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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.

Apoptotic Effects of Junglyeokdaejosape-tang and Junglyeok-tang on A549 lung Cancer Cells (A549 폐암 세포주에 대한 정력대조사폐탕 및 정력탕의 Apoptosis 효과)

  • Yu Byeong-Gil;Kim Myung-Dong;Hwang Tae-Jun;Yoo Yeong-Min;Lee Seon-Goo
    • Journal of Physiology & Pathology in Korean Medicine
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    • v.19 no.5
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    • pp.1204-1212
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    • 2005
  • Lung cancer is one of the common malignant tumors in the world. It occurs more increasingly due to the serious air pollution, heavy smoking, expoure to ionized radiation, pollution with heavy metal, and owing to well advanced diagnostic skill, etc. Also lung cancer has the limitation of medical care because metastasis is already shown up in more than half cases when it is first detected through medical examination. Although it is treated with chemoradiation, the rate of deaths from lung cancer is high as well, because blood has a lot of toxicity which give side effects. So it has a low rate of cure. So, the ways of various treatment is being researched to raise the rate of care and decrease the side effects recently, and one of the results is inducing apoptosis which makes use of molecularbiologic diagnosis of lung cancer's cell and using oriental medicine drugs. The purpose of this study is whether apoptosis would happen on the human lung carcinoma cell by treated with Junglyeokdaejosape-tang, Junglyeok-tang Junglyeokdaejosape-tang and Junglyeok-tang has been prescribed for cough, chest pain, and many other similar cases. Cough and chest pain is shown in early lung cancer. That is why we used these prescriptions. Apoptosis happend on the human lung carcinoma A549 cells treated with Jeongiyeokdaejosapye-tang, Jeonglyeok-tang. The concentration-dependent inhibition of cell viability was observed and apoptosis was confirmed by DNA fragmentation. Bcl-2 and COX-2 mRNA expression decreased, but Bax mRNA expression increased, so it was identified with the case of indomethacin known to enhance apoptotic DNA fragmentation. Also expression of the p21, p53, cyclin E, cyclin D1, cytochrome c, caspase-3, and caspase-9 protein increased and the activity of caspase-3 increased, as well. Last, fragmentation of the PARP was shown. The previous and present results indicated that apoptosis of A549 cells by above-mentioned drugs is associated with the blockage of G1/S progression.

Effective Doses in the Radial Gamma Radiation Field near a Point Source: Gender Difference and Deviation from the Personal Dose Equivalent (점선원 감마선장에서 유효선량의 성별차 및 개연선량당량과의 차이)

  • Chang, Jai-Kwon;Lee, Jai-Ki
    • Journal of Radiation Protection and Research
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    • v.22 no.4
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    • pp.299-307
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    • 1997
  • The individual dose equivalent, $H_p$, effective dose, E, and gender specific effective dose, $E^m$ and E$^f$, were evaluated using the male and female phantoms of MIRD type located in the radial gamma radiation field near a point source. The point sources were placed at the distances of 15, 40 and 100 cm in front of the body at different heights. Two radionuclides, $^{137}Cs$ and $^{131}I$, were selected for the illustrative examples. In terms of the gender specific effective doses, $E^f$ is higher than $E^m$ with a few exceptions, e.g. the case where the point source is at the height of reproductive organs, but the differences from the sex- averaged values are not significant enough to justify use of gender specific dose conversion factors for the radial gamma field. The ratios $H_p$/E were in the range of 1 to 3 depending on the source and dosimeter positions when the dosimeter is worn on the front surface of the torso covering from chest to lower abdomen, but varied from 0.34 to 6.5 in extreme cases. When it is assumed that the typical handling procedure of radioactive source material and the typical dosimeter position(on the chest) be respected, the dosimeters calibrated against the broad parallel field appear to provide estimates with acceptable errors for the effective dose of workers exposed to radial broad gamma field around a point source.

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A Change of the ability of CPR After educations for Some Health College Students and the factors Effects CPR Performance (일부 보건계열 대학생들의 심폐소생술 교육 전·후 비교 및 교육 후 수행능력에 미치는 영향)

  • Park, Sang-Sub;Park, Dae-Sung;Ko, Chong-Hyeon;Kim, Yeong-Ah;Park, Jae-Seong
    • The Korean Journal of Emergency Medical Services
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    • v.12 no.3
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    • pp.17-26
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    • 2008
  • Purpose : This study aimed to make comparison before and after CPR training for health college students (departments of visual optics, occupational therapy, and health welfare administration). The questionnaire consisted of self-efficacy, knowledge, and attitudes toward CPR affect performance ability. Through this study, the researchers tried to provide basic data in developing of cardiopulmonary resuscitation training programs and training methods for health college students. Methods : This study was applied to 83 health college students (departments of visual optics, occupational therapy, and health welfare administration) in G Province. Data were collected from September 22 to October 9, 2008. Analysis was performed by using SPSS WIN 12.0 Version program. Frequency analysis, t-test, ANOVA, and Pearson's correlation coefficients were used. Statistical significance was based on p < .05. Results : 1. There was an increase with(${\rightarrow}$ in) 1.69 point before education and 3.01 after education of artificial ventilation(p < .001). There was an increase in 1.46 point before education and 3.24 after education of airway(p < .001). There was an increase in 1.54 point before education and 2.84 after education of chest compression location(p < .001). There was an increase in 1.97 point before education and 3.13 after education of chest compression(p < .001). 2. The difference between self-efficacy, knowledge, and attitude before and after CPR education, the self-efficacy increased from 2.08 point before education(p < .001 to 3.18 point after education. Knowledge increased from 2.09 point before education(p < .001) to 3.28 point after education. Attitude increased from 1.75 point before education(p < .001 to 3.05 point after education. 3. The correlation between self-efficacy, knowledge, and attitude toward CPR), was not changed before education. After education, knowledge showed quantitative correlation(r = .219, p < .05) with attitude. 4. In regression analysis with the aim of examining influence of self-efficacy, knowledge, and attitude upon performance ability, the effect wasn't given to performance ability before education. After education, the self-efficacy was indicated to have significant effect on performance ability(p < .05).

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Clinical Usefulness of D-dimer Test with Semiquantitative Latex Agglutination Method in Pulmonary Embolism (폐색전증에서 반정량적 Latex 응집법으로 시행한 D-dimer 검사의 유용성에 관한 연구)

  • Kim, Dong Kyun;Chun, Kang Il;Kim, Yang-Ki;Lee, Young-Mok;Kim, Ki Up;Uh, Soo-taek;Kim, Yong Hoon;Park, Choon Sik;Park, No Jin;Choi, Tae Youn
    • Tuberculosis and Respiratory Diseases
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    • v.59 no.6
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    • pp.651-655
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    • 2005
  • Background : Diagnosing a pulmonary embolism is difficult because its presenting symptoms are nonspecific and there are limitations with all of the objective tests. The D-dimer is known to be a marker of the lysis of intravascular cross-linked fibrin as a result of the activation of the endogenous fibrinolytic pathways, and the D-dimer assay is these an objective method for diagnosing a pulmonary embolism. This study assessed the benefits of the D-dimer test for diagnosing a pulmonary embolism using semiquantitative latex agglutination. Methods : The latex agglutination results of 185 patients were retrospectively reviewed. The D-dimer test was performed at the time a pulmonary embolism was suspected. Ninety patients(group I) were diagnosis with PE through spiral chest CT or a chest CT angiogram, perfusion/ventilation scans, and/or pulmonary angiogram. Ninety-five patients (group II) were found not to have a pulmonary embolism through the above tests. Results : The male to female ratio and mean age in groups I and II was 37:55, and 57 years old to 50:45 and 52 years old, respectively. When the cut off value for a positive D-dimer assay was set to $500{\mu}g$, the sensitivity, positive predictive value, negative predictive value and specificity was 86.7%, 61.4%, 79.3%, and 48.4%, respectively. Conclusion : The semiquantitative latex agglutination method in the D-dimer test has a lower sensitivity and negative predictive value than the well known ELISA test particularly for small emboli. Therefore, this test is not a suitable screening test for excluding a pulmonary embolism.

Ischemic Preconditioning and Its Relation to Glycogen Depletion (허혈성 전처치와 당원 결핍과의 관계)

  • 장대영;김대중;원경준;조대윤;손동섭;양기민;라봉진;김호덕
    • Journal of Chest Surgery
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    • v.33 no.7
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    • pp.531-540
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    • 2000
  • Baclgrpimd; Recent studies have suggested that the cardioprotective effect of ischemic preconditioning(IP) is closely related to glycogen depletion and attenuation of intracellular acidosis. In the present study, the authors tested this hypothesis by perfusion isolated rabbit hearts with glucose(G) is closely related to glycogen depletion and attenuation of intracellular acidosis. In the present study, the authors tested this hypothesis by perfusion isolated rabbit hearts with glucose(G)-free perfusate. Material and Method; Hearts isolated from New Zealand white rabbits(1.5~2.0 kg body weight) were perfused with Tyrode solution by Langendorff technique. After stabilization of baseline hemodynamics, the hearts were subjected to 45 min global ischemia followed by 120 min reperfusion with IP(IP group, n=13) or without IP(ischemic control group, n=10). IP was induced by single episode of 5 min global ischemia and 10 min reperfusion. In the G-free preconditioned group(n=12), G depletion was induced by perfusionwith G-free Tyrode solution for 5 min and then perfused with G-containing Tyrode solution for 10 min; and 45 min ischemia and 120 min reperfusion. Left ventricular functionincluding developed pressure(LVDP), dP/dt, heart rate, left ventricular end-distolic pressure(LVEDP) and coronary flow (CF) were measured. Myocardial cytosolic and membrane PKC activities were measured by 32P-${\gamma}$-ATP incorporation into PKC-specific peptide and PKC isozymes were analyzed by Western blot with monoclonal antibodies. Infarct size was determined by staining with TTC(tetrazolium salt) and planimetry. Data were analyzed by one-way analysis of variance (ANOVA) and Turkey's post-hoc test. Result ; In comparison with the ischemic control group, IP significantly enhanced functional recovery of the left ventricle; in contrast, functional significantly enhanced functional recovery of the left ventricle; in contrast, functional recovery were not significantly different between the G-free preconditioned and the ischemic control groups. However, the infarct size was significantly reduced by IP or G-free preconditioning(39$\pm$2.7% in the ischemic control, 19$\pm$1.2% in the IP, and 15$\pm$3.9% in the G-free preconditioned, p<0.05). Membrane PKC activities were increased significantly after IP (119%), IP and 45 min ischemia(145%), G-free [recpmdotopmomg (150%), and G-free preconditioning and 45 min ischemia(127%); expression of membrane PKC isozymes, $\alpha$ and $\varepsilon$, tended to be increased after IP or G-free preconditioning. Conclusion; These results suggest that in isolated Langendorff-perfused rabbit heart model, G-free preconditioning (induced by single episode of 5 min G depletion and 10 min repletion) colud not improve post-ischemic contractile dysfunction(after 45-minute global ischemia); however, it has an infarct size-limiting effect.

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Cytotoxicity of COX-2 Inhibitor (Nimesulide) in Non-small Cell Lung Cancer Cell Line (비소세포폐암 세포주에서 COX-2억제제(Nimesulide)의 세포독성)

  • Park Chan Beom;Jeon Hyun Woo;Jin Ung;Cho Kyu Do;Kim Chi Kyung;Wang Young-Pil
    • Journal of Chest Surgery
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    • v.38 no.4 s.249
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    • pp.263-270
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    • 2005
  • In recent years, a combination of two demographic phenomena, an increased number of older people in the population and an increase in the incidence of lung cancer with age, has made it mandatory to develop therapeutic modalities with less toxicity for the treatment of inoperable elderly patients with lung cancer. Therefore, we investigated the correlation between COX-2 expression and cytotoxicity of Nimesulide, a specific COX-2 inhibitor. Material and Method: Immunohistochemical staining of COX-2 was performed. After exposure of Nimesulide, XTT analysis, FACS analysis and Hoechst staining were carried out. Result: COX-2 protein was expressed in non-treated A549 cells strongly, but not in H1299. Cytotoxicity of Nimesulide against A549 cell and H1299 cell were similar and $IC_{50}$ of Nimesulide in both cell lines were $70.9{\mu}M$ in A549 cell line and $56.5{\mu}M$ in H1299 cell line respectively. FACS analysis showed $G_0/G_1$ arrest in both cell lines and the S phase cell fraction was decreased. Morphologic assessment of apoptosis by Hoechst 33258 staining, many apoptotic cells were detected in both cell lines. Conclusion: Selective COX-2 inhibitor, Nimesulide, can inhibit the proliferation of non-small cell lung cancer cell lines in vitro. Inhibitory effect of Nimesulide are induction of apoptosis and $G_0/G_1$ arrest. There is no correlation between COX-2 expression and cytotoxicity of Nimesulide, a specific COX-2 inhibitor. Therefore, highly selective COX-2 inhibitors such as Nimesulide can be expected to lead to even greater efficacy of their use as adjuncts to various anticancer angents and radiation therapy for the treatment of high-risk patients.

Effect of Antibody Titer on Xenograft Survival in Pig-To-Dog Heterotopic Cardiac Xenotransplantation -Opening of Xenotrasplantation Era- (돼지\longrightarrow개 이소이종심장 이식모델에시 생존에 미치는 항체 역가의 영향 -이종이식시대의 개막-)

  • 이정렬;김희경;김지연;최대영;이재형;위현초;강희정;김영태;강병철
    • Journal of Chest Surgery
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    • v.37 no.5
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    • pp.391-400
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    • 2004
  • Xenotransplantation in discordant species results in immediate and irreversible hyperacute rejection due to natural antibodies, IgM. With this, antibody depletion is one option to reduce hyperacute rejection, we investigated the effect of PCPP (postcentrifugal plasmapheresis) on the depletion of natural antibodies and the effect of antibody titer on xenograft survival. Material and Method: Outbred swines (n=4) weighing 10∼20 kg were used as donors and mongrel dogs (n=4) weighing 25∼30 kg were used as recipients. Recipient canines underwent plasmapheresis (COBE TPE Laboratories, Lakewood. CO, USA). Pre-transplantation PCPP was peformed on day -2 and day 0. There were three groups (Group 0: no PCPP, Group 1: 1 pla sma-volume (PV) at day -2 and 2 PV at day 0, Group 2: 2 PV at day -2 and 2 PV at day 0). A swine heart was heterotopically transplanted into a recipient's abdominal infrarenal aorta and inferior vena cava. Mean percent depletion of total IgM and IgG in plasma of the recipients was calculated. Serum albumin, electrolyte, complement activity and coagulation factors were measured. Histopathologic examination of heart specimens was performed. Result: Mean percent depletion of IgM and IgG were 95.7$\pm$1.2%, 80.5$\pm$2.4% in the group 2 at the end of PCPP. The percent depletion of serum albumin concentration was decreased from 2.8 to 1.4 g/㎗ in the group 1 and 3.0 to 1.5 g/㎗ in the group 2. Complement hemolytic activity was decreased in group 1 and 2, but returned to normal level within 24 hours. Complement hemolytic activity was reduced to 10% of pre-PCPP level in group 2. Serum fibrinogen decreased to 20% or less and was recovered within 24 hours in group 2. Antithrombin III decreased but less than fibrinogen. PT and aPTT were sometimes but not always prolonged during plasmapheresis. After plasmapheresis, PT and aPTT were prolonged beyond the measurable level. D-dimer was not found during PCPP, but appeared and maintained from 10 minutes after trasplantation. Graft Survival time was 5 min in group 0, and it was 90$\pm$0 min in the group 2. Histopathologic changes were more typically characterized by edema, hemorrhages, thrombosis in all groups at the end of experiment. Conclusion: PCPP effectively removed immuoglobulins and reduced the titer of natural antibodies, as a result, significantly prololonged swine heart xenograft survival.

Indicators of Nutritional Status on the Basis of Preschool Children's Anthropomary (학경전 아동의 신체 발달과 영양 상태 지표에 관한 연구)

  • 문현경
    • Journal of Nutrition and Health
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    • v.31 no.8
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    • pp.1283-1294
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    • 1998
  • This study was conducted to investigate nutritional status by anthropometric meas-urement and to assess the validity of indicators for nutritional status of preschool children. Health examinations, including blood and urine tests, were done for 799 childern aged 2 to 6 years. The mean anthropometric measurements of height, weight , head circumference, and chest circumference were 90.2cm, 13.9kg, 47.7cm, 50.9cm for 2 years (n=13), 94.8cm, 15.2kg, 43.3cm, 47.0cm for 3 years(n=125), 100.7cm, 16.4kg, 46.2cm, 50.5cm for 4 years(n=272), 106.5cm, 18.1kg, 48.8cm, 53.8cm for 5 years(n=253), 113.0cm 20.4kg, 50.4cm 56.9cm for 6 years(n=136), respectively. All anthropometric measureemnts were significantly different by age and sex. The results of physical examinations excluding dental examination were normal for all studied children. Urine test results were also normal for the subjects. The most common blood type was A and the rarest one was type AB. The mean hemoglobin concentration was 11.84g/dl for boys(n=398), 11.86g/dl for girls, and 11.85kg/dl for all. The percentage of childeren whose hemoglobin concentration was less than 11.0g/dl was 21.6% for boys and 18.9% for girls. The percentage of children whose height for age, weight for age, and weight for height were in the rang of median $\pm$1 standard devication(S.D) was 71.7% , 65.6%, and 67.0% for each indicator. Obese childeren whose weight for height or weigth for age was more than median +2S.D. were 5.2% while tall children whose height for age was more than median +2S.D, were 1.8% . Past and recent nutritional status of most children(87.2%) were assessed as normal by the combination of three indicators(height for age, weight for age, weight for height). The percentate of coincidence between for height) and one assessed by anthropometric measurements (height for age, weight for age, weight for height) and one assessed by physicial was 59.6%, 69.3% and 67.8% for each indicator, respectively. The distribution of subjects by height for age was significantly different between normal and anemic groups. With these results , the nutritional status of children the studied was normal. The anthropometric measurements reflected the nutritional status of childeren relatively well. In conclusion, in comparison with biochemical meausurements anthropometric measurements are relatively simple and effective indicators for nutritional assessement of children in large health and nutrition suveys in Korea.

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Characterization of Radiation Field in the Steam Generator Water Chambers and Effective Doses to the Workers (증기발생기 수실의 방사선장 특성 및 작업자 유효선량의 평가)

  • Lee, Choon-Sik;Lee, Jai-Ki
    • Journal of Radiation Protection and Research
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    • v.24 no.4
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    • pp.215-223
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    • 1999
  • Characteristics of radiation field in the steam generator(S/G) water chamber of a PWR were investigated and the anticipated effective dose rates to the worker in the S/G chamber were evaluated by Monte Carlo simulation. The results of crud analysis in the S/G of the Kori nuclear power plant unit 1 were adopted for the source term. The MCNP4A code was used with the MIRD type anthropomorphic sex-specific mathematical phantoms for the calculation of effective doses. The radiation field intensity is dominated by downward rays, from the U-tube region, having approximate cosine distribution with respect to the polar angle. The effective dose rates to adults of nominal body size and of small body size(The phantom for a 15 year-old person was applied for this purpose) appeared to be 36.22 and 37.06 $mSvh^{-1}$) respectively, which implies that the body size effect is negligible. Meanwhile, the equivalent dose rates at three representative positions corresponding to head, chest and lower abdomen of the phantom, calculated using the estimated exposure rates, the energy spectrum and the conversion coefficients given in ICRU47, were 118, 71 and 57 $mSvh^{-1}$, respectively. This implies that the deep dose equivalent or the effective dose obtained from the personal dosimeter reading would be the over-estimate the effective dose by about two times. This justifies, with possible under- or over- response of the dosimeters to radiation of slant incidence, necessity of very careful planning and interpretation for the dosimetry of workers exposed to a non-regular radiation field of high intensity.

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