• Journal of The Korean Society of Inherited Metabolic disease
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• v.23 no.1
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• pp.25-30
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• 2023

Leigh syndrome is a rare progressive neurodegenerative mitochondrial disorder with clinical and genetic heterogeneity. Recently, balletic IARS2 variants have been identified in a number of patients presenting broad clinical phenotypes from Leigh and West syndrome to a rare syndrome CAGSSS characterized by cataracts, growth hormone deficiency, sensory neuropathy, sensorineural hearing loss, and skeletal dysplasia syndrome (OMIM#616007). We describe a child with Korean Leigh syndrome with urologic manifestations resulting from a compound heterozygote mutation in IARS2. A 5-year-old girl visited the emergency room with a complaint of abdominal pain accompanied by abdominal distension. Abdominal-pelvic CT showed a markedly distended urinary bladder without definite obstructive lesions. She was diagnosed with neurogenic bladder dysfunction based on a urodynamic study. She had global delayed development due to neurologic regression after 6 months of age and a history of bilateral cataract surgery at the age of 2 years. Her brain magnetic resonance imaging showed symmetrically increased signal intensities in the bilateral putamen and caudate nuclei with diffuse cerebral atrophy. No gene variants were identified through whole-mitochondrial genome analysis. Whole exome sequencing was performed for diagnosis, and compound heterozygous pathogenic variants were identified in IARS2: c.2446C>T (p. Arg816Ter) and c.2450G>A (p. Arg817His). To the best of our knowledge, this is the first case report of bladder dysfunction manifestation in a patient with IARS2-related Leigh syndrome. Thus, it broadens the clinical and genetic spectrum of IARS2-associated diseases.

• Archives of Plastic Surgery
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• v.37 no.6
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• pp.783-787
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• 2010

Purpose: There are many articles describing about Guyon canal compression syndrome. Until recently, most of these articles have been presented about the symptoms of ulnar nerve compression, but there have been no reports about ulnar artery compression. In this article, besides the nerve compression symptoms in the Guyon's canal, we represented the symptoms and treatments based on the ulnar artery obstruction. Methods: Guyon canal is composed of the hamate and pisiform, and the ligaments which connect them. The course of the ulnar nerve and artery, which passes through this narrow canal, is affected by the anatomical structure of the base of the canal. Out of 14 patients (21 cases) were retrospectively reviewed in this study from 2006 to 2009. Of 14 patients, there were 5 men and 9 women with ages between 21 to 61 years old. The symptoms had volar sensory loss of ulnar sided digits, with muscular atrophy of hypothenar muscles. Prior to surgery, most of these patients had vascular disorders which was diagnosed definitively by angiography and electromyogram. Results: The release of Guyon canal and interposition graft of the obstructed arteries was carried out to 11 patients (15 cases) who had artery (vascular) occlusive disorder, and. 12 cases had sympathectomy and interposition graft after resection of obstructed ulnar artery. Six cases had release of carpal tunnel performed simultaneously. There were no major complications after surgery. The circulation of the ulnar artery was improved along with the patients' symptoms. Conclusion: The pre-existing articles about Guyon canal compression syndrome were mainly focused on ulnar nerve compression. This study, which was carried out by our department, showed that most of these patients had ulnar artery obstruction or stenosis simultaneously with ulnar nerve compression. The vascular disorder was corrected by interposition graft after the resection of the site of ulnar artery occlusion. And to conclude, When we resolve the ulnar nerve compression, the proper diagnosis & treatment of impaired ulnar artery circulation should be carried out concomitantly.

• Journal of Korean Medical classics
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• v.26 no.3
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• pp.53-73
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• 2013

Objective : This study will analyze the constitution of Danxihuqianwan(丹溪虎潛丸) with the dragon-tiger and lead-mercury theory of Su Shi(蘇軾). It would help understand more the usage of Danxihuqianwan over the scope of Sa-nam and bo-buk(瀉南補北) theory that has been well known until now. Methods : This study tried to analyze the explanation about Danxihuqianwan in Yixuerumen(醫學入門) that had used the dragon-tiger and lead-mercury concept with the theory of this kind that appears in Sushenliangfang(蘇沈良方). Results & Conclusion: Based on the internal alchemy theory, Su Shi proclaimed that dragon[龍] corresponds to mercury[汞] Jeong(精) Hyul(血), and tiger[虎] corresponds to lead[鉛] Qi(氣). It is said to be in good position that dragon appears from fire and tiger is hidden in water. Su Shi also expalined the relation between dragon and tiger as the relation of water[水] and fire[火] between heart and kidney. As Li Yan(李梴) did, the dragon-tiger theory could be applied to the analysis about Danxihuqianwan, and it explains well the effect of this prescription that makes tiger hidden in water and thus help cure the diseases such as atrophy syndrome.

• Journal of Physiology & Pathology in Korean Medicine
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• v.19 no.1
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• pp.279-283
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• 2005

After initial recovery from acute carbon monoxide(CO) intoxication, some patients occasionally undergo severe neuropsychiatric deterioration, which is called postanomic delayed encephalopathy(sequelae). This is the clinical study about one patient, a 53-year-old woman, diagnosed with delayed encephalopathy after acute CO intoxication. The patient's symptoms were mental dysfunction including memory impairment and disorientation, aphasia, atrophy and weakness throughtout the body. She had completely recovered after an anomic episode, but the neurological symptoms that developed were preceded by an interval of apparent nomality.(the 'lucid interval'). She was characterized as suffering deficiency syndrome of the heart(心虛) and was prescribed for her an Ansinschungnoi-tang(安神淸腦湯), and thereafter her symptoms improved remarkably. For the evaluation of clinical improvement, we use the Modified Barthel Index(MBI).

• Physical Therapy Korea
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• v.2 no.2
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• pp.73-84
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• 1995

Spasticity has been defined as a motor disorder characterised by a velocity-dependent increase in tonic stretch reflexes with exaggerated tendon jerks resulting in hyperexcitability of the stretch reflexes as one component of the upper motor neuron syndrome. Weakness and loss of dexterity, however, are considered to be more disabling to the patient than changes in muscle tone. The discussion includes the important role that alterations in the physiology of motor units, notably changes in firing rates and muscle fiber atrophy, play in the manifestation of muscle weakness. This paper considers both the neural and mechanical components of spasticity and discusses, in terms of clinical intervention, the implications arising from recent research. Investigations suggest that the resistance to passive movement in individuals with spasticity is due not only to neural mechanisms but also to changes in mechanical properties of muscle. The emphasis is on training the individual to gain control over the muscles required for different tasks, and on preventing secondary and adaptive soft tissue changes and ineffective adaptive motor behaviours.

• Journal of Yeungnam Medical Science
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• v.38 no.1
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• pp.27-33
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• 2021

The gut is a complex organ that has played an important role in digestion, absorption, endocrine functions, and immunity. The gut mucosal barriers consist of the immunologic barrier and nonimmunologic barrier. During critical illnesses, the gut is susceptible to injury due to the induction of intestinal hyperpermeability. Gut hyperpermeability and barrier dysfunction may lead to systemic inflammatory response syndrome. Additionally, gut microbiota are altered during critical illnesses. The etiology of such microbiome alterations in critical illnesses is multifactorial. The interaction or systemic host defense modulation between distant organs and the gut microbiome is increasingly studied in disease research. No treatment modality exists to significantly enhance the gut epithelial integrity, permeability, or mucus layer in critically ill patients. However, multiple helpful approaches including clinical and preclinical strategies exist. Enteral nutrition is associated with an increased mucosal barrier in animal and human studies. The trophic effects of enteral nutrition might help to maintain the intestinal physiology, prevent atrophy of gut villi, reduce intestinal permeability, and protect against ischemia-reperfusion injury. The microbiome approach such as the use of probiotics, fecal microbial transplantation, and selective decontamination of the digestive tract has been suggested. However, its evidence does not have a high quality. To promote rapid hypertrophy of the small bowel, various factors have been reported, including the epidermal growth factor, membrane permeant inhibitor of myosin light chain kinase, mucus surrogate, pharmacologic vagus nerve agonist, immune-enhancing diet, and glucagon-like peptide-2 as preclinical strategies. However, the evidence remains unclear.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.13 no.11
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• pp.5305-5310
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• 2012

This study is Carpal tunnel syndrome(CTS) disorder of median nerve at wrist. It is usually diagnosed through clinical manifestation and Nerve Conduction Study(NCS). NCS of the median nerve before and after operation were compared in twenty four patient's with CTS, in order to seventeen patient's evaluate the prognostic value of that findings. Analysis result symptom profile of CTS in total number of patient's 17 (Female:17, Male:0), 21 hands (Rt:9, Lt:4, Both:4), Ages(31~60), Mean duration of symptom months($46.6{\pm}36.1$), Mean interval between 1st and 2nd NCS months($20.5{\pm}7.1$), Sensory symptoms(Tingling:21, Numbness:19, Noctunal paresthesia:17), Motor symptoms(Thenar atrophy:20, Trigger finger:2, Morning stiffness:3), Post-operative symptoms(Free:38.1%, >50% improve:52.4%, <50% improve:9.5%). NCS was normal range after operation than before in Sensory nerve conduction study 4 patients's and Motor nerve conduction study 5 patients. Surgery before and after Sensory nerve action potential (SNAP) responses showed improvement over the previous results. Forward by the patient's occupation and occupation patterns of CTS, other treatment methods and surgical treatment of CTS by comparing the degree of improvement to identify and correct nerve conduction study to judge whether the patient's operation.

• Journal of Korean Neurosurgical Society
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• v.45 no.5
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• pp.293-296
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• 2009

Objective : Pain has long been regarded as a subjective symptom. Recently, however, some regard a type of intractable chronic pain as a disease. Furthermore, chronic persistent pain becomes a cause of permanent impairment (PI) In 6th edition, the American Medical Association (AMA) Guides has rated the pain as a PI. In Korea, pain has been already been rated as a PI. Here, we examined the present status and the prospect of disability evaluation for the pain in Korea. Methods : Pain can be rated as a PI by the Workmen's Compensation Insurance Act (WCIA) and Patriots and Veterans Welfare Corporation Act (PVWCA) in Korea. We examined the definition, diagnostic criteria and grades of the pain related disability (PRD) in these two acts. We also examined legal judgments, which were made in 2005 for patients with severe pain. We also compared the acts and the judgments to the criteria of the 6th AMA Guides. Results : The PRD can berated as one of the 4 grades according to the WCIA. The provisions of the law do not limit the pain only for the complex regional pain syndrome (CRPS). The PRD can berated as one of the 3 grades by the PVWCA. If there were objective signs such as osteoporosis, joint contracture and muscle atrophy corresponding to the CRPS, the grade is rated as 6. When the pain always interferes with one's job except easy work,the grade is rated as high as 5. In Korea, judicial precedents dealt the pain a sa permanent disability in 2005. Conclusion : Although there were no objective criteria for evaluation of the PRD, pain has been already rated as a PI by the laws or judicial precedents, in Korea. Thus, we should regulate the Korean criteria of PRD like the AMA 6th edition. We also should develop the objective tools for evaluation of the PRD near in future.

• The Korean Journal of Nuclear Medicine
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• v.23 no.2
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• pp.149-154
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• 1989

Transient ischemic attacks (TIAs) is a syndrome resulting from brain ischemia lasting less than 24 hours. The mechanisms of TIAs may be similar to those of cerebral embolism and thrombosis, and thus TIAs may be followed by cerebral infarction. Despite the availability of CT scanning, the diagnosis and management of TIAs continue to be difficult. Recently SPECT has been advocated as a diagnostic imaging modality. We performed Tc-99m-HMPAO regional cerebral blood flow (rCBF) SPECT in 24 patients with the clinical diagnosis of TIAs to assess its ability to detect early changes of rCBF, and determine the diagnostic value. Ten men and fourteen women with an average of 51 years (range; 27-74 years) were included. All but 8 patients had normal brain CT prior to SPECT. The two patients had moderate degree of brain atrophy and the 6 patients nonspecific calcifications. Eighteen of the 24 patients had abnormal Tc-99m-HMPAO rCBF SPECT. Fifteen had unilateral involvement and the other three had bilateral involvements. Seventy-five percents of the defects were found in the left cerebral hemisphere. According to the distribution of the lesions (total number: 34 lesions), fourteen were in the parietal, eight in the temporal, and the remainders were elsewhere. Tc-99m-HMPAO rCBF SPECT is sensitive in detecting rCBF abnormalities in patients with TIAs, and represent the most accurate diagnostic tool available in the diagnosis of TIAs.

• Journal of fish pathology
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• v.9 no.2
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• pp.137-145
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• 1996

A specific disease syndrome, which led to massive mortality on larve of rockfish(Sebastes schlegeli) in marine hatcheries at Chungnam area during the period 1995~1996 was studied. The causative agent isolated from the diseased or dead larvae was identified as Vibrio ordalii on the basis of biochemical and biological characteristics. In the experimental challenges aganist 0 and 1 summer fish conducted at two different temperatures as $18^{\circ}C$ and $25^{\circ}C$, Vibrio ordalii showed higher virulence to no summer fish at $18^{\circ}C$ than 1-summer fish at $25^{\circ}C$. These results were consistent to field data obtained during epizootic outbreaks in the farms. Moribund and died larvae presented telangiectasis of secondary gill lamella and brain, dissecting of respiratory epithelium, atrophy of hepatic cells and necrosis of kidney associated with the presence of the bacteria. But the digestive tissue of these fish showed no significant change.