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Systematic Approach for the Diagnosis of IEM in the Neonatal Period (신생아기의 유전성대사이상질환의 체계적 접근방법)

  • Lee, Hong Jin
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.14 no.1
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    • pp.10-18
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    • 2014
  • Recent advances in the diagnosis and treatment of inborn errors of metabolism have improved substantially the prognosis of many of these diseases, if diagnosed early enough before irreversible damage occurs. This makes it essential that the practicing pediatrician, especially neonatologists be familliar with the clinical presentations and systematic approaches of these disorders. Characteristic clinical presentations, methods of systematic approach and typing of various disorders is discussed in this review. The signs of neurological dysfunctions of many IEMs manifesting in the neonatal period is very nonspecific, such as poor feeding, poor sucking, apnea or tachypnea, vomiting, hypertonia, hypotonia, seizure, letharginess, consciousness change and coma. Many other non-metabolic severe disorders of neonatal period such as neonatal sepsis and intracerebral hemorrhage share these nonspecific symptoms. Hyperammonemia, metabolic acidosis, ketosis and hyperlatic acidemia are observed in many of these conditions but there are exceptions in which conditions all basal laboratory tests are normal, such as NKH, sulfite oxidase deficiency and peroxisomal disorders. According to the results of basal laboratory tests, IEMs in the neonatal period can be categorized in to 6 types. Grouping of IEMs into 6 types will make confirmatory tests and early emergency treatment more efficient.

Gaussian Bracket Expressions of Aplanatic and Achromatic Conditions for Telephoto Type Telescope Objective Derived from the Numerical Solution (Aplanat조건과 Achromatwhrjs의 Gauss괄호 표현과 Telephoto형 망언경 대물렌즈의 설계)

  • 임천석
    • Korean Journal of Optics and Photonics
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    • v.4 no.3
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    • pp.243-251
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    • 1993
  • We have derived Gaussian bracket expressions of aplanatic and achromatic conditions and obtained the numerical solutions for each of two modules of the telephoto type telescope objective free from the Seidel first order spherical aberration, coma, and longitudinal chromatic aberration. The system which is for use in sighting a target is optimized within the resolution of eyes. The objective lens satisfying the aplanatic and achromatic condition has f/8.5 with the half field angle 0.$3^{\circ}$, and the telephoto ratio is 0.839 with the focal length of 30 cm.

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A Rare Cause of Recurrent Acute Pancreatitis in a Child: Isovaleric Acidemia with Novel Mutation

  • Sag, Elif;Cebi, Alper Han;Kaya, Gulay;Karaguzel, Gulay;Cakir, Murat
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.20 no.1
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    • pp.61-64
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    • 2017
  • Recurrent acute pancreatic attacks is a rare clinical condition (2-5% of all acute pancreatis) in children and is mainly idiopathic in most cases. Sometimes it may be associated with congenital anomalies, metabolic diseases or hereditary conditions. Isovaleric acidemia (IVA) is a rare autosomal recessive amino acid metabolism disorder associated with isovaleryl coenzyme A dehydrogenase deficiency presenting the clinical findings such metabolic acidosis with increased anion gap, hyperammonemia, ketonemia, hypoglycemia, "the odor of sweaty feet," abdominal pain, vomiting, feeding intolerance, shock and coma. Recurrent acute pancreatitis associated with IVA have been rarely reported. Herein; we report a child who admitted with recurrent acute pancreatic attacks and had the final diagnosis of IVA. Mutation analysis revealed a novel homozygous mutation of (p.E117K [c.349G>A]) in the IVA gene. Organic acidemias must kept in mind in the differential diagnosis of recurrent acute pancreatic attacks in children.

Pathological findings of paraquat poisoning in mice, rats and rabbits (마우스, 랫트 및 토끼에서 paraquat 중독의 병리학적 관찰)

  • Lee, Suk-joo;Cho, Sung-whan
    • Korean Journal of Veterinary Research
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    • v.34 no.2
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    • pp.339-347
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    • 1994
  • This study was carried out to investigate the pathological changes with paraquat(1.1'-dimethyl-4.4'-dipyrildiylium dichloride) administered by intraperitoneally, orally, skin applied in mice, rats and rabbits. Results were obtained as follows; In 2 days after paraquat administration clinical signs anorexia, depression, tachypnea, and tachycardia, respiratory failure, coma and death were observed in mice, rats and rabbits. Compared toxicity of paraquat with mouse were observed toward to paraquat that resistance was strong than rats and rabbits. Also, mortality of skin application were found the low than intraperitoneal and high than oral administration. In the case of gross observation were appear lips moisture in orally administered rats and rabbits by skin application. Lung of all laboratory animals were observed congestion and haemorrhage, swelling or atrophy. In the case of microscopic findings were severe congestion and haemorrhage, interstitial pneumonia of lung. Congestion and haemorrhage of liver, congestion and haemorrhage, renal tubule epithelium necrosis of kidney were observed in mice, rats and rabbits. Skin application group of mice, rats and rabbits showed infiltration of inflammatory cells and folliculitis of epidermis and dermis. Also, in oral administration group showed congestion and haemorrhage, tachment, necrosis of alimentary tract mucosa.

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Oral health and hygiene in the neurosurgical patients in intensive care unit (일부 대학병원 신경외과 중환자실 환자의 구강상태에 관한 연구)

  • Kim, Eun-Kyong;Lee, Hee-Kyung
    • Journal of Korean society of Dental Hygiene
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    • v.15 no.1
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    • pp.147-152
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    • 2015
  • Objectives: The purpose of this study was to investigate the oral health and hygiene in the neurosurgical patients in intensive care unit(ICU). Methods: The subjects were 92 neurosurgical patients in intensive care unit(ICU) from March, 2011 to December, 2012. The oral examination consisted of number of residual teeth, DMFT index, clinical attachment loss, gingival index, plague index, and Candida species colony of tongue and saliva. Plaque was inoculated from tongue and saliva and incubated in 36.5C incubator for 48 hours using $Dentocult^{(R)}$ CA(Orion Diagnostica, Espoo, Finland). Glasgow coma scale(GCS) was measured to evaluate the consciousness of the patients on the basis of medical record. Results: Oral health was poor in clinical attachment loss and gingival index. Oral hygiene in neurosurgical patients in ICU was very poor due to high plaque index and Candida colonization of tongue and saliva. Plague index was closely related to Candida colonization of tongue and saliva(p<0.05). Conclusions: Oral health and hygiene of patients in neurosurgical ICU were very poor. More careful oral hygiene care is very important and necessary to enhance the oral health improvement of the neurosurgical patients in ICU.

Multi-Sequence Signaling Based Asynchronous Trellis-Coded DS/CDMA System (다중 시퀀스 시그날링에 기초한 비동기 트레리스 부호화 DS/CDMA 시스템)

  • Sangho Choe
    • The Journal of Korean Institute of Communications and Information Sciences
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    • v.29 no.3A
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    • pp.248-256
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    • 2004
  • Woerner had suggested an asynchronous trellis-coded DS/CDMA system based on a multi-sequence signaling, biorthogonal sequence, which is superior to single sequence signaling, such as M-ary PSK, due to their better cross-correlation properties. This paper analyzes and compares system performance between OPSM, a recently-presented multi-sequence signaling scheme, and biorthogonal sequence signaling. Interuser interference moments of the two schemes are derived and compared which verifies that OPSM, having smaller signature sequences per symbol than biorthogonal signaling, reduces cross-correlation. Numerical results compare the power and spectral efficiency of asynchronous trellis-coded DS/CDMA systems based on multi-sequence signaling.

VERIFICATION OF COSMOS CODE USING IN-PILE DATA OF RE-INSTRUMENTED MOX FUELS

  • Lee, Byung-Ho;Koo, Yang-Hyun;Cheon, Jin-Sik;Oh, Je-Yong;Joo, Hyung-Kook;Sohn, Dong-Seong
    • Proceedings of the Korean Nuclear Society Conference
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    • 2002.05a
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    • pp.242-242
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    • 2002
  • Two MIMAS MaX fuel rods base-irradiated in a commercial PWR have been reinstrumented and irradiated at a test reactor. The fabrication data for two MOX roda are characterized together with base irradiation information. Both Rods were reinstrumented to be fitted with thermocouple to measure centerline temperature of fuel. One rod was equipped with pressure transducer for rod internal pressure whereas the other with cladding elongation detector. The post irradiation examinations for various items were performed to determine fuel and cladding in-pile behavior after base irradiation. By using well characterized fabrication and re-instrumentation data and power history, the fuel performance code, COSMOS, is verified with measured in-pile and PIE information. The COMaS code shows good agreement for the cladding oxidation and creep, and fission gas release when compared with PIE dad a after base irradiaton. Based on the re-instrumention information and power history measured in-pile, the COSMOS predicts re-instrumented in-pile thermal behaviour during power up-ramp and steady operation with acceptable accuracy. The rod internal pressure is also well simulated by COSMOS code. Therfore, with all the other verification by COSMOS code up to now, it can be concluded that COSMOS fuel performance code is applicable for the design and license for MaX fuel rods up to high burnup.

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Tidal Dwarf Galaxies around a Post-Merger Galaxy, NGC 4922

  • Sheen, Yun-Kyeong;Jeong, Hyun-Jin;Yi, Suk-Young K;Ferreras, Ignacio;Lotz, Jennifer M.;Olsen, Knut A.G.;Dickinson, Mark;Barnes, Sydney;Lee, Young-Wook;Park, Jang-Hyun;Ree, Chang-H.
    • Bulletin of the Korean Space Science Society
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    • 2009.10a
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    • pp.35.2-35.2
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    • 2009
  • One possible channel for the formation of dwarf galaxies involves birth in the tidal tails of interacting galaxies. We report the detection of a bright UV tidal tail and several young tidal dwarf galaxy candidates in the post-merger galaxy NGC 4922 in the Coma cluster. Based on a two-component population model (combining young and old stellar populations), we find that its light predominantly comes from young stars (a few Myr old). The Galaxy Evolution Explorer (GALEX) ultraviolet data played a critical role in the parameter (age and mass) estimation. Our stellar mass estimates of the tidal dwarf galaxy candidates are ~10^{6-7} M_sun, typical for dwarf galaxies.

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Riemerella anatipestifer infection in domestic ducks (육용오리에서 Riemerella anatipestifer 감염증례)

  • Lee, Jong-Jin;Kim, Hwan-Hee;Byun, Chul-Sub;Park, Jae-Myoung
    • Korean Journal of Veterinary Service
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    • v.31 no.1
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    • pp.37-42
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    • 2008
  • Riemerellosis, contagious disease of domestic ducks in Korea, occurred in December of 2007 in a farm located in Eumsung, Chungbuk province. The affected ducks were 22 days old and the owner reported that almost one hundred of ducks had died everyday for a few days after infection. Clinical signs were listlessness, ocular and nasal discharge, greenish diarrhea, ataxia, tremor of head and legs, and coma. On necropsy we found fibrinous exudate, which involved serosal surfaces in general, but most evidences were in the pericardial cavity and over the surface of the liver. The causative agent was isolated from the liver of the affected ducks and identified as Riemerella anatipestifer using biochemical tests. Also, the isolate was susceptible to ampicillin, cephalothin, sulfamethazole/trimethoprim, Florfenicol among the 23 species antibiotics (AST Discs, OXOID) used in our laboratory. Further studies should be needed for the more effective control and better epidemiological information such as pathogenicity, serotype, genotype and treatment.

The Effects of SWS(Sahyang·Woohwang·Samchilkeun) on Hyperlipidemia and Brain Damage (사향(麝香)·우황(牛黃)·삼칠근(三七根) 복합방(複合方)이 고지혈증(高脂血症) 및 뇌손상(腦損傷)에 미치는 영향(影響))

  • Park, Jung-yang;Kim, Byeong-tak
    • Journal of Haehwa Medicine
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    • v.8 no.1
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    • pp.425-449
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    • 1999
  • For the evaluation of the effect on SWS, experiments were made on hyperlipidemia induced by hypercholesterol diet, inhibitory reaction to human platelet aggregation, Pulmonary thrombosis induced by collagen and epinephrine, global cerebral ischemia induced by KCN, brain ischemia induced by MCA occlusion, cytotoxicity of PC12 cells induced by amyloid ${\beta}$ protein(25-35), and NO production in RAW cells stimulated by lipopolysaccharide. The results were obtained as follows : 1. In the experiment on hyperlipidemia, the level of serum total cholesterol, phospholipid, and LDL-cholesterol were significantly decreased while the level of triglyceride, VLDL-cholesterol, and HDL-cholesterol had no significant change. 2. In the experiment on inhibitory reaction to platelet aggregation, SWS inhibited platelet aggregation induced by ADP(36.05%), by collagen(20.4%), and by thrombin(0.6%). 3. In the experiment on pulmonary thrombosis induced by collagen and epinephrine, the protective effect was found(37%). 4. In the experiment on global cerebral ischemia, coma duration induced by KCN changed insignificantly. 5. In the experiment on MCA occlusion, the change of neurologic grades on hind limb was significant only after the operation. Besides brain ischemic area and edema ratio were significantly decreased. 6. In the experiment on cytotoxicity of PC 12 cells induced by amyloid ${\beta}$ protein, the significant protective effect was found as concentration increases. 7. In the experiment on NO production in RAW cells stimulated by lipopolysaccharide, NO was significantly decreased. According to the results, it is expected that SWS might be effective on hyperlipidemia and brain damage.

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