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A Case of Partial DiGeorge Syndrome in Prematurity (미숙아에서 발견된 부분형 DiGeorge 증후군 1례)

  • Sung, Tae Jung;Ko, Eun Young;Kim, Dal Hyon;Oh, Ji Eun;Kwon, Young Se;Lim, Dae Hyun;Son, Byong Kwan
    • Clinical and Experimental Pediatrics
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    • v.45 no.3
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    • pp.383-389
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    • 2002
  • We experienced a case of partial DiGeorge syndrome in a $35^{+5}$ week premature female infant presented with micrognathia, fish-shaped mouth, beaked nose, nasal regurgitation, obstructive sleep apnea, velopharyngeal insufficiency and late onset hypocalcemic seizures. The chromosome 22q11 microdeletion was found by the FISH method. The lab findings showed serum calcium level of 4.4 mg/dL, ionized calcium level of 0.49 mg/dL, phosphorous level of 7.5 mg/dL, magnesium level of 1.3 mg/dL and PTH-RIA level of <1 pq/mL. Initial treatment was done with 10% calcium gluconate infusion and magnesium sulfate followed by oral calcium gluconate and low phosphorousformula milk feeding. The serum calcium level was normalized in 6 days. Nasal regurgitation, desaturation with obstructive sleep apnea continued. T-cell functions & numbers(CD 3, CD 4, CD 8)were decreased but Ig G/A/M levels were normal. No visible signs of thymus shadow were seen in either chest X-ray & chest MRI. Electrocardiography and echocardiography showed normal heart. Kidney ultrasonographby showed right side mild hydronephrosis. Neurosonography was normal but EEG showed electrical partial seizure. Hearing assessment by BERA showed mild to moderate hearing impairment. Velopharyngoplasty is scheduled for further treatment. A brief review of literature was made.

Effect of Serum magnesium Concentration on Postoperative Arrhythmias after Open Heart Surgery (혈중 마그네슘 농도가 개심술후 부정맥의 발생에 미치는 영향)

  • Kang, Chang-Hyun;Huh, Jae-Hak;Kim, Ki-Bong;Kim, Won-Gon;Ahn, Hyuk;Kim, Joo-Hyun;Kim, Chong-Whan
    • Journal of Chest Surgery
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    • v.33 no.1
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    • pp.51-59
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    • 2000
  • Background: Magnesium is one of the important intracellular cations. Hypomagnesemia is common after an open heart surgeryand may affect the development of posoperative arrhythmias. The aims of this study were to identify 1) the severity of the hypomagnesemia 2) the adequate dose of the magnesium replacement and 3) the effect of magnesium replacement on the postoperative arrhythmias. Material and Method: The serum magnesium level was measured in 20 patients in whom magnesium was replaced postopertively(6gm at the operative day 4gm at the 1st postoperative day and 2gm at the 2nd postoperative day) and compared with that of the 13 patients in whom magnesium was not replaced postopertively The serum magnesium level was normalized after magnesium replacement. We analyzed the development of arrhythmias in the patients groups who did not receive magnesium and were operated on between Oct. 1994 and Oct. 1995(Group I; n=206) and who received the magnesium postoperatively and were operated on between Nov. 1995 and Aug. 1996(Group II; n=133) Result: There were no differences in the preoperative risk factors and the rate of postoperative supraventricular or ventricular tachyarrhythmia occur-rences irrespectivel of the magnesium replacement. Magnesium replacement could prevent the aggrevation of the occurrence of postoperative arrhythmias in high risk groups of ventricular tachyarrhytnmia in old age but magnesium could not prevent postoperative arrhythmia in other high risk groups. Conclusion: The magnesium replacement after open heart surgery could prevent the development of ventricular tachyarrhythmias especially in old age groups but could not prevent atrial tachyarrhthmias.

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Study on Dosimetric Properties of Radiophotoluminescent Glass Rod Detector (유리선량계의 선량 특성에 관한 연구)

  • Rah, Jeong-Eun;Shin, Dong-Oh;Hong, Ju-Young;Kim, Hee-Sun;Lim, Chun-Il;Jeong, Hee-Gyo;Suh, Tea-Suk
    • Journal of Radiation Protection and Research
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    • v.31 no.4
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    • pp.181-186
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    • 2006
  • A radiophotoluminescent glass rod detector (GRD) system has recently become commercially available. We investigate the dosimetric properties of the GRD regarding the reproducibility of signal, dose linearity and energy dependence. The reproducibility of five measurements for 50 GRDs is presented by an average of one standard deviation of each GRD and it is ${\pm}1.2%$. It is found to be linear in response to doses of $^{60}Co$ beam in the range 0.5 to 50 Gy with a coefficient of linearity of 0.9998. The energy dependence of the GRD is determined by comparing the dose obtained using cylindrical chamber to that by using the GRD. The GRD response for each beam is normalized to the response for a $^{60}Co$ beam. The responses for 6 and 15 MV x-ray beams are within ${\pm}1.5%$ (1SD). The energy response of GRD for high-energy photon is almost the same as the energy dependence of LiF:Mg:Ti (TLD-100)and shows little energy dependence unlike p-type silicon diode detector. The GRDs have advantages over other detectors such diode detector, and TLD: linearity, reproducibility and energy dependency. It has been verified to be an effective device for small field dosimetry for stereotactic radiosurgery.

Clinical Features and Natural Course of Benign Transient Hyperphosphatasemia in Children (소아 양성 일과성 고인산분해효소혈증의 임상양상 및 자연경과에 대한 고찰)

  • Oh, Dae-Sung;Han, Jae-Joon;Yang, Hye-Ran;Chang, Ju-Young;Ko, Jae-Sung;Choi, Jung-Yun;Seo, Jeong-Kee
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.10 no.2
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    • pp.173-178
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    • 2007
  • Purpose: This study was conducted to analyze the clinical manifestation and natural course of benign transient hyperphosphatasemia (BTH) in children. Methods: A total of 17 children diagnosed with BTH between June 2006 and July 2007, were included in this study. Clinical records and laboratory tests were analyzed retrospectively for all children. Results: The mean age of the patients was $26.9{\pm}13.8$ months (range: 9 to 49 months). Nine of the 17 BTH cases (52.9%) occurred in patients that had previously suffered from infectious diseases. While the patients were suffering from BTH, the mean serum level of alkaline phosphatase (ALP) was $2,500.2{\pm}1,165.1$ U/L, however, these elevated serum ALP levels normalized within 7 weeks of recovery in all cases. In addition, a seasonal peak in the onset of BTH was observed from September to December. Further, the measurement of ALP isoenzymes in 7 of the patients with BTH, revealed a marked elevation of bone fraction. Conclusion: It is important for pediatricians to understand the clinical manifestation and benign course of BTH to avoid unnecessary diagnostic evaluation in children showing a transient increase in ALP activities.

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Differentiation between Glioblastoma and Primary Central Nervous System Lymphoma Using Dynamic Susceptibility Contrast-Enhanced Perfusion MR Imaging: Comparison Study of the Manual versus Semiautomatic Segmentation Method

  • Kim, Ye Eun;Choi, Seung Hong;Lee, Soon Tae;Kim, Tae Min;Park, Chul-Kee;Park, Sung-Hye;Kim, Il Han
    • Investigative Magnetic Resonance Imaging
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    • v.21 no.1
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    • pp.9-19
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    • 2017
  • Background: Normalized cerebral blood volume (nCBV) can be measured using manual or semiautomatic segmentation method. However, the difference in diagnostic performance on brain tumor differentiation between differently measured nCBV has not been evaluated. Purpose: To compare the diagnostic performance of manually obtained nCBV to that of semiautomatically obtained nCBV on glioblastoma (GBM) and primary central nervous system lymphoma (PCNSL) differentiation. Materials and Methods: Histopathologically confirmed forty GBM and eleven PCNSL patients underwent 3T MR imaging with dynamic susceptibility contrast-enhanced perfusion MR imaging before any treatment or biopsy. Based on the contrast-enhanced T1-weighted imaging, the mean nCBV (mCBV) was measured using the manual method (manual mCBV), random regions of interest (ROIs) placement by the observer, or the semiautomatic segmentation method (semiautomatic mCBV). The volume of enhancing portion of the tumor was also measured during semiautomatic segmentation process. T-test, ROC curve analysis, Fisher's exact test and multivariate regression analysis were performed to compare the value and evaluate the diagnostic performance of each parameter. Results: GBM showed a higher enhancing volume (P = 0.0307), a higher manual mCBV (P = 0.018) and a higher semiautomatic mCBV (P = 0.0111) than that of the PCNSL. Semiautomatic mCBV had the highest value (0.815) for the area under the curve (AUC), however, the AUCs of the three parameters were not significantly different from each other. The semiautomatic mCBV was the best independent predictor for the GBM and PCNSL differential diagnosis according to the stepwise multiple regression analysis. Conclusion: We found that the semiautomatic mCBV could be a better predictor than the manual mCBV for the GBM and PCNSL differentiation. We believe that the semiautomatic segmentation method can contribute to the advancement of perfusion based brain tumor evaluation.

Effects of Dietary Taurine Supplementation on Growth Performance, Serum Constituents and Antibody Production of Broilers

  • Lee, Der-Nan;Cheng, Yeong-Hsiang;Chuang, Yu-Shuan;Shive, Jiing-Lin;Lian, Yuh-Ming;Wei, Hen-Wei;Weng, Ching-Feng
    • Asian-Australasian Journal of Animal Sciences
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    • v.17 no.1
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    • pp.109-115
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    • 2004
  • Three experiments were conducted to evaluate the effects of taurine (Tau) supplements on broiler growth performance, serum constituents and antibody production. In Exp. 1, 3 day old chicks received a basal diet supplemented with Tau at 0, 0.10, 0.20, 0.30 or 0.40% for 6 weeks. Although dietary Tau supplementing at 0.30 or 0.40% enhanced feed conversion and reduced feed consumption during 0 to 3 weeks (p<0.05), neither serum total cholesterol or anti-Newcastle disease virus (NDV) titer were affected. In Exp. 2, dietary Tau supplement at 0.25-0.75% enhanced feed conversion of broilers during 0 to 3 weeks, but daily gain and feed consumption were not affected. The 0.75% Tau supplement group displayed lower serum total cholesterol at 6 weeks (p<0.05) comparing with the control group but no difference in anti-NDV titers. In Exp. 3, broilers were treated with dietary Tau of 0 or 0.50% combined with low (0/0%), medium (0.18/0.08%), or high (0.36/0.16%) methionine (Met) levels for 6 weeks (0 to 3/3 to 6 weeks). The addition of Met significantly improved daily gain and feed conversion of broilers during 0 to 3 weeks (p<0.01). Dietary Tau interacted significantly with Met on daily gain and feed consumption. Broiler serum amino acids revealed that Met supplements only increased serum Met level, but only serum Tau level was enhanced as given dietary Tau supplementation. The broilers receiving Tau normalized serum triglycerides level by feeding with the low Met diet and tended to display higher anti-NDV titers (p<0.10). The experimental results suggest that the growth response obtained by Tau supplements results partly from interactions with sulfur amino acids. However, the modulation of the broiler lipid metabolism may be responsible for dietary Tau.

Characteristics of Tropical Cyclones over the Western North Pacific in 2008 (2008년 태풍 특징)

  • Cha, Eun-Jeong;Hwang, Ho-Seong;Yang, Kyung-Jo;Won, Seong-Hee;Ko, Seong-Won;Kim, Dong-Ho;Kwon, H. Joe
    • Atmosphere
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    • v.19 no.2
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    • pp.183-198
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    • 2009
  • The purpose of this study is to summarize the tropical cyclone (TC) activity of 2008 over the western North Pacific including the verification of the official track and intensity forecast errors of these TCs. The TC activity - frequency, Normalized Typhoon Activity (NTA), and life span - was lower than 58-year (1951-2008) average. 22 tropical cyclones of tropical storm (TS) intensity or higher formed in the western North Pacific and the South China Sea in 2008. The total number is less than 58-year average frequency of 26.4. Out of 22 tropical cyclones, 11 TCs reached typhoon (TY) intensity, while the rest 11 TCs only reached severe tropical storm (STS) and tropical storm (TS) intensity - six STS and five TS storms. One typhoon KALMAEGI (0807) among them affected the Korea peninsula. However, no significant impact - casualty or property damage - was reported. On average of 22 TCs in 2008, the Korea Meteorological Administration (KMA) official track forecast error for 48 hours was 229 km. There was a big challenge for individual cyclones such as 0806 FENGSHEN and 0817 HIGOS presenting significant forecast error, with both intricate tracks and irregular moving speed. The tropical cyclone season in 2008 began in April with the formation of NEOGURI (0801). In May, four TCs formed in the western North Pacific in response to enhanced convective activity. On the other hand, the TC activity was very weak from June to August. It is found that the unusual anti-cyclonic circulation in the lower level and weak convection near the Philippines are dominant during summertime. The convection and atmospheric circulation in the western North Pacific contributed unfavorable condition for TC activity in the 2008 summertime. The 2008 TC activity has continued the below normal state since mid 1990s which is apparent the decadal variability in TC activity.

Increase of Growth on Cool Season Turfgrass by Foliar Application of Preparations of Turfgrass Fertilizer during Fall Season (잔디 비료 제형의 엽면 살포가 가을철 한지형 잔디의 생장 증대)

  • Koo, Jun Hwak;Heo, Hyug Jae;Kim, Yang Sun;Yun, Jeong Ho;Chang, Seog Won;Lee, Seong Jun;Chang, Taehyun
    • Weed & Turfgrass Science
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    • v.3 no.1
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    • pp.41-49
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    • 2014
  • Four preparations of liquid turfgrass fertilizers were tested cool season turfgrasses during fall season. The preparations of PS-A, PS-B, PH-C and PH-D for commercial products were contained with essential nutrient elements, seaweed extract, amino acids, and humus substance. Growth and quality of creeping bentgrass (Agrostis palustris Huds cv. Penn-A1) and Kentucky bluegrass (Poa pratensis L.) mixture (Midnight 33%, Moonlight 33%, Prosperity 33%) were evaluated by normalized difference vegetation index (NDVI), root length and fresh weight, shoot density, turf color, and chlorophyll content. Three foliar sprays of 4 preparations with an interval of 7 days were made in the fall of 2013 in the sod production field at Hapchun, Korea. No significant difference among 3 preparations was found in NDVI of creeping bentgrass. However, PS-A was significantly increased NDVI of Kentucky bluegrass. Two to three applications of PS-A significantly increased chlorophyll content and turf color. Three foliar sprays of PS-A and PS-B were significantly increased the shoot density of Kentucky bluegrass on 20 days after final application. These results may indicate that the use of some preparation is beneficial in producing higher quality turfgrass sod with better color and chlorophyll content during fall season.

The Ornithine Transcarbamylase (OTC) Deficiency Identified by a Novel Mutation (새로운 유전자 돌연변이로 확진된 Ornithine Transcarbamylase (OTC) 결핍증 1례)

  • Song, Ari;Lee, Kiwuk;Yang, Aram;Kim, Jinsup;Park, Hyung-Doo;Cho, Sung Yoon;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.16 no.3
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    • pp.148-154
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    • 2016
  • A urea cycle disorder is a condition caused by a defect of the enzymes in the urea cycle, and deficiency of ornithine transcarbamylase (OTC), which converts carbamoyl phosphate and ornithine into citrulline, is the most common type of the disorder. OTC deficiency induces the accumulation of precursors of urea, ammonia, and glutamine, leading to neurological symptoms including hypotonia, respiratory failure, seizure, lethargy, and coma and sometimes to death. Because OTC deficiency is inherited in an X-linked manner, typical symptoms such as vomiting, poor feeding, and lethargy appear mainly in male neonates. We recently had a case that presented with neonatal onset lethargy, vomiting, and apnea in a 4-day-old boy. He was diagnosed with OTC deficiency by biochemical phenotype, including hyperammonemia and an increased orotic acid level in the urine. Genetic analysis of the OTC gene showed a novel mutation c.780_781insCAGGCAGTGT (p.Ile261Glnfs*35). He was treated for hyperammonemia using continuous venovenous hemofiltration (CVVH) at 118 hours after birth. After 4 days of CVVH, his consciousness and blood ammonia concentration were normalized, and he was discharged at the age of 53 days. At around 12 months of age, bilateral femur fractures and osteomyelitis occurred in this patient. Two months after the fracture, he died of septic shock, insulin-resistant hyperglycemia, and multi-organ failure.

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Two Cases of Invasive Pseudomonas aeruginosa Infection that Developed in the Apparently Immunocompetent Infants (면역 기능의 이상이 발견되지 않은 영아에서의 침습성 녹농균 감염증 2례)

  • Kang, Min Jae;Kim, So Hee;Kim, Nam Hee;Lee, Jin-A;Eun, Byung Wook;Choi, Eun Hwa;Lee, Hoan Jong
    • Pediatric Infection and Vaccine
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    • v.13 no.2
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    • pp.180-185
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    • 2006
  • Invasive Pseudomonas infections most often occur in the immunocompromised patients and are associated with high mortality rate. Rarely this disease may develop in healthy infants and children. We report two cases of invasive Pseudomonas aeruginosa infections that were diagnosed in otherwise healthy infants. The first case was a previously healthy 5-month-old infant with ecthyma gangrenosum and septicemia. She presented with fever, swelling of left periorbital area and multiple erythronodular skin lesions. Each skin lesion formed a black eschar surrounded by an erythematous areola over time. Cultures of blood, urine and discharge from skin lesions grew P. aeruginosa. On the day of visit, she showed pancytopenia which was normalized after 10 days. The patient responded well to the management with ceftazidime and tobramycin. The other case was a previously healthy 9-month-old infant with community-acquired pneumonia. He was referred from an outside hospital with fever and cough. Chest x-ray revealed pneumonic infiltrations on both lower lungs with pleural effusion on the right side. Cultures of blood and pleural fluid grew P. aeruginosa. Chest CT performed on the ninth day demonstrated pneumatoceles, lung abscess and necrosis of lung parenchyma. He was managed with ceftazidime and amikacin for 50 days. No residual pulmonary complications were noted during the three month follow-up. Laboratory results to evaluate immunologic defects of phagocytic cells, complement components and T- and B-lymphocytes were all within normal range in both patients. It should be kept in mind that Pseudomonas can be, though uncommon, a cause of community-acquired invasive infections in the previously healthy infants.

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