• Title/Summary/Keyword: liver disorder

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Spontaneous Perforation of the Bile Duct (담관의 자연 천공)

  • Yoo, Soo-Young;Park, Yong-Tae;Choi, Seung-Hoon;Hwang, Eui-Ho
    • Advances in pediatric surgery
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    • v.2 no.2
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    • pp.143-147
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    • 1996
  • Spontaneous perforation of the bile duct in children is a very rare disorder. We experienced a 6 year-old girl with spontaneous perforation of the right hepatic duct. The patient was initially misdiagnosed as hepatitis because of elevation of liver enzyme and then as appendicitis because of fluid collection in the pelvic cavity demonstrated by ultrasonogram. A laparoscopic exploration was done and no abnormal findings were detected except bile-stained ascites. Peritoneal drainage was performed and the patients seemed to improve clinically. Abdominal pain, distention and high fever developed after removal of the drains. DISIDA scan showed a possible of bile leak into the peritoneal cavity. ERCP demonstrated free spill of dye from the right hepatic duct. At laparotomy, the leak was seen in the anterior wall of the right hepatic duct 2cm above the junction of the cystic duct and common hepatic duct. The perforation was linear in shape and 0.8cm in size. The patient underwent cholecystectomy, primary closure of the perforation and T-tube choedochostomy. We could not identify the cause of the perforation; however, the T-tube cholangiography taken on the 42nd postoperative day showed a little more dilatation of the proximal common bile duct compared with the cholangiography taken on the 14th day. Long-term follow-up of the patient will be necessary because of the possibility for further change of the duct.

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A bibliographic study about the meaning of Hwa and Hwa-byung (화(火) 및 화병(火病)의 의의(意義)에 관한 문헌적(文獻的) 고찰(考察))

  • Park Jing-Sung;Lyu Yeong-Soo
    • Journal of Oriental Neuropsychiatry
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    • v.8 no.1
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    • pp.183-200
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    • 1997
  • I got the following result from the bibliographic study about the meaning of Hwa(火) and Hwa- byung(火病) at the concept and the aspect of pathological and etiological factors.The results are as follows.1. The pathological characteristics of Hwa(火) mean the inflammed state, the excited mental condition and perinervous hyperfunction caused by exopathy produced by any of the six extermal etiological factors(外感六淫) and Hwa caused by interior injury(內傷火熱).2. Hwa caused by the fire emotions(五志之火); anger, gladness, contemplation, sadress, fear by menta complications brings to Hwa caused by the five viscera(五臟之火) and Hwa caused by the stagnation of the liver - energy(肝鬱之火) is the rpresentive Hwa caused by the five viscera and the six hollow organs(贓腑之火).3. A symptom caused by a pathogenic Hwa(火證) is understood in a pathological sense involved an inflammed disease and is considered as a pathological state of excess syndrome(實證) caused by exterior Hwa( 外火).4. The pathologic process of Hwa-byung(火病) as the mental disease is based upon our people's emotion; Han(恨) to be pathologized without being overcome and can be considered as the process of Hwa caused by long-term(鬱久而火化).5. Hwa-byung(火病) means a psychosomatic disorder resulting from stress reaction caused by personal relations in the climacterium.Form the above mentioned study, the meaning of Hwa(火) and Hwa-byung(火病) is set theoretically and it also needs to be maintained reserching and studying about Hwa-byung(火病) consistantly.

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Molecular Genetic Testing and Diagnosis of Wilson Disease (윌슨병의 진단과 분자유전학적 검사)

  • Seo, Jeong Kee
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.11 no.sup1
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    • pp.72-82
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    • 2008
  • Wilson disease (WD) is an autosomal recessive disorder of copper metabolism that results in accumulation of copper primarily in the liver, the brain and the cornea. Mutations in the WD gene, ATP7B cause failure of copper excretion from hepatocyte into bile and a defective synthesis of ceruloplasmin. More than 370 mutations are now recognized, scattering throughout the ATP7B gene. Since WD has protean clinical presentations, awareness of WD in clinical practice is important for the early diagnosis and prevention of accumulated copper toxicity. None of the laboratory parameters alone allows a definite diagnosis of WD. There are numerous pitfalls in the diagnosis of WD. Low serum ceruloplasmin concentrations, increased 24 hour urinary copper excretion, increased hepatic copper concentrations and the presence of Kayser-Fleischer rings in the cornea are major diagnostic points. A combination of any two of these 4 laboratory findings is strong support for a diagnosis of WD. Molecular methods are now being used to aid diagnosis. Molecular genetic testing has confirmed the diagnosis in individuals in whom the diagnosis is not clearly established biochemically and clinically. Siblings should be screened for WD once an index case has been diagnosed. Discrimination of heterozygotes from asymptomatic patients is essential to avoid inappropriate lifelong therapy for heterozygotes. Genetic testing, either by haplotype analysis or by mutation analysis, is the only reliable tool for differentiating heterozygote carriers from affected asymptomatic patients. Currently, genetic testing is of limited value in the primary diagnosis. However, genetic testing will soon play an essential role in diagnosing WD as rapid advancement of biomedical technology will allow more rapid, easier and less expensive mutation detection.

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Tetany in a 13-Year-Old Girl with Wilson's Disease (테타니가 발생한 윌슨병 1예)

  • Ra, Chae-Ik;Kim, Sang-Yong;Koh, Hong
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.14 no.1
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    • pp.86-90
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    • 2011
  • Wilson's disease is an autosomal recessive disorder of copper metabolism consequence of which leads to accumulation of copper in the liver, brain, cornea and other tissues. The manifestations are more likely to be hepatic in the early childhood and neurological in the adolescents. In addition, the abnormalities that develop during disease progression may result in other manifestations such as hematologic, endocrine, or renal findings. We report a thirteen year-old girl who manifested tetany shortly after the initial diagnosis of Wilson's disease. Despite aggressive calcium, magnesium and vitamin D replacement, the hypocalcemia and hypomagnesemia did not respond to the therapy promptly. It took more than three weeks for blood levels of the minerals to be normal. We concluded that tetany occurred in our patient because of hypoparathyroidism as a rare complication of Wilson disease, vitamin D deficiency resulting from various conditions, and inconclusive hypomagnesemia.

Phenotypic Characterization of MPS IIIA (Sgshmps3a/ Sgshmps3a) Mouse Model

  • Park, Sung Won;Ko, Ara;Jin, Dong-kyu
    • Journal of mucopolysaccharidosis and rare diseases
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    • v.4 no.1
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    • pp.26-36
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    • 2018
  • Mucopolysaccharidosis IIIA is a heritable neurodegenerative disorder resulting from the dysfunction of the lysosomal hydrolase sulphamidase. This leads to the primary accumulation of the complex carbohydrate heparan sulphate in a wide range of tissues and CNS degeneration. Characterization of animal model is the beginning point of the therapeutic clinical trial. Mouse model has a limitation in that it is not a human and does not have all of the disease phenotypes. Therefore, delineate of the phenotypic characteristics of MPS IIIA mouse model prerequisite for the enzyme replace treatment for the diseases. We designed 6-month duration of phenotypic characterization of MPS IIIA mouse biochemically, behaviorally and histologically. We compared height and weight of MPS IIIA mouse with wild type from 4 weeks to 6 months in both male and female. At 6 months, we measured GAG storage in urine kidney, heart, liver, lung and spleen. The brain GAG storage is presented with Alcian blue staining, immunohistochemistry, and electron-microscopy. The neurologic phenotype is evaluated by brain MRI and behavioral study including open field test, fear conditioning, T-maze test and Y-maze test. Especially behavioral tests were done serially at 4month and 6month. This study will show the result of the MPS IIIA mouse model phenotypic characterization. The MPS IIIA mouse provides an excellent model for evaluating pathogenic mechanisms of disease and for testing treatment strategies, including enzyme or cell replacement and gene therapy.

Effects of Gyejigamchoyonggolmoryeo-tang on sleep latency and total duration of sleep induced by thiopental sodium in mice (계지감초용골모려탕(桂枝甘草龍骨牡蠣湯)이 치오펜탈 나트륨으로 유도된 수면의 입면 및 수면 시간에 미치는 영향)

  • Kim, Tae Yeon;Leem, Kanghyun
    • The Korea Journal of Herbology
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    • v.35 no.6
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    • pp.29-34
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    • 2020
  • Objectives : Gyejigamchoyonggolmoryeo-tang (GT, Guizhigancaolonggumulitang in Chinese) is a herbal medicine to be prescribed for insomnia caused by anxiety induced by Heart-Heat and elevated Liver-Yang. In the present study, the onset time (sleep latency) and the duration time of sleep were measured to find out the sleep inducing effects of GT. The expression of c-fos immunohistochemistry was also measured at the ventrolateral preoptic area (VLPO) and tuberomammillary nucleus (TMN) site in brain. Methods : The onset time (sleep latency) and the duration time of sleep were measured 30 minutes after thiopental sodium injection. Thereafter, brain tissue was obtained and c-fos immunohistochemistry was performed on the VLPO and TMN sites. Results : GT statistically significantly reduced the sleep latency required to enter sleep, and significantly increased sleep duration time. GT significantly increased the number of c-fos immunohistochemical staining-positive cells in the sleep-inducing center (VLPO), whereas GT significantly decreased the number of c-fos immunohistochemical staining-positive cells in the arousal center (TMN). Conclusions : It could be concluded that the GT shortened the sleep latency and increased the duration time for sleep induced by thiopental sodium. And it was confirmed that the mechanism was to stimulate the sleep-inducing center (VLPO) and suppress the arousal center (TMN) in the brain. The results of this study are considered to be useful as scientific evidence that can be used clinically for the treatment of insomnia caused by anxiety.

A Clinical Study of Insomnia in 33 Admission Cases (불면증(不眠症)을 주소(主訴)로 입원(入院)한 환자(患者) 33례(例)에 대(對)한 임상적(臨床的) 고찰(考察))

  • Choi Byung-Man;Lee Sang-Ryong;Kim Myung-Jin
    • Journal of Oriental Neuropsychiatry
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    • v.12 no.1
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    • pp.169-182
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    • 2001
  • The clinical study was carried out the 33 patients with insomnia who were treated in Daejeon University Oriental Hospital from 17 March 1997 to 12 May 2001. The results were summarized as follows. 1. The ratio of male and female was 10:23 and sleep initiation insomnia and sleep maintenance insomnia were the highest frequence(97%), the beginning of insomnia was frequent at 30s(27.3%). 2. Living accident by mental attack was the most inducing factor and many patients came to our hospital by way of west-neuropsychiatry(55.6%). 3. In admission period most of the patients were within 15 days(78.8%) and Liver-Qi- depression was the main cause and in classification of Four Human coporeal constitution the number of patients Sho-Eum-In(少陰人) was remarked mostly and in distribution of the prescription drugs of growing heart and warming gall bladder such as GUIBIONDAMTANG(歸脾溫膽湯) and ONDAMTANGGAMI (溫膽湯加味) were many, 4. Relatively most patients were well treated(69.7%) and insomnia was mainly caused by Anxiety Disorder and Depression and Hwabyoung and in the age distribution the highest frequence was 30s but we know insomnia appeared at all ages. 5. In distribution of the period of the clinical history was various and within 15 days were all improved and the treatments group of drug and acupuncture and aid treatments etc. and hypnotics was more improved than not used hypnotics treatments group but using hypnotics was temporal. I.

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A Pediatric Case of Primary Sj$\ddot{o}$gren's Syndrome Associated with Nephrogenic Diabetes Insipidus and Renal Tubular Acidosis (신성요붕증과 신세뇨관산증을 동반한 일차성 쇠그렌 증후군의 1례)

  • Choi, Jong Won;Jung, You Jin;Suh, Jin Soon;Park, So Hyun;Koh, Dae Kyun
    • Childhood Kidney Diseases
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    • v.16 no.2
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    • pp.126-131
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    • 2012
  • Sj$\ddot{o}$gren's syndrome (SS) is an autoimmune disorder primarily affecting the salivary and lacrimal glands. In addition, extra-glandular manifestations involving the lungs, liver, kidneys, pancreas, skin and central nervous system were reported in patients with SS. These extra-glandular manifestations are not rare in adult patient, but are very rare in pediatric SS. Renal manifestations are relatively common in adult SS, but are rarely reported in childhood SS. We experienced a girl with primary SS manifested with nephrogenic diabetes insipidus and renal tubular acidosis.

A Case of Successful Endoscopic and Conservative Treatment for Intentional Ingestion of Sharp Foreign Bodies in the Alimentary Tract (고의로 섭취한 커터칼날의 내시경 및 보존적 치료 증례 보고)

  • Park, Jong-Min;Kim, Seong Yup;Chung, Il Yong;Kim, Woo-Shik;Shin, Yong-Chul;Kim, Yeong Cheol;Park, Sei Hyeog
    • Journal of Trauma and Injury
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    • v.26 no.4
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    • pp.304-307
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    • 2013
  • Food bolus impaction is the most common cause of esophageal foreign body obstruction in adults. Other causes include intentional ingestion in psychiatric patients or prison inmates. We experienced successful treatment of a patient with intentional ingestion of multiple sharp foreign bodies(25 cutter and razor fragments). A 47-year-old male patient who was suffering from chronic alcoholism was admitted, via the emergency room, with dysphagia and neck pain. He was suffering from alcoholic liver cirrhosis and psychiatric problems, such as chronic alcoholism, anxiety disorder and insomnia. The patient had intended to leave the hospital after having swallowed the sharp objects. Plain radiographs and computed tomography (CT) scan showed multiple, scattered metal fragments in the esophagus, stomach, and small bowel. We performed emergent endoscopy and successfully removed one impacted blade in the upper esophagus using by a snare with an overtube. The rest of the fragments had already passed through the pylorus, so we could not find them with endoscopy. We checked the patient with simple abdominal radiographs and careful physical examinations every day. All remaining fragments were uneventfully excreted through stool during the patient's 6 day hospital stay. Finally, we were able to confirm the presence of the objects in the stool, and radiographs were negative. The patient was discharged without complications after 14 days hospital stay and then was followed by the Department of Psychiatry.

Anti-Inflammatory Effects of Haliotidis Concha (석결명(石決明)의 항염증효과(抗炎症效果))

  • Moon, Soo-Young;Kim, Young-Woo;Kim, Sang-Chan
    • The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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    • v.26 no.4
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    • pp.70-80
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    • 2013
  • Objectives : Haliotidis Concha has been used to treat various human diseases such as liver dysfunction and inflammatory disorder. Although it has been shown the effects of Haliotidis Concha on the various diseases, it has almost not been studied about the anti-inflammatory effects of the Haliotidis Concha and its mechanisms. Methods : This research investigated the effects of the Haliotidis Concha ethanol extract (HCE) on the production of nitric oxide (NO) and prostaglandin $E_2$ ($PGE_2$) as well as tumor necrosis factor-alpha (TNF-${\alpha}$). The protein expressions of inducible nitric oxide synthase (iNOS) and cyclooxygenase-2 (COX-2) were assayed by immunoblot analyses, and the productions of NO, $PGE_2$ and TNF-${\alpha}$ were assessed by ELISA. Results : Haliotidis Concha decreased the production of NO and $PGE_2$, and inhibited the expression iNOS and COX-2 proteins in a concentration-dependent manner in LPS-treated Raw 264.7 cells. HCE suppressed the ability of LPS to activate the signaling pathways of nuclear factor kappa B (NF-${\kappa}B$) as indicated by HCE inhibited nuclear NF-${\kappa}B$ level and I-${\kappa}B{\alpha}$ phosphorylation. Also, HCE inhibited mitogen-activated protein kinases (MAPKs). Conclusions : HCE repressed the production of LPS-inducible NO, $PGE_2$ and TNF-${\alpha}$, which may be mediated by inhibition of NF-${\kappa}B$ translocation. This study suggest the use for the treatment of acute inflammatory disorders.