• Journal of Korean Neurosurgical Society
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• 제56권6호
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• pp.492-495
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• 2014

A ruptured distal lenticulostriate artery (LSA) aneurysm is detected occasionally in moyamoya disease (MMD) patients presented with intracerebral hemorrhage. If the aneurysm is detected in hemorrhage site on angiographic evaluation, its obliteration could be considered, because it rebleeds frequently, and is associated with poorer outcome and mortality in MMD related hemorrhage. In this case report, the authors present two MMD cases with ruptured distal LSA aneurysm treated by endovascular embolization.

• Clinical and Experimental Pediatrics
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• 제55권2호
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• pp.48-53
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• 2012

Purpose: Gaucher disease is caused by a ${\beta}$-glucocerebrosidase (GBA) deficiency. The aim of this study is to investigate the clinical and genetic characteristics according to subtypes of Gaucher disease in the Korean population. Methods: Clinical findings at diagnosis, $GBA$ mutations, and clinical courses were reviewed in 20 patients diagnosed with Gaucher disease. Results: Eleven patients were diagnosed with non-neuronopathic type, 2 with acute neuronopathic type, and 7 with chronic neuronopathic type. Most patients presented with hepatosplenomegaly, thrombocytopenia, and short stature. In the neuronopathic group, variable neurological features, such as seizure, tremor, gaze palsy, and hypotonia, were noted at age $8.7{\pm}4.3$ years. B cell lymphoma, protein-losing enteropathy, and hydrops fetalis were the atypical manifestations. Biomarkers, including chitotriosidase, acid phosphatase, and angiotensin-converting enzyme, increased at the initial evaluation and subsequently decreased with enzyme replacement treatment (ERT). The clinical findings, including hepatosplenomegaly, thrombocytopenia, and skeletal findings, improved following ERT, except for the neurological manifestations. L444P was the most common mutation in our cohort. One novel mutation, R277C, was found. Conclusion: Although the clinical outcome for Gaucher disease improved remarkably following ERT, the outcome differed according to subtype. Considering the high proportion of the neuronopathic form in the Korean population, new therapeutic strategies targeting the central nervous system are needed, with the development of a new scoring system and biomarkers representing clinical courses in a more comprehensive manner.

• Asian Pacific Journal of Cancer Prevention
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• 제16권18호
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• pp.8041-8045
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• 2016

Cervical cancer is one of the most common cancers in women worldwide. During their life time the vast majority of women become infected with human papillomavirus (HPV), but interestingly only a small portion develop cervical cancer and in the remainder infection regresses to a normal healthy state. Beyond HPV status, associated molecular characterization of disease has to be established. However, initial work suggests the existence of several different molecular classes, based on the biological features of differentially expressed genes in each subtype. This suggests that additional risk factors play an important role in the outcome of infection. Host genomic factors play an important role in the outcome of such complex or multifactor diseases such as cervical cancer and are also known to regulate the rate of disease progression. The aim of this review was to compile advances in the field of host genomics of HPV positive and negative cervical cancer and their association with clinical response.

• IMMUNE NETWORK
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• 제7권4호
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• pp.167-172
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• 2007

Various cell types that express regulatory function may influence the pathogenesis of most and perhaps all infections. Some regulatory cells are present at the time of infection whereas others are induced or activated in response to infection. The actual mechanisms by which different types of infections signal regulatory cell responses remain poorly understood. However a most likely mechanism is the creation of a microenvironment that permits the conversion of conventional T cells into cells with the same antigen specificity that have regulatory function. Some possible means by which this can occur are discussed. The relationship between regulatory cells and infections is complex especially with chronic situations. The outcome can either be of benefit to the host or damage the disease control process or in rare instances appears to be a component of a finely balanced relationship between the host and the infecting agent. Manipulating the regulatory cell responses to achieve a favorable outcome of infection remains an unfulfilled objective of therapeutic immunology.

• 대한자기공명의과학회:학술대회논문집
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• 대한자기공명의과학회 2003년도 제8차 학술대회 초록집
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• pp.38-39
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• 2003

To evaluate the clinical characteristics and outcome of the patients with disseminated pilocytic astrocytoma compared to non-disseminated disease and to discuss treatment options for disseminated disease. We Identified patients with disseminated pilocytic astrocytoma from our Tumor Registry over 21 years of period and reviewed medical records and neuroimages to determine location of the tumor, pattern of dissemination, clinical characteristics, treatment, and outcome. 24 controls without dissemination, matched for age at diagnosis, tumor histology, and primary location were selected from the same registry and case/control comparison was done using Kaplan-Meier survival analysis.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제10권1호
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• pp.1-10
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• 2007

Outcome of liver transplantation for children with end liver disease has been improved markedly during last two decades. Improvement of immunosuppressive agent and its strategy to use in children, development of innovative surgical technique, and better understanding of the course of liver transplantation attributed to better outcome of pediatric liver transplantation. Therefore this review article will focus on the problems which can occur during pre- and post-transplantation period, current strategy to use immunosuppressive agent for the better understanding of pediatrician who is not involved in transplantation but takes care of the children pre- and postoperatively.

• Journal of Preventive Medicine and Public Health
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• 제40권4호
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• pp.273-277
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• 2007

The purpose of establishing the circulatory disease surveillance system in Korea is to ensure that the problems of circulatory disease importance are being monitored efficiently and effectively. The goals of circulatory disease surveillance system are to monitor the epidemiological trends of circulatory disease and to evaluate the outcome of health activity for controlling circulatory diseases. Surveillance system are being updated to achieve the needs for the integration of the surveillance and information system, the establishment of data standards, the electronic exchange of data, and changes in the goals of circulatory disease surveillance system to facilitate the response of this system to manage the national health problem effectively. This article provides the target diseases and determinant indicators to be monitored, structure of circulatory disease surveillance system, and many tasks and related activities that should be applied to this system.

• Journal of Korean Neurosurgical Society
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• 제58권5호
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• pp.401-411
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• 2015

More than 10 years have passed since lumbar total disc replacement (LTDR) was introduced for the first time to the world market for the surgical management of lumbar degenerative disc disease (DDD). It seems like the right time to sum up the relevant results in order to understand where LTDR stands on now, and is heading forward to. The pathogenesis of DDD has been currently settled, but diagnosis and managements are still controversial. Fusion is recognized as golden standard of surgical managements but has various kinds of shortcomings. Lately, LTDR has been expected to replace fusion surgery. A great deal of LTDR reports has come out. Among them, more than 5-year follow-up prospective randomized controlled studies including USA IDE trials were expected to elucidate whether for LTDR to have therapeutic benefit compared to fusion. The results of these studies revealed that LTDR was not inferior to fusion. Most of clinical studies dealing with LTDR revealed that there was no strong evidence for preventive effect of LTDR against symptomatic degenerative changes of adjacent segment disease. LTDR does not have shortcomings associated with fusion. However, it has a potentiality of the new complications to occur, which surgeons have never experienced in fusion surgeries. Consequently, longer follow-up should be necessary as yet to confirm the maintenance of improved surgical outcome and to observe any very late complications. LTDR still may get a chance to establish itself as a substitute of fusion both nominally and virtually if it eases the concerns listed above.

• Journal of Korean Neurosurgical Society
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• 제42권6호
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• pp.436-440
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• 2007

Objective : Spinal cord hemangioblastoma is an uncommon vascular neoplasm with a benign nature and is associated with von Hippel-Lindau (VHL) disease in 20-30% of patients. Total removal of these tumors without significant neurological deficit remains a great challenge. The purpose of this study was to investigate the efficacy of VHL mutation analysis and to evaluate surgical outcome of patients with spinal cord hemangioblastomas. Methods : This study included nine patients treated for spinal cord hemangioblastomas at our institute between December 1994 and March 2006. There were four male and five female patients. Mean age was 37.8 years. The mean follow-up period was 22.4 months. Magnetic resonance imaging (MRI) of the complete neuraxis was done in all cases and VHL mutation analysis was performed in three cases for a definite diagnosis. Results : Six patients had intramedullary tumor, and the remaining patients had intradural extramedullary lesions. Five patients were associated with VHL disease. The von Hippel-Lindau mutation analysis was done in three patients and two of them showed VHL gene abnormality. Tumors were located in the cervical cord in five cases and in the thoracic cord in four cases. All patients underwent surgical intervention, and total removal was achieved in six cases. All patients showed improvement or, at least, clinically stationary state. Surgical complications did not develop in any cases. Conclusion : Spinal hemangioblastoma in this series has been safely and effectively removed via a posterior approach. Postoperatively, clinical outcome was excellent in the majority of cases. The VHL mutation analysis was useful in patients with family history and in those with multiple hemangioblastomas.

• 한국임상수의학회지
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• 제34권1호
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• pp.1-6
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• 2017

Heartworm disease (HWD) in dogs is a life-threatening mosquito-borne disease resulting in right-sided congestive heart failure and inflammatory pulmonary disease. Due to complications from adulticidal therapy with melarsomine, slow kill protocol either with preventive dose of ivermectin or combined with doxycycline has been proposed for an alternative adultcidal therapy in dogs with HWD. Therefore, this study evaluated the clinical outcome of adultcidal therapy in dogs with class II stage of HWD after treating either American Heartworm Society (AHS) or slow kill protocol for 10 months. Clinical outcome after therapy was evaluated by clinical, radiographic and echocardiographic examination along with hematology before (D0) and after therapy (D300). Although clinical signs associated with HWD were all resolved after therapy in both groups, the infection was not cleared out 67% of dogs treated by slow kill protocol at the end of therapy. Furthermore, pulmonary arterial flow of acceleration time to ejection time ratio (AT/ET) and the right pulmonary artery distensibility index (RPADI) have been firstly used for detecting pulmonary hypertension in this study group. The pulmonary hypertension was more common in dogs with mild clinical signs, although tricuspid and pulmonary regurgitation were not detectable in most dogs in this study. Our study findings suggested that the slow kill protocol might not be efficacious enough to clear out HWD in dogs and more attention on the presence of pulmonary hypertension might be necessary for effective management of HWD in dogs.