenty-seven infants who received phototherapy were investigated. Thirth-eight normal infants without phototherapy served as controls. All the infants of the two groups were fed artificially and then dialy riboflavin intake in each was more than 0.3mg. Riboflavin was determined from the degree of flavin adenine dinucleotide saturation of erythrocyte glutathione reductase, a method shown to reflect riboflavin nutritional status on the neonates. This is expressed as the activity coefficient. The mean activity coefficient of erythrocyte glutathione reductase in control group was 1.15+/-0.16. In phototherapy group, mean activity coefficient of just prior to the initiation and just after cessation of phototherapy were similar with the mean activity coefficient of control group and there was no significant increment of activity coefficient after phototherapy. There was no significant difference between the changes of activity coefficient according to light intensity. And also there was no significnat difference between the changes of activity coefficient according to duration of phototherapy and according to total phototherapeuti cdose.
Absorption ; Erythrocytes ; Flavin-Adenine Dinucleotide ; Glutathione Reductase ; Humans ; Hyperbilirubinemia ; Hyperbilirubinemia, Neonatal* ; Infant ; Infant, Newborn ; Nutritional Status ; Phototherapy* ; Reference Values ; Riboflavin Deficiency ; Riboflavin*

This study was done to evaluate the health and nutritional status of female college students in Seoul. The subjects were 63 healthy college students aged 20 to 29 years. Their body composition, dietary intakes, clinical blood indices were investigated. Their body composition was determined by means of a multifreqency bioelectrical impedance analysis. Their dietary intake was determined using 3-day record method and their nutrient intake was analyzed by Computer Aided Nutritional analysis program for professional (CAN-pro). Their hemoglobin, hematocrit, red blood cell (RBC), white blood cell (WBC) and mean corpuscular volume (MCV) were determined by semi-automated microcell counter (F-520). Their plasma total cholesterol, TG, and HDL-cholesterol levels were measured using test kits. All data were statistically analyzed by SAS PC package program. Their average consumption of calcium, iron vitamin A, vitamin B2 and niacin were 63.3%, 65.0%, 85.2%, 89.2% and 95.2% of RDA, respectively. The overall mean values of the hematological indices in the female college students were within the normal range. However anemic subjects with hemoglobin (< 12 g/dl) and hematocrit (< 36%) accounted for about 20% of the subjects. The mean levels of total cholesterol, HDL-cholesterol and TG were 188.4 mg/dl, 69.9 mg/dl and 67.4 mg/dl, respectively. The percentages of the subjects with plasma total cholesterol level (> 200 mg/dl) and LDL-cholesterol (> 130 mg/dl) were about 41% and 30.4%, respectively. The data showed a significantly positive correlation between either body fat (%) or BMI and TG. However, there was a significantly negative correlation between either body fat (%) or BMI and HDL-cholesterol. These overall results suggest that it is necessary for college women to be educated regarding consuming more iron and vitamin C and less fat, in order to prevent iron deficiency anemia and/or cardiovascular diseases in later life.
Adipose Tissue ; Anemia, Iron-Deficiency ; Ascorbic Acid ; Body Composition* ; Calcium ; Cardiovascular Diseases ; Cholesterol ; Eating* ; Electric Impedance ; Erythrocyte Indices ; Erythrocytes ; Female* ; Hematocrit ; Humans ; Iron ; Leukocytes ; Niacin ; Nutritional Status ; Plasma ; Reference Values ; Riboflavin ; Seoul ; Vitamin A

OBJECTIVEGlutaric aciduria type II, or multiple acyl-CoA dehydrogenase deficiency is an autosomal recessively inherited defect of mitochondrial energy metabolism. The authors report two cases of late-onset glutaric aciduria type II, and evaluate the procedures for the diagnosis and treatment of this rare disease.

METHODSThe clinical and biochemical characteristics of 2 patients with late-onset glutaric aciduria type II were documented. Case 1 presented with lipid storage myopathy for 3 years. Case 2 presented with intermittent episodes of non-ketotic hypoglycemia and muscle weakness for 9 years. The diagnosis of the 2 cases was confirmed with gas chromatography/mass spectrometry analysis of urine samples. Riboflavin supplementation and a low-fat, low-protein, high-carbohydrate diet were initiated as soon as the diagnosis was made.

RESULTSOrganic acid analysis on both untreated cases revealed massive glutaric acid with elevated concentrations of isovalerylglycine, isobutyrylglycine, ethylmalonic acid, adipic acid, suberic acid and other dicarboxylic acids. The clinical manifestations were improved remarkably after the administration of riboflavin and diet control. Consistent improvements of sera enzymes and urine organic acids were observed during the course of treatment.

CONCLUSIONPatients with unexplained myopathy, metabolic acidosis or hypoglycemia should be carefully screened for inherited metabolic disorders. Riboflavin in conjunction with appropriate diet control is an effective therapeutic regime for patients with late-onset glutaric aciduria type II.

Acyl-CoA Dehydrogenase ; deficiency ; Adolescent ; Age of Onset ; Child ; Female ; Gas Chromatography-Mass Spectrometry ; Glutarates ; urine ; Humans ; Metabolism, Inborn Errors ; diagnosis ; drug therapy ; urine ; Photosensitizing Agents ; therapeutic use ; Riboflavin ; therapeutic use ; Treatment Outcome

BACKGROUNDLate-onset multiple acyl-CoA dehydrogenase deficiency (MADD) is the most common type of lipid storage myopathies in China. Most patients with late-onset MADD are well responsive to riboflavin. Up to now, these patients are often treated with glucocorticoids as the first-line drug because they are misdiagnosed as polymyositis without muscle biopsy or gene analysis. Although glucocorticoids seem to improve the fatty acid metabolism of late-onset MADD, the objective evaluation of their rationalization on this disorder and comparison with riboflavin treatment are unknown.

METHODSWe performed a historical cohort study on the efficacy of the two drugs among 45 patients with late-onset MADD, who were divided into glucocorticoids group and riboflavin group. Detailed clinical information of baseline and 1-month follow-up were collected.

RESULTSAfter 1-month treatment, a dramatic improvement of muscle strength was found in riboflavin group (P < 0.05). There was no significant difference in muscle enzymes between the two groups. Significantly, the number of patients with full recovery in glucocorticoids group was less than the number in riboflavin group (P < 0.05). On the other hand, almost half of the patients in riboflavin group still presented high-level muscle enzymes and weak muscle strength after 1-month riboflavin treatment, meaning that 1-month treatment duration maybe insufficient and patients should keep on riboflavin supplement for a longer time.

CONCLUSIONSOur results provide credible evidences that the overall efficacy of riboflavin is superior to glucocorticoids, and a longer duration of riboflavin treatment is necessary for patients with late-onset MADD.

Adolescent ; Adult ; Age of Onset ; Child ; Cohort Studies ; Female ; Glucocorticoids ; therapeutic use ; Humans ; Lipid Metabolism, Inborn Errors ; therapy ; Male ; Multiple Acyl Coenzyme A Dehydrogenase Deficiency ; drug therapy ; Muscular Dystrophies ; therapy ; Riboflavin ; therapeutic use ; Young Adult

OBJECTIVETo identify pathogenic mutation in a boy affected with riboflavin responsive-multiple acyl-CoA dehydrogenase deficiency (RR-MADD).

METHODSThe patient was initially diagnosed as primary carnitine deficiency (PCD) and has been treated with carnitine supplementation for 7 years. Clinical manifestations and characteristics of fibula muscle specimen were analyzed. Potential mutation in electron transfer flavoprotein dehydrogenase (ETFDH) gene (for the patient and his parents) and carnitine transfer protein gene (SLC22A5) (for the patient) was screened.

RESULTSElectronic microscopy of the muscle specimen has suggested lipid storage myopathy. Mutation analysis has found that the patient carried compound heterozygous mutations, c.250G>A and c.380T>C, in exon 3 of the ETFDH gene, whilst his father and mother were heterozygous for the c.380T>C and c.250G>A mutations, respectively. Screening of the SLC22A5 gene has yielded no clinically meaningful result. After the establishment of diagnosis of RR-MADD, the condition of the patient has improved greatly with supplementation of high doses of riboflavin along with continuous carnitine supplement.

CONCLUSIONThe c.250G>A (p.Ala84Thr) mutation of exon 3 of the ETFDH gene has been a hot spot in Southern Chinese population, whilst the c.380T>C (p.Leu127Pro) is rarely reported. Our case has suggested that therapeutic diagnosis cannot substitute genetic testing. The mechanism for having stabilized the patient with only carnitine supplementation for 7 years needs further investigation.

Adolescent ; Adult ; Base Sequence ; Child ; DNA Mutational Analysis ; Electron-Transferring Flavoproteins ; genetics ; metabolism ; Female ; Humans ; Iron-Sulfur Proteins ; genetics ; metabolism ; Male ; Molecular Sequence Data ; Multiple Acyl Coenzyme A Dehydrogenase Deficiency ; enzymology ; genetics ; metabolism ; Muscle, Skeletal ; metabolism ; Organic Cation Transport Proteins ; genetics ; metabolism ; Oxidoreductases Acting on CH-NH Group Donors ; genetics ; metabolism ; Riboflavin ; metabolism ; Solute Carrier Family 22 Member 5