• Journal of Genetic Medicine
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• v.16 no.1
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• pp.31-38
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• 2019

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a maternally inherited mitochondrial disorder of which m.3243A>G is the most commonly associated mutation, resulting in an inability to meet the energy requirements of various organs. MELAS poses a diagnostic challenge owing to its multiple organ involvement and great clinical variability due to its heteroplasmic nature. We report three cases from a family who were initially misdiagnosed with myasthenia gravis or undiagnosed. Although there is no optimal consensus treatment approach for patients with MELAS because of the disease's heterogeneity, our 21-year-long therapy regimen of ${\text\tiny{L}}-arginine$, ${\text\tiny{L}}-carnitine$, and coenzyme Q10 supplementation combined with dietary management appeared to provide noticeable protection from the symptoms and complications. Prompt early diagnosis is important, as optimal multidisciplinary management and early intervention may improve outcomes.

• Korean Journal of Bronchoesophagology
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• v.13 no.2
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• pp.77-81
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• 2007

Foreign body aspiration is a cause of the accidental death at home. Therefore, early intervention and proper management is important. A bronchoscopy is indicated whenever there is a suggestive history and medical opinion. Occasionally, foreign body removal with bronchoscopy may be fail. But, on the situation, there is no definite recommended standard management. We experienced two cases of bronchial foreign body could not be removed with bronchoscopy at first intervention. The one was diagnosed too late. Endobronchial granulation tissue and edema made it impossible to find the foreign body at first bronchoscopy. After steroid and antibiotic therapy, foreign body could be removed with secondary bronchoscopy. Another was bronchial foreign body jammed tightly bronchus intermedius. Even after medical therapy, patient got aggravated. So foreign body was removed with bronchotomy.

• Journal of Chest Surgery
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• v.29 no.4
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• pp.440-443
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• 1996

Some tricuspid valve endocarditis can be controlled effectively with specific antibiotic treatment. However, surgical intervention Is necessary when there are continuing sepsis, moderate or severe heart failure, multiple pulmonary emboli, and echocardiographycally demonstrated vegitations. We are repoting a 19 year-old male patient who was admitted for the treatment of infective endocarditis. He previously had an operation for ventriculer septal defect (perimembranous type) about 9 years ago . An echocardiogram showed a large vegetation on the anterior cusp area and a left to right shunt through VSD, which was previously closed with dacron patch. A valve replacement in addition to antibiotic therapy was recommended for the patient. The patient underwent on operation : tricuspid valve replacement was done with 51. Jude medical valve prosthesis (33 mm), and in addition to above procedure, removal of vegetation and direct closure of VSD were done Postoperative echocardiogram showed that replaced tricuspid valve functioned well and vegeta ion and shunt flow were not observed. The patient recovered without complication and discharged at Postoperative day 25. Early aggressive surgical intervention is indicated to optimize surgical results, and this case seems to be a typical right sided bacterial endocarditis, which is caused by residual VSD. We are reporting a case of tricuspid valve endocarditis with a review of the literature. (Korean J Thorax Cardiovasc Surg 1996 ; 29: 440-3)

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.18 no.2
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• pp.117-122
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• 2007

Practice parameters for non-pharmacological treatment of children and adolescents with pervasive developmental disorders are based on the scientific literature for evidence-based practices. Appropriate educational and behavioral interventions are important in improving the long-term outcome in pervasive developmental disorders. Early and sustained intervention appears to be particularly important. The goal for interventions is to gain pragmatic skills for verbal communication, playing with peers, daily living routines, self-management, and social adaptation. Appropriate involvement and collaboration with parents and family are essential for well-functioning intervention programs. The life-long nature of autism implies that the clinician should maintain an active role in long-term treatment planning and family support. Vocational training and training for more independent living are important for adolescents with autism. Professionals should be knowledgeable about local and national resources and opportunities for family support as well as support of the individual.

• Korean Journal of Health Education and Promotion
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• v.30 no.1
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• pp.65-81
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• 2013

Objectives: The present study was carried out to develop and evaluate comprehensive health care program to prevent infectious disease and promote health in child-care centers by Doctor of Korean medicine. Methods: A nonequivalent control group pretest-posttest design study was conducted on 568 children and 85 child care teacher at 12 child care facilities for 12 weeks from July to October 2012. The program was consist of management, education, screening under concepts of traditional preventive medicine, Yangsaeng and Chimibyeong. Children's medical utilization due to infectious disease and attendance means functional status were measured by reports from parents. The Difference in difference(DID) estimator was applied data analysis, and added Zero-inflated negative binomial regression model. Also, attitudes on the infection of teacher was measured and analyzed through t-test. Results: After the intervention, the total medical utilization due to infectious disease decreased, but not significantly. Total absence, early leave and lateness decreased significantly. But, Attitude on the infection of child care teacher was not changed. The parent's satisfaction showed positive overall. Conclusions: The intervention program may be effective in preventing infectious disease and managing health in child-care center partially. To measure long-term effect, long-term study improved is requested.

• Archives of Plastic Surgery
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• v.34 no.3
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• pp.319-324
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• 2007

Purpose: Necrotizing fasciitis is a rapid progressive, life-threatening disease. Many organisms have been identified for causative pathogens of necrotizing fasciitis. And necrotizing fasciitis caused by Vibrio vulnificus is a matter of grace concern in Southwest seashore of Korea recently. But concrete analysis between these pathogens was not executed yet. Methods: Sixty necrotizing fasciitis patients were included in this study. We divided the patients into 2 groups: Group A was the case which Vibrio vulnificus was cultured for causative pathogen of necrotizing fasciitis, and Group B was the case of other organisms. And we analysed each group for treatment, progression and prognosis. Results: There was no significant difference between two groups in total hospital stay but there was a great difference in ICU stay and progression to septic shock. Also, there was a great difference in mortality within 48 hours but there was no difference in mortality of 48 hours after hospital visit. Conclusion: This indicates that intial management of necrotizing fasciitis cased by Vibrio vulnificus is the key of treatment. So initial medical management with early surgical intervention is necessary for necrotizing fasciitis.

• The Korean Journal of Medicine
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• v.93 no.6
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• pp.565-570
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• 2018

Postcardiac injury syndrome (PCIS) is an inflammatory process that usually occurs within 1 to 6 weeks after an injury to the pericardium, epicardium, or myocardium. As more interventions are performed for complicated coronary artery obstructive lesions, there have been some recent reports on PCIS following percutaneous coronary intervention (PCI). The medical management of PCIS depends on nonsteroidal anti-inflammatory drugs (NSAIDs), in addition to colchicine or steroids. An 80-year-old male patient underwent a PCI. Unfortunately, the guidewire piercing failed but he showed no immediate signs of complication. However, 5 hours after the procedure, he complained of chest discomfort. An electrocardiogram showed widespread ST elevation. Chest X-ray and computed tomography showed pulmonary congestion with pleural effusion, while thoracic echocardiography showed a moderate amount of pericardial effusion. NSAIDs were initiated, but there was no improvement of symptoms. We describe an unusual case of atypical earl onset PCIS after PCI, recovered rapidly by steroids.

• Journal of mucopolysaccharidosis and rare diseases
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• v.5 no.1
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• pp.8-11
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• 2021

Glutaric aciduria type 1 (GA1; OMIM #231670) is a rare autosomal recessive-inherited neurometabolic disorder caused by the deficiency of glutaryl-CoA dehydrogenase (GCDH), which is encoded by the GCDH gene. It results in the accumulation of glutaric acid (GA), 3-hydroxyglutaric acid (3-OH-GA), glutaconic acid, and glutarylcarnitine (C5DC). These metabolites are considered to damage the striatum through an excitotoxic mechanism. The treatments of GA1 known to date are metabolic maintenance treatment based on a low-lysine diet and emergency treatment during acute illness. However, treatment after the onset of neurological symptoms has limited effectiveness and is associated with poor outcomes, and the effect of treatment and disease course after treatment are not good. After the implementation of newborn screening, the incidence of acute encephalopathic crisis fell to 10%-20% with early diagnosis, preventative dietary management, and aggressive medical intervention during acute episodes. Recently, several cohort studies have been published on the natural course and treatment of GA1 patients. This mini review will cover the clinical symptoms, natural history, and treatment of GA1 through a literature review.

• Journal of Korean Neurosurgical Society
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• v.30 no.6
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• pp.729-733
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• 2001

Objective : Antifibrinolytic treatment after aneurysmal subarachnoid hemorrhage has been shown to have no significant effect on outcome since a reduction in the rate of rebleeding was offset by an increase in the incidence of hydrocephalus and ischemic events. As the results of early aneurysm surgery and a change of strategy in the intensive medical treatment, outcome in patients with cerebral ischemia has been improved. On the other hand, rebleeding still remains as a major cause of death. A short course of tranexamic acid(TA) was tried to study its efficacy and safety in reducing the incidence of rebleeding before aneurysm surgery. Methods : A total of 507 patients with ruptured cerebral aneurysm operated within 3 days after the attack from 1990 to 1999 were included in this study. Group A consisted of 302 consecutive patients treated from 1990 through 1995 served as control. Two hundred-five patients in group B were treated with TA from 1996 through 1999. Both groups were evaluated for comparability of demographic and clinical variables including age, Hunt-Hess grade, Fisher grade, aneurysm location, hypertension, day of surgery, and initial hydrocephalus. The relationships of TA with rebleeding, ischemia, and chronic hydrocephalus were also studied. Results : There was no significant difference in patient demographics and clinical characteristics between group A and group B. Sixteen patients(5.3%) suffered a recurrent hemorrhage in group A and three(1.5%) in group B(p<0.05). Chronic hydrocephalus requiring a shunt was found in a significantly greater proportion in group B than in group A(p<0.05). The incidence of cerebral ischemia was not elevated in group B compared with group A. Conclusion : Considering the fact that the reduction of fatal rebleeding outweighed the increased incidence of hydrocephalus, the authors believe that a short course of TA is beneficial in diminishing the risk of rebleeding prior to early surgical intervention.

• Journal of Gastric Cancer
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• v.6 no.3
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• pp.193-197
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• 2006

Melanoma is a malignant neoplasm of melanocytes most frequently arising from the skin, but primary melanoma can also arise from the mucosa of the gastrointestinal tract. Gastrointestinal melanomas are most commonly metastases from a cutaneous melanoma. Primary melanoma of the stomach is rare and carries a poor prognosis. Reported here is the case of a 75-year-old man with a primary gastric melanoma who presented with a melena, abdominal pain, and weight loss. Most cases of melanoma are treated by excision of the primary tumor. Patients with melanoma have been treated with adjuvant chemotherapy, radiation therapy, and immunotherapy. None of these modalities has been demonstrated to prolong the survival rate. To improve long-term disease-free survival, early diagnosis and surgical intervention are very important.