• 제목/요약/키워드: AGE-R

검색결과 3,624건 처리시간 0.032초

단독성 고메티오닌혈증 환아들의 임상적 특성과 유전자 분석 (Clinical Findings and Genetic Analysis of Isolated Hypermethioninemia Patients in Korea)

  • 유상수;이민희;이정호;이동환
    • 대한유전성대사질환학회지
    • /
    • 제13권2호
    • /
    • pp.98-103
    • /
    • 2013
  • Purpose: MAT-I/III deficiency by MAT1A gene mutation causes isolated hypermethioninemia, which is considered to be a clinically benign disease. But in some patients, mental retardation, developmental delay, myelination disorder may be shown. This study was performed to find out the clinical manifestations and genetic characteristics of patients with isolated hypermethioninemia. Methods: Clinical, biochemical and genetic analysis were done to 10 patients with isolated hypermethioninemia who were referred to department of pediatrics, Soonchunhyang University Hospital from March 1999 to March 2012. Results: At first visit, all patients' mean plasma methionine level was 5.5 mg/dL (2.1-14.6) and there were no increase of amino acid levels including homocystine in all patients. Serum homocysteine level was evaluated in seven patients who visited after year 2003, and ranged from 4.96 to $11.15{\mu}mol/L$ (normal < $25{\mu}mol/L$). Methionine restricted diet was started to all patients. Nine patients who managed regularly showed normal development, but one patient whose initial plasma methionine level was 14.6 mg/dL showed language delay at 1 year of age and was diagnosed as mild mental retardation (IQ=66) at 6 years of age. Genetic analysis was done to eight patients, R264H mutation was identified in seven patients. Also, both R299C and R356Q mutation were identified in one patient. Conclusion: Clinical findings in patients with isolated hypermethioninemia were generally good, but one patient showed mental retardation and language difficulty. R264H mutation which usually inherits as an autosomal dominant trait was most frequently found in our patients, and R299C/R356Q mutation were also identified.

  • PDF

Cerebral Oxygenation during Apnea in Preterm Infants: Effects of Accompanying Peripheral Oxygen Desaturation

  • Choi, Seo Hee;Lee, Juyoung;Nam, Soo Kyung;Jun, Yong Hoon
    • Neonatal Medicine
    • /
    • 제28권1호
    • /
    • pp.14-21
    • /
    • 2021
  • Purpose: Premature infants have immature respiratory control and cerebral autoregulation. We aimed to investigate changes in cerebral oxygenation during apnea with and without peripheral oxygen desaturation in premature infants. Methods: This prospective observational study was conducted at Inha University Hospital. Near-infrared spectroscopy (NIRS)-monitored regional cerebral oxygen saturation (rScO2) and pulse oximeter-monitored peripheral oxygen saturation (SpO2) were assessed during the first week of life in 16 stable, spontaneously breathing preterm infants. Apneic episodes that lasted for ≥20 seconds or were accompanied by desaturation or bradycardia were included for analysis. The average rScO2 value during the 5-minute prior to apnea (baseline), the lowest rScO2 value following apnea (nadir), the time to recover to baseline (recovery time), the area under the curve (AUC), and the overshoot above the baseline after recovery were analyzed. Results: The median gestational age and birth weight of the infants were 29.2 weeks (interquartile range [IQR], 28.5 to 30.5) and 1,130 g (IQR, 985 to 1,245), respectively. A total of 73 apneic episodes were recorded at a median postnatal age of 2 days (IQR, 1 to 4). The rScO2 decreased significantly following apneic episodes regardless accompanied desaturation. There were no differences in baseline, nadir, or overshoot rScO2 between the two groups. However, the rScO2 AUC for apnea with desaturation was significantly higher than that for apnea without desaturation. Conclusion: Cerebral oxygenation can significantly decrease during apnea, especially when accompanied by reduced SpO2. These results add the evidence for the clinical utility of NIRS in monitoring premature infants.

동해안 도루묵, Arctoscopus japonicus의 연령과 성장 (Age and growth of the sandfish, Arctoscopus japonicus in the East Sea of Korea)

  • 양재형;이성일;차형기;윤상철;장대수;전영열
    • 수산해양기술연구
    • /
    • 제44권4호
    • /
    • pp.312-322
    • /
    • 2008
  • The age and growth of the sandfish, Arctoscopus japonicus were investigated from samples of the eastern sea danish seine and gill net fishery in the East Sea of Korea from February, 2004 to December, 2007. Ages were determined from annuli in otoliths and annuli were formed between December and February once a year. Also, the main spawning period was estimated to be between December and January, thus rings were considered to be annual marks. For the relationship between fork length and total weight, a multiplicative error structure was assumed because variability in growth increased as a function of the length. The relationship between fork length and total weight were $TW=0.0083FL^{3.01}(R^2=0.966)$ for female and $TW=0.0079FL^{3.04}(R^2=0.969)$ for male. The variability in growth was constant as a function of age, revealing an additive error structure. The von Bertalanffy growth parameters estimated by a non-linear method were $L_{\infty}=31.6cm$, K=0.266/year, $t_0=-0.835$ years for female and $L_{\infty}=27.7cm$, K=0.273/year, $t_0=-0.806$ years for male. Growth at age between male and female had a significant difference(P<0.001).

심실중격결손증의 임상적 고찰 (The Clinical Analysis of Ventricular Septal Defect Review of 97 cases)

  • 백광제
    • Journal of Chest Surgery
    • /
    • 제18권2호
    • /
    • pp.139-150
    • /
    • 1985
  • From 1977 through 1984, 97 patients of V.S.D. were treated surgically at Department of Thoracic and Cardiovascular Surgery, Korea University college of Medicine. Among 97 patients, 3 patients were treated by PDA ligation, 3 patients were treated by PDA ligation and subclavian flap aortoplasty, 1 patient were treated by pulmonary artery banding. All of the above patients were analyzed clinically. The results were as follows; 1. Of the patients, 52 patients were male [55.5%] and 45 patients were female [44.5%]. Their age ranged from 7 days to 32 year, and the mean age was 9 year of age and 28.9% of patients were between 4 and 8 year of age. 2. The most common clinical symptoms were frequent U.R.l. and D.O.E.. 3. The most common chest PA findings were cardiomegaly and increased pulmonary vascularity. 4. Associated anomaly was founded in 27 cases and PDA was most common associated anomaly and others were A.S.D., pulmonary stenosis, aortic regurgitation, D.C.R.V.. 5. Pulmonary hypertension was founded in 37 patients and it`s incidence was increased by patient age and shunt amount. 6. On Kirklin`s anatomical classification, type 11 defect was most common [45.5%], and type 1 was 35.5%, and type 111 was 4.4%, and type 1V was 4.4%. 7. Mean E.C.C. time was 69.1 min. and varied by closing method and associated anomaly as in case of simple closure; 47.8 min., in case of patch closure; 77.2 min., in cases with associated anomaly; 92.7 min.. 8. Mean postoperative ventilatory assisted time was 7.3 hour and varied by preoperative pulmonary artery pressure and E.C.C. time, as the group with pulmonary hypertension; 10.5 hour, the group without pulmonary hypertension; 5.5 hour, the group of short E.C.C time [within 1 hour]; 4.4 hour, the group of long E.C.C. time [over 1 hour]; 8.4 hour. 9. Overall operative mortality was 9.3% [9 cases].

  • PDF

한국 남해 연안 눈볼대(Doederleinia berycoides)의 연령과 성장 (Age and Growth of Blackthroat Seaperch Doederleinia berycoides in the South Sea of Korea)

  • 최정화;최승희;김영혜;이동우;류동기
    • 한국수산과학회지
    • /
    • 제45권3호
    • /
    • pp.246-252
    • /
    • 2012
  • The age and growth of the blackthroat seaperch Doederleinia berycoides was investigated based on samples captured in the southern seas of Korea from January to December, 2006. Age was estimated by analyzing otolith growth rings. The spawning period was extrapolated from August to November based on monthly changes in the gonad somatic index. The relationship between total length and otolith radius was analyzed separately for each sex with TL=-15.6174+10.3274R for females and TL=-4.4083+7.0749R for males. From the parameters calculated using average total length and weight when the year ring was formed, the growth of D. berycoides was expressed by von Bertalanffy growth equations as $L_t=34.71(1-e^{-0.2557(t+0.2078)})$, $W_t=713.85(1-e^{-0.2557(t+0.2078)})^{3.1972}$ for females, $L_t=27.37(1-e^{-0.3388(t+0.7362)})$, $W_t=353.91(1-e^{-0.3388(t+0.7362)})^{3.1647}$ for males, and $L_t=34.20(1-e^{-0.2530(t+0.2871)})$, $W_t=674.10(1-e^{-0.2530(t+0.2871)})^{3.1171}$ for pooled sexes, where L is total length at age t.

수령간 상관을 이용한 잣나무 수고 생장의 조기선발 효율 추정 (Estimates of Early Selection Efficiency for Height Growth Using Age-Age Correlation in Pinus koraiensis)

  • 한상억;이재선
    • 한국산림과학회지
    • /
    • 제84권3호
    • /
    • pp.356-360
    • /
    • 1995
  • 잣나무림에 있어서 목재 및 종자 생산이 중요 경영 목적이므로 이러한 형질을 개량하기 위한 선발육종이 진행중에 있다. 본 연구는 잣나무의 유전모수 및 조기선발 효율 등을 추정하여 1세대 채종원을 전진세대 채종원으로 유도하는데 필요한 자료와 정보를 얻기 위하여 수행하였다. 1986년 50가계로 된 풍매 차대검정림을 3곳에 조성하여 6년간(5~10년생) 수고 생장을 조사하여 분석한 결과, LAR에 대한 수령간 유전 및 표현형 상관의 단순직선회귀는 유의차가 있었으며($r^2=0.7862$, 0.8256), 성숙기를 20년으로 가정하였을 때 조림 후 3년생이 1.96으로 조기선발 효율이 가장 높은 수령으로 추정되어 조기선발이 가능하였다.

  • PDF

Effect of Anionic Alkali Mineral Complex Barodon$^{(R)}$ on Body Growth and Testicular Development in Rats

  • Chung, Y.C;Kim, C.K.;Kim, I.;Kim, K.S.;J.W. Ryu;S.E. Yeon;Lee, E.S.;Park, S.I.;K.S. Jeon
    • 한국동물번식학회:학술대회논문집
    • /
    • 한국동물번식학회 2001년도 춘계학술발표대회
    • /
    • pp.39-39
    • /
    • 2001
  • Effects of Barodon$^{(R)}$ on body growth, histological changes in seminiferous tubules of testes and serum level of testosterone and FSH were examined in juvenile rats from 5 to 38 wks of age. All rats supplied feed and Barodon-added water(0, 0.1, 0.15, 0.3 and 1.0%) available ad libitum. DNA distribution of testicular cells from control rats obtained by flow cytometry was characterized by 4 peaks representing I : elongated, II round spermatids(1N), III: a variety of 2N cells and IV: 4N cells. Frequencies of 4 testicular cell types were calculated using cumulative DNA frequency distributions. Body growth from 18wk of age was significantly accelerated in rats supplied water with 0.15% Barodon compared to other groups. Testes weights tended to be greater in 0.15% Barodon-treated rats than in control, without significant difference. Diameter of seminiferous tubules advanced in 0.15-0.3% Barodon till 26 wk of age, but there was not significant different. Proportion of spermatids in seminiferous tubules was 48.4% at 6wk of age(round: 81.2% and elongated: 18.8% as proportion of total spermatids) and frequency of spermatids was higher in 0.3% Barodon group(57.1%) than in control(44.0%), but there was no significant difference. Serum testosterone of all groups significantly elevated at 18wk of age and level in 0.15% Barodon group was greatly higher than in others. Serum FSH at 10wks was greatly higher in 0.1-0.3% Barodon groups compared to control, but there were no significant differences. It is concluded that 0.15-0.3% Barodon treatment tended to induce precocious puberty in rats.

  • PDF

재가 중.노년 여성의 골밀도와 낙상두려움 및 낙상효능감에 관한 연구 (Fear of Falling and Falls Efficacy with Bone Mineral Density in the Middle and Old Aged Women)

  • 이혜영;박원숙;양형인
    • 근관절건강학회지
    • /
    • 제16권1호
    • /
    • pp.5-12
    • /
    • 2009
  • Purpose: The purpose of this study was to investigate bone mineral density(BMD) and fear of falling and falls efficacy in the middle and old aged women over 50 years. Methods: The subjects consisted of 409 women. One-way ANOVA, Pearson's correlations and multiple regression were used to test the BMD, fear of falling and falls efficacy scale by using SPSSWIN 12.0. The BMD of the calcaneus were measured with peripheral dual energy x-ray absorptiometry(DEXA). Results: The average age was 63 years old and the average T-score was -3.21 in patient with osteoporosis, -1.72 with osteopenia, and .13 with normal. There were significant differences in the status of the BMD according to age(p=.000), height(p=.000), weight(p=.000), married status(p=.000), age of menarche(p=.002), and menopause(p=.002). The fear of falling was related with falls efficacy(r=-.247, p=.01), BMD(r=-.337, p=.01). Falls efficacy($\beta$=-.21, p=.000)and BMD($\beta$=-.26, p=.000) were predicting variables of fear of falling. The model explained 13% of the variance in fear of falling(F=27.38, p=.000). Conclusion: Fear of falling and falls efficacy were related with the bone mineral density. Falls efficacy and BMD may be useful for the predicting fear of falling for women in middle and old age. Further studies with assessment of fall-related risk-factors and a longitudinal study are necessary to assess with falls efficacy, and BMD with age.

Zygomaticotemporal suture maturation evaluation in Chinese population using cone-beam computed tomography images

  • Yifan Li;Ruomei Li;Jiajun Shi;Yuhua Shan;Zhenqi Chen
    • 대한치과교정학회지
    • /
    • 제53권4호
    • /
    • pp.232-240
    • /
    • 2023
  • Objective: This study aimed to evaluate the zygomaticotemporal suture (ZTS) maturation, analyze the age distribution patterns of ZTS maturation stages, and investigate the relationship between ZTS and cervical vertebral maturation (CVM). Methods: A total of 261 patients who underwent cone-beam computed tomography (112 males, mean age, 13.1 ± 3.3 years; 149 females, mean age, 13.7 ± 3.1 years) were examined to evaluate the ZTS stages. The ZTS stages were defined based on a modified method from previous studies on zygomaticomaxillary sutures. Differences between groups and correlations between indicators were analyzed using the Spearman correlation test, intraclass coefficient of correlation (ICC), one-way analysis of variance and rank sum test. Statistical significance was set at p < 0.05. The diagnostic value of CVM stages in identifying ZTS maturation stages was evaluated using positive likelihood ratios (LRs). Results: A positive relationship was found between the ZTS and CVM stage (r = 0.747, ICC = 0.621, p < 0.01) and between the ZTS stage and chronological age (r = 0.727, ICC = 0.330, p < 0.01). Positive LRs > 10 were found for several cervical stages (CSs), including CS1 and CS2 for the diagnosis of stage B, CS1 to CS3 for the diagnosis of stages B and C, and CS6 for the diagnosis of stages D and E. Conclusions: The ZTS maturation stage may be more relevant to the CVM stage than to the chronological age. The CVM stages can be good indicators for clinical decisions regarding maxillary protraction, except for CS4 and CS5.

Influence of Grain Processing and Dietary Protein Degradability on Nitrogen Metabolism, Energy Balance and Methane Production in Young Calves

  • Pattanaik, A.K.;Sastry, V.R.B.;Katiyar, R.C.;Lal, Murari
    • Asian-Australasian Journal of Animal Sciences
    • /
    • 제16권10호
    • /
    • pp.1443-1450
    • /
    • 2003
  • Crossbred (Bos taurus${\times}$Bos indicus) calves were used from birth till 14 weeks of age to evaluate three sources of protein that differed in ruminal degradability viz. groundnut cake alone (HD) or in combination with cottonseed meal (MD) and meat and bone meal (LD), when fed along with two sources of non-structural carbohydrates viz. raw (R) and thermally processed (P) maize. Twenty four new born calves were arranged in six groups in a $3{\times}2$ factorial design and fed on whole milk up to 56 d of age. All the different calves received calf startes along with green oats (Avena sativa) from 14 d of age onwards free-choice. A metabolism trial of 6d starters duration, conducted after 90 d of experimental feeding, revealed greater (p<0.05) digestibility of DM, OM, total carbohydrates, NDF and ADF in calves fed on the P diets than on the R diets promoting greater (p<0.05) metabolizable energy intake. The digestibility of NDF was higher (p<0.01) on LD diets where as calves on MD diets exhibited significantly lower digestibility of ADF (p<0.01). The retention of nitrogen per unit metabolic body size was significantly (p<0.05) higher on the LD-P diet than on the diet HD-P which, in turn, was higher (p<0.05) than that of HD-R. Nitrogen retention as percentage of intake was significantly greater (p<0.05) on LD-P than on LD-R diets (52.2 vs. 36.4%). Also, P fed calves utilized nitrogen more efficiently than the R fed as shown by retention of significantly greater proportions of intake (47.4 vs. 40.9%) and absorbed (65.8 vs. 59.5%) nitrogen. Calorimetric evaluation of the diets through open-circuit respiration chamber revealed that the dietary treatments had no impact on methane production by calves. The intake of DE and ME was improved (p<0.01) because of maize processing resulting in greater (p<0.01) retention of energy. The protein degradability exerted no influence on the partitioning or retention of energy. A significant interaction between cereal and protein types was evident with respect to retention of both nitrogen (p<0.01) and energy (p<0.05). In conclusion, no discernible trend in the influence of cereal processing was apparent on the dietary protein degradability, but the positive effect of cereal processing on energy retention diminished with the increase in dietary undegradability.