• Clinical and Experimental Pediatrics
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• 제57권9호
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• pp.410-415
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• 2014

Purpose: Neurofibromatosis 1 (NF1) is an autosomal dominant condition caused by an NF1 gene mutation. NF1 is also a multisystem disorder that primarily affects the skin and nervous system. The goal of this study was to delineate the phenotypic characterization and assess the NF1 mutational spectrum in patients with NF1. Methods: A total of 42 patients, 14 females and 28 males, were enrolled in this study. Clinical manifestations and results of the genetic study were retrospectively reviewed. Results: Age of the patients at the time of NF1 diagnosis was $15.8{\pm}14.6$ years (range, 1-62 years). Twelve patients (28.6%) had a family history of NF1. Among the 42 patients, $Caf\acute{e}$-au-lait spots were shown in 42 (100%), neurofibroma in 31 (73.8%), freckling in 22 (52.4%), and Lisch nodules in seven (16.7%). The most common abnormal finding in the brain was hamartoma (20%). Mental retardation was observed in five patients (11.9%), seizures in one patient (2.4%), and plexiform neurofibromas (PNFs) in four patients (9.5%). One patient with PNFs died due to a malignant peripheral nerve sheath tumor in the chest cavity. Genetic analysis of seven patients identified six single base substitutions (three missense and three nonsense) and one small deletion. Among these mutations, five (71.4%) were novel (two missense mutations: p.Leu1773Pro, p.His1170Leu; two nonsense mutations: $p.Arg2517^*$, $p.Cys2371^*$; one small deletion: $p.Leu1944Phefs^*6$). Conclusion: The clinical characteristics of 42 Korean patients with NF1 were extremely variable and the mutations of the NF1 gene were genetically heterogeneous with a high mutation-detection rate.

• Toxicological Research
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• 제13권4호
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• pp.317-322
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• 1997

Acute delayed neurotoxicity of KH-502 [O.O-Diethyl O-(1-phenyyl-3-trifluoromethyl-5-pyrazoyl) thiophosphoric acid ester], an insecticide synthesized newly in Korea, was studied in White Leghorn hens. The doses were determined on the basis of preliminary $LD_{50}$ study. High, middle and low doses were determined to be 1123 mg/kg, 762 mg/kg and 518 mg/kg, respectively. The animals were pretreated with atropine (30 mg/kg) prior to administration of KH-502. The chemical was administrated at the first and 21st day of the study. As positive controls, animals were admlnistrated with triorthocresylphosphate (TOCP 1000 mg/kg and 500 mg/kg). Animals administrated with TOCP or KH-502 were sacrificed by perfusion-fixation at 21st and 42nd day of the study, respectively. The central and peripheral nerve tissues were routinely treated for microscopic observation. As results, eight, three, one, and one chickens died within 2 day after adminiatration with signs of cholinergic acute toxicity in high, middle low and TOCP dose-group (500 mg/kg), respectively. No abnormal clinical signs were observed in the survived chickens administrated with KH-502 in the duration of the study. The chickens in positive control groups showed ataxia and incoordination at the 14th day after administration of TOCP. From necropsy, macroscopic changes were not observed in all groups including positive control groups. Histopathologically, oxonal swelling with myelin loss, focal gliosis, distention around axonal space were observed in the spinal cords of the chickens administrated with TOCP 1000 mg/kg. The lesions were distinct in the dorsal and lateral funiculi of cervical spinal cord, in the lateral and ventral funiculi of thoracic spinal cord and in ventral funiculi of lumbosacral spinal cord. Axonal swelling and mlcrogliosis were infrequently observed in the chickens of other groups including negative control one. However, they were nonspecifically distributed in the spinal cords. In this study, we concluded that the new chemical, KH-502 did not have acute delayed neurotoxicity in White Leghorn hens.

• 동의생리병리학회지
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• 제21권2호
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• pp.432-437
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• 2007

Reactive oxygen species (ROS) are toxic agents that may be involved in various neurodegenerative diseases. Recent studies indicate that ROS are also involved in persistent pain through a spinal mechanism. In the present study, whole cell patch clamp recordings were carried out on substantia gelatinosa (SG) neurons in spinal cord slice of neonatal rats to investigate the effects of ROS on neuronal excitability and excitatory synaptic transmission. In current clamp condition, tert-buthyl hydroperoxide (t-BuOOH), an ROS donor, induced a electrical hyperexcitability during t-BuOOH wash-out followed by a brief inhibition of excitability in SG neurons. Application of t-BuOOH depolarized membrane potential of SG neurons and increased the neuronal firing frequencies evoked by depolarizing current pulses. Phenyl-N-tert-buthylnitrone (PBN), an ROS scavenger, antagonized t-BuOOH induced hyperexcitability. IN voltage clamp conditions, t-BuOOH increased the frequency and amplitude of spontaneous excitatory postsynaptic currents (sEPSCs). In order to determine the site of action of t-BuOOH, miniature excitatory postsynaptic currents (mEPSCs) were recorded. t-BuOOH increased the frequency and amplitude of mEPSCs, indicating that it may modulate the excitability of the SG neurons via pre- and postsynaptic actions. These data suggest that ROS generated by peripheral nerve injury can induce central sensitization in spinal cord.

• 대한수의학회지
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• 제32권4호
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• pp.609-627
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• 1992

The study was carried out to obtain the basic data for types, incidence and histopathological features of the spontaneous tumors of the specific pathogen free (SPF) Ktc : ICR mice bred in a barrier system in Technology Research Center, Korea Research Institute of Chemical Technology. One hundred of the mice consisted of 50 males and 50 females were examined for 18 months. 1. The overall incidence rate of spontaneous tumors was 51(51%) of 100 heads tested. The male mice showed slightly higher incidence(28%) than the female(23%), and the incidence rate and the number of affected organs were increased with the increasing age of mice. 2. The incidence rate of primary tumor was 59(59%) of 100 heads tested, consisted of 30 cases (50.8%) of benign tumors and 29 cases(49.2%) of malignant tumors. Among the malignant tumors twenty cases were metastasized to various organs. 3. In tumor incidence rates by systems and organs, the male mice showed the high incidence rate in the liver (18%), hematopoietic system (16% ) and lung (14%), while the female mice, in the hematopoietic system(18%), lung(12%), liver(8%) and uterus(8%). 4. The tumors showing the particularly low incidence rates (<1.0%) were rhabdomyosarcoma in the skeletal muscle, malignant schwannoma in the peripheral nerve, cortical adenoma in the adrenal gland, transitional cell carcinoma in the urinary bladder, tubular cell adenoma in the kidney and adenoma in the pituitary gland and harderian gland.

• Tuberculosis and Respiratory Diseases
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• 제50권2호
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• pp.258-264
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• 2001

저자들은 비염과 기관지 천식의 병력이 있으면서 열감, 설사, 신경염 증상과 함께 양측성 흉막액과 심낭액 그리고 페침윤을 보인 환자에서 흉박액 성분검사상 저당, 산성 삼출액 소견과, 폐조직 검사상 호산구 침윤과 혈관염 및 육아종성 염증 소견을 보인 Churg-Strauss 증후군 1예를 경험하였기에 이에 문헌고찰과 함께 보고하는 바이다.

• Tuberculosis and Respiratory Diseases
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• 제53권2호
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• pp.183-189
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• 2002

저자들은 기침, 객담, 그리고 양측 하지의 부종과 저린감을 주소로 내원한 환자를 신경과 근육의 조직 검사를 시행하여, 폐장 및 신경근계를 침범한 Churg-Strauss 증후군으로 확진하여 스테로이드로 성공적으로 치료한 1 예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• 한국전문물리치료학회지
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• 제5권3호
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• pp.11-20
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• 1998

The purposes of this study were to determine the effect of different degrees of severity of diabetic neuropathy on balance function, and to evaluate dynamic balance and functional performance in diabetes patients. Twenty-four subjects with diabetes mellitus were divided into three groups according to results of sensory nerve conduction study. All subjects were evaluated for dynamic balance which was measured using computerized dynamic posturography, and functional performance which was measured using the Berg balance scale. One-way analysis of variance was used to determine whether there were any statistically differences of dynamic balance function and functional performance among the three groups. The Spearrnan's rank correlation was used to determine statistical significance between dynamic balance and age. The results were as follows: 1. Dynamic balance measured using computerized dynamic posturography was significantly lower in the no response group than in the normal amplitude group (p<0.05). 2. Functional performance tested by the Berg balance scale was not statistically different among the three groups (p>0.05). 3. an inverse relationship was found between dynamic balance measured using computerized dynamic posturography and age (r=-0.68, p<0.05). These results suggest that patients with severe diabetic neuropathy have loss of dynamic balance function. Therefore, patients with severe diabetic neuropathy need to have their balance evaluated and receive appropriate education.

• 근관절건강학회지
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• 제10권1호
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• pp.44-53
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• 2003

Purpose : This study was to identify Korean terms appropriate for a new scale using pain terms to assess the pain of the arthritis patients. Method : This study was conducted by secondary analysis of two previous studies, one from Kim's data (1994) and the other from Lee and Choi's(1988). The subjects were 482 patients with arthralgia. Data were analyzed by SPSS Win 11.0, for frequencies, percentages, and means. Results : Five pain groups were sorted out from the sensory pain category, three in the affective pain category, and two in the miscellaneous pain category by selecting groups of pain terms expressed more than 50% by all subjects. Local inflammatory repeated pain terms, simple irritative pain terms, superficial pressure pain terms, traction pain terms, and peripheral nerve terms were included in the sensory pain category, fatigue-related pain terms, digestion-related terms, and suffering-related terms in the affective category, and dull pain terms and body cavity pressure pain category. Among five sample groups terms selected by more than 3 groups with more than 30% of patients were dugumhada, sikungorinda, wooksinwooksinhada, nurudusi apuda, and dangginda in the sensory pain category; nogonhage apuda, gobukhada, and jajungsurupge apuda in the affecte pain category; and buctjockgigunhada and manjimyon apuda in the miscellaneous pain category. Conclusion : Ten pain groups were tentatively selected out of 20 pain groups; 10 pain terms composed of 1 pain term from each group were selected out of 90 pain terms from thesecondarydataofarthriticpainpatients. Implications More detailed clinical studies are needed for the refinement of the scale.

• 한국생물물리학회:학술대회논문집
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• 한국생물물리학회 2003년도 정기총회 및 학술발표회
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• pp.55-55
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• 2003

P2X$_3$ receptor, a member of P2 purine receptors, is a ligand-gated ion channel activated by extracellular ATP as an endogenous ligand, and highly localized in peripheral and central sensory neurons. The activation of P2X3 receptor by ATP as the pronociceptive effect has been known to initiate the pain signaling involved in chronic inflammatory nociception and neuropathic pain by nerve injury, implicating the possibility of new drug development to control pains. In this study, we have developed a two electrode voltage clamp (TEVC) assay system to evaluate the inhibitory activity of several newly synthesized PPADS and a novel non-ionic antagonist against ATP activation of human P2X3 receptor. PPADS derivatives include several pyridoxine and pyridoxic acid analogs to study the effects of phosphate and aldehyde functional groups in PPADS. All new PPADS analogs were less potent than PPADS at human P2X$_3$ receptors, however, LDD130, a non-ionic analog showed potent antagonistic property with $IC_{50}$/ of 8.34 pM. In order to uncover the structure activity relationships of LDD130, and design new structural analogs, we synthesized and investigated a few structural variants of LDD130, and the results will be discussed in this presentation.

• Journal of Acupuncture Research
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• 제18권6호
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• pp.215-224
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• 2001

Objcetive : Neuropathic pain sometimes arises from a partial peripheral nerve injury. This kind of pain is usually accompanied by spontaneous burning pain, allodynia and hyperalgesia. It has been well known that acupuncture is effective to the pain control from ancient time in Asia. However, it is not clear whether acupuncture can control neuropathic pain. The aim of the present study is to examine if acupuncture stimulation may be effective to the mechanical allodynia in a rat model of neuropathic pain. Methods : To produce neuropathic pain, under sodium pentobarbital anesthesia, the right superior caudal trunk was resected between the S3 and S4 spinal nerves. After the neuropathic surgery, we examined if the animals exhibited the behavioral signs of mechanical allodynia. The mechanical allodynia was assessed by stimulating the tail with von Frey hair (bending force : 2.0g). three or 6 weeks after the neuropathic surgery, acupuncture stimulation was delivered to Houxi (SI 3) as the following parameters (2HZ frequency, 0.07mA intensity and 3msec duration) for 30 minutes. Results : The stimulation of Houxi (SI 3) acupoint relieved the behavioral signs of mechanical allodynia. Conclusion : Our results suggest that acupuncture can control the mechanical allodynia of neuropathic pain.