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Chung KW Hyun YS Lee HJ Jung HK Koo H Yoo JH Kim SB Park CI Kim HN Choi BO 《Journal of the peripheral nervous system : JPNS》2011,16(2):143-146
Various phenotypes have been reported in Charcot-Marie-Tooth (CMT) disease carrying mutations in the ganglioside-induced differentiation-associated protein 1 (GDAP1) gene. Here, we report two recessive intermediate Charcot-Marie-Tooth (RI-CMT) patients with GDAP1 missense mutations: a His256Arg homozygous mutation (c.767A>G + c.767A>G) and compound mutations of heterozygous Pro111His (c.332C>A) and Val219Gly (c.656T>G). The Pro111His and Val219Gly are unreported mutations, but the His256Arg was previously reported. In both patients, histopathological findings showed well-documented features of mixed demyelinating and axonal neuropathies, and nerve conduction velocities fall in the intermediate range. In addition, the patterns of fatty substitutions in leg magnetic resonance imaging (MRI) were different by the mutation sites within the same GDAP1 gene. 相似文献
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Yong-Soo Lee Sang-Beom Han Yong-Hwan Mo Seul-Gi Lee Deok-Hye Park JiHyun Song Seongho Hong Kyung-Won Park 《RSC advances》2020,10(64):39121
For the removal of volatile organic compounds (VOCs) from environmental systems, gaseous benzene, a model VOC, was adsorbed on naturally Fe-containing activated carbon and subsequently, decomposed in the presence of de-ionized water, and low (0.03%, pH 6.5) and high (30%, pH 2.5) concentration H2O2 solutions. The intermediates produced during benzene decomposition were analyzed and compared using gas chromatography-mass spectrometry. After the decomposition process, the activated carbon sample was air dried. Three cycles were carried out with de-ionized water and low and high concentration H2O2 solutions as oxidants. The adsorption capacity of the activated carbon sample treated with DI water gradually decreased as the number of cycles increased. On the other hand, the benzene adsorption capacity of the activated carbon samples treated with the H2O2 solutions was improved due to the relatively higher specific surface areas of these samples. After treatment with the low-concentration H2O2 solution, intermediates such as glyoxylic acid, oxalic acid, phenol, malonic acid, and pyrocatechol were observed. After treatment with high-concentration H2O2 solution, intermediates such as glyoxylic acid, formic acid, and acetic acid were formed. With increasing H2O2 concentration, the number and the molecular weight of the intermediate formed by the oxidative degradation of benzene, simultaneously decreased. The Fenton reaction induced by naturally Fe-containing activated carbon and H2O2 could lead to more efficient decomposition of benzene.For the removal of volatile organic compounds from environmental systems, gaseous benzene, a model VOC, was adsorbed on naturally Fe-containing activated carbon and subsequently, decomposed in the presence of H2O2 solution. 相似文献
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Hak Young Rhee Hye-Yeon Choi Sang-Beom Kim Won-Chul Shin 《Journal of thrombosis and thrombolysis》2010,30(2):229-232
Recurrent cerebral infarctions developed in a patient with idiopathic thrombocytopenic purpura (ITP). At the time of the first
stroke, there were large thrombi in the right proximal internal carotid artery (ICA) and an occlusion of the right terminal
ICA. The occlusion was recanalized by intra-arterial infusion of urokinase. After 20 months, he suffered another ischemic
attack. The autoantibodies in ITP directed against antigens presented on both platelets and endothelial cells might induce
the endothelial damage, thrombus formation, and embolic occlusion of the artery. 相似文献
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Lee SW Song CY Gi YH Kang SB Kim YS Nam SW Lee DS Kim JO 《World journal of gastroenterology : WJG》2008,14(18):2935-2938
Angiosarcoma is a rare tumor that account for less than 1% of all sarcomas. Although hepatic angiosarcoma usually presents with unspecific symptoms, it rapidly progresses and has a high mortality. We report a rare case of primary hepatic angiosarcoma manifested as recurrent hemoperitoneum. 相似文献
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Young Hwa Kim Hwa Kyung Chung Kee Duk Park Kyoung-Gyu Choi Seung-Min Kim Il-Nam Sunwoo Young-Chul Choi Jeong-Geun Lim Kwang Woo Lee Kwang-Kuk Kim Dong Kuk Lee In Soo Joo Ki-Han Kwon Seok Beom Gwon Jae Hyeon Park Dae-Seong Kim Seung Hyun Kim Woo-Kyung Kim Bum Chun Suh Sang-Beom Kim Nam-Hee Kim Eun Hee Sohn Ok-Joon Kim Hyun Sook Kim Jung Hee Cho Sa-Yoon Kang Chan-Ik Park Jiyoung Oh Jong Hyu Shin Ki Wha Chung Byung-Ok Choi 《JOURNAL OF CLINICAL NEUROLOGY》2012,8(2):139-145
Background and Purpose
Charcot-Marie-Tooth disease (CMT) type 1A (CMT1A) is the demyelinating form of CMT that is significantly associated with PMP22 duplication. Some studies have found that the disease-related disabilities of these patients are correlated with their compound muscle action potentials (CMAPs), while others have suggested that they are related to the nerve conduction velocities. In the present study, we investigated the correlations between the disease-related disabilities and the electrophysiological values in a large cohort of Korean CMT1A patients.Methods
We analyzed 167 CMT1A patients of Korean origin with PMP22 duplication using clinical and electrophysiological assessments, including the CMT neuropathy score and the functional disability scale.Results
Clinical motor disabilities were significantly correlated with the CMAPs but not the motor nerve conduction velocities (MNCVs). Moreover, the observed sensory impairments matched the corresponding reductions in the sensory nerve action potentials (SNAPs) but not with slowing of the sensory nerve conduction velocities (SNCVs). In addition, CMAPs were strongly correlated with the disease duration but not with the age at onset. The terminal latency index did not differ between CMT1A patients and healthy controls.Conclusions
In CMT1A patients, disease-related disabilities such as muscle wasting and sensory impairment were strongly correlated with CMAPs and SNAPs but not with the MNCVs or SNCVs. Therefore, we suggest that the clinical disabilities of CMT patients are determined by the extent of axonal dysfunction. 相似文献19.
Sang-Beom Lee Hyung-Ki Park Jae-Chil Chang So-Young Jin 《Journal of Korean Neurosurgical Society》2011,50(2):130-133
Spinal epidural lipomatosis (SEL) is an overgrowth of the normally encapsulated adipose tissue in the epidural space around the spinal cord in the thoracic and lumbar spine causing compression of the neural components. Idiopathic SEL in non-obese patients is exceptional. Idiopathic SEL can result in thoracic myelopathy and lumbar radiculopathy. A thoracic radiculopathy due to idiopathic SEL has not been reported yet. We report a case of idiopathic SEL with intractable chest pain and paresthesia. We suggest that idiopathic SEL should be considered as a cause of chest pain. 相似文献
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Jhang Ho Pak Dong-Wook Kim Ju Hyun Moon Joo-Hyun Nam Jong-Hyeok Kim Jung Won Ju Tong-Soo Kim Sang-Beom Seo 《Parasitology research》2009,104(5):1035-1046
Clonorchiasis is associated with bile duct malignancy and the subsequent development of cholangiocarcinoma. Although this
is likely caused by adult Clonorchis sinensis and its excretory–secretory products (ESP), the precise molecular mechanisms remain obscure. To evaluate the effect of C. sinensis infection on differential gene expression in host hepatocytes, we examined the kinetics of changes in gene expression in
the human cholangiocarcinoma cell line HuCCT1 treated with ESP at different times. Using complementary DNA microarrays containing
23,920 human genes of known function, we initially identified 435 genes altered by ESP treatment. Of these, 31 were up-regulated
and 35 were down-regulated more than twofold in a time-dependent manner following ESP treatment. Clustering of these genes
by function revealed that several were involved in the cell cycle, oncogenesis, protein modification, immunity, signal transduction,
cell structure, and developmental processes. These findings should provide a fundamental basis for further analysis of the
molecular pathways and mechanisms involved in C. sinensis infection of host cells. 相似文献