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Liver Involvement in Children with Alpha-1 Antitrypsin Deficiency: A Multicenter Study

  • Cakir, Murat;Sag, Elif;Islek, Ali;Baran, Masallah;Tumgor, Gokhan;Aydogdu, Sema
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.23 no.2
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    • pp.146-153
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    • 2020
  • Purpose: Alpha-1 antitrypsin deficiency (A1ATD) in one of the most common genetic causes of liver disease in children. We aimed to analyze the clinical characteristics and outcomes of patients with A1ATD. Methods: This study included patients with A1ATD from five pediatric hepatology units. Demographics, clinical findings, genetics, and outcome of the patients were recorded (n=25). Results: Eight patients (32.0%) had homozygous PiZZ genotype while 17 (68.0%) had heterozygous genotype. Patients with PiZZ genotype had lower alpha-1 antitrypsin levels than patients with PiMZ genotype (37.6±7.7 mg/dL vs. 66.5±22.7 mg/dL, p=0.0001). Patients with PiZZ genotype were diagnosed earlier than patients with PiMZ genotype, but this was not significant (13±6.8 months vs. 23.7±30.1 months, p=0.192). Follow-up revealed the death of one patient (12.5%) with a homozygous mutation, and revealed that one patient had child A cirrhosis, five patients (62.5%) had chronic hepatitis, and one patient (12.5%) was asymptomatic. Nine of the 17 patients with a heterozygous mutation had chronic hepatitis (52.9%), two (11.7%) had child A cirrhosis, and six (35.2%) were asymptomatic. Overall, 18 (72%) of the 25 children had liver pathology in the long-term. Conclusion: Although prevalence is rare, patients with liver disorders should be checked for alpha-1 antitrypsin levels. Moreover, long-term follow-up is essential because most patients have a liver pathology.

No Adverse Outcomes of Video-Assisted Thoracoscopic Surgery Resection of cT2 Non-Small Cell Lung Cancer during the Learning Curve Period

  • Bilgi, Zeynep;Batirel, Hasan Fevzi;Yildizeli, Bedrettin;Bostanci, Korkut;Lacin, Tunc;Yuksel, Mustafa
    • Journal of Chest Surgery
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    • v.50 no.4
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    • pp.275-280
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    • 2017
  • Background: Video-assisted thoracoscopic surgery (VATS) anatomic lung resections are gradually becoming the standard surgical approach in early-stage non-small cell lung cancer (NSCLC). The technique is being applied in cases of larger tumors depending on the experience of the surgical team. The objective of this study was to compare early surgical and survival outcomes in patients undergoing anatomic pulmonary resections using VATS and thoracotomy techniques for clinical T2 NSCLC during the adaptation period of the surgical team to the VATS approach. Methods: The data of all patients who underwent anatomic pulmonary resection for NSCLC using VATS and open techniques since April 2012 were recorded to create a prospective lung cancer database. Clinical T2 NSCLC patients who underwent VATS anatomic lung resection were identified and compared with cT2 patients who underwent open resection. Results: Between April 2012 and August 2014, 269 anatomical resections for NSCLC were performed (80 VATS and 189 thoracotomy). Thirty-four VATS patients who had clinical T2 disease were identified and stage-matched to thoracotomy patients. The average tumor diameter was comparable ($34.2{\pm}11.1{\times}29.8{\pm}10.1mm$ vs. $32.3{\pm}9.8{\times}32.5{\pm}12.2mm$, p=0.4). Major complications were higher in the thoracotomy group (n=0 vs. n=5, p=0.053). There was no 30-day mortality, and the 2-year survival rate was 91% for VATS and 82% for thoracotomy patients (p=0.4). Conclusion: VATS anatomic resections in clinical T2 NSCLC tumors are safe and have perioperative and pathologic outcomes similar to those of thoracotomy, while remaining within the learning curve.

Comparison of Two Methods for Heparin Sensitivity; Activated Partial Thromboplastin Time Assay using in vitro Heparin-spiked Sample and Anti-Xa Assay using in vivo Heparin-treated Sample

  • Koo, Bon-Kyung;Kwon, Eui-Hoon;Ryu, Kwang-Hyun;Yun, Jae-Won;Kim, Hee-Jin
    • Korean Journal of Clinical Laboratory Science
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    • v.43 no.4
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    • pp.133-137
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    • 2011
  • The monitoring of heparin therapy is using almost aPTT assay. This study is compare to estimating aPTT therapeutic range using in vitro heparin-spiked sample and aPTT therapeutic range using in vivo heparin-treated sample. Normal pooled plasma was collected from 20 healthy representative individuals. 11 concentration of heparinized plasmas from 0 U/mL to 1.0 U/mL at intervals of 0.1 U/mL made by addition of heparin to normal pooled plasma were measured aPTT. The aPTT therapeutic range was performed through correlation analysis between heparin level 0.2 to 0.4 U/mL and aPTT. 30 plasmas from patients on heparin therapy were measured aPTT and anti-Xa activity. The aPTT therapeutic range was performed through correlation analysis between anti-Xa activity 0.3 to 0.7 U/mL and aPTT. The aPTT therapeutic range corresponded by heparin level-vs-aPTT value regression analysis was 60.7 to 102.4 seconds. The aPTT therapeutic range corresponded by anti-Xa activity-vs-aPTT value regression analysis was 85.3 to 147.5 seconds. The validation of heparin sensitivity using in-vitro heparin sample was not considered. The establishing aPTT therapeutic range is recommended anti-Xa activity using in-vivo sample.

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Influence of Sunflower Whole Seeds or Oil on Ruminal Fermentation, Milk Production, Composition, and Fatty Acid Profile in Lactating Goats

  • Morsy, T.A.;Kholif, S.M.;Kholif, A.E.;Matloup, O.H.;Salem, A.Z.M.;Elella, A. Abu
    • Asian-Australasian Journal of Animal Sciences
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    • v.28 no.8
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    • pp.1116-1122
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    • 2015
  • This study aimed to investigate the effect of sunflower seeds, either as whole or as oil, on rumen fermentation, milk production, milk composition and fatty acids profile in dairy goats. Fifteen lactating Damascus goats were divided randomly into three groups (n = 5) fed a basal diet of concentrate feed mixture and fresh Trifolium alexandrinum at 50:50 on dry matter basis (Control) in addition to 50 g/head/d sunflower seeds whole (SS) or 20 mL/head/d sunflower seeds oil (SO) in a complete randomized design. Milk was sampled every two weeks during 90 days of experimental period for chemical analysis and rumen was sampled at 30, 60, and 90 days of the experiment for ruminal pH, volatile fatty acids (tVFA), and ammonia-N determination. Addition of SO decreased (p = 0.017) ruminal pH, whereas SO and SS increased tVFA (p<0.001) and acetate (p = 0.034) concentrations. Serum glucose increased (p = 0.013) in SO and SS goats vs Control. The SO and SS treated goats had improved milk yield (p = 0.007) and milk fat content (p = 0.002). Moreover, SO increased milk lactose content (p = 0.048) and feed efficiency (p = 0.046) compared to Control. Both of SS and SO increased (p<0.05) milk unsaturated fatty acids content specially conjugated linolenic acid (CLA) vs Control. Addition of SS and SO increased (p = 0. 021) C18:3N3 fatty acid compared to Control diet. Data suggested that addition of either SS or SO to lactating goats ration had beneficial effects on milk yield and milk composition with enhancing milk content of healthy fatty acids (CLA and omega 3), without detrimental effects on animal performance.

Effect of Activation Method and Culture Medium on the Development of Porcine Nuclear Transfer Embryo using Fetal Fibroblast

  • Im, Gi-Sun;Yang, Byoung-Chul;Park, Jin-Ki;Kim, Hyun-Ju;Chang, Won-Kyung;R. S. Prather;B. N. Day
    • Proceedings of the KSAR Conference
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    • 2001.03a
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    • pp.66-66
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    • 2001
  • Since the first birth of pig derived from embryonic cells by nuclear transfer, many researches to produce cloned pig have been carried out. Recently, two reports about the birth of somatic cell cloned pigs using in vivo oocytes and also Betthauser et al. (2000) reported the birth of somatic cell cloned pigs using in vitro oocytes. So here we investigated the effect of activation method and culture medium on in vitro development of porcine nuclear transfer embryo using fetal fibroblast. Oocytes derived from slaughter house obtained ovaries were matured for 42 to 44 h in TCM 199. Matured oocytes were denuded using 0.1% hyaluronidase and then Oocytes with the first polar body were used for enucleation by aspirating the first polar body and adjacent cytoplasm in TCM 199 supplemented with 7.5 $\mu\textrm{g}$ cytochalasin B. Petal fibroblast cells were prepared from 35 days old fetus. To be used as donor cells, fetal fibroblast cells were serum starved for 3 to 5 days and then isolated into single co:1 by trypsinization. Nuclear transfer embryos were fused using 2 times 1.25㎸ for 30$mutextrm{s}$. Fused NT embryos were activated with calcium ionophore (CI) and 6-dimethyl-aminopurine (6-DMAP). Activated oocytes were cultured in NCSU 23 or BECM 3 for 6 days. There was no significant difference between chemical activation and no chemical activation for blastocyst development rate(11.6 vs. 14.8%). However, cell number was significantly higher when NT embryos were activated with CI and 6-DMAP (31.2 vs. 22.6). When NT embryos were cultured in NCSU 23 or BECM 3, blastocyst development rate was 16.4 and 13.2%, respectively, and cell number was 31.5 and 24.1, respectively. These results suggest that chemical activation after fusion and culture in NCSU 23 could increase cell number of porcine NT embryos.

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Development of Vitrified Bovine Oocytes following Intracytoplasmic Sperm Injection (ICSI)

  • Yeo, H-J;Ock, S-A;Yea, E-H;Lee, H-J;Choe, S-Y;Park, G-J
    • Proceedings of the KSAR Conference
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    • 2001.03a
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    • pp.6-6
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    • 2001
  • Oocyte freezing has become a prevalent source for related reproductive technologies. This study was carried out to evaluate viability of post-thawed bovine oocyte injected DTT-treated sperm following by two different activation stimuli (Group 1, 5 M ionomycin, 5 min + CR1aa, 3 h . 1.9 mM dimetylaminopurine (DMP), 3 h; group 2, ionomycin + 10 $\mu\textrm{g}$/$m\ell$ cycloheximide(CHX), 5h). The techniques of ultra-rapid freezing used in this study were essentially similar to those of described by Vajta et al (Theriogenology 1999; 52:939-948), Denuded oocytes at 22 h of culture were exposed to cryoprotectant (3.2 M Ethylene glycol, 2.36 M DMSO, 0.6 M sucrose), and followed by freezing in electron microscopic grid. After thawing the oocytes were transferred back into the drop of maturation medium and cultured for additional 2 h before being subjected to ICSI. All eggs were then cultured in CRlaa medium, and transferred into M199+10% FCS on day 4. The culture was maintained until day 9. In Experiment 1, frozen-ICSI eggs were compared on development into blastocyst to those of unfrozen and IVF control. Those eggs were activated with the method of group 2. A higher proportion of unfrozen-ICSI and IVF eggs developed into cleavage and blastocysts than of frozen-ICSI eggs (65% and 13%; 71% and 23% vs. 39% and 8%; P<0.05). In Experiment 2, development and ploidy of embryos made from group 1 were compared to those from group 2. Between groups there did not differ on the rates of development, however, chromosomal abnormality in group 1 was significantly higher than in group 2 (49% vs. 30%; P<0.05). The present result suggests that frozen bovine oocytes can be used for ICSI.

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Eye-movements in reading easy and difficult texts (난이도가 다른 덩이글 읽기에서의 안구운동 양상)

  • Yoon, Nak-Yeong;Koh, Sung-Ryong
    • Korean Journal of Cognitive Science
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    • v.20 no.3
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    • pp.291-307
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    • 2009
  • This study investigated global and local characteristics of eye movement while 30 college students read easy and difficult Korean texts. It was found that readers who read the difficult text fixated longer for about 217ms and made shorter saccades of about 3.7 characters while readers who read the easy one fixated for about 190ms and made saccades of about 4.8 characters. Single fixation times and gaze durations in the difficult text were longer than those in the easy one(227ms vs. 195ms; 266ms vs. 210ms). In both easy and difficult texts, the effects of word frequency and eojeol length were found. In addition, the differences in fixation times according to word frequency and length were larger in the difficult text.

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Effect of L-Carnitine and Source of Dietary Fat on Growth Performance and Serum Biochemical Parameters of Piglets Weaned at 35 Days of Age

  • Li, Defa;Qiao, Q.;Johnson, E.W.;Jiang, J.;Wang, F.;Blum, R.;Allee, G.
    • Asian-Australasian Journal of Animal Sciences
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    • v.12 no.8
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    • pp.1263-1272
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    • 1999
  • The effects of carnitine in diets with or without added fat (5% lard or soybean oil) were evaluated in 72 Large White ${\times}$ Landrace ${\times}$ Duroc pigs weaned at 35 days of age. Pigs were fed a 1.30% lysine corn-soybean basal diet+15% dried whey+4% fish meal with carnitine at 0 or 50 mg/kg and either 0% added fat, 5% soybean oil or 5% lard for 6 weeks in a $2{\times}3$ factorial trial (6 treatments, 3 pens per treatment, 4 pigs per pen). Addition of carnitine increased average daily gain (ADG) and average daily feed intake (ADFI) in the second two weeks of the six-week trial and overall, but had no significant effect on feed per gain (F/G). Lard alone depressed ADG (p<0.05) in the last two weeks of the trial and overall, but the ADG for pigs fed lard+carnitine was similar to the control. Lard reduced feed intake in the first two weeks of the trial (p<0.05). Carnitine reduced the percentage of pigs with poor (ADG<375 g/d) growth (15 vs 40%; p<0.05). The greater uniformity of growth was most evident in low-weaning-weight pigs in the second period (16 vs 62%, p<0.005). Addition of fat did not produce any positive effect on uniformity and had no interaction with carnitine on uniformity. Carnitine addition increased serum total carnitione and short-chain acyl-carnitine levels (p<0.05), but did not modify free carnitine levels. Serum carnitine levels were lower at weaning than at 14, 28, or 39 days after weaning (p<0.05). Carnitine increased serum protein levels on day 14 (p<0.05). Addition of fat in the form of soybean oil or lard did not improve piglet growth performance. Addition of 50 mg/kg of carnitine to the diet of weanling pigs enhanced postweaning performance.

A lifelong exposure to a Western-style diet, but not aging, alters global DNA methylation in mouse colon

  • Choi, Sang-Woon;Tammen, Stephanie A;Liu, Zhenhua;Friso, Simonetta
    • Nutrition Research and Practice
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    • v.9 no.4
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    • pp.358-363
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    • 2015
  • BACKGROUND/OBJECTIVES: Previous studies have indicated that when compared to young mice, old mice have lower global DNA methylation and higher p16 promoter methylation in colonic mucosa, which is a common finding in colon cancer. It is also known that a Western-style diet (WSD) high in fat and calories, and low in calcium, vitamin D, fiber, methionine and choline (based on the AIN 76A diet) is tumorigenic in colons of mice. Because DNA methylation is modifiable by diet, we investigate whether a WSD disrupts DNA methylation patterns, creating a tumorigenic environment. SUBJECTVIES/METHODS: We investigated the effects of a WSD and aging on global and p16 promoter DNA methylation in the colon. Two month old male C57BL/6 mice were fed either a WSD or a control diet (AIN76A) for 6, 12 or 17 months. Global DNA methylation, p16 promoter methylation and p16 expression were determined by LC/MS, methyl-specific PCR and real time RT-PCR, respectively. RESULTS: The WSD group demonstrated significantly decreased global DNA methylation compared with the control at 17 months (4.05 vs 4.31%, P = 0.019). While both diets did not change global DNA methylation over time, mice fed the WSD had lower global methylation relative to controls when comparing all animals (4.13 vs 4.30%, P = 0.0005). There was an increase in p16 promoter methylation from 6 to 17 months in both diet groups (P < 0.05) but no differences were observed between diet groups. Expression of p16 increased with age in both control and WSD groups. CONCLUSIONS: In this model a WSD reduces global DNA methylation, whereas aging itself has no affect. Although the epigenetic effect of aging was not strong enough to alter global DNA methylation, changes in promoter-specific methylation and gene expression occurred with aging regardless of diet, demonstrating the complexity of epigenetic patterns.

Effect of Dietary Fibers in Rice and Barley on Lipid and Cadmium Metabolism in the Rat (쌀과 보리의 식이 섬유가 흰쥐의 지방 및 Cadmium 대사에 미치는 영향)

  • 김미경
    • Journal of Nutrition and Health
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    • v.30 no.3
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    • pp.252-265
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    • 1997
  • This study was performed to investigate effect of dietary fibers in rice and barley on glucose, lipid and cadmium (Cd) metabolism in the rat. Fifty-six male Spague-Dawley rats weighing 244.6$\pm$2.7g were blocked into eight groups according to body weight and raised for four weeks with diets containing 0 or 0.04%(w/w) CdCl2 and four different carbohydrate sources, starch, rice flour, barley flour and mixture of rice and barley flour(7 : 3, w/w). Total dietary fibers and $\beta$-glucan contents of barley were about three times higher than those of rice (10.75% vs. 3.94%, 3.11% vs. 1.06%, respectively). Food intake, weight gain, food efficiency ratio, liver and kidney weights were lower in Cd exposed groups, and barley group among Cd exposed animals showed highest weight gain, food efficiency ratio and organ weights. Fasting serum glucose levels were not significantly different among groups, Serum cholesterol level was lowest in Cd exposed barley group. Serum HDL-cholesterol level was higher in none-Cd exposed starch and barley groups, and HDL-cholesterol : total cholesterol ratios were higher in none-Cd rice and mixed flour groups than other groups. Liver total lipid and triglyceride levels were lowest in barley groups regardless of Cd administration. Fecal total lipid, cholesterol and triglyceride excretions were high in barley and mixed flour groups. Liver Cd concentrations were low in Cd exposed barley and mixed flour groups. In Cd exposed barley group, fecal weight and Cd excretion were highest and Cd retention ratio was lowest among groups. Small intestine metallothionein(MT) concentration was highest in Cd exposed rice group, and kidney MT concentration was highest in Cd exposed barley group. In conclusion, cereals showed different effects on lipid and Cd metabolism that might be mediated by dietary fibers in cereals. Especially $\beta$-glucan-rich barley group showed greatest lipid and Cd lowering effects by increasing fecal lipids and Cd excretions.

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