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양현웅,김연수,문희석,박기오,이엄석,김선문,서승원,성재규,나병규,이승민,이병석,김남재,이헌영 충남대학교 의과대학 지역사회의학연구소 2001 충남의대잡지 Vol.28 No.1
Background/Aims : Wilson's disease in an autosomal recessive genetic disorder which is characterized by hepatic and neurologic manifestations due to defect in copper metabolism. The progression of hepatic damage and critical neurologic complication can be prevented by early diagnosis and treatment. Our study investigated the usefulness of clinical manifestations and laboratory findings in Wilson's disease to early diagnosis of wilson's disease. Method : We investigated the 10 patients who are diagnosed as Wilson's disease at Chungnam national university Hospital during the recent 5 years for clinical manifestation, laboratory findings and treatment. Results : 1. Females are 9 patients(90%) among 10 objective patients and the median age of patients is 30.2±14 years. The second and third decade are the more common prevalent and 5 patients(50%) have family history. 2. The clinical manifestations of Wilson's disease at time of diagnosis are hepatic manifestation in 9 patients (90%) except presymptomatic patient and the psycho-neurologic manifestations are appeared in 5 patients(50%). Three patients have the secondary amenorrhea, hemolytic anemia and recurrent spontaneous abortions in each case. 3. The serum ceruloplasmin concentrations were lower than 20mg/dl in 8 patient(80%) including presymptomatic patient and the 24 hours urine copper excretion is above 100μg/24hrs in 7 patients(70%). 4. Seven patients(70%) with symptomatic Wilson's disease have the detectable Kayser-Fleischer ring in slit lamp examinations but one presymptomatic patient has no Kayser-Fleischer ring. 5. The serum level of ceruloplasmin is increased in 3 patients (42.8%) among 7 patients who are had been treated by penicillamine(one patient is not followed up serum ceruloplasmin) and the 24 hours urine copper excretion was decreased in 6 patient(75%) among 8 patients treated by penicillamine. 6. Five patients died with hepatic failure related to uncompensated liver cirrhosis. Conclusion : The slit lamp examination should be recommended in patients with hepatic and neurologic disease of unknown etiology and sibling of the patients wilson's disease. The irrerersible damage of organs would be prevented by aggressive treatment at early state in patients diagnosed as Wilson's disease.
Case Reports : Pregnancy in a Woman with Wilson`s Disease Treated with Zinc: A Case Report
( Sanjoy Kumar Bhattacharyya ),( Shyama Prasad Saha ),( Jaydeep Samanta ),( Atanu Roychowdhuri ) 대한산부인과학회 2012 Obstetrics & Gynecology Science Vol.55 No.3
Wilson`s disease is a rare hereditary disorder of copper metabolism affecting 30 per million populations. Copper get deposited in liver, brain and kidney due to decreased hepato-cellular excretion and leading to diverse clinical manifestations. Patient may remain apparently asymptomatic or may present with fulminant liver disease or neuropsychiatric illness. Reproductive outcome in untreated Wilson`s disease is poor. Women remaining untreated either suffer from infertility or experience recurrent pregnancy losses. Invention of copper chelating agents and their usage in these women resulted in successful pregnancy outcome. Penicillamine or zinc salts can be used as copper chelator in pregnancy with Wilson`s disease with equal effectiveness in respect to pregnancy outcome. We report a case of previously undiagnosed Wilson`s disease with three consecutive pregnancy losses who achieved a live birth after successfully treated with zinc salts. The several points regarding antenatal care, drug therapy and optimum time and mode of delivery for the woman with Wilson`s disease are discussed here.
고태현,엄재용,채진석,손성세,최익선,Go, Tae-hyun,Eom, Jae-yong,Chae, Jin-suk,Shon, Sung-se,Choi, Ik-sun 대한침구의학회 2004 대한침구의학회지 Vol.21 No.5
Objectives : Wilson's disease is an autosomal recessive abnormality in the hepatic excretion of copper that results in toxic accumulation of the metal in liver, brain, and other organs. The purpose of this case study is to show a case with Wilson's disease treated with acupuncture therapy. Methods : We experienced a 17 year old male patient with a Wilson's disease whose main symptoms are neurological symptoms, such as spasticity, quadripleia and dysphagia. The patient was treated with acupuncture therapy for 3 weeks. Results : Spasticity was assessed by the modified Ashworth scale in an every week. 1. Lt. elbow, wrist and ankle joint improved Gr.III to Gr.II. 2. Rt. each joints and Lt. knee joint seemed to improve a little but no grade changed. Conclusions : This study is just one case and the period of acupuncture therapy is short, which make this case study less sufficient to decide the effect of acupuncture therapy. However, in this case study, acupuncture therapy seems somewhat effective to neurological symptoms of Wilson's disease, such as spasticity and quadriplegia. We suggest that oriental medicine should be studied to cure Wilson's disease from now on.
고흥 世明대학교 한의학연구소 2003 韓醫學硏究所 論文集 Vol.5 No.-
Though we diagnosis wilson's disease as up-stirring of liver(肝風內動) for neurological symptoms , calming the liver to stop the wind(平肝熄風) drug's high copper content could increase wilson's symptoms. We diagnosis wilson's disease as damp-heat syndrome(濕熱), heat-clearing(淸熱)·removing the poisonous quality of any substance(解毒)·diuresis(利水)· obstruction-removing prescription(通腑) method decrease symptoms by copper discharge through the bile and disturbance in intestine absorption. We supposed that heat-clearing(淸熱)·removing the poisonous quality of any substance(解毒)·diuresis(利水)· obstruction-removing prescription(通腑) method therapy could be supportive therapy in wilson's disease by copper discharge ability equal to zinc sulfate and low side effect in a long time medicinal therapy.
Case Report : Successful pregnancy outcome in a Korean patient with symptomatic Wilson``s disease
( Hyun Joo Lee ),( Won Joon Seong ),( Seong Yeon Hong ),( Jin Young Bae ) 대한산부인과학회 2015 Obstetrics & Gynecology Science Vol.58 No.5
Wilson``s disease is an inherited disease of copper metabolism leading to the toxic accumulation of copper, primarily in the liver and brain. Although the literature shows successful outcomes after proper treatment, pregnant patients with Wilson``s disease still need close monitoring and management. Here, we report the case of a successful pregnancy in a Korean woman with Wilson``s disease. A 33-year-old primigravid patient with Wilson``s disease visited our antenatal clinic. Of her own volition, she had stopped her medication 2 years earlier. Oral zinc oxide therapy was started, and she was closely monitored throughout her pregnancy. She delivered a healthy female infant weighing 3.13 kg through a cesarean section. After delivery, the clinical course of both the mother and the baby were uneventful. We review crucial points in the treatment and the management dilemmas raised by the patient.
한성식(SS Han),권혜경(HK Kwon),김세광(SK Kim),이경진(KJ Lee),손용석(YS Sohn),문성은(SE Moon) 대한산부인과학회 1999 Obstetrics & Gynecology Science Vol.42 No.6
Wilson`s disease associated with pregnancy is not common and the underlying mechanism triggering the event is not known at present. There are few reports of successful pregnancies in women with untreated Wilson`s disease. Recently we experienced a 28-year-old pregnant woman with Wilson`s disease who had liver cirrhosis with hemolytic anemia. The patient had a successful pregnancy outcome with no complication.
뇌하수체 호르몬의 분비 이상을 보인 Wilson 씨 병
김대현(Dae Hyun Kim),권범찬(Bum Chan Kweon),이한균(han Keun Lee),남근하(Keun Ha Nam),김석준(Seog Jun Kim),이동욱(Dong Wook Lee),박찬규(Chan Gyu Park),이창기(Chang Gi Lee),이상문(Sang Moon Lee),이중기(Choong Ki Lee) 대한내과학회 1997 대한내과학회지 Vol.52 No.3
Wilson`s disease is a hereditary disorder in hepa- tic excretion of copper that results in toxic accumulations of the metal in liver, brain and other organs. Though there have been some reports of Wilson's disease with hepatic, neurologic, psychiatric symptoms, or hemolytic anemia, a case of Wilson's disease with abnormal secretion of pituitary hormones is, to our best knowledge, the first report in Korea. We report a case of Wilson`s disease with abnormal secretion of pituirary hormones, chronic active hepatitis with feature of early cirrhosis and hemolytic anemia in 18-year-old man showing delayed puberty. And also we describe a case of successful desensitization to penicillamine in a penicillamine hypersensitivity.
간세포암이 병발한 윌슨병(Wilson`s disease)
김휘영 ( Hwi Young Kim ),박주경 ( Joo Kyung Park ),심주현 ( Joo Hyun Shim ),이정훈 ( Jeong Hoon Lee ),김윤준 ( Yoon Jun Kim ),윤정환 ( Jung Hwan Yoon ),이효석 ( Hyo Suk Lee ) 대한내과학회 2005 대한내과학회지 Vol.69 No.-
In Wilson`s disease, liver cirrhosis is a common complication. However, hepatocellular carcinoma rarely develops in Wilson`s disease, in contrast to other liver diseases, such as postnecrotic cirrhosis or hemochromatosis. It has been speculated that the accumulated copper in the liver may prevent the development of hepatocellular carcinoma and the use of chelating agents may decrease the copper in the liver resulting in the development of carcinoma. However, there are various evidences contradicting this hypothesis. We report a 45-year-old male patient of Wilson`s disease with hepatocellular carcinoma, and the literature was reviewed. (Korean J Med 69:S782-S786, 2005)