• Clinical and Experimental Pediatrics
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• 제59권sup1호
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• pp.161-164
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• 2016

Guillain-$Barr{\acute{e}}$ syndrome and acute transverse myelitis manifest as demyelinating diseases of the peripheral and central nervous system. Concurrency of these two disorders is rarely documented in literature. A 4-year-old girl presenting with cough, fever, and an impaired walking ability was admitted to hospital. She had no previous complaints in her medical history. A physical examination revealed lack of muscle strength of the lower extremities and deep tendon reflexes. MRI could not be carried out due to technical problems; therefore, both Guillain-$Barr{\acute{e}}$ syndrome and acute transverse myelitis were considered for the diagnosis. Intravenous immunoglobulin treatment was started as first line therapy. Because this treatment did not relieve the patient's symptoms, spinal MRI was carried out on the fourth day of admission and demyelinating areas were identified. Based on the new findings, the patient was diagnosed with acute transverse myelitis, and high dose intravenous methylprednisolone therapy was started. Electromyography findings were consistent with acute polyneuropathy affecting both motor and sensory fibers. Therefore, the patient was diagnosed with concurrency of Guillain-$Barr{\acute{e}}$ syndrome and acute transverse myelitis. Interestingly, while concurrency of these 2 disorders is rare, this association has been demonstrated in various recent publications. Progress in diagnostic tests (magnetic resonance imaging and electrophysiological examination studies) has enabled clinicians to establish the right diagnosis. The possibility of concurrent Guillain-$Barr{\acute{e}}$ syndrome and acute transverse myelitis should be considered if recovery takes longer than anticipated.

• Clinical and Experimental Pediatrics
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• 제57권9호
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• pp.416-419
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• 2014

X-linked adrenoleukodystrophy (X-ALD) is a rare peroxisomal disorder, that is rapidly progressive, neurodegenerative, and recessive, and characteristically primary affects the central nervous system white matter and the adrenal cortex. X-ALD is diagnosed basaed on clinical, radiological, and serological parameters, including elevated plasma levels of very long chain fatty acids (VLCFA), such as C24:0 and C26:0, and high C24:0/C22:0 and C26:0/C22:0 ratios. These tests are complemented with genetic analyses. A 7.5-year-old boy was admitted to Department of Pediatrics, Chungnam National University Hospital with progressive weakness of the bilateral lower extremities. Brain magnetic resonance imaging confirmed clinically suspected ALD. A low dose adrenocorticotropic hormone stimulation test revealed parital adrenal insufficiency. His fasting plasma levels of VLCFA showed that his C24:0/C22:0 and C26:0/C22:0 ratios were significantly elevated to 1.609 (normal, 0-1.390) and 0.075 (normal, 0-0.023), respectively. Genomic DNA was extracted from peripheral whole blood samples collected from the patient and his family. All exons of ABCD1 gene were amplified by polymerase chain reaction (PCR) using specific primers. Amplified PCR products were sequenced using the same primer pairs according to the manufacturer's instructions. We identified a missense mutation (p.Arg163Leu) in the ABCD1 gene of the proband caused by the nucleotide change 488G>T in exon 1. His asymptomatic mother carried the same mutation. We have reported an unpublished mutation in the ABCD1 gene in a patient with X-ALD, who showed increased ratio of C24:0/C22:0 and C26:0/C22:0, despite a normal VLCFA concentrations.

• 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.

• Applied Microscopy
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• 제33권4호
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• pp.291-298
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• 2003

스트레스는 교감신경계와 부신을 활성화시킨다. 본 연구에서는 고염식이가 흰 생쥐의 부신수질에 미치는 영향을 tyrosine hydroxylase (TH) 면역염색법과 투과 전자현미경을 이용한 미세구조적 관찰로 규명하였다. 1. 부신수질의 TH 면역반응은 고염식이 후 증가하였으며 특히 고염식이를 4주간 시행한 경우보다 4일간 시행한 흰 생쥐에서 더 강하게 관찰되었다. 2. TH 면역반응은 부신수질을 구성하는 세포들 중 노르아드레날린 세포의 세포질과 과립들에서 주로 나타났다. 3. 고염식이를 시행한 흰 생쥐 부신수질의 노르아드레날린 세포에서는 세포질 내에 비어있는 공간들, 분비과립이 팽대되어 과립 내 물질이 한 쪽으로 치우친 형태 및 손상된 미토콘드리아등의 특징들이 관찰되었다. 이와 같은 결과는 고염식이가 스트레스를 유발하는 요인이 될 수 있음을 시사해준다.

• 한국산업보건학회지
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• 제30권3호
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• pp.270-279
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• 2020

Objective: The aim of this study was to propose revision of the occupational exposure limit(OEL) for 1-Bromopropane(1-BP) following a review of the appropriateness of the standard in light of increasing epidemiological data and handling risk. Materials and Methods: The results of toxicity and epidemiologic investigations for 1-BP and agencies' OELs were compared and reviewed through a literature review. In order to investigate the status of 1-BP handling in South Korea, data from work environment actual condition survey results and work environment measurement results were used. Results: The toxicity of 1-BP, such as central nervous system(CNS) damage, peripheral neuropathy, hematological adverse effects, and developmental and reproductive toxicity(male and female) has been reported. ACGIH recommends 0.1 ppm as a TLV-TWA value, but the OEL of South Korea stands at 25 ppm, which is 250 times higher than the TLV-TWA. Although 1-BP is a specially managed substance under the Industrial Safety and Health Law, the currently applied OEL cannot be said to be a safe level based on the results of epidemiological studies to date. In a work environment measurement in 2017, the total number of samples was 626, which were derived from 78 industries, and the average concentration was 1.173 ppm(standard deviation 2.88). Conclusions: To protect the health of workers handling 1-BP, estimated to be 780 in South Korea, it is necessary to strengthen the OEL(TWA) to a level of 0.3 ppm(lower than the 0.34 ppm with known toxic effects), which is believed to be safe as a result of epidemiological investigation. "Skin" notation should be recommended.

• Safety and Health at Work
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• 제11권2호
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• pp.173-177
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• 2020

Background: Indoor air dampness microbiota (DM) is a big health hazard. Sufficient evidence exists that exposure to DM causes new asthma or exacerbation, dyspnea, infections of upper airways and allergic alveolitis. Less convincing evidence has yet been published for extrapulmonary manifestations of dampness and mold hypersensitivity syndrome). Methods: We investigated the prevalence of extrapulmonary in addition to respiratory symptoms with a questionnaire in a cohort of nurses and midwives (n = 90) exposed to DM in a Helsinki Obstetric Hospital. The corresponding prevalence was compared with an unexposed cohort (n = 45). Particular interest was put on neurological symptoms and multiple chemical sensitivity. Results: The results show that respiratory symptoms were more common among participants of the study vs. control cohort, that is, 80 vs 29%, respectively (risk ratio [RR]: 2.56, p < 0.001). Symptoms of the central or peripheral nervous system were also more common in study vs. control cohort: 81 vs 11% (RR: 6.63, p < 0.001). Fatigue was reported in 77 vs. 24%, (RR: 3.05, p < 0.001) and multiple chemical sensitivity in 40 vs. 9%, (RR: 3.44, p = 0.01), the so-called "brain fog", was prevalent in 62 vs 11% (RR: 4.94, p < 0.001), arrhythmias were reported in 57 vs. 2.4% (RR: 19.75, p < 0.001) and musculoskeletal pain in 51 vs 22% (RR: 2.02, p = 0.02) among participants of the study vs. control cohort, respectively. Conclusion: The results indicate that the exposure to DM is associated with a plethora of extrapulmonary symptoms. Presented data corroborate our recent reports on the health effects of moist and mold exposure in a workplace.

• The Korean Journal of Pain
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• 제25권1호
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• pp.7-15
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• 2012

Background: Neuropathic pain is a chronic pain due to disorder in the peripheral or central nervous system with different pathophysiological mechanisms. Current treatments are not effective. Analgesic drugs combined can reduce pain intensity and side effects. Here, we studied the analgesic effect of nimesulide, nefopam, and morphine with different mechanisms of action alone and in combination with other drugs in chronic constriction injury (CCI) model of neuropathic pain. Methods: Male Wistar rats (n = 8) weighing 150-200 g were divided into 3 different groups: 1- Saline-treated CCI group, 2- Saline-treated sham group, and 3- Drug-treated CCI groups. Nimesulide (1.25, 2.5, and 5 mg/kg), nefopam (10, 20, and 30 mg/kg), and morphine (1, 3, and 5 mg/kg) were injected 30 minutes before surgery and continued daily to day 14 post-ligation. In the combination strategy, a nonanalgesic dose of drugs was used in combination such as nefopam + morphine, nefopam + nimesulide, and nimesulide + morphine. Von Frey filaments for mechanical allodynia and acetone test for cold allodynia were, respectively, used as pain behavioral tests. Experiments were performed on day 0 (before surgery) and days 1, 3, 5, 7,10, and 14 post injury. Results: Nefopam (30 mg/kg) and nimesulide (5 mg/kg) blocked mechanical and thermal allodynia; the analgesic effects of morphine (5 mg/kg) lasted for 7 days. Allodynia was completely inhibited in combination with nonanalgesic doses of nefopam (10 mg/kg), nimesulide (1.25 mg/kg), and morphine (3 mg/kg). Conclusions: It seems that analgesic drugs used in combination, could effectively reduce pain behavior with reduced adverse effects.

• 약학회지
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• 제55권4호
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• pp.277-283
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• 2011

Simultaneous monitoring of catecholamines and serotonin with their appropriate extraction from the biological samples is required in order to understand thoroughly the regulation of the central and peripheral nervous system. In the present research the segmented gradient elution with the solid phase extraction using a C18 cartridge rather than the previous isocratic elution with alumina extraction is successfully employed to determine norepinephrine (NE), epinephrine (E), dopamine (DA), serotonin (5HT), 3,4-dihydroxyphenylacetic acid (DOPAC) and 5-hydroxyindoleacetic acid (5HIAA) simultaneously within 20 minutes using 3,4-dihydroxyhydrocinnamic aicd as the internal standard (IS). Linearities were obtained in the concentration range between $5{\times}10^{-6}M$ and $1{\times}10^{-4}M$ for all 7 compounds with detection limits of 0.6~1.9 ${\mu}M$. The present HPLC/ECD method yielded reasonable accuracy (relative error; -1.4~1.1%) and precision (relative standard deviation; 0.4~1.9%) for 9 measurements of the standard solution consisting of NE, E, DA, 5HT, DOPAC and 5HIAA compounds. Recoveries of catecholamines, serotonin and their metabolites from human serum were in the range of 57%~86%. While the concentrations of NE and 5HT in the serum of normal Sprague-Dawley rat were found as $1.4{\times}10^{-6}M$ and $2.6{\times}10^{-6}M$, respectively, the contents of NE and 5HT in the serum of the stressed rat were increased 5.6 times and 1.4 times more, respectively.

• Tuberculosis and Respiratory Diseases
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• 제52권2호
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• pp.166-173
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• 2002

저자들은 급성 호흡부전으로 발현되어 특별한 원인 없이 말초혈액의 호산구 증가와 골수의 호산구 증식, 그리고 심장 및 폐장의 침범 소견이 있는 환자에서 부신 피질호르몬 치료로 호전을 보였던 특발성 과호산구성 증후군 환자를 경험하였기에 보고하는 바이다.

• The Korean Journal of Pain
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• 제20권2호
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• pp.148-153
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• 2007

Background: Complex regional pain syndrome (CRPS) is not regarded as an impairment in Korea. Guidelines for rating this impairment are under development by the Korean Academy of Medical Science based on that of the American Medical Association (AMA). However, no studies have been done on the validity of these guidelines in Korea. We therefore evaluated the validity of these guidelines using the criteria from the chapter on the central and peripheral nervous system (CNS-PNS class) and the worksheet for calculating total pain-related impairment score (TPRIS class). Methods: TPRIS and CNS-PNS classes were calculated through interviews of 28 CRPS patients. The correlation between the two classes was calculated. Results: TPRIS class and CNS-PNS class were well correlated (r = 0.593, P < 0.05). Conclusions: Both TPRIS or CNS-PNS classes were well correlated and could be used for evaluation of impairment. However, the CNS-PNS class is simpler and quicker to complete.