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RESTORATION OF THE UPPER CENTRAL INCISOR FOR PATIENTS WITH CEREBRAL PALSY : A REPORT OF 3 CASES (뇌성마비 환자의 전치부 수복)

  • Yoon, Hae-Jeong;Jung, Bok-Yeong;Kim, Seung-Hye;Song, Je-Seon;Lee, Jae-Ho
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.6 no.1
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    • pp.19-22
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    • 2010
  • Cerebral palsy is an umbrella term encompassing a group of non-progressive non-contagious motor conditions that cause physical disability in human development. Motor disorder of cerebral palsy is often accompanied by disturbances of sensation, perception, cognition, communication, behavior and seizure disorder. Disharmony of motor function leads to frequent falling down. Moreover patients have high prevalence of class II malocclusion. Compared to normal patients, the patients with cerebral palsy tends to have high prevalence of recurrent trauma and bruxism which make restoration of the anterior tooth more difficult. This case report is consisted of three cases of cerebral palsy patients who have challenging problems with restoration of anterior teeth.

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A case of Menkes disease with unusual hepatomegaly (멘케스병에서 간비대를 보인 1례)

  • Jeong, Go Un;Cho, Anna;Hwang, Hee;Hwang, Yong Seung;Kim, Ki Joong;Chae, Jong Hee;Seo, Jeong Kee
    • Clinical and Experimental Pediatrics
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    • v.51 no.5
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    • pp.538-541
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    • 2008
  • Menkes disease is an X-linked recessive copper transport disorder characterized by neurological deterioration, connective-tissue damage, and abnormal hair growth. It is caused by the mutation of the ATP7A gene. This report describes a four-month-old boy with neurological symptoms typical of Menkes disease plus unusual liver involvement. He developed seizures at three months of age and exhibited hypotonia, cephalhematoma, a sagging face, redundant and hypopigmented skin, and abnormal hair growth. In addition, he had unexplained hepatomegaly and high hepatic transaminase. We confirmed the diagnosis of Menkes disease by mutation analysis of the ATP7A gene. To exclude other possible causes for the hepatic abnormalities, a liver biopsy was performed, revealing intracytoplasmic cholestasis, focal spotty necrosis, and minimal lobular activity. The patient's liver involvement may be an underestimated complication of Menkes disease.

Results of Anxiety Disorder Screening Programat the 2013 Mental Health Exposition in Seoul (2013년 정신건강박람회 불안 장애 선별검사 결과)

  • Choi, Kangrok;Kim, Daeho;Seo, Ho Jun;Huh, Hyu Jung;Lee, Dong-Woo;Chae, Jeong Ho
    • Anxiety and mood
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    • v.9 no.2
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    • pp.147-153
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    • 2013
  • Objective : Despite the chronic nature and high social costs, individuals with anxiety disorders seldom seek treatment. Thus, education for public awareness and screening for the illness is tremendously important for mental health professionals. This study summaries and presents the results from Anxiety Disorder Screening Program during the Mental Health Exposition held in Seoul in April, 2013. Methods : We analyzed the data from 116 participants who agreed and completed the screening questionnaires during their visits to two-day Anxiety Disorder Screening Program prepared by the Korean Academy of Anxiety Disorder. The questionnaire comprised of modified Mobility inventory for agoraphobia, Contents of worries, Penn State Worry Questionnaire, Life Events Checklist, and Abbreviated Posttraumatic Stress Disorder Checklist. Results : Participants demonstrated high rates of anxiety symptoms and possible anxiety disorders. Experience of panic attack was reported by 45%, lifetime and 16% in the past month by respondents. Phobia was reported by 46%. Participants had an average of 3.3 pathological worries and among those, social or interpersonal content was most common (46%). At least one lifetime traumatic event was reported by 64%. By the cut-off scores in the literature, 46% had possible generalized anxiety disorder and 58% possible posttraumatic stress disorder. Conclusion : Our results suggested that many visitors to Anxiety Disorder Screening Program were in fact treatment seeking after experiencing some forms of anxiety symptoms. Further efforts for delivery of medical information and increasing public awareness for anxiety disorders are needed.

A Case of asymptomatic Short-chain Acyl-CoA Dehydrogenase Deficiency (무증상의 경쇄 acyl-CoA 탈수소효소 결핍증 1례)

  • Lee, Hwapyung;Kim, Jinsup;Huh, Rimm;Cho, Sung Yoon;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.15 no.2
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    • pp.98-100
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    • 2015
  • Short-chain acyl-CoA dehydrogenase (SCAD) deficiency is a rare mitochondrial fatty-acid oxidation disorder that is inherited as an autosomal recessive pattern. SCAD deficiency is caused by mutations in the ACADS gene (Acyl-CoA Dehydrogenase, Short-chain, OMIM #606885), which encodes SCAD, the mitochondrial enzyme that catalyzes the first reaction in the beta-oxidation of fatty acids four to six carbons in length. Here, we describe one Korean pediatric case of SCAD deficiency, which was diagnosed during newborn screening through tandem mass spectrometry. An increased concentration of butyrylcarnitine was detected on the newborn screening test, and the urine organic acid analysis showed increased urinary excretion of ethylmalonic acid. The patient has been asymptomatic and has shown normal growth and development by 8 months of age without any intervention during follow-up period.

Short-chain Acyl-CoA Dehydrogenase Deficiency in an Asymptomatic Neonate (무증상 신생아에서 진단된 경쇄 acyl-CoA 탈수소효소 결핍증 1례)

  • Lee, Yeonhee;Kim, Jinsup;Huh, Rimm;Cho, Sung Yoon;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.15 no.2
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    • pp.93-97
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    • 2015
  • Short-chain acyl-CoA dehydrogenase (SCAD) deficiency is an autosomal recessive hereditary metabolic disorder of mitochondrial fatty acid beta-oxidation. Mutations in the ACADS gene cause short-chain acyl-CoA dehydrogenase deficiency, which is characterized by developmental delay, hypotonia, seizure, and hypoglycemia. Here, we describe one Korean pediatric case of SCAD deficiency, which was diagnosed during newborn screening by tandem mass spectrometry and confirmed by molecular analysis. The level of C4 was typically elevated 5.23 mg/dL (reference range <1.5 mg/dL). This patient had a homozygous mutation [c.1031A>G, p. E344G] in ACADS. Therefore, we present a case of SCAD deficiency in an otherwise healthy neonate and her subsequent development and growth over four years.

One Case of Dizziness Patients Suggested Benign Paroximal Positional Vertigo treated by Jaeumkunbitang-gamibang and Dix-hallpike maneuver (양성 발작성 자세변환성 현훈(Benign Paroximal Positional Vertigo, BPPV)으로 의심되는 현훈증 환자를 자음건비탕(滋陰健脾湯) 가미방(加味方)과 Dix-hallpike Maneuver로 치료한 치험 1례)

  • Shin, Sun-Ho;Jeong, Yong-Jun
    • The Journal of Internal Korean Medicine
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    • v.21 no.1
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    • pp.181-184
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    • 2000
  • Dizziness is one of common diseases clinically, it is defined as a hallucination or an illusion of motion that causes sensation disorder of circumstance, and described as circulatory. rotatory leaning. shaking sensation. In particular, benign paroximal positional vertigo(BPPV) is one of peripheral vertigo, it causes dizziness due to debris which has collected within a part of the inner ear. Chemically, debris are small crystals of calcium carbonate. They are derived from structures in the ear called 'otoliths' that have been damaged by head injury, infection, or other disorder of the inner ear, or degenerated because of advanced age. The symptoms of BPPV include dizziness or vertigo, lightheadedness, imbalance, and nausea, Activities which bring on symptoms will vary in each person, but symptom are almost always precipitated by a position change of the head or body. As for treatment of vertigo, it is differentiated as excess in the upper and deficiency in the lower(上實下虛) and treated in oriental medicine and are used to stability. antihistaminics . anticolinergics . vestibule control drug of GABA system in western medicine. Moreover, Dix-hallpike maneuver is applicated in diagnosis and treatment of BPPV patients. A case of dizziness patient suggested benign paroximal positional vertigo who is diagnosed as weakly dizziness(虛暈)showed prominent improvement by Jaeumkunbitang-gamibang(滋陰建脾湯) and Dix-hallpike maneuver.

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Early Result of Bentall Operation (Bentall 술식의 조기 성적)

  • 송명근
    • Journal of Chest Surgery
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    • v.31 no.2
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    • pp.113-117
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    • 1998
  • From October 1993 to April 1997, 37 patients(30 male and 7 female patients), ranging in age from 23 to 73 years, were operated for annuloaortic ectasia with or without aortic dissection. Four patients were in New York Heart Association Class I, 10 in Class II, 20 in Class III, and 3 in Class IV. In cases of annuloaortic ectasia with aortic dissection, the surgical treatment in all cases consisted of total replacement of the ascending aorta with composite graft and reimplantation of the coronary arteries to the tube graft. The postoperative complication, as postoperative bleeding or LV dysfunction, was 18%(7 cases) and late mortality for entire group was 2.7%(one death). The period of follow-up ranged from 1 month to 36 months(average 9.6 months). In conclusion, Bentall operation for annuloaortic ectasia with or without aortic dissection is reliable method with low mortality and excellent short-term results.

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The Effect of Gabapentin for the Clinical Symptoms in the Traumatic Neuropathic Pain (외상성 신경병증성 통증의 각 임상증상에 대한 Gabapentin의 효과)

  • Kim, Yeung-Ki;Cho, Yun-Woo
    • Journal of Yeungnam Medical Science
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    • v.21 no.1
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    • pp.82-90
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    • 2004
  • Background: Gabapentin is widely used for the relief of neuropathic pain. But, there is no study of gabapentin in relation to traumatic neuropathic pain. The aim of this study is to assess the efficacy and effectiveness of gabapentin for the various clinical symptoms of traumatic neuropathic pain Materials and Methods: 50 patients with traumatic nerve injury were assigned to receive gabapentin, titrated to 900 mg/day over 9 days, followed by further increases to a maximum of 2400 mg/day. Continuous pain, paroxysmal pain, allodynia and thermal evoked pain were measured in mean daily pain scores, based on the 11-point Likert scale. The primary efficacy parameter was compared from the baseline to the final study week. Results: Over the 4.5 week study, this pain score decreased by 2.6 points in the continuous pain, 3.6 points in the paroxysmal pain, 3.1 points in the allodynia, and 2.5 points in the thermal evoked pain. The percentage of patients with over 50% improvement in pain scores was 33% in the continuous pain, 67% in the paroxysmal pain, 53% in the allodynia and 36% in the thermal evoked pain. There was no significant correlation between the effect of gabapentin and the time difference of the onset of symptoms and start of medication. Conclusions: This study shows that gabapentin reduced neuropathic pain in patients with traumatic peripheral nerve injury. Among the various characteristics of neuropathic pain, the reduction of paroxysmal pain and allodynia was greatest.

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Assessment of the Relationship between Vigabatrin and Visual Field Defect in Children (항경련제 Vigabatrin이 시야에 미치는 영향)

  • Chung, Sa Jun;Chung, Yong Hwan;Cho, Eu Hyun
    • Clinical and Experimental Pediatrics
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    • v.45 no.12
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    • pp.1546-1550
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    • 2002
  • Purpose : The authors carried out this study to determine the relationship between vigabatrin (VGB) and visual field defect.. Methods : Seventy eight patients older than 8 years who had epilepsy which had developed and been diagnosed, and were receiving add-on therapy, were the subjects of this study. If suspicious results were obtained from the initial test with the Humphrey automatic perimeter, the patient was tested again with the Goldman perimeter. Follow-up examinations were performed on these patients after 6 months. Results : In this study, five of the 78 patients had suspicious primary test results, but upon the second examination they were all found to be normal. Thus there were no patients with visual field defects. Conclusion : VGB is a drug which may cause visual field defects, but in this study no patients presented with this symptom. Instead of limiting the use of VGB due to the adverse effect of visual field defect in the initial treatment of partial seizure and infantile spasm untreatable with other medication, if used with care it may not cause serious problems. Screening for visual defect is recommended, and in patients taking VGB regular examination is necessary.

A Case of Influenza-associated Encephalopathy (인플루엔자 관련 뇌증 1례)

  • Song, Yeoni;Choi, Chang Hwan;Choi, Jong Woon;Kim, Se Young;Kim, Hyun Soo;Kim, Yeol;Im, Dong Jin
    • Clinical and Experimental Pediatrics
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    • v.46 no.10
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    • pp.1024-1028
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    • 2003
  • Influenza-associated encephalopathy is regarded as one of the major neurologic disease entities along with those of Reye syndrome, acute necrotizing encephalopathy, and myelitis which are known to be related to influenza virus, mostly type A. And it is being actively researched in Japan as it has caused a tremendous increase in the number of deaths from 1997 to 2002, but it has not been yet reported in the Korean pediatric medical community. It attacks those previously healthy children, who have not been vaccinated. Patients start with such symptoms as fever and common respiratory problems, but within 24 to 48 hours they suffer from seizures with acute mental deterioration, become worse, and suffer multiple organ failures including marked elevated transaminase levels as well as coagulopathy. It induces deaths in a couple of days after the symptoms appear or remains a serious neurologic sequelae. Confirmative diagnosis is used to demonstrate influenza viral infection. We report here a 37 month aged boy who was admitted to our hospital during the last influenza season under the diagnosis of influenza associated encephalopathy on the basis of serologic testing by hemagglutinin inhibition(HI). This is the first report confirmed by increased antibody titer of the influenza A virus in Korea.