• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.19 no.1
• /
• pp.76-81
• /
• 2016

Carnitine palmitoyltransferase 1A (CPT1A) is an enzyme functioning in mitochondrial fatty acid oxidation (FAO) of the liver. Patients with CPT1A deficiency have impaired mitochondrial FAO and display hypoketotic hypoglycemia and hepatic encephalopathy as typical manifestations. In this report, we present a case of CPT1A deficiency presenting jaundice as the first manifestation. A 1.9 years old boy showed jaundice and elevated levels of free and total carnitine were observed. From direct sequencing analysis of CPT1A, two novel mutations, c.1163+1G>A and c.1393G>A (p.Gly465Arg), were identified. At the age of 2.2 years, hypoglycemia, tachycardia, and altered mental status developed just after cranioplasty for craniosynostosis. High glucose infusion rate was required for recovery of his vital signs and mentality. Diet rich in high carbohydrate, low fat and inclusion of medium chain triglyceride oil resulted in improvement in cholestatic hepatitis and since then the boy has shown normal growth velocity and developmental milestones to date.

• Journal of the Korean Society of Fisheries and Ocean Technology
• /
• v.57 no.3
• /
• pp.214-227
• /
• 2021

The distribution of tidal current and tidal induced residual current, topographical eddies and tidal residual circulation in the waters surrounding the Geumo Island-An Island channel were identified through numerical model experiments and vorticity balance analysis. Tidal current flows southwest at flood and northeast at ebb along the channel. The maximum flow velocity was about 100-150 cm/s in neap and spring tide. During the flood current in the neap tide, clockwise small eddies were formed in the waters west of Sobu Island and southwest of Daebu Island, and a more grown eddy was formed in the southern waters of Geumo Island in the spring tide. A small eddy that existed in the western waters of Chosam Island during the ebb in neap tide appeared to be a more grown topographical eddy in the northeastern waters of Chosam Island in spring tide. Tidal ellipses were generally reciprocating and were almost straight in the channel. These topographical eddies are made of vorticity caused by coastal friction when tidal flow passes through the channel. They gradually grow in size as they are transported and accumulated at the end of the channel. When the current becomes stronger, the topographic eddies move, settle, spread to the outer sea and grow as a counterclockwise or clockwise tidal residual circulation depending on the surrounding terrain. In the waters surrounding the channel, there were counterclockwise small tidal residual circulations in the central part of the channel, clockwise from the northeast end of the channel to northwest inner bay of An Island, and clockwise and counterclockwise between Daebu Island and An Island. The circulation flow rate was up to 20-30 cm/s. In the future, it is necessary to conduct an experimental study to understand the growth process of the tidal residual circulation in more detail due to the convergence and divergence of seawater around the channel.

• Nuclear Engineering and Technology
• /
• v.50 no.7
• /
• pp.1138-1147
• /
• 2018

Delayed hydride cracking (DHC) is an important failure mechanism for Zircaloy tubes in the demanding environment of nuclear reactors. The threshold stress intensity factor, $K_{IH}$, and critical hydride length, $l_C$, are important parameters to evaluate DHC. Theoretical models of them are developed for Zircaloy tubes undergoing non-homogenous temperature loading, with new stress distributions ahead of the crack tip and thermal stresses involved. A new stress distribution in the plastic zone ahead of the crack tip is proposed according to the fracture mechanics theory of second-order estimate of plastic zone size. The developed models with fewer fitting parameters are validated with the experimental results for $K_{IH}$ and $l_C$. The research results for radial cracking cases indicate that a better agreement for $K_{IH}$ can be achieved; the negative axial thermal stresses can lessen $K_{IH}$ and enlarge the critical hydride length, so its effect should be considered in the safety evaluation and constraint design for fuel rods; the critical hydride length $l_C$ changes slightly in a certain range of stress intensity factors, which interprets the phenomenon that the DHC velocity varies slowly in the steady crack growth stage. Besides, the sensitivity analysis of model parameters demonstrates that an increase in yield strength of zircaloy will result in a decrease in the critical hydride length $l_C$, and $K_{IH}$ will firstly decrease and then have a trend to increase with the yield strength of Zircaloy; higher fracture strength of hydrided zircaloy will lead to very high values of threshold stress intensity factor and critical hydride length at higher temperatures, which might be the main mechanism of crack arrest for some Zircaloy materials.

• Clinical and Experimental Pediatrics
• /
• v.49 no.3
• /
• pp.305-311
• /
• 2006

Purpose : GnRH analogues(GnRHa) are used to treat central precocious puberty(CPP). However, in some patients, the GV decrease is so remarkable that it impairs predicted adult height(PAH); and there fore, the addition of growth hormone(GH) is suggested. We analysed the growth changes during two years and final adult height(FAH) in girls with idiopathic CPP treated with combined therapy, compared with those of girls treated with GnRHa alone. Methods : For the analysis, we classified the patients, who was treated for longer than two years, into three groups depending on the initial PAH and combination of GH; PAH_L, treated with GnRHa and PAH less than midparental height(MPH) - 5 cm. PAH_H, treated with GnRHa and PAH greater than MPH - 5 cm. GnRHa＋GH, combined GH treatment, regardless of PAH before treatment. We analysed the GV and PAH change during the first two years and FAH. Results : In PAH_L, the PAH(SDS) at first year of therapy was significantly increased to $153.5{\pm}6.5cm(-1.4{\pm}1.3)$ from $149.7{\pm}6.4cm(-2.1{\pm}1.3)$ before treatment(P=0.004). In PAH_H, there was no significant increase in PAH during the two years of treatment. During the first year of combination of GH and GnRHa, GV and PAH increased significantly. We observed significant increases in FAH, comparing to the initial PAH in the PAH_L and GnRHa＋GH groups. The height gains(FAH - initial PAH) were significantly higher in the PAH_L and GnRHa＋GH groups than that in the PAH_H group. Conclusion : This study suggests the FAH and height gains are improved in patients, whose predicted adult height before treatment was shorter than those with higher predicted adult height, with the treatment of GnRHa alone or in combination with GH. GH could not improve the final adult height, but compensated the growth in patients whose growth velocity was decelerated by GnRHa alone.

• Journal of the Korean Institute of Landscape Architecture
• /
• v.47 no.5
• /
• pp.66-77
• /
• 2019

This study investigated the feasibility of wintering evergreen broad-leaf trees in the Incheon coastal area through a climate analysis. The coldest monthly mean air temperature ranged from $-2.9^{\circ}C{\sim}-1.6^{\circ}C$. The warmth index of the coastal area of Incheon ranged from $98.89^{\circ}C{\cdot}month-109.03^{\circ}C{\cdot}month$, while the minimum air temperature year ranged from $-13.9^{\circ}C{\sim}-3.6^{\circ}C$. This proved that the Incheon coastal area was not suitable for evergreen broad-leaf trees to grow as the warmth index ranges from $101.0^{\circ}C{\cdot}month{\sim}117.0^{\circ}C{\cdot}month$, and the temperature year-round is $-9.2^{\circ}C$ or higher. This suggests the coastal areas of Incheon is not suitable for the growth of evergreen broad-leaf trees, however some evergreen broad-leaf trees lived in some parts of the area. Wind speed reduction and temperature effect simulations were done using Landschaftsanalyse mit GIS program. As a result of the simulations of wind speed reduction and temperature effects affecting the evergreen broad-leaf trees, it was discovered that a coastal wind velocity of 8.6m/sec was alleviated to be 5m/sec~7m/sec when the wind reached the areas where evergreen broad-leaf trees were present. It was also discovered that species that grew in contact with buildings benefited from a temperature increase of $1.1^{\circ}C{\sim}3.4^{\circ}C$ due to the radiant heat released by the building. Simulation results show that the weather factors affecting the winter growth damages of evergreen broad-leaved trees were wind speed reduction and local warming due to buildings. The wind speed reduction by shielding and local warming effects by buildings have enabled the wintering of evergreen broad-leaved trees. Also, evergreen broad-leaved trees growing in the coastal area of Incheon could be judged to be gradually adapting to low temperatures in winter. This study reached the conclusion that the blockage of wind, and the proximity of buildings, are required for successfully wintering evergreen broad-leaf trees in the coastal area of Incheon.

• Journal of the Korean Crystal Growth and Crystal Technology
• /
• v.31 no.1
• /
• pp.8-15
• /
• 2021

TiO2 has been used in various fields such as solar cells, dental implants, and photocatalysis, because it has high physical and chemical stability and is harmless to the body. TiO2 nanofibers which have a large specific surface area also show a good reactivity in bio-friendly products and excellent photocatalysis in air and water purification. To fabricate TiO2 nanofibers, an electrospinning method was used. To observe the diameter of TiO2 nanofibers with fabrication variables, the fabrication variables was divided into precursor composition variables and process variables and microstructure was analyzed. The concentrations of PVP (Polyvinylpyrrolidone) and TTIP (Titanium(IV) isopropoxide) were selected as precursor composition variables, and inflow velocity and voltage were also selected as process variables. Microstructure and crystal structure of TiO2 nanofibers were analyzed using FE-SEM (Field emission scanning electron microscope) and XRD (X-ray diffraction), respectively. As-spun TiO2 nanofibers with an average diameter of about 0.27 ㎛ to 1.31 ㎛ were transformed to anatase TiO2 nanofibers with an average diameter of about 0.22 ㎛ to 0.78 ㎛ after heat treatment of 3 hours at 450℃. Anatase TiO2 nanofibers with an average diameter of 0.22 ㎛ can be expected to improve the photocatalytic properties by increasing the specific surface area. To change the average diameter of TiO2 nanofibers, the control of precursor composition variables such as concentrations of PVP and TTIP is more efficient than the control of electrospinning process variables such as inflow velocity and voltage.

• Clinical and Experimental Pediatrics
• /
• v.51 no.3
• /
• pp.315-322
• /
• 2008

Purpose : Prader-Willi syndrome (PWS) is a complex genetic disorder, caused by the deletion of the paternally derived 15q11-13 region or the maternal uniparental disomy of chromosome 15 (mUPD(15)). In this study, we compared phenotypic differences between those patients whose disease was caused by microdeletion and those caused by mUPD(15). In addition, a comparison of the efficacy of growth hormone (GH) therapy between these two PWS genotypes was analyzed. Methods : Fifty-three patients were diagnosed as having PWS based on molecular and cytogenetic analyses and clinical features. Data that included maternal age, birth weight, a feeding problem in the neonatal period, cryptorchidism, developmental delay or mental retardation, short stature, hypopigmentation, changes in height, weight, and body mass indexes (BMI) before and after GH treatment were obtained by a retrospective review of medical records. The data from the patients with microdeletion were compared with those from the patients with mUPD(15). Results : Of the 53 patients with genetically confirmed PWS, 39 cases had microdeletion and 14 mUPD(15). Maternal ages were significantly higher in the mUPD(15) group, and hypopigmentation and a feeding problem in the neonatal period were more frequent in the microdeletion group. Growth hormone was administered to 20 patients [14 with microdeletion, 6 with mUPD(15)]. There were no differences between the two groups in height velocity, weight and height SDS, and BMI after GH therapy. Conclusion : Phenotype and genotype correlations were observed in Korean PWS patients, such as more advanced maternal ages in the mUPD(15) group and more feeding problems and hypopigmentations in the microdeletion group. Further long-term prospective studies are needed to correlate other aspects of the phenotypes.

• Journal of Chest Surgery
• /
• v.34 no.10
• /
• pp.754-762
• /
• 2001

Background: To evaluate the efficacy of arterial switch operation for transposition of great arteries, serial echocardiographic studies were performed in 8 patients who underwent the surgery between 1989 and 1998 at Dept. of Thoracic & Cardiovascular Surgery, Yeungnam University Hospital in Daegu City, Korea. Material and Method: Follow-up period ranged from 6 months to 11 years(average of 4.1 years). Body weight ranged from 2.6kg to 4.8kg, with average of 3.6kg. 5 of 8 patients were preoperatively diagnosed as TGA+VSD, and 3 as TGA+IVS. LV function was evaluated by the measurement of LV shortening fraction, LVSTI, and LVEF. RVSTI was also measured. Postoperative function of valve and growth of great vessels were analyzed by the measurement of PSPGV, valvular regurgitation, LA/AO ratio, root dimension of aorta and pulmonary artery, comparing with the age matched controls, respectively. Result: LVEF had an average of 65.0+9.03% which is tended to increase serially. LVAOPG had an average of 15.9mmHg. RVPAPG, 27.5mmHg. From the measurement of aortic root dimension of 6 patients at end-systole, aortic root growth was assumed to increase more than the mean value of normal growth. PA root dimension at end-systole showed a similar growth progress when compared with age matched normal controls. Postoperative pulmonic valve regurgitation was noted in 5 of total 8 patients, in which 1 patient who showed grade 2 and 4 showed below grade 1. AR, in 6 patients and all grade 1 Except 1 patient, all the valvular regurgitations were below grade 1, which was presumed to be clinically insignificant.

• Journal of agriculture & life science
• /
• v.46 no.6
• /
• pp.25-31
• /
• 2012

This study aimed to investigate the effect of night temperature on the productivity, growth, and fruit characteristics of tomatoes and set the optimal night air temperature. Tomatoes (Solanum lycopersicum Mill. cv. Superdoterang and cv. Rapito) were grown at 5, 10, 15, and $20^{\circ}C$ during night time and ventilation temperature point during day time set $27^{\circ}C$. Depending on night air temperature, plant height, leaf area, fresh and dry weight of each organ, leaf, stem, and root were changed. Condition of high night temperature resulted in promotion of tomatoes growth with high dry weight and fresh weight. However, the dry weight under night temperature $20^{\circ}C$ decreased, especially, in 'Superdoterang'. On changes of yield, 'Rapito' showed higher productivity than 'Superdoterang' and the highest productivity point for nigh temperature is $13.32^{\circ}C$ for 'Superdoterang' and $14.25^{\circ}C$ for 'Rapito'. Although the total yield decreased from the point, the daily productivity increased with increasing night temperature, the highest point for nigh temperature is $14.45^{\circ}C$ for 'Superdoterang' and $16.46^{\circ}C$ for 'Rapito'. High temperature deceased the fruit weight and increased total soluble solid content in fruits. It is concluded that the night air temperature changes productivity of tomato and the temperatures between productivity and growth velocity have differences depending on different cultivars. scores than the other beef jerky samples (p<0.05).

• Clinical and Experimental Pediatrics
• /
• v.48 no.2
• /
• pp.191-196
• /
• 2005

Purpose : Short stature is one of the characteristic features of Turner syndrome. We investigated the factors affecting final adult height(FAH) in patients with Turner syndrome. Methods : The study group was comprised of 60 patients who were diagnosed with Turner syndrome by chromosomal study and clinical phenotypes and attained FAH. Data were obtained from retrospective review of the medical records. We analyzed the factors influencing FAH in growth hormone(GH) treated and GH untreated groups. Results : Sixty patients were enrolled; 48 patients received GH treatment, and 12 patients did not. Mean duration of GH treatment was 35.8 months(range 4 to 120 months), and mean dosage of GH was $0.8{\pm}0.2IU/kg/wk$ in GH treated group. Mean growth velocity was $5.6{\pm}2.0cm/yr$, which was significantly higher than that during pretreatment period. In the GH treated group, mean chronological age, bone age, mean height, and height standard deviation(SD) score at GH treatment were $12.2{\pm}2.7yr$ $10.3{\pm}2.5yr$ $127.5{\pm}10.1cm$ and $-3.1{\pm}1.1$, respectively. In the GH treated group, the mean FAH and SD score of FAH were $146.9{\pm}5.8cm$ and $-2.7{\pm}1.2$, respectively, which showed significant differences compared with those of the GH untreated group. Analyzing the factors affecting FAH in GH-treated patients, only the SD score of height at the time of treatment was significantly related to FAH. Conclusion : GH treatment leads to an increment in FAH in patients with Turner syndrome. Average FAH gain was as much as 5.8 cm. SD score of height at the time of GH treatment was the only factor influencing FAH.