• Journal of Korean Dental Science
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• 제16권2호
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• pp.204-210
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• 2023

Lesch-Nyhan syndrome is a rare X-linked recessive disorder characterized by a deficiency of the enzyme hypoxanthine-guanine phosphoribosyl transferase, which is important in the metabolism of purines. Prevalence of Lesch-Nyhan syndrome is 1:100,000 to 1:380,000 live births, and Lesch-Nyhan syndrome mainly affects males. It is characterized by neurologic dysfunction, uric acid overproduction, and cognitive and behavioral disturbances. The self-mutilation is the most distinctive symptom in Lesch-Nyhan syndrome, appearing about 1 year of age. It is expressed as persistent bites in the oral mucosa, tongue, lips, and fingers, which leads to the total or partial destruction of the affected tissues. Several medical and dental management methods might be performed in Lesch-Nyhan syndrome patients. Dental approaches to prevent injuries caused by self-mutilation include treatments with oral appliances such as mouth guards or lip bumpers, extraction, and orthognathic surgery. This report described two brothers with Lesch-Nyhan syndrome, who injured themselves on oral tissues. The methods of tooth reduction and extraction were performed to prevent injuries caused by self-mutilation.

• Journal of Korean Neurosurgical Society
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• 제46권5호
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• pp.468-471
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• 2009

Objective : The adolescent presentation of tethered cord syndrome (TCS) is well-recognized, but continues to pose significant diagnostic and management controversies. The authors conducted a retrospective study of clinical outcomes after surgical intervention in 24 school-aged children, adolescents, and young adults with TCS. Methods : All 83 patients with a lipomyelomeningocele (LMMC) underwent untethering surgery for caudal cord tethering between 1987 and 2007. The clinical charts and follow-up data were reviewed. Of these patients, 24 school-aged children, adolescents, and young adults with TCS were studied with respect to the clinical, radiologic, pathologic features, and surgical outcomes. Results : Untethering procedures were performed in 24 patients (age range, 7-25 years) for TCS of various origins (lipoma, lipomyelomeningocele, and tight filum terminale). Specific circumstances involving additional tugging of the already tight conus, and direct trauma to the back precipitated the onset of symptom in 50% of the patients. Diffuse and non-dermatomal leg pain, often referred to the anorectal region, was the most common presenting symptom. Progressive sensorimotor deficits in the lower extremities, as well as bladder and bowel dysfunction, were also common findings, but progressive foot and spinal deformities were noted less frequently. The most common tethered lesions were intradural lipomas, thickened filum and fibrous band adhesions into the placode sac. The surgical outcome was gratifying in relation to pain and motor weakness, but disappointing with respect to resolution of bowel and bladder dysfunction. Of the 24 patients with TCS, pre-operative deficits improved after surgery in 14 (58.3%). remained stable in 8 (33.4%). and worsened in 2 (8.3%). Conclusion : The pathologic lesions of tethered cord syndrome in school-aged children, adolescents, and young adults, are mostly intradural lipomas and tight filum. It is suggested that the degree of cord traction results in neurologic dysfunction in late life due to abnormal tension, aggravated by trauma or repeated tugging of the conus during exercise. Early diagnosis and adequate surgical release might be the keys to the successful outcome in school-aged children, adolescents, and young adults with TCS.

• Annals of Clinical Neurophysiology
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• 제3권2호
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• pp.136-142
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• 2001

Background : Nervous system dysfunction is a major complication of end stage renal disease. Although severe neurologic symptoms are partially or completely reversed by adequate hemodialysis, even optimally dialyzed patients will usually not return to normal neurocognitive function. To investigate the influence of chronic renal failure and hemodialysis on higher cognitive function electrophysiologically, we studied auditory P300 event-related potentials in 14 hemodialysis patients and 14 age- and sex-matched normal healthy controls. Methods : The subjects consisted of 14 patients(M: 6, F: 8) with chronic renal failure(CRF) for 1 to 10 years and 14 age- and sex-matched healthy controls(M: 5, F: 9). For the reliability of study, patients with diabetes mellitus, abnormal brain CT findings, or low mini-mental state score(below 20) were excluded. Event related potentials(ERPs) for hemodialysis patients were performed at pre- and post-hemodialysis. To obtain ERPs, subjects underwent 2-tone auditory discrimination test(oddball paradigm). Results : Although the age(control: $48.79{\pm}10.31years$, CRF: $51.21{\pm}7.61years$) and mini-mental state score(control: $27.00{\pm}1.71points$, predialysis CRF: $25.07{\pm}3.58points$) were not different in normal control and CRF groups significantly(P>0.05), P300 latencies at Cz(control: $288.11{\pm}17.36msec$, predialysis CRF: $332.35{\pm}42.34msec$) were significantly delayed(P<0.05)and the duration of Trail making test A was significantly prolonged(control: $64.2{\pm}24.2sec$, CRF: $118.9{\pm}101sec$) in CRF group. P300 latencies between pre- and post-hemodialysis CRF patients(predialysis CRF: $332.35{\pm}42.34msec$, postdialysis CRF: $325.82{\pm}38.69msec$) were not significantly different. The P300 latency was not related with the duration of CRF(Spearman's correlation test, r=0.25, P>0.05) and the frequency of hemodialysis(Spearman's correlation test, r=0.28, P>0.05). Conclusions : From these results, we suggest that P300 latency is valuable in evaluating cognitive brain dysfunction in patients with CRF and hemodialysis does not have a significant effect on cognitive brain dysfunction in patients with CRF.

• 가정∙방문간호학회지
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• 제6권
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• pp.78-87
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• 1999

From 1990 to 1997 Home Care Education Programs have been offered at 11 Home Care Education Institutes. But there have been no revisions in the program. Especially in the clinical practicum, the Ministry of Health and Welfare proposed 248 hours as 'Family Nursing and Practice'. But each of institutes has developed their own program, and the clinical practicum is very different. Institutions for home care practice have been very limited and even now only 40 hospitals started home care in the second demonstration project. A few community-based institutions have also been offering home care services. This study was conducted to analyze home care clinical practicum offered at Y university, and plan for a revised home care clinical practicum. Y university clinical practicum was revised to include 'visits to community institutions', 'laboratory practice', 'hospital practice', 'discharge planning and home care practice', and 'home care specialty practice'. The results of the evaluation and plan for a revision are as follows: 'Visits to community institutions' is a practice that helps the students understand community resources which are available to home care nurses, and as an orientation to institutions for practice. 'Laboratory practice' is to used to improve nursing skills that are applicable to home care. Problems that the students identified in the laboratory practice were 'lack of opportunity for individual practice', and 'inadequate theoretical preparation for practice'. To address these problems the basic nursing skills laboratory was open and could be used freely by the home care students, and practice could be done after the theoretical lectures. 'Hospital practice' is a practicum in which the students apply nursing skills to patients and to obtain assessment skills for discharge planning. Using a preceptorship, five days for hospital practice should be offered. 'Discharge planning and home care practice' was done at Wonju Christian Hospital. This institute is too far away that this practice should be provided at different institutions as soon as it is possible to contract with home care institutions. Patients groups in different home care institutions are very different, so the 'Home care specialty practice' should be done after analyzing patient groups and choosing specialty areas. These areas are' care of patients with respiratory dysfunction', 'care of patients with neurologic dysfunction', 'care of cancer patients', 'care of patients on dialysis', and 'wound care'. This practice should be offered with a preceptorship, so preceptors, clinical directors, and students should meet for home visit orientation.

• 한방재활의학과학회지
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• 제24권1호
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• pp.47-53
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• 2014

Objectives The aim of this review is to analyse the study tendency in papers related with sibjeondaebotang (Shiquan dabutang) which are published in Korea from 2000 to 2013. Methods We searched the four electronic database (NSDL, RISS, Korean traditional knowledge portal, OASIS) and checked relevant Korean journals from 2000 to 2013. We classified the papers by publish date, speciality, study method, and field of study, and analysed the study tendency. Results 1. 4 papers were published annually on average. 2. After classifying papers by the speciality of journal, continuous study was followed not only in korean medicine, but also in many specialities like Dietetics, Biotechnology and Pharmacology. 3. In study methods, clinical case was 29%, in vivo was 26%, and in vitro was 26%. 4. After classifying papers by field of study, beneficial effect was 62%, toxicity was 14%, qualitative analysis was 9%, and adverse effect and pharmacology was 3%. 5. In beneficial effect, it is effective in antioxidation, treatment and prevention of neurologic disease, skin disease, gynecologic disease and so on. It has also an effect in enhancing the immune system, improving the dysfunction of organs, and it can also be used for anticancer and anti-metastatic purpose. Conclusions These results suggest that Sibjeondaebotang (Shiquan dabutang) can be used as cure medicine, not just as herbal tonic, but there are not sufficient evidence based papers, so there should be further studies in order to establish Sibjeondaebotang as a cure medicine.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제30권6호
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• pp.482-487
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• 2004

Aims: This study was designed to determine the incidence of altered sensation in patients undergoing orthognathic surgery. Method: Seventy two patients who underwent orthognathic surgery between January, 1999 and December, 1999 constituted the study group. Seven patients were excluded because of lack of follow up. Sixty five patients were followed using objective and subjective neurologic testing during the period immediately following operation, 1 month, 2 months, 6 months, and 1 year postoperatively. Age ranged from 17 to 38 years, with a mean of 24.5 years. Male patients were 21, female 44. Twenty eight bilateral sagittal splitting ramus osteotomy(BSSRO) of mandible were performed, 35 BSSRO with genioplasty, 2 genioplasties. Information on the degree of intraoperative nerve encounter was obtained from the surgical reports in 47 patients and was divided into the following three categories: (1) the nerve was not encountered in 23 patients; (2) the nerve was exposed in 11 patients; (3) the nerve was exposed and repositioned from the proximal segment in 13 patients. Results: Four patients reported altered nerve sensation of lower lip and/or chin(6.2%) at final follow up. Two patients underwent BSSRO and the other two patients BSSRO with genioplasty. Three of the patients underwent nerve exposure during the operation. Conclusion: We suggest that the nerve exposure during the operation might be partly responsible for nerve dysfunction after orthognathic surgery.

• The Korean Journal of Pain
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• 제10권1호
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• pp.5-10
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• 1997

Background : The Current Perception Threshold (CPT) provides an objective, quantitative gauge of sensory nerve integrity which is obtainable from any cutaneous site. CPT measurement can confirm and quantify or rule out dysfunction of nerve through comparison with established normative values ($Neuval^{TM}$ CPT database). The aim of this study is to compare collected data from Korean adults with $Neuval^{TM}$ CPT database. Method : Normative data from 5 standard test site in face, hand, toe were obtained from 50 healthy adults. Three frequencies(5, 250, 2000 Hz) were stimulated with $Neuromoter^{(R)}$ CPT device. Results : The results of our data were statistically significantly different than Neuval data except in face, but within normal range. Sensory Threshold increased as the frequency of the stimulus changed from 5 Hz to 250 Hz to 2000 Hz., and from face to hand to toe. Conclusion : CPT testing is a valuable neurologic testing modality that is noninvasive and highly reliable for diagnosis and evaluation of sensory nerves where neuropathy is suspected.

• Journal of Korean Neurosurgical Society
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• 제29권8호
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• pp.1037-1042
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• 2000

Objective : The goal of this study was to recognize the clinical feature and associated risk factors in spinal epidural abscess(SEA), and to improve the outcome accordingly. Methods : A retrospective study was performed in 14 patients with SEA who underwent surgical intervention at our hospital between 1990 and 1999. Results : After a mean follow-up period of 10.2 months(range, 1-57 months), 8 patients had no or minimal deficits, 4 patients had severe paresis or plegia and/or bowel/bladder dysfunction, and 2 patients died due to medical complications. Staphylococcus aureus was the predominant pathogen, isolated in 9 patients(64.3%). Cervical and thoracic spinal epidural abscesses showed a tendency to develop rapidly and were associated with severe neurological deficits. Conclusion : Thoracic spinal epidural abscesses were associated with a poorer prognosis than those in other regions. Therefore, it should be treated more aggressively. Good neurological recovery can be obtained despite severe neurologic deficit when treated by early diagnosis and prompt surgical intervention.

• Journal of Korean Neurosurgical Society
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• 제58권1호
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• pp.22-29
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• 2015

Objective : Perinatal hypoxic-ischemic encephalopathy (HIE) and prolonged febrile seizures (pFS) are common neurologic problems that occur during childhood. However, there is insufficient evidence from experimental studies to conclude that pFS directly induces hippocampal injury. We studied cognitive function and histological changes in a rat model and investigated which among pFS, HIE, or a dual pathologic effect is most detrimental to the health of children. Methods : A rat model of HIE at postnatal day (PD) 7 and a pFS model at PD10 were used. Behavioral and cognitive functions were investigated by means of weekly open field tests from postnatal week (PW) 3 to PW7, and by daily testing with the Morris water maze test at PW8. Pathological changes in the hippocampus were observed in the control, pFS, HIE, and HIE+pFS groups at PW9. Results : The HIE priming group showed a seizure-prone state. The Morris water maze test revealed a decline in cognitive function in the HIE and HIE+pFS groups compared with the pFS and control groups. Additionally, the HIE and HIE+pFS groups showed significant hippocampal neuronal damage, astrogliosis, and volume loss, after maturation. The pFS alone induced minimal hippocampal neuronal damage without astrogliosis or volume loss. Conclusion : Our findings suggest that pFS alone causes no considerable memory or behavioral impairment, or cellular change. In contrast, HIE results in lasting memory impairment and neuronal damage, gliosis, and tissue loss. These findings may contribute to the understanding of the developing brain concerning conditions caused by HIE or pFS.

• 대한핵의학회지
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• 제26권1호
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• pp.26-32
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• 1992

Minimal deterioration of cerebral perfusion or microanatomical changes were undetectable on conventional Brain CT or MRI. So evaluation of focal functional changes of the brain parenchyme is essential in chronic head injury patients, who did not show focal anatomical changes on these radiological studies. However, the patients who had longstanding neurologic sequelae following head injury, there had been no available imaging modalities for evaluating these patients precisely. Therefore we tried to detect the focal functional changes on the brain parenchyme using $^{99m}Tc-HMPAO$ Brain SPECT on the patients of chronic head injuries. Twenty three patients who had suffered from headache, memory dysfunction, personality change and insomnia lasting more than six months fellowing head injury were included in our cases, which showed no anatomical abnormalities on Brain CT or MRI. At first they underwent psychological test whether the symptoms were organic or not. Also we were able to evaluate the cerebral perfusion changes with $^{99m}Tc-HMPAO$ Brain SPECT in 22 patients among the 23, which five patients were focal and 17 patients were nonfocally diffuse perfusion changes. Thus we can predict the perfusion changes such as local vascular deterioration or functional defects using $^{99m}Tc-HMPAO$ Brain SPECT in the patients who had suffered from post-traumatic sequelae, which changes were undetectable on Brain CT or MRI.