Journal of The Korean Society of Inherited Metabolic disease
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v.12
no.1
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pp.35-41
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2012
Since the causative gene, SLC25A13 which encodes citrin, was discovered in 1999, over 500 cases with citrin deficiency have been identified. Two phenotypes can occur by citrin deficiency, neonatal intrahepatic cholestasis by citrin deficiency (NICCD) and adult-onset type II citrullinemia (CTLN2). Some patients with NICCD develop CTLN2 in their later lives. Although cholestatic jaundice is spontaneously resolved within the first year of life in most cases with NICCD, a few cases experience progressive hepatic failure. In this report, two neonates with severe type of NICCD were described. Both cases exhibited neonatal cholestatic jaundice, hyperammonemia and severe coagulopathy. Of note, plasma citrulline and blood galactose levels were extremely high. Serum ${\alpha}$-fetoprotein, plasma methionine, arginine, and threonine-to-serine ratio were elevated as well. SLC25A13 mutations were found in all the four alleles of both patients. With the commencement of lactose-free formula, coagulopathy and hyperammonemia were resolved, and galactose level was normalized. Currently, no factor has been identified to predict the prognosis of NICCD. More experiences are needed to build up the adequate therapeutic strategies for severe type of NICCD. Our experience, however, indicates that the degree of citrullinemia and galactosemia might reflect the severity.
Proceedings of the Korean Society for Agricultural Machinery Conference
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1996.06c
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pp.977-986
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1996
The uncertainty of rains at the onset of wet season (WS) and the drought risk involved hinder growing more than one rainfed lwoland rice crop per year. Establishing transplanted rice well into the WS leaves insufficient moisture in the soil for subsequent crop. Rice establishment early in the season gives the farmer better opportunities to grow a crop after rice. An experiment was conducted startign in 1993 to evaluate dry seeding of rice through slit soil seeding. It is done utilizing the vertical metering slit seeder for conserving soil moisture coming from the first rains in the early WS to sustain germination and establishment of the seeding at least until the succeeding rains under therainfed lowland (RL) environment. The treatment consisted of slit-seeding the PSBRc 14 into the tilled and nontilled plots at 100kg/ha and at depths of <10 mm (shallow seeding) and 60-70m (deep seeding). The control treatment was broadcast seeded on tilled soil and harrow to cover the seeds The superior crop establishment observed in 1995 WS experiment on nontilled, slit-seeded plots confirmed the results observed in 1993 WS and 1994 WS experiments. Emergence in deep seeding was not significantly different from shallow seeding in nontilled plots giving an average yield of 2.1 t/ha in all slit-seeded plots. This offers an advantage of reduced energy in put in nontilled shallow seeding. However, heavy weed infestation has to be addressed at the early stage of rice in nontilled soil to get the full advantage from slit seeding. The consistently better crop establishment observed in slit seeding over that of broadcast seeding in the WS of 1993, 1994 and 1995 also demonstrates that the slit seeding technology can be adopted with confidence in the rainfed lowland field condition to reduce the risks involved in broadcast seeding.
Aseptic meningitis, the most common infection of the central nervous system, is an acute illness mostly caused by enteroviruses. Cerebrospinal fluid(CSF) has been used for the detection of enteroviral RNA but the detection has been mostly performed in a single CSF specimen obtained during the illness. A major objective was to evaluate the relation of sampling time to the recovery of enteroviral RNA in CSF. Thirty seven CSF specimens were obtained from 24 patients between May and August 1993, when an outbreak of asceptic meningitis by echovirus type 9 occurred. Enteroviral RNA in CSF was detected by polymerase chain reaction(PCR). Data about onset of symptom development were obtained by review of medical records. Enteroviral RNA was detected by PCR in 29 of 37 CSF specimens. PCR yielded positive results in 4 of 5 CSF specimens obtained on day 1 to 3, 10 of 11 on day 4 to 6, 8 of 10 on day 7 to 9, 6 of 8 on day 10 to 12, 1 of 3 on day 13 to 15 postonset. Of 11 patients from each of whom more than one CSF were obtained on different day postonset, PCR yielded positive resutls in 2 of 3 cases in whom enteroviral RNA detection was negative in the first CSF. These results indicate that two or more CSF specimens obtained within 12 days postonset are required for improving the accuracy of the diagnosis of enteroviral meningitis.
A study was conducted to provide comprehensive information on epidemiological state of crippled schoolchildren in Chungnam area from November 23,1979 to June 19,1980. In this study, two step survey was conducted upon whole primary, middle and high school, and subsequent selected 537 cases in total 3,063 crippled children. The results obstained are as follows; 1. The prevalence rate of orthopedic defect children was 3.7 per 1,000 and dominant male as 6:4. 2. It was relatively higher the school grade and prevalence rate more increased. Prevalence rate was highest in high school as 4.7 per 1,000, middle in middle school as 4.4 per 1,000 and lowest in primary school as 3.1 per 1,000. 3. The most predominant group was $15{\sim}19$ age as 52.9 percent. 4. The most common job of their parent was agriculture as 52.9 percent and next job sales and service. Economic state of Cripple's parent have revealed no particular difference. 5. In a family, the most common incidence rate was first children in both sex and decreased by ordered sibling. 6. Poliomyelitis was the most common cause as 71.5 percent and next cerebral palsy 11.9 percent. 7. The most common affected site was lower extremity (Right: 42.3%, Left: 35.5%) and next upper extremity. Right was more dominant as 55 : 45. 8. Intelligence was decresed in cerebral palsy predominantly than other cause of defect, including poliomyelitis. 9. For the treatment of their condition at onset of illness, crippled children was utilized private clinic and herb medicine, but was changed tendency of treatment as acupucture and moxibustion after diagnosis of cause of defect.
The recent development of cardiovascular surgery as well as aortoarteriogaphy has been established excellent operative result with great aid of limb-salvage. However, less consideration or less experience still exists on the regard of vascular accident and vascular disease, as well as vascular surgery in Korea. During the last 13 years, we experienced only two cases of aorto-iliac occlusion,acute and chronic, regardless of having had more than 300 cases of mitral valvotomy and gradual increasing tendency of arteriosclerosis and hypertension in Korea. Therefore it is noteworthy to report the cases in order to promote the consideration for vascular surgery. Case 1; 52 year old female who had 20 years history of mitral stenosis with uricular fibrillation and received medical treatment for recent 1 year in the medical department. 10 days before admission, acute saddle emboli developed and 15 days after the onset, embolectomy through both common femoral arteries on the groin and abdominal approach was made. The progression of emboll to the right popliteal bifurcation was found by arteriography on operating table and retrograde flushing with heparin solution by the polyethylene catheter inserted through posterior tibial artery. The operation was successful, but 9 hours after operation sudden death occurred. Considering this case, first, mitral valvotomy already before might prevent peripheral embolizatlon, secondarily, the more early detection and surgery might also prevent the progression of emboli. Thirdly, although preoperative or postoperatlve heparinization is controversial for mitraI stenosis, heparinization might prevent additional emboli to vital organs in this case Cases 2; 66 year old female who had 4 years history of left hip and calf intermittent claudication and has had rest pain, inability to walk and ischemic necrosis on the the left leg since last 3 months prior to admission to the orthopedic department under the suspicion of herniated disc. Absence of pulsation on the groin and aortography evidenced aortoillac occlusion predominantly on the left side. Thromboendarterectomy was made and the operative result was successful with absence of claudication, healing of ulcer and aortographic patency of occlusive site. This chronic occlusion is considered to result from arteriosclerosis in origin with the evidence of moderate hypertension, x-ray evidence of calcified plaque on the aortic knob and operative finding of palpable plaques.
A study of the non-linear optical properties of nanocrystal-Si embedded in SiO2 has been performed by using the z-scan method in the nanosecond and femtosecond ranges. Substoichiometric SiOx films were grown by plasma-enhanced chemical-vapor deposition(PECVD) on silica substrates for Si excesses up to 24 at/%. An annealing at $1250^{\circ}C$ for 1 hour was performed in order to precipitate nanocrystal-Si, as shown by EFTEM images. Z-scan results have shown that, by using 5-ns pulses, the non-linear process is ruled by thermal effects and only a negative contribution can be observed in the non-linear refractive index, with typical values around $-10-10cm^2/W$. On the other hand, femtosecond excitation has revealed a pure electronic contribution to the nonlinear refractive index, obtaining values in the order of 10-12 cm2/W. Simulations of heat propagation have shown that the onset of the temperature rise is delayed more than half pulse-width respect to the starting edge of the excitation. A maximum temperature increase of ${\Delta}T=123.1^{\circ}C$ has been found after 3.5ns of the laser pulse maximum. In order to minimize the thermal contribution to the z-scan transmittance and extract the electronic part, the sample response has been analyzed during the first few nanoseconds. By this method we found a reduction of 20% in the thermal effects. So that, shorter pulses have to be used obtain just pure electronic nonlinearities.
Chromosome condensation and swelling of the donor nucleus have been known as the early morphological indicators of chromatin remodelling after injection of a foreign nucleus into an enucleated recipient cytoplasm. The effects of non-preactivation and electrical preactivation of recipient cytoplasm, prior to fusing a donor nucleus, on the profile of nuclear remodelling in the nuclear transplant rabbit embryos were evaluated. The embryos of 16-cell stage were collected and synchronized to G1 phase of 32-cell stage. The recipient cytoplasms were obtained by removing the first polar body and chromosome mass by non-disruptive microsurgical procedure. The separated G1 phase blastomeres of 32-cell stage were injected into non-preactivated recipient cytoplasms. Otherwise, the enucleated recipient cytoplasms were preactivated by electrical stimulation and the separated G1 phase blastomeres of 32-cell stage were injected. After culture until 20h post-hCG injection, the nuclear transplant oocytes were electrofused by electrical stimulation. The nuclei of nuclear transplant embryos fused into non-preactivated and/or preactivated recipient cytoplasm were stained by Hoechst 33342 at 0, 1.5, 2, 4, 6, 8, 10 hrs post-fusion and were observed under an fluorescence microscopy. Accurate measurements of nuclear diameter were revealed with an ocular micrometer at 200$\times$. Upon blastomere fusion into non-preactivated recipient cytoplasm, a prematurely chromosome condensation at 1.5 hrs post-fusion and nuclear swelling at 8 hrs post-fusion were occurred as 91.6% and 86.1%, respectively. But the nuclei of nuclear transplant embryos fused into preactivated recipient cytoplasm, as o, pp.sed to non-preactivated recipient cytoplasm, were not occurred chromosome condensation and extensive nuclear swelling. Nuclear diameter fused into non-preactivated and preactivated recipient cytoplasm at hrs post-fusion was 30.2$\pm$0.74 and 15.2$\pm$1.32${\mu}{\textrm}{m}$, respectively. These results indicated that onset of unclear condensation and swelling which was associated with oocytes activation were critical steps in the process of chromatin swelling. Futhermore, complete reprogramming seemed only possible after remodelling of the donor nucleus by chromosome condensation and nuclear swelling.
The objective of this study was to evaluate the effects of mirror therapy on motor function recovery following a stroke through a systemic review and meta-analysis. In total, nine of the 48 studies were identified from search engines between 1997 and 2011, as well as from a review of the reference lists of each identified study. The quality of each study was assessed using Jadad scale, and the effect size was calculated as a Cohen's effect size using MetaAnalyst (Beta 3.13). The overall effect size of the mirror therapy was 2.005 (95% confidence interval=1.041~2.970) in a random-effects model. This finding suggests that mirror therapy is beneficial for improving motor function following a stroke. The results from the subgroup analysis according to categorical variables were as follows: First, the effect size was larger for an onset time of less than 1 year (1.166) than for a duration of 1 year or more (.668). Second, the effect size of unpublished dissertations (1.610) was larger than published articles (1.221). Third, motor recovery of upper extremities (1.609) had a greater effect than motor recovery of lower extremities (.903). The major limitation of this study is the relatively small study population. Therefore, further individual studies of mirror therapy should be conducted in order to generalize the effects. In addition, mirror therapy supervised by a physical therapist should be recognized as a potential approach to manage motor function following a stroke and recommended to patients to improve their motor function.
Journal of the Korea Society of Computer and Information
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v.13
no.6
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pp.59-68
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2008
In this paper, we propose a method for selecting meaningful event packets from which can receive before anything else from seismograph according to allotted priority and estimate epicenter using selected packets. Event packets which received from each station will be evaluated with their onset time, signal period and SNR by statistical method and will be selected packets related with real earthquake's P-wave. And estimated epicenters using by 'Application of epicenter estimation using first P arrivals'. With local earthquakes occurred in 2007 were announced by KMA, collected event packets on earthquake happened date and selected p-wave related packets and estimated epicenter. After result of experiment, if an earthquake occurred within seismic networks, can estimate epicenter with small misfits just after event packets arrived from above 4 stations. Considering average distance of each station, in case of using all stations' data include other organization, can estimate and alert rapidly. It show this method is useful when construct a local earthquake early warning system later.
Time-varying tempo of a song is one of the error sources for the identification of a note duration in automatic music recognition. This paper proposes an improved music transcription scheme equipped with the identification of note duration considering the time-varying tempo. In the proposed scheme the measures are found at first and the tempo, the playing time of each measure, is then estimated. The tempo is then used for resizing each IOI(Inter Onset Interval) length and considered to identify the accurate note duration, which increases the degree of correspondence to the music piece. In the experiment the proposed scheme found the accurate measure position for 14 monophonic children songs out of 16 ones recorded by men and women. Also, it achieved about 89.4% and 84.8% of the degree of matching to the original music piece for identification of note duration and pitch, respectively.
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