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PREVENTION OF SELF-MUTILATION IN PATIENT WITH LESCH-NYHAN SYNDROME: A CASE REPORT (Lesch-Nyhan 증후군 환아의 자해 예방)

  • Lee, Ji-Hyun;Kim, Ji-Hoon;Kim, Jae-Moon;Kim, Shin;Jeong, Tae-Sung
    • Journal of the korean academy of Pediatric Dentistry
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    • v.32 no.2
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    • pp.306-311
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    • 2005
  • Lesch-Nyhan syndrome is a rare disorder of purine metabolism, first described in 1964. The incidence is estimated to be 1:100,000 birth. It is an X-linked recessive disorder in which affected males have a virtually complete deficiency of the enzyme hypoxanthine guanine phosphorybosyl transferase(HGPT). This enzyme deficiency gives rise to excessive uric acid production and consequent hyperuricemia. Lesch-Nyhan syndrome is clinically characterized by mental retardation, choreoathetosis, spastic cerebral palsy, and severe self-mutilation behavior. Patient with Lesch-Nyhan syndrome mostly bite their lip, tongue and finger. In severe cases, partial or total amputation of the lip and tongue is common. Self-inflicted bites are often further complicated by secondary infection to injuried site as well as pain. And tissue loss by biting results in esthetic problems. This report presents a Lesch-Nyhan syndrome patient with self-mutilation, who have a destruction of perioral tissue, especially the lower lip. He was treated successfully with soft mouthguard, psychological and pharmacological method.

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Evaluation of Using Pattern of the Topical Agents in Korean Community (한국 외래형 국소용 피부 외용제의 사용 현황에 대한 기초 평가)

  • Cheon, Young Ju;Kim, Sug Hyun;Kim, Ok Ju;Hong, Se Hwa;Yang, Jin Wook;Kim, Jung Tae;Lim, Sung Cil
    • YAKHAK HOEJI
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    • v.58 no.6
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    • pp.378-386
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    • 2014
  • Topical agents including of steroids are easily exposed to Korean patients in community setting. Those topical agents must be use cautiously because can cause adverse drug reaction unlike other oral medication. We evaluate the using pattern topical agents of Korean in community setting. We performed a questionnaire survey in three different pharmacies (general community pharmacy, dermatology clinic oriented pharmacy, general hospital oriented pharmacy) and in three university town. The survey asked necessity of topical agents, the number of applications per day, application period, site, and exist of steroids ingredient, etc. Total 330 people was participated in the survey from May 1st to July 31st, 2014. We analysed the survey by the frequency of each question and cross-analysis of the compliance between general public and pharmacy groups. In results, Korean people used topical agents for sudden skin diseases (66.9%), was gotten by dermatologist's prescription (89.1%), and did not know their diagnosis (39.2%). The people applied topical agents on face (22.3%), less one half FTU (finger tip unit) per application (61.9%), less than one week (62.9%). Though they was received drug education from pharmacist (52.2%), did not heard about adverse effects (78.4%). And compliance of applied times in pharmacy group was higher than in-non-pharmacy group (56% vs 38%, p=0.002). The highest compliance between 3 pharmacy subgroup was general community pharmacy (76.3% vs 61.6%, 61.2%, p=0.039). Compliance of applied duration was no difference between groups. Majority of respondents did not know diversity of ingredients, potency, application area of topical steroids, and replied to feel some vague reluctance and to think so dangerous about topical steroids. In conclusion, Korean people in community use topical agents well but still some exposed to risk from topical agents.

Cardiovascular Responses over the Time Course during Muscle Group III Stimulation in Prehypertensive Individuals (고혈압 전단계자들에 대한 골격근 Group III 자극 시 시간에 따른 심혈관 반응)

  • Park, Won-Il;Park, Si-Young;Choi, Hyun-Min;Lee, Joon-Hee;Jeon, Jong-Mok;Kim, Jong-Kyung;Shim, Jae-Kun;Nho, Ho-Sung
    • Journal of Life Science
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    • v.19 no.11
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    • pp.1568-1574
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    • 2009
  • The purpose of this study was to investigate whether group III muscle afferents play an important role eliciting abnormal blood pressure response mediated during passive muscle stretch in prehypertensive individuals. Eleven middle-aged prehypertensive men (average BP 133/80 mmHg) and nine middle-aged normotensive men (average BP 119/74 mmHg) participated in this study. After 1 min rest baseline data collection, the subject's foot was flexed (dorsiflexion) by an automated cybex for one minute. Systolic blood pressure (SBP), diastolic blood pressure (DBP), mean arterial blood pressure (MAP), heart rate, stroke volume, cardiac output, and total peripheral resistance were continuously measured on a beat-by-beat basis from a finger via a Finapres device for 1 minute. To evaluate the role of mechanoreflex, a component of exercise pressor reflex, SBP, DBP, and MAP responses over the course of time were examined. The results showed that the pressor response mediated by the muscle mechanoreflex was faster in prehypertensive individuals compared to the normotensive individuals. The substantial pressor response was observed within mean 20 sec of the onset of passive stretch in prehypertension, while mean 45 sec in normotension (p<0.05). It is concluded that excessive pressor response produced during exercise in prehypertension may be due to the dysfunction of the mechano-receptors.

Comparison of Methods to Confirm the Cervical Epidural Space (경추 경막외강 확인법의 비교 연구)

  • Ok, Si Young;Chun, Hae Rim;Baek, Young Hee;Kim, Sang Ho;Kim, Soon Im;Kim, Sun Chong;Park, Wook;Song, Dan
    • The Korean Journal of Pain
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    • v.22 no.2
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    • pp.158-162
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    • 2009
  • Background: The loss of resistance (LOR) method is most commonly used to identify the epidural space. This method is thought to rely on the penetration of the ligamentum flavum. Unfortunately the exact morphology of the ligamentum flavum is variable at different vertebral levels. Especially, it has been pointed out that the lower cervical ligamentum flavum may be discontinuous in the midline in up to 50% of patients. Thus, the LOR method may be inaccurate to confirm the cervical epidural space. The aim of this study is to determine which method is the safest and most exact for confirming the cervical epidural space. Methods: 100 adult, chronic renal failure patients who were undergoing an arteriovenous bridge graft for hemodialysis at the upper arm under cervical epidural anesthesia were recruited for this study. During the cervical epidural puncture, we identified the cervical epidural space by subjectively feeling the resistance with using a finger just through the ligamentum flavum, and we also used the drip infusion method, the loss of resistance method using air, and the hanging drop method. By using 5 grades, we classified the extent of whether or not the techniques were effective. Results: Using the drip infusion method, we identify the epidural space in all the patients as +/++ grade. The catheter insertion method was also successful in identifying those epidural spaces over a ${\pm}$ grade. The pseudo LOR was over ${\pm}$ grade in 47 patients. Conclusions: The combined LOR/hanging drop with drip infusion method is useful for confirming the cervical epidural space.

The Effects of One to One Interactive Picture Book Reading on Two-year-olds' Verbal & Nonverbal Reading Response and Teachers' Language Teaching Efficacy (일대일 상호작용을 통한 그림책 읽기가 만 2세 영아의 언어적·비언어적 읽기 반응과 교사의 언어교수효능감에 미치는 효과)

  • Yoo, Kyung Hee;Choi, Naya
    • Korean Journal of Childcare and Education
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    • v.9 no.5
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    • pp.251-276
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    • 2013
  • This study was performed in order to examine the effects of one to one interactive picture book reading on infants' reading response and teachers' language teaching efficacy. A total of 50 2-year-olds from 8 child care centers in Incheon were divided into an experimental group and a control group. The interactive picture book reading activities were carried out once a week during 12 weeks. The infants in the experimental group read one-to-one with teachers, and their counterparts read the same book in a group. The change in teachers' language teaching efficacy was examined, and their subjective perception was evaluated through in-depth interview. As a result of this research, the infants in the experimental group showed significantly increased verbal response, compared to the control group, both in the total score and most sub-factors such as naming, responding, asking, demanding, spontaneous utterance, and decontextualized utterance. They also represented a significant rise, compared to the control group, in the total score of non-verbal response and factors like finger indicating, imitating, and accepting. Finally, the teachers who led the experimental group showed increased language teaching efficacy, and evaluated the effects of interactive picture book reading with infants very positively.

The New Finding on BOLD Response of Motor Acupoint KI6(照海) by fMRI (fMRI를 이용하여 수지굴신운동(手指屈伸運動)과 조해(照海)(KI6) 자침(刺鍼)에 의(依)한 대뇌운동피질(大腦運動皮質)의 활성변화(活性變化)에 관(關)한 비교(比較) 연구(硏究))

  • Kwon, Cheol-hyeon;Lee, Jun-beom;Hwang, Min-seob;Yoon, Jong-hwa
    • Journal of Acupuncture Research
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    • v.21 no.6
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    • pp.177-186
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    • 2004
  • Introduction : Recent studies Suggested that there is a strong correlation between acupuncture stimulation and its related cortical activation. Anther study showed that either positive or negative BOLD effects could be observed depending on anatomical structure in acupuncture stimulation. In ttis study, we investigated a new acupoint $KI_6$ (照海), which was known as motor-related acupoint and obtained an evidence that the stimulation of $KI_6$ resulted in either negative or positive BOLD response to stimulation. Methods & Results : 1. Subjects and paradigms : Two separate stimulation paradigms were performed on five healthy (aged 22-23 yrs) in this study. First, the paradigm of acupuncture stimulation was that the acupuncture needle was inserted in acupoints $KI_6$, which is located in lateral side of the foot and then continuously twisted(補瀉를 除外한 捻轉法) for 70 seconds for 10 cycles of activation. During rest period (70 seconds), the needle was completed removed from acupoint. Total 60 cycles were performed and 10 images were obtained per cycle. Second, nonacupoint was randomly selected and the same paradigm was performed as acupoint stimulation. The stimulation protocol comprised 10 cycles of alternating. activation and rest (10 images per cycle). Total 60 cycles were performed and each cycle take about 1.5 sec for motor task. Subjects take an at least 15 minutes break before starting anther paradigm. 2. fMRI mapping : Multi-slice functional images were obtained on a 1.5T Magnetom Vision MRI scanner (Simens Medical, Erlangen, Germany) equipped with high performance whole-body gradients. The BOLD T2 * - weighted images were acquired with acho planar imaging sequence (TR = 1.2 sec, TE = 60 msec, and flip angle = $90_{\circ}$). The other sequence parameter are : FOV = 210 mm, matrix=$64{\times}128$ or $64{\times}64$, slice number=10 and slice thickness = 5 or 8 mm. the anatomic images were obtained with Spin-echo T1-weighted images. The resulting images were then anaiyzed with STIMULATE (CMRR, U. of Minnesota) to generate functional maps using a student T-test (p < 0.005) and cluster analysis. Both positive and negative response were evaluated. Conclusions : We have observed the activation of the motor cortex by stimulating motor-related acupoint ($KI_6$). Among five subjects, negative BOLD response was shown in four and positive response in one. All subjects showed positive response to conventional finger flexion-extension task. To understand the detailed mechanisms of correlation between acupuncture stimulation and BOLD fMRI changes and two typs of response, further study strongly required.

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Symbol Timing Alignment and Combining Technique in Rake Receiver for cdma2000 Systems (cdma2000 시스템용 레이크 수신기에서의 심볼 정렬 및 컴바이닝 기법)

  • Lee, Seong-Ju;Kim, Jae-Seok;Eo, Ik-Su;Kim, Gyeong-Su
    • Journal of the Institute of Electronics Engineers of Korea TC
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    • v.39 no.1
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    • pp.34-41
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    • 2002
  • In the conventional rake receiver structure for the IS-95 CDMA system, each finger has its own time-deskew buffer or FIFO that aligns the multipath signals to the same timing reference in order to combine symbols. This architecture is not a burden to the rake receiver design mainly because of the small number and size of the buffers. However, the number and size of the buffers are significantly increased in the cdma2000 system which adopts multiple carriers and the small spreading gain for a higher rate in data services. In order to decrease the number of buffers, we propose a new model of the time-deskew buffers, which combines the symbols as well as realigns them at the same time. Our architecture reduces the hardware complexity of the buffers by about more than 60% and 70% compared with the conventional one when we consider each rake receiver has three and four independent fingers, respectively. Moreover, the proposed algorithm is very useful not only to the cdma2000 rake receiver but also to the receiver with many fingers in order to increase the BER performance.

CHANGES IN THE SHAPE AND ULTRASTRUCTURE OF THE ARTICULAR DISC OF THE RAT MANDIBULAR JOINT WITH AGING (가령에 따른 백서 악관절 원판의 형태 및 미세구조적 변화)

  • Suh, Hye-Kyung;Kyung, Hee-Moon;Sung, Jae-Hyun;Bae, Yong-Chul
    • The korean journal of orthodontics
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    • v.24 no.2
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    • pp.331-348
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    • 1994
  • The purpose of this study was to investigate changes in the shape and ultrastructure of the articular disc of the rat mandibular joint with aging. Mechanical stress applied to the articular disc changes during neonatal, suckling, juvenile, adult and senile stages. Mandibular joints of 6 groups of rats(1-, 7-, 17-, 27-, 55-day and over-1-year groups) were removed en bloc and processed for light and electro microscopic study. The changes in the shape of articular disc were examined by light microscope in each group. Structural and ultrastructural changes in the articular disc were examined by light and electron microscope in each group. The results were as follows : In the 1-day and 7-day groups, the articular disc was long and slender in shape and the articular disc was not fitted with the shape of the mandibular fossa and condyle. However' after that time, the anterior and posterior portions of the articular disc were more bulged and the middle portion was shorter and biconcave. Thus the articular disc was well fitted with the shape of the mandibular fossa and condyle. The cell density decreased with aging. In the l -day and 7-day groups, the Golgi apparatus, rough endoplasmic reticulum and free ribosome, which are involved in the synthesis of intracellular and extracellular matrix, were developed. In the 17-day, 27-day and 55-day groups, not only the cell organelles involved in the synthesis of the intracellular and extracellular matrix but also the cell organelles involved in the remodeling of the extracellular matrix(i.e., finger-like cell process, lysosome and mitochondria)were well developed. With advancing age, intracytoplasmic microfilaments were more accumulated and condroid cells increased. In the over-1-year group, the majority of cells of the articular disc were chondroid cells. The majority of cytoplasmic compartment were filled with intracytoplasmic microfilaments and cell organelles were not developed. Therefore, metabolic activities of the cell was markedly reduced and cells contained structures enduring mechanical stress, and cells which were in the process of degeneration were observed occasionally.

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CLINICAL APPLICATION OF MODIFIED FR-4 (Modified FR-4의 임상적용례)

  • Song, Jae-Hyuk;Lee, Keung-Ho;Choi, Yeong-Chul
    • Journal of the korean academy of Pediatric Dentistry
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    • v.28 no.2
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    • pp.323-328
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    • 2001
  • Anterior open bite is one in which the teeth in the anterior portion of the maxilla and mandible are vertically apart and lack the overlapping necessary for the incisive function when the mandible is in closed position. Anterior open bite is a result of the interaction of many different etiologic factors including thumb and finger sucking, lip and tongue habits, airway obstruction, skeletal growth abnormalities and its tendency may appear with any type of skeletal patterns, such as Class I, II or III malocclusion types. Though the treatment methods for anterior open bite are various, the conventional FR-4, designed by Rolf Fr$\"{a}$nkel, is known to be effective in treating open bite cases with Class I or II skeletal patterns. It is due to that an incidence of skeletal Class II is high in the Occidentals, and open bite is accompanied by these malocclusion type in many cases. However, an incidence of skeletal Class III is high in the Orientals, and open bite is sometimes accompanied by skeletal Class III in many cases. Although the use of the conventional FR-4 was effective in the treatment of open bite, skeletal Class III would be worsened. So, a modified FR-4(placing the labial bow in the lower, the labial pads in the upper) was designed for the treatment of patients showing skeletal Class III and open bite.

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Joint Symptoms During Antituberculous Chemotherapy (결핵 치료 중 나타나는 관절 증상)

  • Kim, Sang-Cheol;Baik, Jae-Joong;Lee, Tae-Hoon;Chung, Yeon-Tae
    • Tuberculosis and Respiratory Diseases
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    • v.49 no.2
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    • pp.162-168
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    • 2000
  • Background : Joint symptoms frequently occur in the course of antituberculous chemotherapy and tend to be ignored and overlooked, but in some cases, they are often very troublesome in obstructing ordinary life. Joint symptoms that develop during antituberculous chemotherapy need to be understood, but there are few materials describing them systematically. Method : This study enrolled 33 patients with tuberculosis treated with first line antituberculous agents for more than 6months. In the course of treatment, joint symptoms not associated with specific cause, such as preexisting joint disease or trauma, were investigated and compared with thæe of the asymptomatic group. We confirmed the incidence of joint symptoms and factors associated with them. Results : Nineteen of 33 patients (58%) had joint symptoms. Joint symptoms developed 1.9$\pm$1.4 months after the beginning of chemotherapy and lasted for 3.6$\pm$2.5months. In 18 of 19 symptomatic patients, multiple joints were involved: shoulder (10 patients, 53%), knee (10, 53%), finger (6, 32 %). Joint symptoms were expressed as pain (19 patiens, 100%), stiffness (7, 37%) and/or swelling (3, 16%). Fourteen patients (74%) took analgesics to relieve their symptoms and in 2 patients, antituberculous agents were discontinued because of the severity of their symptoms. The symptoms seem to be caused by agents other than pyrazinamide, but it was very difficult to identify the definite causative agent. In age, sex, underlying disease and serum uric acid level, no significant differences were noted between the two groups. Conclusions : Although joint symptoms are common during antituberculous chemotherapy, their development is difficult to predict. Because some joint symptoms can become very bothersome, the physician should pay close attention to these symptoms.

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