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Targeted busulfan and fludarabine-based conditioning for bone marrow transplantation in chronic granulomatous disease

  • Ju, Hee Young;Kang, Hyoung Jin;Hong, Che Ry;Lee, Ji Won;Kim, Hyery;Song, Sang Hoon;Yu, Kyung-Sang;Jang, In-Jin;Park, June Dong;Park, Kyung Duk;Shin, Hee Young;Kim, Joong-Gon;Ahn, Hyo Seop
    • Clinical and Experimental Pediatrics
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    • v.59 no.sup1
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    • pp.57-59
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    • 2016
  • Chronic granulomatous disease (CGD) is a primary immunodeficiency disease caused by impaired phagocytic function. Hematopoietic stem cell transplantation (HSCT) is a definitive cure for CGD; however, the use of HSCT is limited because of associated problems, including transplantation-related mortality and engraftment failure. We report a case of a patient with CGD who underwent successful HSCT following a targeted busulfan and fludarabine reduced-toxicity myeloablative conditioning. Intravenous busulfan was administered once daily for 4 consecutive days (days -8 to -5), and the target area under the curve was $75,000{\mu}g{\cdot}hr/L$. Fludarabine ($40mg/m^2$) was administered once daily for 6 consecutive days from days -8 to -3. Antithymocyte globulin (2.5 mg/kg/day) was administered from days -4 to -2. The patient underwent successful engraftment and did not have any severe toxicity related to the transplantation. Conditioning with a targeted busulfan and fludarabine regimen could provide a better outcome for HSCT in CGD, with close regulation of the busulfan dose.

Regional Myocardial Blood Flow Estimation Model Using Rubidium-82 Dynamic Myocardial PET and Modified Double Integration Method (Rubidium-82 심근 Dynamic PET 영상과 변형이중적분법을 이용한 국소 심근 혈류 예측 모델)

  • Park, Yong-Woo;Lee, Jae-Sung;Lee, Tae-Hoon;Kwak, Cheol-Eun;Lee, Dong-Soo;Kang, Keon-Wook;Park, Jae-Hyung;Jeong, Jae-Min;Chung, June-Key;Lee, Myung-Chul;Seo, Joung-Don;Min, Byoung-Goo;Koh, Chang-Soon
    • Proceedings of the KOSOMBE Conference
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    • v.1995 no.11
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    • pp.157-158
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    • 1995
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Apocrine Gland Adenocarcinomas in 4 Dogs

  • Oh, Ye-In;Seo, Kyoung-Won;Han, Sei-Myoung;Sohn, Suh-Young;Kim, Hyung-Jin;Chung, Jin-Young;Hong, Soo-Ji;Pyoun, Hanna;Kim, Dae-Yong;Lee, Chang-Woo;Yoon, Jung-Hee;Hwang, Cheal-Yong;Youn, Hwa-Young
    • Proceedings of the Korean Society of Veterinary Clinics Conference
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    • 2008.10a
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    • pp.151-151
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    • 2008
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Breast Sparganosis Presenting with a Painless Breast Lump: Report of Two Cases

  • Oh, Moon Young;Kim, Kyoung-Eun;Kim, Min Jung;Chu, Ajung;Lee, Jong Yoon;Park, Jeong Hwan;Kim, Jongjin;Hwang, Ki-Tae
    • Parasites, Hosts and Diseases
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    • v.57 no.2
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    • pp.179-184
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    • 2019
  • Sparganosis is a parasitic infestation caused by sparganum, a plerocercoid tapeworm larva of the genus Spirometra. Since the first case of human sparganosis reported in 1908, sparganosis has been a global disease, and is common in China, Japan, and Southeast Asian countries. Consumption of raw snakes, frogs, fish, or drinking contaminated beverages are sources of human infections. Human sparganosis usually manifests in subcutaneous fat in areas such as the abdomen, genitourinary tract, and limbs. Breast sparganosis cases are rare, representing less than 2% of total cases of human infections. Complete surgical extraction of the sparganum is the treatment of choice. Because of the rarity of the disease, clinical suspicion is vital to reach the diagnosis of breast sparganosis. Here we report 2 rare cases of breast sparganosis presenting with a painless breast lump, both treated with surgical excision and sparganum extraction.

Clinical Outcomes of Coil Embolization for Unruptured Intracranial Aneurysms Categorized by Region and Hospital Size : A Nationwide Cohort Study in Korea

  • Bong-Gyu Ryu;Si Un Lee;Hwan Seok Shim;Jeong-Mee Park;Yong Jae Lee;Young-Deok Kim;Tackeun Kim;Seung Pil Ban;Hyoung Soo Byoun;Jae Seung Bang;O-Ki Kwon;Chang Wan Oh
    • Journal of Korean Neurosurgical Society
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    • v.66 no.6
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    • pp.690-702
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    • 2023
  • Objective : To analyze the outcomes of coil embolization (CE) for unruptured intracranial aneurysm (UIA) according to region and hospital size based on National Health Insurance Service data in South Korea. Methods : The incidence of complications, including intracranial hemorrhage (ICRH) and cerebral infarction (CI), occurring within 3 months and the 1-year mortality rates in UIA patients who underwent CE in 2018 were analyzed. Hospitals were classified as tertiary referral general hospitals (TRGHs), general hospitals (GHs) or semigeneral hospitals (sGHs) according to their size, and the administrative districts of South Korea were divided into 15 regions. Results : In 2018, 8425 (TRGHs, 4438; GHs, 3617; sGHs, 370) CEs were performed for UIAs. Complications occurred in 5.69% of patients seen at TRGHs, 13.48% at GHs, and 20.45% at sGHs. The complication rate in TRGHs was significantly lower than that in GHs (p=0.039) or sGHs (p=0.005), and that in GHs was significantly lower than that in sGHs (p=0.030). The mortality rates in TRGHs, GHs, and sGHs were 0.81%, 2.16%, and 3.92%, respectively, with no significant difference. Despite no significant difference in the mortality rates, the complication rate significantly increased as the number of CE procedures per hospital decreased (p=0.001; rho=-0.635). Among the hospitals where more than 30 CEs were performed for UIAs, the incidence of CIs (p=0.096, rho=-0.205) and the mortality rates (3 months, p=0.048, rho=-0.243; 1 year, p=0.009, rho=-0.315) significantly decreased as the number of CEs that were performed increased and no significant difference in the incidence of post-CE ICRH was observed. Conclusion : The complication rate in patients who underwent CE for UIA increased as the hospital size and physicians' experience in conducting CEs decreased. We recommend nationwide quality control policies CEs for UIAs.

Blood-Brain Barrier Disruption in Mild Traumatic Brain Injury Patients with Post-Concussion Syndrome: Evaluation with Region-Based Quantification of Dynamic Contrast-Enhanced MR Imaging Parameters Using Automatic Whole-Brain Segmentation

  • Heera Yoen;Roh-Eul Yoo;Seung Hong Choi;Eunkyung Kim;Byung-Mo Oh;Dongjin Yang;Inpyeong Hwang;Koung Mi Kang;Tae Jin Yun;Ji-hoon Kim;Chul-Ho Sohn
    • Korean Journal of Radiology
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    • v.22 no.1
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    • pp.118-130
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    • 2021
  • Objective: This study aimed to investigate the blood-brain barrier (BBB) disruption in mild traumatic brain injury (mTBI) patients with post-concussion syndrome (PCS) using dynamic contrast-enhanced (DCE) magnetic resonance (MR) imaging and automatic whole brain segmentation. Materials and Methods: Forty-two consecutive mTBI patients with PCS who had undergone post-traumatic MR imaging, including DCE MR imaging, between October 2016 and April 2018, and 29 controls with DCE MR imaging were included in this retrospective study. After performing three-dimensional T1-based brain segmentation with FreeSurfer software (Laboratory for Computational Neuroimaging), the mean Ktrans and vp from DCE MR imaging (derived using the Patlak model and extended Tofts and Kermode model) were analyzed in the bilateral cerebral/cerebellar cortex, bilateral cerebral/cerebellar white matter (WM), and brainstem. Ktrans values of the mTBI patients and controls were calculated using both models to identify the model that better reflected the increased permeability owing to mTBI (tendency toward higher Ktrans values in mTBI patients than in controls). The Mann-Whitney U test and Spearman rank correlation test were performed to compare the mean Ktrans and vp between the two groups and correlate Ktrans and vp with neuropsychological tests for mTBI patients. Results: Increased permeability owing to mTBI was observed in the Patlak model but not in the extended Tofts and Kermode model. In the Patlak model, the mean Ktrans in the bilateral cerebral cortex was significantly higher in mTBI patients than in controls (p = 0.042). The mean vp values in the bilateral cerebellar WM and brainstem were significantly lower in mTBI patients than in controls (p = 0.009 and p = 0.011, respectively). The mean Ktrans of the bilateral cerebral cortex was significantly higher in patients with atypical performance in the auditory continuous performance test (commission errors) than in average or good performers (p = 0.041). Conclusion: BBB disruption, as reflected by the increased Ktrans and decreased vp values from the Patlak model, was observed throughout the bilateral cerebral cortex, bilateral cerebellar WM, and brainstem in mTBI patients with PCS.

2020 Clinical Practice Guideline for Percutaneous Transthoracic Needle Biopsy of Pulmonary Lesions: A Consensus Statement and Recommendations of the Korean Society of Thoracic Radiology

  • Soon Ho Yoon;Sang Min Lee;Chul Hwan Park;Jong Hyuk Lee;Hyungjin Kim;Kum Ju Chae;Kwang Nam Jin;Kyung Hee Lee;Jung Im Kim;Jung Hee Hong;Eui Jin Hwang;Heekyung Kim;Young Joo Suh;Samina Park;Young Sik Park;Dong-Wan Kim;Miyoung Choi;Chang Min Park
    • Korean Journal of Radiology
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    • v.22 no.2
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    • pp.263-280
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    • 2021
  • Percutaneous transthoracic needle biopsy (PTNB) is one of the essential diagnostic procedures for pulmonary lesions. Its role is increasing in the era of CT screening for lung cancer and precision medicine. The Korean Society of Thoracic Radiology developed the first evidence-based clinical guideline for PTNB in Korea by adapting pre-existing guidelines. The guideline provides 39 recommendations for the following four main domains of 12 key questions: the indications for PTNB, pre-procedural evaluation, procedural technique of PTNB and its accuracy, and management of post-biopsy complications. We hope that these recommendations can improve the diagnostic accuracy and safety of PTNB in clinical practice and promote standardization of the procedure nationwide.