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The Complex Surgical Management of the First Case of Severe Combined Immunodeficiency and Multiple Intestinal Atresias Surviving after the Fourth Year of Life

  • Guana, Riccardo;Garofano, Salvatore;Teruzzi, Elisabetta;Vinardi, Simona;Carbonaro, Giulia;Cerrina, Alessia;Morra, Isabella;Montin, Davide;Mussa, Alessandro;Schleef, Jurgen
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.17 no.4
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    • pp.257-262
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    • 2014
  • Severe combined immunodeficiency (SCID) is a life-threatening syndrome of recurrent infections and gastro-intestinal alterations due to severe compromise of T cells and B cells. Clinically, most patients present symptoms before the age of 3 months and without intervention SCID usually results in severe infections and death by the age of 2 years. Its association with intestinal anomalies as multiple intestinal atresias (MIA) is rare and worsens the prognosis, resulting lethal. We describe the case of a four year-old boy with SCID-MIA. He presented at birth with meconium peritonitis, multiple ileal atresias and underwent several intestinal resections. A targeted Sanger sequencing revealed a homozygous 4-bp deletion ($c.313{\Delta}TATC$; p.Y105fs) in tetratricopeptide repeat domain 7A (TTC7A). He experienced surgical procedures including resection and stricturoplasty. Despite parenteral nutrition-associated liver disease, the patient is surviving at the time of writing the report. Precocious immune system assessment, scrutiny of TTC7A mutations and prompt surgical procedures are crucial in the management.

The Clinical Effect of Drinking Habit in Acute Stroke Patients (급성기 중풍환자에서 음주습관이 중풍의 발생양상에 미치는 임상적 영향)

  • Choi, Dong-Jun;Hyun, Jin-Ho;Shin, Won-Yong;Kim, Yong-Hyung;Kang, A-My;Lee, Won-Chul;Jun, Chan-Yong;Jo, Ki-Ho;Han, Chang-Ho
    • The Journal of Internal Korean Medicine
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    • v.28 no.1
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    • pp.92-96
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    • 2007
  • Objectives : This study investigated the clinical effect of a drinking habit in acute stroke patients. Methods : 409 acute stroke patients were included from October 2005 to October 2006. Patients were hospitalized within 14 days after the onset of stroke at DongGuk University International Hospital, Kyungwon University In-cheon Oriental Medical Hospital, or Department of Cardiovascular and Neurologic Diseases (Stroke Center), Kyung Hee University Oriental Hospital. We investigated general characteristics, drinking habit, and stroke subtype by TOAST classification. Results : Among drinking subjects, hemorrhagic stroke was more frequent than ischemic stroke (odds ratio 3.04), and less in small vessel occlusion than others (odds ratio 1.84). Ischemic stroke was associated with a longer (30 yrs) drinking habit than hemorrhagic stroke. Conclusions : To acquire more concrete conclusions on this theme, we need further and larger scale research.

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Endovascular treatment of Takayasu arteritis in a middle-aged woman with syncope and limb claudication: a case report

  • Ha-Young Choi;Sunggun Lee;Jino Park;Yeo-Jeong Song;Dong-Kie Kim;Ki-Hun Kim;Sang-Hoon Seol;Doo-Il Kim;Seunghwan Kim
    • Journal of Yeungnam Medical Science
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    • v.40 no.4
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    • pp.448-453
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    • 2023
  • Takayasu arteritis (TA) is a disease that causes inflammation and stenosis of medium to large blood vessels. We report a case of a 50-year-old female patient with newly developed hypertension, syncope, and claudication of the extremities. Total occlusion of the left subclavian artery at the origin was found and significant stenosis of the right common iliac artery was revealed by hemodynamic analysis. She was successfully treated with percutaneous angioplasty for multiple peripheral arterial diseases and was finally diagnosed with TA. In consultation with a rheumatologist, medical treatment for TA was initiated, the patient's hypertension disappeared, and her claudication symptoms improved.

A Study on the Cognizance of Hospital and oriental Hospital Workers for Medical Device Safety Information Monitoring (의료기기 안전성 정보 모니터링에 대한 한병병원 및 병원 종사자의 인지도 조사연구)

  • Kim, Ji Hyun;Nam, Ki Chang;Kim, Hojun;Nam, Yeon Gyo;Kim, You Jin;jo, Eun hye;Kwon, Bum Sun
    • Journal of Biomedical Engineering Research
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    • v.42 no.3
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    • pp.86-93
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    • 2021
  • Purpose: The purpose of this study is to find out the cognizance of medical device safety information (MDSI) monitoring in the hospital and oriental hospital workers, and the different aspect of MDSI between oriental medical devices and medical devices. Methods: The survey was performed both in the oriental medicine hospital and general hospital. The survey had 16 items; 2 items basic questions, 5 items in the awareness of MDSI, 5 items in the education of MDSI, 4 items in the necessity of defining oriental medical devices and differences between general and oriental medical devices. A total of 120 hospital worker were participated; 60 oriental medicine hospital workers and 60 general hospital workers. They had worked in the oriental medicine or general hospital associated with 'Medical Device Safety Monitoring Center, Dongguk University Ilsan Hospital' in 2019 and 2020. Results: The cognizance of MDSI was high both in oriental medicine hospital and general hospital workers and there were no significant differences between oriental medicine and general hospital workers. When we divided the hospital workers into the senior workers who had worked for over 3 years and junior workers for less than 3 years, the senior workers had higher awareness of MDSI than junior workers. However, the cognizance of education of MDSI was high which was not different between senior and junior workers. Both hospital workers thought that it was necessary to define oriental medicine device legally and the oriental medical device might have low risk and less side effect than medical device. Conclusion: The cognizance of MDSI was high and there was no significant differences between oriental medicine and general hospital workers. Because the senior hospital workers had higher recognition of MDSI, we need to provide the continuous education program for junior hospital workers. Although oriental medical device are thought to be safer than medical device, we need to have a legal definition.

Introduction of brain computer interface to neurologists

  • Kim, Do-Hyung;Yeom, Hong Gi;Kim, Minjung;Kim, Seung Hwan;Yang, Tae-Won;Kwon, Oh-Young;Kim, Young-Soo
    • Annals of Clinical Neurophysiology
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    • v.23 no.2
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    • pp.92-98
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    • 2021
  • A brain-computer interface (BCI) is a technology that acquires and analyzes electrical signals from the brain to control external devices. BCI technologies can generally be used to control a computer cursor, limb orthosis, or word processing. This technology can also be used as a neurological rehabilitation tool for people with poor motor control. We reviewed historical attempts and methods toward predicting arm movements using brain waves. In addition, representative studies of minimally invasive and noninvasive BCI were summarized.

Voice Change Due to Paratracheal Air Cysts

  • Rhee, Youn Ju;Han, Sung Joon;Chong, Yoo Young;Cho, Hyun Jin;Kang, Shin Kwang;Lee, Choong-Sik;Kang, Min-Woong
    • Journal of Chest Surgery
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    • v.49 no.4
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    • pp.313-316
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    • 2016
  • Paratracheal air cysts are a rare entity in which cystic formation occurs adjacent to the trachea. Most patients with paratracheal air cysts are asymptomatic, and the cysts are detected incidentally on chest radiograph or computed tomography (CT) scan. Most symptomatic patients complain of pulmonary symptoms or repeated respiratory infection. Rarely, the air cysts can lead to paralysis of the recurrent laryngeal nerve as a result of direct compression. We report a case of a 59-year-old male patient who presented with voice change, and the cause was identified as paratracheal air cysts on a chest CT scan. Surgical resection via video-assisted mediastinoscopy was performed, and the voice recovered immediately after the operation.

A Pulmonary Sarcoidosis Manifesting as a Rare Atypical Pattern and Distribution (드문 비전형적인 형태와 분포를 보인 폐 유육종증 1예)

  • Hong, Sun-Hwa;Kang, Eun-Young;Woo, Ok Hee;Yong, Hwan Seok;Oh, Yu-Whan;Shin, Bong Kyung;Shim, Jae Jeong
    • Tuberculosis and Respiratory Diseases
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    • v.64 no.3
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    • pp.236-239
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    • 2008
  • A unique case of atypical pulmonary sarcoidosis in a 62-year-old man complaining of dyspnea is presented. Chest CT scan showed an unusual pattern and distribution of pulmonary sarcoidosis manifesting mainly as reticular densities, interlobular septal thickening, and ground-glass opacities, in the subpleural and lower lung predominancy. However, a surgical lung biopsy revealed classical findings of sarcoidosis. Knowledge of this atypical pulmonary involvement may improve understanding sarcoidosis as the great masquerader.

A Case of Recurrent Pneumonitis Caused by Bojungikgitang (Bu-Zhong-Yi-Qi-Tang) (보중익기탕(補中益氣湯) 복용 후 재발생한 미만성 간질성 폐렴 1예)

  • Kim, Seung Gu;Kang, Gun Hi;Kim, Jun Jae;Park, Hyun;Baek, Na Na;Choi, Sang Bong;Shin, Eun Ah;Kim, Joung Sook;Park, I Nae;Jeung, Hoon;Hur, Jin Won;Lee, Sung Soon;Lee, Hyun Kyung;Kim, Joo In;Lee, Young Min;Lee, Hyuk Pyo;Yum, Ho Kee;Choi, Soo Jeon
    • Tuberculosis and Respiratory Diseases
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    • v.65 no.5
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    • pp.416-420
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    • 2008
  • Many classes of drug, such as antineoplastic drugs and antiarrhythmic drugs, have potential to induce interstitial lung disease. Herbal medicines are also believed to have the potential to induce pneumonitis. However, to our knowledge, there are no reports of pneumonitis caused by herbal medications in the Korean medical database. We report a case of recurrent pneumonitis caused by a self rechallenge of the Herbal medicine Bojungikgitang (Bu-Zhong-Yi-Qi-Tang : Hochu-ekki-to).

Involvement of Cdc25c in Cell Cycle Alteration of a Radioresistant Lung Cancer Cell Line Established with Fractionated Ionizing Radiation

  • Li, Jie;Yang, Chun-Xu;Mei, Zi-Jie;Chen, Jing;Zhang, Shi-Min;Sun, Shao-Xing;Zhou, Fu-Xiang;Zhou, Yun-Feng;Xie, Cong-Hua
    • Asian Pacific Journal of Cancer Prevention
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    • v.14 no.10
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    • pp.5725-5730
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    • 2013
  • Cancer patients often suffer from local tumor recurrence after radiation therapy. Cell cycling, an intricate sequence of events which guarantees high genomic fidelity, has been suggested to affect DNA damage responses and eventual radioresistant characteristics of cancer cells. Here, we established a radioresistant lung cancer cell line, A549R, by exposing the parental A549 cells to repeated ${\gamma}$-ray irradiation with a total dose of 60 Gy. The radiosensitivity of A549 and A549R was confirmed using colony formation assays. We then focused on examination of the cell cycle distribution between A549 and A549R and found that the proportion of cells in the radioresistant S phase increased, whereas that in the radiosensitive G1 phase decreased. When A549 and A549R cells were exposed to 4 Gy irradiation the total differences in cell cycle redistribution suggested that G2-M cell cycle arrest plays a predominant role in mediating radioresistance. In order to further explore the possible mechanisms behind the cell cycle related radioresistance, we examined the expression of Cdc25 proteins which orchestrate cell cycle transitions. The results showed that expression of Cdc25c increased accompanied by the decrease of Cdc25a and we proposed that the quantity of Cdc25c, rather than activated Cdc25c or Cdc25a, determines the radioresistance of cells.