• Title/Summary/Keyword: Myasthenia gravis

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Guillain-barré Syndrome after Multiple Bee Stings (다발성 벌 자상에 의한 길랑 바레 증후군 1례)

  • Jin, Sang-Chan
    • Journal of The Korean Society of Clinical Toxicology
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    • v.16 no.1
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    • pp.57-59
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    • 2018
  • Severe systemic responses including neurologic complications such as myasthenia gravis, myeloradiculopathy, optic neuropathy, parkinsonism, stroke and Guillain-$barr{\acute{e}}$ syndrome can occur after bee stings. This case describes a 78-year-old female who presented with symptoms of acute progressive bilateral symmetrical weakness in both lower legs after multiple bee stings. Nerve conduction study findings were consistent with acute sensorimotor axonal neuropathy and recovered by treatment with intravenous immunoglobulin. This case highlights that bee stings can result in acute onset Guillain-$barr{\acute{e}}$ syndrome, although the pathophysiologies of bee venoms need to be investigated accurately.

Engineering Cell Therapies for Autoimmune Diseases: From Preclinical to Clinical Proof of Concept

  • Sangwook Oh;Aimee S. Payne
    • IMMUNE NETWORK
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    • v.22 no.5
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    • pp.37.1-37.16
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    • 2022
  • Autoimmune diseases are caused by a dysfunction of the acquired immune system. In a subset of autoimmune diseases, B cells escaping immune tolerance present autoantigen and produce cytokines and/or autoantibodies, resulting in systemic or organ-specific autoimmunity. Therefore, B cell depletion with monoclonal Abs targeting B cell lineage markers is standard care therapy for several B cell-mediated autoimmune disorders. In the last 5 years, genetically-engineered cellular immunotherapies targeting B cells have shown superior efficacy and long-term remission of B cell malignancies compared to historical clinical outcomes using B cell depletion with monoclonal Ab therapies. This has raised interest in understanding whether similar durable remission could be achieved with use of genetically-engineered cell therapies for autoimmunity. This review will focus on current human clinical trials using engineered cell therapies for B cell-associated autoimmune diseases.

The Significance of Acetylcholine Receptor Autoantibody Test (아세틸콜린 수용체 항체(Acetylcholine receptor autoantibody) 검사의 의의)

  • Yoo, Soh-Yeon;Lim, Soo-Yeon;Pack, Song-Ran;Seo, Mi-Hye;Moon, Hyung-Ho;You, Sun-Hee
    • The Korean Journal of Nuclear Medicine Technology
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    • v.15 no.1
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    • pp.113-116
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    • 2011
  • Purpose: Acetylcholine receptor antibodies cause acetylcholine receptor loss, which is responsible for failure of the neuromuscular junction in the acetylcholine receptor autoantibody. The disease characterized by muscle weakness and fatigue, myasthenia gravis(MG) occurs when the body inappropriately produces antibodies against acetylcholine receptors, and thus inhibits proper acetylcholine signal transmission. And this reason, the measurement of acetylcholine receptor antibodies can be of considerable value in disease diagnosis. Methods: From 2010. August to September, we tested orderd AchRAb 19 samples to get the results. 1. Pipette $5{\mu}{\ell}$ undiluted patient sera and kit control and add 125I AChR $50{\mu}{\ell}$ and incubate at R.T for 2 hours. 2. Pipette $50{\mu}{\ell}$ of anti-human IgG into each tube, and incubate at $2{\sim}8^{\circ}C$ for 2 hours. 3. Pipette $25{\mu}{\ell}$ precipitation enhancer into each tube and add 1mL washing solution into all tubes. 4. Centrifuge each tube for 20minutes at $2{\sim}8^{\circ}C$ at 1500g. 5. Aspirate or decant the supernatant. 6. Pipette 1 mL washing solution into all tubes and resuspend the pellet and repeat centrifugation. 7. Aspirate or decant the supernatant and count all tubes on a gamma counter. Results: Cut off value is 0.2 nmol/L and the results taken below 0.2 nmol/L are negative, the results above that identified as being positive values. We assayed the 19 patients samples and got 7 positive results. Of which, 6 patients were diagnosed as MG.(85.7%). Conclusions: Acetylcholine Receptor autoantibody test is intended for use by persons only for the quantitative determination of it in human serum. Even if measurement of the antibodies is not a routine test, it can be of considerable value in disease diagnosis.

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The result of Radiotherapy in Malignant Thymona (흉선암의 방사선치료 성적)

  • Ahn, Sung-Ja;Park, Charn-Il
    • Radiation Oncology Journal
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    • v.8 no.2
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    • pp.225-230
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    • 1990
  • Twenty one patients of malignant thymoma treated with curative aim at the Department of Therapeutic Radiology of Seoul National University Hospital from 1979 to 1987 were analysed retrospectively. The 3 year overall and relapse free survival rate was $80.5\%\;and\;78.6$, respectively. Myasthenia gravis (MG) was seen in $43.5\%$ at presentation and disappeared in $40\%$ (4/10) after radiotherapy with or without operation. The 3 year cumulative survival rate with and without MG was $90\;and\;78.8\%$, respectively. We could consider that MG was no longer adverse prognostic factor. The complete response rate after partial resection was $100\%$ (3/3), and that after biopsy was $20\%$(3/15). The overall local control rate including complete and partial response rate ($33\;vs\;56\%$)was$89\%$ and the 3 year actuarial survival rate by the response rate was $88.9\%\;and\;81.7\%$, respectively. There was no statistically significant survival difference between two groups. The crude rate of relapse at 3 years was $23.8\%$ (5/21), and $80\%$ (4/5) were locoregional failures. All failures were observed in biopsy only group, while no failure was observed in resected group. The major pattern of the treatment failure was the locoregional failure and the distant metastases was rarely observed.

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Surgical Treatment of Thymoma (흉선종의 외과적 치료)

  • Jang, Jae-Han;Kim, Min-Ho;Jo, Jung-Gu
    • Journal of Chest Surgery
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    • v.29 no.4
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    • pp.427-432
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    • 1996
  • Eighteen patients operated on for thymoma from 1985 to 1994 were evaluated, 3 with myasthenia gravis and 15 without. Masaoka's clinical staging revealed stage I disease in 5(27.8%), stage ll in 6 (33.3%), stage 111 in 6(33.3%), and stage IV in 1 (5.6%). Of the 18 patients, 10 had surgical resection as the only treatment and the remaining 8 (stage II : 1 patient, stage III : 6 patients, stage IV: 1 patient) had surgical resection with adjuvant preoperative or postoperative radiotherapy and/or chemotherapy There was no operative mortality. Currently, 15 patients are alive, 3 are dead. The mean follow up time for all patients was 3.4 years. Acturlal survival at 5 years was 82.2 $\pm$ 9% for all patients' 100% for those in stage I and II, and 62.5% for those in stage III. Pleural and mediastinal recurrence developed in one patient in stage II which was considered to have noninvasive disease on the operating field. Two patients underwent reoperation for recurrence of thymoma a intervals of 14 months and 52 months. Clinical stage and resectability had a significant prognostic value(p < 0.05). The presence of myasthenia gravis is no longer considered as an adverse factor in survival.

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Bilateral Vocal Cord Paralysis : A 10-year Review of 42 Patients (양측성대마비의 10년간 경험 분석)

  • 김광문;김세헌;최홍식;최흥식;조정일;이준협
    • Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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    • v.8 no.1
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    • pp.75-81
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    • 1997
  • The clinical investigation was done in the 42 patients with bilateral vocal cord palsy who visited the otorhinolaryngologic department of the Yongdong Severance Hospital during the 10-year period between August 1986 to August 1996. On the sex and age distribution, the ratio of male to female patients was 2.8 : 1 and the age was evenly distributed and average was 46 years old. Of their chief complaints, dyspnea was the most common symptom. Among the position of the paralyzed vocal cords, paramedian position was most common. The most common causes of the bilateral vocal cord palsy was idiopathic Other causes include iatrogenic, prolonged intubation, head & neck trauma, brain tumor, Myasthenia Gravis, and mediastinitis. Our treament results were as follows. Recovery rate of idiopathic bilateral vocal cord palsy was 77.7% and recovery period after bilateral vocal cord palsy was shortened remarkedly after use with steroid. We performed laser arytenoidectomy in patients with irreversible idiopathic vocal cord palsy, neural injury, and cricoarytenoid joint fixation. Decannualtion was possible to be carried out in 86% of the patients and none of complication except for 1 case of aspiration developed. Thus we concluded that it was meaningful surgical treatment of bilateral vocal cord palsy.

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A Case of Lymphocytic Interstitial Pneumonia (Lymphocγtic Interstitial Pneumonia 1예)

  • Suh, Yo-Ahn;Kim, Sang-It;Kim, Dae-Han;Kwak, Jin-Young;Lee, Jae-Cheol;Baek, Hee-Jong;Chung, Jin-Haeng
    • Tuberculosis and Respiratory Diseases
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    • v.51 no.4
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    • pp.390-394
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    • 2001
  • Lymphocytic interstitial pneumonia(LIP) is characterized by a massive infiltration of the interstitium of the lung by mature lymphocytes, plasma cells and reticuloendothelial cells. LIP may be associated with autoimmune diseases including Sj$\check{o}$grens syndrome, SLE, myasthenia gravis, pernicious anemia, autoimmune hemolytic anemia, and HIV or an EB virus infection. There is a possibility of LIP progressing to a pulmonary or systemic lymphoma. The therapeutic response to corticosteroids and/or immunosuppressive drugs varies. Here we report a case of LIP that was diagnosed by an open lung biopsy and clonality study. The patient was a 36 year-old man without autoimmune disease or HIV infection. He was admitted as a result of severe hypoxemia showing $PaO_2$ of 48.3mmHg. The patient was treated with corticosteroids after the diagnosis and had fully recovered without a sequalae or relapse.

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Differential Diagnosis between Neurogenic and Functional Dysphonia (신경성 발성장애와 기능성 발성장애의 감별 진단)

  • Kim, So Yean;Lee, Sang Hyuk
    • Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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    • v.28 no.2
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    • pp.71-78
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    • 2017
  • Voice disorder is classified into three categories, structural, neurogenic and functional dysphonia. Neurogenic dysphonia refers to a disruption in the nerves controlling the larynx. Common examples of this include complete or partial vocal cord paralysis, spasmodic dysphonia. Also it occurs as part of an underlying neurologic condition such as Parkinson's disease, myasthenia gravis, Lou Gehrig's disease or disorder of the central nervous system that causes involuntary movement of the vocal folds during voice production. Functional dysphonia is a voice disorder in the absence of structual or neurogenic laryngeal characteristics. A near consensus exist that Muscle tension dysphonia (MTD) is functional voice disorder wherein hyperfunctional laryngeal muscle activity whereas Spasmodic dysphonia (SD) is neurogenic, action-induced focal laryngeal dystonia including several subtype. Both Adductor type spasmodic dysphonia (AdSD) and MTD may be associated with excessive supraglottic contraction and compensation, resulting in a strained voice quality with spastic voice breaks. It makes these two disorders extremely difficult to differentiate based on clinical interpretation alone. Because treatment for AdSD and MTD are quite different, correct diagnosis is important. Clinician should be aware of the specific vocal characteristics of each disease to improve therapeutic outcome.

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Clinical evaluation of thymic tumors: a report of 10 cases (흉선에 발생한 종양 10례에 대한 임상적 고찰)

  • Gwak, Mun-Seop;Lee, Hong-Gyun
    • Journal of Chest Surgery
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    • v.16 no.4
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    • pp.571-583
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    • 1983
  • Thymic tumor is the most common neoplasm originating in the anterior mediastinum. Histologically, thymic tumors and tumorlike conditions have been classified into thymic cyst, thymolipoma, true thymic hyperplasia, thymoma and carcinoid tumor of the thymus gland. We have experienced several tumors and a few tumorlike conditions of the thymus gland in 10 patients: thymoma 6, thymic hyperplasia 2, carcinoid tumor 1 and thymic cyst 1. The age distributions were ranging from 30 to 60 years except for one 3 year old child [malignant thymoma, lymphocytic type], and male to female ratio was 1:1.5. In 6 cases, thymic lesions were removed through lateral thoracic incision [right 2, left 4] because the mediastinal masses were growing far into the pleural space, meanwhile, medially located lesions [4 cases] through median sternotomy incision. Among these 10 patients, 3 were malignant thymomas, in which 2 were died of recurrence after tumor resection. Myasthenia gravis was all noted in 3 females [thymoma 2, thymic follicular hyperplasia 1], in which only two revealed remission in symptoms following thymectomy. The authors would like to recommend early radical thymectomy through median sternotomy incision whenever patients demonstrate suspicious thymic tumor lesions on the chest roentgenogram or generalized myasthenic symptoms.

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Malignant Thymoma Diagnosed by Fine Needle Aspiration Cytology - A Case Report- (세침흡인 세포검사로 진단된 악성 흉선종 - 1예 보고 -)

  • Kang, Kyung-Ha;Sohn, Jin-Hee;Kim, Duck-Hwan;Pang, Sung-Suk;Yang, Seong-Eun;Suh, Jung-Il
    • The Korean Journal of Cytopathology
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    • v.5 no.2
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    • pp.189-193
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    • 1994
  • Malignant thymoma is usually manifested as a mediastinal mass. Occasiomally, these tumors may be associated with a variety of systemic syndromes, such as myasthenia gravis, pure red cell aplasia or hypogammaglobulinemia However, it is generally accepted that thymomas rarely metastasize and the frequency of extrathoracic metastasis has been reported as 1 to 15%. In general, nuclear changes of tumor cells such as enlarged size, irregular shape and altered textures are determining factors in the assessment of the grade of malignancy. We experienced a case of malignant thymoma of anterior mediastinum in a 35-year-old woman. After 3 years, she developed recurrent malignant thymoma in the right supraclavicular area, diagnosed by aspiration biopsy cytology.

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