• 대한유전성대사질환학회지
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• 제11권1호
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• pp.88-98
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• 2011

Lesch-Nyhan syndrome is a X-linked recessive disorder caused by a deficiency of the enzyme hypoxanthine-guanidine phosphoribosyltransferase (HPRT), enzyme to recycle purines. Case history: born induced vaginal delivery at 40 weeks complicated by premature membrane ruputure, body weight 2.820 gm. He showed failure to thrive showing severe protein aversion like milk products and pink daper. Developmental delay revealing rolling over at 10.5 month, followed by regression. Seizure at 2 months, His poor oral feeding was lifelong problem. Weak crying, spastic, choreoathetoid movement. Self mutilating behavior noted and diagnosed at age 3 years. No family history of consanguinity and neurological disorders. Method: Laboratory test, physical exam, imaging study and molecular. Clinical follow up Treat ment with allopurinol. Result: uric acid 10.5 mg/dL (N 3.5-7.9), APRT 151.1uM/ min/ml pro(25.7-101), HPRT 7.6 (N 233.5-701) and c.151C>T hemizygote (p,Arg51X). Abdominal sonogram showed staghorn calculi in both kidneys, brain MRI brain atrophy. Clinical follow up showed, seizure at 2 mo, developmental delay (head control and, rolling over at at 11mo, pointing body part at 2 yr 7 mo, eye hand coordination at 2 y 11mo,creeping at 3 y 7 mo, speaking words at 6 y 6 mo ),and developmental regression at 3 yr of age. Sleeping problem including insomnia and severe constipation. Self mutilating behavior (lip bite) started at 2.5 yr, neurologic sx including intermittent upward gaze accompanied by swallowing difficulty at 3 y 7 mo grand mal seizure at 4.5 yr and spastic extremity and trunchal hypotonia and choleoathetoid movement and ataxia at 6.5 yr. Scoliosis with severe spasticity at 9 yr 9 mo. Acute life threatening episode with irregular breathing at 9 yr and 9 mo, Emaciation and nephrolithiasis and recurrent pneumonia. Died suddenly at 10 yr 3 mo. Conclusion: life long feeding problem, chronic gut motility dysfunction, sleeping difficulty and progressing neurologic deterioration and nephrolithiasis despite normal serum uric acid maintence by allopurinol treatment.

• 한국임상수의학회지
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• 제33권1호
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• pp.30-33
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• 2016

혀 뿌리 부위의 종양, 특히 종양이 정상으로 보이는 점막 아래에 위치해 있는 경우 발견하고 진단하기가 어렵다. 전산화단층촬영법, 특히 조영후 영상은 혀의 종양을 평가하고 경부 림프절을 포함한 인근 조직을 평가하기에 유용하다. 그러나 종양을 확진하기 위해서는 조직병리검사가 필요하다. 본 증례는 개에서 혀의 뿌리부터 몸통에 걸쳐 종양이 발생하였으며 환자는 유연과다 및 섭식장애 증상으로 전원되었다. 구강검사 결과 혀의 운동성 장애가 관찰되었으며 전산화단층촬영으로 혀 종양이 확인되었다. 2달 뒤 사후에 개의 혀에서 조직을 채취하여 조직병리검사를 실시한 결과 혀의 종양은 편평세포암종으로 진단되었다.

• 대한한방내과학회지
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• 제36권4호
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• pp.527-546
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• 2015

Objectives The purpose of this study was to investigate the correlation between gastric emptying measured by ultrasonography and Korean medical instruments of diagnosis and assessment in functional dyspepsia (FD) patients. Among the subgroups of FD, postprandial distress syndrome (PDS) is related to gastric motility disorder.Methods Ten patients with FD and particularly with PDS as well as 10 healthy controls were enrolled in the study from September to November 2015. The gastric emptying shown as the half-life of gastric volume (T_1/2) was measured by ultrasonography. The severities of spleen qi deficiency and dyspepsia symptoms were assessed by a spleen qi deficiency questionnaire (SQDQ) and the Nepean Dyspepsia Index-Korean version (NDI-K), respectively. In addition, a food retention questionnaire (FRQ), a damum questionnaire (DQ), a cold and heat questionnaire (CHQ), a deficiency and excess questionnaire (DEQ), and a visual analogue scale (VAS) of distention and fullness were completed by every participant.Results In comparison with the control group, the FD group showed significantly higher scores for the SQDQ, NDI-K, FRQ, DQ, DEQ, and VAS of distention and fullness. T_1/2 was also significantly higher in the FD group than in the control group. There were significant correlations between T_1/2 and the SQDQ score. However, there were no significant correlations between T1/2 and other questionnaire scores except for one item of the NDI-K.Conclusions According to these findings, it was determined that measuring gastric emptying using ultrasonography could be a quantitative indicator to diagnose spleen qi deficiency in FD patients.

• 한국약용작물학회지
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• 제24권6호
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• pp.471-478
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• 2016

Background: Constipation is one of the most common functional gastrointestinal disorder. The present study examined the ability of water extract of fermented (FRC) and non-fermented (NFRC) roasted Cassia tora to improve intestinal function and reduce constipation in a rat constipation model. Methods and Results: Different concentration of FRC and NFRC were orally administered loperamide (5 mg/kg; LOP) reduced the number, weight, and water content of feces, as well as intestinal transit motility. However, 24 h-(24 hour fermented roasted-Cassia tora) 300 mg/kg FRC administration increased the number, weight, and water concent of feces, compared to that seen in the LOP group, and also improve intestinal transit mitility and, the thickness of distal colon and mucous fluid. Conclusions: The results of the present study indicated that LOP-induced constipation was improved by treatment with FRC. Therefore FRC could be used to develop functional foods or natural medicine for constipation. However, further study is needed to clarify how fermentation improves the medicinal properties of roasted C. tora.

• Journal of Acupuncture Research
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• 제25권5호
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• pp.139-149
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• 2008

Objectives : The aim of this study was to compare the effect of needle retention(NR) and electro-acupuncture of low(EA(L)) and high(EA(H)) frequencies at $SP_6$ and Sham point in rats. Methods : Intestinal hypermotility was induced by feeding carbachol and experimental groups divided mainly into 7 groups which were normal, holder, control, acupuncture in normal state of rats, pre-treatment of acupuncture(NR, EA) in hypermotility, post-treatment of acupuncture(NR, EA) in hypermotility. We fed charcoal to them after the treatment and measured the travel rate of charcoal in the gastrointestinal track so that which treatment affected more in intestinal hypermotility. Results : As the following study, each acupuncture ways of EA(L) had significant effect of decreasing travel rate on intestinal hypermotility than EA(H) and NR. The comparison between pre-treatment and post-treatment, pre-treatment had slight more effect than post-treatment but not significantly. There was more affected at $SP_6$ than Sham point on this study. Conclusions : There were 21 groups to find out which treatment was best to slow down the intestinal motility and $SP_6$-EA(L)-C had significant effect compared with control group at the figure than any other groups. That meant $SP_6$ had effect on gastric disorder such as intestinal hypermotility and its' effect had more prevention than cure. Further study was needed to have more precise effect of EA and $SP_6$.

• 대한핵의학회지
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• 제26권2호
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• pp.299-306
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• 1992

당뇨병 환자에서 $^{99m}Tc-colloid$ labeled scrambled egg와 hamberger를 이용한 고형식의 위배출 양상의 변화를 동위원소를 이용하여 고찰한 결과 당뇨병성 신경증의 정도, 특히 자율신경계 침습 유무, 이환기간 및 망막증의 정도와 관련이 있었고 혈당농도 및 조절상태와는 무관한 것으로 나타나 당뇨병 환자에서 신경장애가 심할수록 이환기간이 길수록 위장운동 장애가 심한 것을 나타내있다. 당뇨병성 위마비증도 자율신경계 침습이 있는 경우에 잘 발생하였고 Cisapride는 당뇨병 환자의 위 배출시간 및 위저류율을 호전시켰다. $^{99m}Tc-colloid$ labeled scrambled egg와 hamberger를 이용한 고형식의 핵의학적 정사 방법은 barium을 이용한 방사선학적 청사보다 정량화하기 쉽고 생리 식염수 부하검사보다 비침습적이며 당뇨병 환자의 실재 일회 식사량과 비슷한 500kCal의 열량으로 임상상을 잘 반영한다고 사료된다.

• 대한핵의학회지
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• 제26권1호
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• pp.86-94
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• 1992

Quantitative analysis of gallbladder emptying is important in diagnosis of motility disorder of gallbladder and in studies of biliary physiology. However, the normal range of gallbladder ejection fraction (GBEF) has not been determined yet and the best method for stimulating the gallbladder to contract has not been elucidated adequately. The purpose of this study was to compare the gallbladder emptying effect of the fatty meal ingestion with that of the continuous infusion of cholecystokinin (CCK) and to establish the normal GBSF values of normal subjects. Quantitative hepatobiliary scan with $^{99m}Tc-DISIDA$ after a fatty meal was performed for 22 normal healthy volunteers. Among them, 10 subjects repeated the test with a fatty meal. Again, for 7 subjects quantitative heaptobiliary scan with an infusion of CCK (sincalide) at a rate of 20 ng/kg/hr for 45 minutes was performed repeatedly. The results were as follows. 1) With a fatty meal, the mean GBEF was $89.6{\pm}8.2%$ in 22 normal subjects, and there was no difference between subjects. 2) With a continuous infusion of CCK, the mean GBEF was $62.4{\pm}16.6%$ in 7 normal subjects, and there was a significant difference between subjects(p<0.05). 3) The reproducibility of GBEF by a fatty meal was significantly higher than by an infusion of CCK (p < 0.05). 4) The mean GBEF by a fatty meal was significantly higher than that by an infusion of CCK (p < 0.05). We concluded that a fatty meal is superior to a continuous infusion of CCK for inducing gall-bladder contraction because that induces more complete emptying and the response is more reproducible and constant.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제23권6호
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• pp.521-530
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• 2020

Purpose: Malnutrition is a common feature in critically ill children. Enteral nutrition (EN) is the main strategy to nutritionally support critical ill children, but its use can be hindered by the development of intolerance. The study aimed to assess the effectiveness and safety of amoxicillin/clavulanate (A/C) to treat EN intolerance. Methods: We retrospectively evaluated patients admitted to the pediatric intensive care unit from October 2018 to October 2019. We conducted a case-control study: in the first 6 months (October 2018-April 2019) we implemented the nutritional protocol of our Institution with no drug, whereas in the second half (May 2019-October 2019) we employed A/C for 1 week at a dose of 10 mg/kg twice daily. Results: Twelve cases were compared with 12 controls. At the final evaluation, enteral intake was significantly higher than that at baseline in the cases (from 2.1±3.7 to 66.1±27.4% of requirement, p=0.0001 by Wilcoxon matched-pairs signed rank test) but not in the controls (from 0.2±0.8 to 6.0±14.1% of the requirement, p=NS). Final gastric residual volume at the end of the observation was significantly lower in the cases than in the controls (p=0.0398). The drug was well tolerated as shown by the similar safety outcomes in both cases and controls. Conclusion: Malnutrition exposes critically ill children to several complications that affect the severity of disease course, length of stay, and mortality; all may be prevented by early EN. The development of intolerance to EN could be addressed with the use of A/C. Future prospective clinical trials are needed to confirm these conclusions.

• 대한후두음성언어의학회지
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• 제23권1호
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• pp.33-42
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• 2012

Background and Objectives : Dysarthria refers to speech disorder that causes difficulties in speech communication due to paralysis, muscle weakening, and incoordination of speech muscle mechanism caused by damaged central or peripheral nerve system. Pitch, strength and speed are influenced by dysarthria during detonation due to difficulties in muscle control. As evaluation items, alternate motion rate and diadochokinesis have been commonly used, and articulation is also an important evaluation items. The purpose of this study is to find acoustic characteristics on sound production of dysarthria patients. Materials and Methods : Research subjects have been selected as 20 dysarthria patients and 20 subjects for control group, and voice sample was composed of bilabial, alveolar sound, and velar sound in diadochokinetic rate, while consonant articulation test was composed of bilabial plosive, alveolar plosive, velar plosive. Analysis items were composed of 1) speaking rate, energy, articulation time of diadochokinesis, 2) voice onset time (VOT), total duration (TD), vowel duration (VD), hold of plosives. Results and Conclusions : The number of diadochokinetic rate of dysarthria was smaller than control group. Both control group and dysarthria group was highly presented in the order of /t/>/p/>/k/. Minimum energy range per cycle during diadochokinetic rate of dysarthria group was smaller than control group, and presented statistical significance in /p/, /k/, /ptk/. Maximum energy range was larger than control group, and presented statistical significance in /t/, /ptk/. Articulation time, gap, total articulation time during diadochokinetic rate of dysarthria group was longer than control group and presented statistical significance. The articulation time was presented in both control group and dysarthria group in the order of /k/>/t/>/p/, while Gap was presented in the order of /p/>/t/>/k/ for control group and /p/>/k/>/t/ for dysarthria group. VOT, TD, VD regarding plosives of dysarthria group were longer than control group. Hold showed large deviation compared to control group that had appeared due to declined larynx and articulation organ motility.

• 핵의학기술
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• 제18권2호
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• pp.8-16
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• 2014

간담도 스캔에서 GBEF의 산출은 정적영상법과 동적영상법으로 가능하다. 이번 연구를 통해 영상수집 방법에 따른 GBEF 차이를 알아보고, 정확한 GBEF를 산출하기 위한 방법을 강구하고자 한다. 본원의 환자 27명을 대상으로 하였으며, $^{99m}Tc$-mebrofenin을 정맥주사한 후 60분 동안은 정적영상법으로 영상을 수집하고 담낭이 잘 관찰되면 지방식(우유 $200m{\ell}$, 삶은 계란 1개)을 섭취하도록 하였다. 지방식 섭취 후 동적 영상법으로 60 sec/frame, 30분간 영상을 수집하고, 종료 후 60분 영상과 같은 조건으로 90분 영상을 수집하였다. 정적 GBEF는 지방식 전 후 정적 영상의 담낭계수를 이용하여 산출하고, 동적 GBEF는 시간-방사능곡선 상에 $T_{max}$와 $T_{min}$의 담낭계수를 이용하여 산출하였다. 또한 정적영상법의 지방식 전 영상과 동적영상법의 1분째 영상을 이용하여 지방식을 섭취하는 동안의 담즙배출량(초기담즙배출량)을 산출하였다. 결과는 $T_{max}$가 1분 이하인 경우는 20 case였으며, 정적영상법이 평균 $11.4{\pm}6.7%$ 높게 산출되었다(동적 : $40.1{\pm}21.7%$, 정적 : $51.5{\pm}23.6%$). $T_{max}$가 1분을 초과한 경우는 7 case였으며, 동적영상법이 평균 $-9.7{\pm}4.9%$ 높게 산출되었다(동적 : $31.0{\pm}19.7%$, 정적 : $21.3{\pm}19.4%$). 정확한 비교를 위해 영상법에 따른 담즙배출차이와 초기배출량을 지방식 전 담낭계수와의 비율로 부터 산출하였다. $T_{max}$가 1분 이하인 경우, 영상법간 차이는 평균 $16.7{\pm}13.6%$, 초기배출량은 평균 $12.7{\pm}13.8%$로, 오차범위 이내에서 일치하였다. 이는 영상법간 GBEF의 차이가 동적영상법이 시작되기 전에 이미 배출된 담즙때문이라는 것을 의미한다. $T_{max}$가 1분을 초과한 경우, 영상법간 차이는 평균 $-14.3{\pm}7.3%$, 초기배출량은 $-5.9{\pm}3.9%$로, 8.4% 차이가 났다. 이는 지방식 섭취를 시작한 시점부터 동적영상법을 시작한 후 일정 시점($T_{max}$)까지 계속 담즙이 집적되고 있음을 의미하며, 이 때문에 영상법간에 GBEF가 차이가 나는 것으로 판단된다. 정확히 30분 동안의 GBEF 산출하기 위해서, $T_{max}$가 9.5분 이상인 4 case를 추세 분석하여 GBEF를 산출한 결과, 평균 $57.2{\pm}20.4%$로, 정적영상법 (평균 $37.8{\pm}20.9%$) 보다는 20%정도, 동적영상법(평균 $42.0{\pm}16.2%$) 보다는 15% 정도 높게 산출되었다. 정적영상법은 비교적 간단하게 GBEF을 산출할 수 있으며, 환자의 움직임 등 여러 가지 오류로부터 영향을 적게 받는다는 장점이 있다. 반면에 $T_{max}$ 이전에 담낭자극을 시행한 경우이거나 환자의 상태에 따라서 담낭자극에 반응이 느린 경우엔 GBEF가 낮게 산출된다. 동적영상법은 담낭에서 담즙이 배출되는 양상을 분석할 수 있다는 장점이 있다. 배출이 지연되는 경우에도 T-A curve를 통해 $T_{max}$ 시점을 알 수 있기 때문에 정확한 GBEF를 산출할 수 있다. 하지만 지방식을 섭취한 후에 검사가 시작되기 때문에 지방식을 섭취하는 동안에 배출되는 답즙이 제외되어 GBEF가 낮게 산출된다. 연구 결과 간담도 스캔을 통해 정확한 GBEF를 산출하기 위해서는 우선적으로 동적영상법을 시행하여 T-A curve를 통해 담즙배출양상($T_{max}$의 시점)을 확인한 후에 두 영상법 중 적합한 GBEF를 선택하는 것이 바람직하다고 본다.