Patients with lysosomal acid lipase (LAL) deficiency and glycogen storage disease (GSD) demonstrated hepatomegaly and dyslipidemia. In our case, a 6-year-old boy presented with hepatosplenomegaly. At 3 years of age, GSD had been diagnosed by liver biopsy at another hospital. He showed elevated serum liver enzymes and dyslipidemia. Liver biopsy revealed diffuse microvesicular fatty changes in hepatocytes, septal fibrosis and foamy macrophages. Ultrastructural examination demonstrated numerous lysosomes that contained lipid material and intracytoplasmic cholesterol clefts. A dried blood spot test revealed markedly decreased activity of LAL. LIPA gene sequencing identified the presence of a novel homozygous mutation (p.Thr177Ile). The patient's elevated liver enzymes and dyslipidemia improved with enzyme replacement therapy. This is the first report of a Korean child with LAL deficiency, and our findings suggest that this condition should be considered in the differential diagnosis of children with hepatosplenomegaly and dyslipidemia.
Park, Sun-Young;Seo, Dae-Young;Suh, Kwang-Sun;Ly, Sun-Yung
Journal of Nutrition and Health
/
v.40
no.2
/
pp.138-146
/
2007
Food irradiation has been steadily increased in many countries concomitantly with increasing international trades. Harmful contaminants naturally occurred from foods which contain high levels of unsaturated fatty acids that are easily oxidized can affect the human anti-oxidation system through the generation of free radicals. Moreover, previous studies proved that ${\gamma}$-irradiation may cause production of free radicals in food. We investigated the effect of ${\gamma}$-irradiated soybeans in relation to oxidative stress in mice. Oxidative index of mice was evaluated by TBARS, DNA fragmentation in various organs such as blood lymphocytes, liver and kidney. Forty male ICR mice were equally divided into 4 groups and fed control diet or ${\gamma}$-irradiated diet containing 50% soybeans (5, 10, and 20 kGy, respectively) for 8 weeks. Peroxide values of the irradiated diets were higher than that of the non-irradiated one and increased according to the storage period. There was no significant difference in weight gain as well as in TBARS value in plasma and kidney of all groups. Liver TBARS value of the group fed with irradiated diet at 20 kGy increased significantly compared with the control group (p < 0.05). DNA oxidative damage as measured by alkaline comet assay showed that % tail DNA in the blood lymphocytes of 5 kGy and 10 kGy groups increased significantly over the control group (p < 0.05). Also, tail moments of 5 kGy and 10 kGy groups were higher than that of the control group. Ultrastructural examination shows myeline figures and swollen mitochondria in parietal and intestinal epithelial cells of the group fed with irradiated diet. Therefore, considering unsaturated fatty acid content, consumption of soybeans ${\gamma}$-irradiated with over 20 kGy or repeatedly may decrease the body's antioxidant mechanism.
This study was performed to determine the iron status of the adolescent Korean girls before and after menarche. The 101 subjects aged 11-13 years who attending in an elementary school in Mokpo were recruited. They were divided into pre-menarche (A) group or post-menarche (B) group based on their menstruation status. The latter subjects were sub-divided into one of the four groups according to the times of their menstruation B-I( $\geq$ 3 times), B-II (4-6 times), B-III (7-9 times) or B-W ( $\geq$ 10 times). In the total subjects, dietary iron intake, 11.3 mg/day, was below the Korean RDA for iron, the percentage of heme iron to total iron intake, 15%, and the bioavailability of dietary iron, 12.3%, seemed to be low. And their body iron storage, 140.8 mg, seemed to be insufficient. However, they tended to meet body's iron requirement in the cell level. Red blood cell number (RBC), hematocrit (Hct), and hemoglobin (Hb) level in the total subjects were 4.5 1012/I, 39.3%, and 13.0 g/㎗, respectively. The subjects in B group had lower (p<0.05) RBC and Hct compared to those in A group and the prevalence of iron-deficiency anemia tended to be high. Serum iron, ferritin, and soluble transferrin receptor (sTfR) and sTfR:ferritin ratio were 86.7 $\mu\textrm{g}$/d, 17.6 $\mu\textrm{g}$/l, 3.58 mg/1, and 230, respectively. Those four indices were not significantly different among the groups. The results of this study imply that, although there a tendency to affect negatively iron status, menstrual blood loss in adolescent females does not deteriorate obviously their iron status during the relatively short period up to 1 you. However, it should be better to improve their iron status after starting menarche by increasing iron intake, especially heme-iron, and enhancing factors for iron absorption.
This research was conducted to investigate the physiological consequences of undernourished yak. Twelve Maiwa yak ($110.3{\pm}5.85kg$) were randomly divided into two groups (baseline and starvation group). The yak of baseline group were slaughtered at day 0, while the other group of yak were kept in shed without feed but allowed free access to water, salt and free movement for 9 days. Blood samples of the starvation group were collected on day 0, 1, 2, 3, 5, 7, 9 and the starved yak were slaughtered after the final blood sample collection. The liver and muscle glycogen of the starvation group decreased (p<0.01), and the lipid content also decreased while the content of moisture and ash increased (p<0.05) both in Longissimus dorsi and liver compared with the baseline group. The plasma insulin and glucose of the starved yak decreased at first and then kept stable but at a relatively lower level during the following days (p<0.01). On the contrary, the non-esterified fatty acids was increased (p<0.01). Beyond our expectation, the ketone bodies of ${\beta}$-hydroxybutyric acid and acetoacetic acid decreased with prolonged starvation (p<0.01). Furthermore, the mRNA expression of lipogenetic enzyme fatty acid synthase and lipoprotein lipase in subcutaneous adipose tissue of starved yak were down-regulated (p<0.01), whereas the mRNA expression of lipolytic enzyme carnitine palmitoyltransferase-1 and hormone sensitive lipase were up-regulated (p<0.01) after 9 days of starvation. The phosphoenolpyruvate carboxykinase and pyruvate carboxylase, responsible for hepatic gluconeogenesis were up-regulated (p<0.01). It was concluded that yak derive energy by gluconeogenesis promotion and fat storage mobilization during starvation but without ketone body accumulation in the plasma.
Nine tons of fresh rice straw from early-maturing varieties was ensiled with 50 g urea $kg^{-1}$ DM straw in plastic bags immediately after threshing. Five months after storage, preserved straw was used to partially replace elephant grass (Pennisetum purpureum, Schumach) for lactating cows. Eight crossbred Holstein lactating cows (75% of Holstein blood) in their second to fourth lactation and in mid-lactation were arranged in a balanced design with two squares consisting of $4\;periods{\times}4\;treatments$ (100% grass ad lib. as a control; 75% grass+urea treated fresh rice straw (UTrFRS) ad lib.; 50% grass+UTrFRS ad lib.; 25% grass+UTrFRS ad libitum) in one square. A concentrate supplement was given at a rate of 400 g per day per kg of milk produced. Samples of fresh straw taken in the field and UTrFRS and elephant grass taken at feeding time were evaluated in a degradation trial with 3 fistulated heifers (undefined blood ratio of crossbred of Sindhi and local yellow cattle). Straw preserved for 5-9 months was in nearly all cases of good quality. Crude protein (CP) content was increased 2.1 fold and 48 h dry matter loss (DML) was 20% higher compared to dry straw. Elephant grass cultivated intensively was low in DM content and 10% higher in 48 h DML compared to UTrFRS. Dry matter intake (DMI) was higher for the mixture of UtrFRS and Elephant grass, and highest when one-third of the roughage was UTrFRS. Higher DMI of mixed roughage diets was probably due to the low DM content of elephant grass in the sole grass roughage diet. Increasing substitution of elephant grass with UTrFRS up to 75% of the roughage component increased milk fat content and had no effect on milk yield and other milk composition parameters. Feeding UTrFRS, partially replacing elephant grass in the diets of lactating cows in the dry season can reduce the cost of roughage.
Journal of the Korea Institute of Information and Communication Engineering
/
v.18
no.12
/
pp.3029-3034
/
2014
The paper describes the scleral diagnostic system implementation for human eyes by using the scleral color code and vessels sign pattern code generations. The system is based on the high performance DSP image signal processor, programmable gain control for preprocessing and RISC SD frames storage. RGB image signals are optimized by PGC, the edge image is detected form the gray image converted. The processing algorithms are executed by scleral color code generation and scleral vessels sign pattern code creation for discriminating and matching. The scleral symptomatic color code is generated by YCbCr values at memory map tolerated and the vessel sign pattern code is created by digitizing the 24 clock and 13 ring zones, overlay matching and tolerances. The experimental results for performance are that the system runs 40ms, and the color and pattern for diagnostic errors are around 20% and 24% on average. The system and technique enable a scleral diagnosis with subdividing the patterns and patient database.
This study was designed to develop a microencapsulated, water-soluble isoflavone for application into milk and to examine the hypocholesterolemic effect of such a milk product in a rat diet. The coating material was medium-chain triglyceride (MCT) and the core material was watersoluble isoflavone. The microencapsulation efficiency was 70.2% when the ratio (w/w) of coating material to core material was 15:1. The isoflavone release from the microcapsules was 8% after 3-day storage at $4^{\circ}C$. In in vitro study, 4.0-9.3% of water-soluble isoflavone in simulated gastric fluid was released in the pH range of 2 to 5 after 60 min incubation; however, in simulated intestinal fluid at pH 8, 87.6% of isoflavone was released from the capsules after 40 min incubation time. In sensory analysis, the scores of bitterness, astringency, and off-taste in the encapsulated isoflavone-added milk were slightly, but not significantly, different from those in uncapsulated, isoflavone-added milk. In blood analysis, total cholesterol was significantly decreased in the isoflavone-added group compared with that in the control after 6-week feeding. Therefore, this study confirmed the acceptability of MCT as a coating material in the microencapsulation of water-soluble isoflavone for application into milk, although a slight adverse effect was found in terms of sensory attributes. In addition, blood total cholesterol was lowered in rats which had been fed a cholesterol-reduced and microencapsulated, isoflavoneadded milk for 6 weeks.
This study was performed to investigate the effects of laying productivity and egg quality according to providing germinated and fermented soybean (GFS) as feed additive. Among the strain, we selected Monascus purpureus KCCM 12002 so that inoculated in soybean and fermented for 48 h at $20^{\circ}C$. A total of two-hundred forty 70-wk-old Hy-Line Brown layers were divided into four groups (4 treatment${\times}$6 replication${\times}$10 birds each) and fed diets containing 0 (as control) (T1), 0.5% (T2), 1.0% (T3) or 2.0% GFS (T4) for 6 wk. The laying productivity, egg quality and blood property in the egg yolk were experimented. There were no significant differences in the laying productivity, relative liver and spleen weights, egg yolk color and eggshell strength among another groups. The eggshell color, eggshell thickness and haugh unit significantly increased in the GFS-supplemented group (p<0.05) compared to control. However, no significant differences were observed in the blood property after supplementation. The amount of lactic acid bacteria present during storage increased by providing of GFS (p<0.05) compare to control group. Our study results suggested that GFS can be used as a favorable feed additive and feedstuff for the productivity of high quality eggs and promoted relative industry.
Objective: This study was conducted to investigate the supplementary effect of a phytogenic blend (SPA: a mixture containing fermented Schisandra chinensis pomace, fermented Pinus densiflora needle extract, and Allium tuberosum powder in the ratio of 2:2:1) on egg production, egg quality, blood constituents, and visceral organs in laying hens. Methods: A total of 135 Hy-line brown laying hens (48-wk-old) were randomly allocated to three dietary treatments with 5 replicates of 9 hens. The control group (CON) was fed a basal diet (no exogenous SPA addition) and the experimental groups were fed the basal diet containing SPA at the level of 0.1% and 0.3% for 6 weeks. Results: The feed intake was significantly improved in SPA supplemented groups as compared with the control (p<0.05). However, egg production, daily egg mass, and feed conversion ratio were not different among the dietary treatments (p>0.05). For egg quality traits, only Haugh unit (HU) was significantly improved in SPA (0.3%) (p<0.05) as compared with other groups. However, HU was not affected during 4-wk of storage at 18℃ among the dietary treatments (p>0.05). Furthermore, SPA supplementation did not affect the blood biochemical constituents except for the phosphate content, which was significantly higher in SPA groups than the CON group (p<0.05). There were no significant differences in visceral organ characteristics and immune indicators (immunoglobulin A [IgA], IgG, and IgM) in SPA or CON groups. Conclusion: This study suggested that the supplementation of SPA may have beneficial effects on feed intake and egg quality in laying hens.
Platelet products are used to treat hemorrhagic or platelet dysfunction diseases. Plateletpheresis involves collecting the platelet components of blood using an apheresis blood-collection system. Various indicators are available for evaluating the qualities of the apheresis platelets. The productivity of platelet collection is evaluated through both the collection efficiency and collection rates. Platelet storage quality can be evaluated in vitro using several indicators, including visual appearance, metabolic activities, volume, platelet count, white blood cell count, microparticles, and various platelet activation markers. Platelet activation markers have been used as indicators of storage quality in various studies. Post-transfusion platelet quality can be evaluated based on the corrected count increment and the percentage of platelet recovery. Although various studies have investigated the aspects of plateletpheresis, no article has systemically presented assessments of the platelet products obtained from different plateletpheresis devices. The present study provides a review of plateletpheresis, including the specifics of the process, the types of devices employed, the platelet quality, the overall efficacy, and the evaluation indicator qualities. Furthermore, the differences in functionality among the different apheresis devices are discussed. Although adverse reactions to the citrate anti-coagulant have been reported, apheresis processing may provide a safer option for donors who are at a high risk for presyncopal or syncopal reactions related to whole blood collection.
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