• Title/Summary/Keyword: Rare disease

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Treatment Outcomes of Brain metastasis from Papillary Thyroid Cancer (갑상선 유두암 뇌전이의 치료 효과)

  • Bae, Hyeonwoo;Kim, Seok-Mo;Kim, Soo Young;Chang, Ho Jin;Kim, Bup-Woo;Lee, Yong Sang;Chang, Hang-Seok;Park, Cheong Soo
    • Korean Journal of Head & Neck Oncology
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    • v.34 no.1
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    • pp.9-13
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    • 2018
  • Background/Objectives: Brain metastasis (BM) is a rare form of distant metastasis with papillary thyroid cancer (PTC). Patients with BM of PTC carry a poor prognosis. The aim of this study was to contribute to the understanding of this disease by analyzing patients with BM of PTC. Materials & Methods: Between March 2003 and December 2013, the patient database was conducted to identify thyroid cancer patients treated. Among the 22,758 thyroid cancer patients, 14 (0.06 %) were identified to have metastasis to the brain during follow-up. The medical records of 14 patients with BM were retrospectively reviewed, focusing on the following: patient characteristics, synchronous or previous distant metastasis, treatments including whole brain radiotherapy (WBRT), stereotactic radiosurgery (SRS) and surgery, and characteristics on radiologic findings, time interval between first diagnosis of primary thyroid cancer and BM and survival after BM. Results: The mean age at initial diagnosis and BM were $50.9{\pm}15.8years$ and $61.3{\pm}12.7years$. The mean duration between initial diagnosis and BM was $10.4{\pm}7.9years$. Patients were treated with varied combinations of surgery, SRS and WBRT except 4 patients who had refused treatment. The median overall survival (OS) time after BM diagnosis was 10 months (range 1 - 19). Patients receiving treatment (WBRT and/or surgery, SRS) had a significant longer median OS of 16.5 months in comparison to 3.5 months for those treated without treatment. (p = 0.005) Conclusion: Patients who received aggressive treatment had a longer OS than those with only supportive care. Treatment such as surgery, SRS and WBRT should be considered in patients with BM.

Case report : The Bisphosphonate-associated Osteonecrosis of the Jaw(BONJ) (증례 보고: 악골에 발생한 비스포스포네이트 관련 골괴사증 (BONJ))

  • Kim, Kyun-Yo;Ko, Yu-Jung;Hur, Yun-Kyung;Choi, Jae-Kap
    • Journal of Oral Medicine and Pain
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    • v.34 no.3
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    • pp.295-301
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    • 2009
  • Recently, bisphosphonate-associated osteonecrosis of the jaw(BONJ) is added to the list of diseases of the oromaxillofacial region. BONJ is defined as exposed bone in the jaw that does not heal within 8 weeks after identification, in a patient who has been received to bisphosphonates and has not taken radiation therapy to the craniofacial region. Bisphosphonates binded to bone mineral are concentrated in highly active remodeling site, reside in the skeleton for a long time, and do a role as powerful inhibitors of bone resorption. As the patients receiving bisphosphonates therapy grow in number, the patients of BONJ would go on increasing in Korea. We would like to present two patients who were suspected to BONJ, describe the outline of BONJ, and mention importance of our understanding about BONJ. BONJ is rare disease, but once it develops, its prognosis is very poor. Our adequate understanding of BONJ is necessary to prevent it and cope with it properly.

THE LONG-TERM GROWTH OF HOMOGENEOUS EMBRYO TOOTH GERM TRANSPLANTED INTO THE MAXILLA OF A RAT (흰쥐의 악골에 동종 이식한 태아 치아싹의 장기간 발육)

  • Ko, Dong-Hyun;Chung, Han-Sung;Kim, Seong-Oh;Lee, Jae-Ho;Choi, Hyung-Jun;Choi, Byung-Jai
    • Journal of the korean academy of Pediatric Dentistry
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    • v.34 no.1
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    • pp.53-61
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    • 2007
  • In case of missing tooth caused by dental caries or periodontal disease, it can be restored by various methods, and there has been much interest in implant and tooth transplantation. The success of tooth transplantation is going to be attained through the knowledge of growth, development and calcification of tooth. Tooth transplantation has been experimented in vivo and in vitro. Many animals such as rats, mice, cats and dogs are used for tooth transplantation experiment in vivo. In most experiments, tooth was transplanted into the extraoral site, but rare into the intraoral site In this study, to observe the capacity of formation and mineralization of tooth germ, first molar of a matured white rat was extracted and the cap stage tooth germ of a 13.5 Embryonic day embryo rat was transplanted into the extracted socket. The rats were killed 6 months later and the radiographical and histological results are as followings. 1. Tooth germ transplanted for 2 and 6 months are developing calcified tooth material such as dentin, cementum, pulp tissue, and epithelium around enamel space in the maxilla was seen. 2. The epithelium around enamel space was located beneath the oral epithelium and contained connective tissue and periodontal ligament. 3. Tooth formation was progressed as transplantation period but the size of newly formed tooth was small and the shape of tooth was incomplete.

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A Case of Pulmonary Gangrene Associated with Obstructive Pneumonia Due to Non-small Cell Lung Carcinoma (위치에 따라 이동하는 종괴를 포함한 공동으로 진행된 비소세포폐암에 동반된 폐렴)

  • Kim, Sung-Jun;Um, Tae-Chan;Moon, Kwie-Ae;Kim, Phil-Ho;Kim, Sang-Hyun;Jeoung, Byung-Oh;Lee, Hyuk-Pyo;Kim, Joo-In;Yum, Ho-Kee;Choi, Soo-Jeon
    • Tuberculosis and Respiratory Diseases
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    • v.46 no.4
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    • pp.591-595
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    • 1999
  • Pulmonary gangrene is a rare complication of severe pulmonary infection in which a pulmonary segment or lobe is sloughed. It is a part of a spectrum of disease in which lung tissue is devitalized(such as necrotizing pneumonia, pulmonary abscess), but apart from them, pulmonary gangrene has more extensive area of necrosis and thrombosis of large vessels plays a prominent role in the pathogenesis. We experienced a case of pulmonary gangrene in 71 year old female obstructive pneumonia patient with non-small cell lung carcinoma. She complained high fever, chill and despite treatment with antibiotics, pneumonia progressed to empyema. At that time chest radiograph showed a large cavity including sloughed lung tissue, freely moving to dependent position at both lateral decubitus view. RML and RLL were resected and compression of pulmonary vessels by enlarged lymph nodes was observed. Defervescence was obtained immediate postoperative period and the patient was discharged after infection control with antibiotics, chest tube drainage. The perivascular lymph nodes dissected during lobectomy were proved to be reactive hyperplasias. We speculated that the carcinoma caused obstructive pneumonia, in turn, resulted in reactive hyperplasia of the draining lymph nodes surrounding the large vessels and finally the lung tissues supplied by them necrotized and sloughed.

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A Case of Interstitial Pneumonitis Caused by Leflunomide (Leflunomide에 의한 약물유발 간질성 폐렴 1예)

  • Lee, Jung Hwa;Cheon, Won Seok;Seo, Young Il;Eom, Kwang-Seok;Jang, Seung Hun;Bahn, Joon-Woo;Kim, Dong-Gyu;Jung, Ki-Suck
    • Tuberculosis and Respiratory Diseases
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    • v.58 no.1
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    • pp.83-88
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    • 2005
  • Leflunomide is a new disease modifying anti rheumatic drug (DMARD) for the treatment of active rheumatoid arthritis. Its mechanism of action differs from other DMARDs in that it inhibits the de novo pyrimidine synthesis by inhibiting dihydroorotate dehydrogenase and therefore prevents the proliferation of activated lymphocytes. As it has been prescribed worldwide, there is a great deal of much concerns regarding its potential adverse effects. Because leflunomide has an active metabolite with a long elimination half-life of approximately 2 weeks, serious adverse reactions may occur even after the leflunomide treatment has been stopped. The profile of serious reactions includes liver dysfunction, hematological disorders, severe skin reactions and respiratory dysfunction. Respiratory dysfunctions with leflunomide therapy are very rare and its incidence is lower than that of methotrexate therapy. However, there are reports in Japan showing that 5 patients died of interstitial pneumonitis and another 11 patients developed serious lung complications associated with leflunomide. This suggests the possibility of fatal respiratory toxicity of leflunomide. There are no reports of interstitial pneumonitis associated with leflunomide in Korea. We report a case of a 62-year old woman who developed interstitial pneumonitis, which might have been induced by leflunomide during the treatment of rheumatoid arthritis.

Two Cases of Diagnosis and Removal of Endobronchial Hamartoma by Cryotherapy via Flexible Bronchoscopy

  • Sim, Jae Kyeom;Choi, Jong Hyun;Oh, Jee Youn;Cho, Jae Young;Moon, Eul Sun;Min, Hye Sook;Lee, Byung Hyun;Park, Min Seon;Hur, Gyu Young;Lee, Sung Yong;Shim, Jae Jeong;Kang, Kyung Ho;Min, Kyung Hoon
    • Tuberculosis and Respiratory Diseases
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    • v.76 no.3
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    • pp.141-145
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    • 2014
  • Although endobronchial hamartoma is a rare benign tumor, most patients with endobronchial hamartoma have respiratory symptoms such as obstructive pneumonia, hemoptysis, cough, or dyspnea due to bronchial obstruction. It can cause irreversible post-obstructive pulmonary destruction, thus early diagnosis and treatment is very important. Recently, there have been cases of neodymium-doped yttrium aluminum garnet (Nd:YAG) laser and electrocautery procedures for bronchoscopic treatment of malignant or benign central airway obstruction with comparable therapeutic efficacy and few complications. Bronchoscopic cryotherapy is a newly developed technique for management of central airway obstruction. Moreover, it provides diagnostic methods with improving diagnostic yield and safety. We report two cases of endobronchial hamartoma, each diagnosed and definitively treated with bronchoscopic techniques. Endobronchial biopsy and removal was successfully performed by cryotherapy via flexible bronchoscopy without notable complications. Follow-up bronchoscopic examinations excluded residual or recurrent disease.

A Case of Endobronchial Lipoma Causing Right Middle and Lower Lobes Collapse and Bronchiolitis Obliterans-organizing Pneumonia (우중하엽 폐허탈 및 폐쇄세기관지기질화 폐렴을 유발한 기관지 지방종 1예)

  • Son, Ji Young;Jung, Ji Ye;Ha, You Jung;Hong, Soo Jung;Jung, Min Kyu;Chung, Moon Jae;Seo, Yong Sung;Moon, Ji Ae;Byun, Min Kwang;Park, Byung Hoon;Moon, Jin Wook;Park, Moo Suk;Kim, Young Sam;Chang, Joon;Kim, Sang Kyum;Chung, Kyung Young;Kim, Se Kyu
    • Tuberculosis and Respiratory Diseases
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    • v.65 no.4
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    • pp.313-317
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    • 2008
  • Lipoma is a common neoplasm in soft tissues. However, an intrapulmonary lipoma is a rare benign tumor. Patients with a bronchial lipoma might have a malignant potential related to their smoking history due to the case reports of lung cancer accompanied with lipoma. Endobronchial lipoma can cause irreversible parenchymal lung damage if not diagnosed and treated early. Therefore, it should initially be treated by fiberoptic bronchoscopy or surgery depending on the status of distal parenchymal lung damage. Bronchiolitis obliterans with organizing pneumonia (BOOP) is a pathological syndrome that is defined by the presence of buds of granulation tissue consisting of fibroblasts and collagen within the lumen of the distal air spaces. BOOP is caused by drug intoxication, connective tissue disease, infection, obstructive pneumonia, tumors, or an unknown etiology. We encountered a 58 year-old male patient with endobronchial lipoma, causing the collapse of the right middle and lower lobes, and BOOP due to obstructive pneumonia.

Systemic Lupus Erythematosus Associated with Interstitial Pneumonia and Achalasia (식도 이완 불능증과 간질성 폐렴을 동반한 전신성 홍반성 낭창)

  • Kwon, Hye Lee;Hong, Kyung Wook;Lim, Seung Jin;Park, So Young;Bae, Young Deok;Kim, Kyung Ho;Choi, Jeong Hee;Mo, Eun Kyung;Park, Yong Bum
    • Tuberculosis and Respiratory Diseases
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    • v.65 no.4
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    • pp.323-327
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    • 2008
  • Systemic lupus erythematosus (SLE) is a multisystem disorder where the etiology is not clearly known. Symptomatic chronic interstitial pneumonitis is an uncommon manifestation, with a reported prevalence of 3~13%. Achalasia is rare disease that presents with failure in the relaxation of the esophagus sphincter. A 22-year-old woman was admitted to our hospital because of fever, cough and dyspnea. The patient had a history of pericardial effusion and Raynaud's phenomenon. The results of laboratory tests indicated the presence of lymphopenia and included positive antibody tests for antinuclear antibody and anti Sm antibody. A chest X-ray demonstrated the presence of peribronchial infiltration on both lung fields. A Chest CT image showed interlobar septal thickening, ground-glass opacity and a honeycomb appearance in both lung fields and esophageal dilatation with air fluid level. An esophagogram showed the presence of dilated esophagus ends that represented the non-relaxed lower esophageal sphincter. Manometry demonstrated incomplete sphincter relaxation. The case was diagnosed as systemic lupus erythematosus associated with interstitial pneumonia and achalasia.

Combined Arthroscopic Gross Total Synovectomy and Radiotherapy for Diffuse Pigmented Villonodular Synovitis of the Knee: A Report of 2 Cases (무릎관절의 미만형 색소 융모 결절성 활액막염에서 관절경을 이용한 육안적 활액막 전 절제술 후 방사선치료: 증례보고)

  • Chang, Sei-Kyung;Kim, Jae-Hwa;Ha, Doo-Hoe;Kang, Hae-Youn;Chong, So-Young;Shin, Hyun-Soo
    • Radiation Oncology Journal
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    • v.24 no.3
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    • pp.201-206
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    • 2006
  • Pigmented villonodular synovitis (PVNS) is a rare proliferative disease involving synovial membranes. Natural history and etiology of PVNS are not well known. PVNS presents as localized or diffuse tumor like nodular lesion of the synovial lining of the joint and the synovial spaces adjacent to the joints. Though histologically benign, it is a very aggressive lesion, capable of bone destruction and widespread infiltration of surrounding tissues. Standard therapy is surgical resection, but due to the infiltrative growth, the recurrence rate is significantly high. After several relapses surgical treatment of diffuse PVNS becomes difficult and may require amputation of the involved limb. Radiotherapy can provide an effective treatment option for patients with large lesions or lesions which are not suitable for surgery, after incomplete resection to prevent relapses or to avoid amputation. We report 2 cases of diffuse PVNS in the knee joint treated with arthroscopic gross total synovectomy and radiotherapy.

Research on Ginseng Production During the Past 20 years (인삼재배 분야의 과거 20년 연구)

  • Park, Hoon
    • Journal of Ginseng Research
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    • v.20 no.4
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    • pp.472-500
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    • 1996
  • Researches on mineral nutrition, physiology and phyrsiological diseases, . cultivaction methods. brceding. pest control quality management and extension during 1976-1995 in Korea were reviewed Review in brceding and pest control was restricted to the researches directely related to cultivaction. Mineral nulrient up take. partion and varicos factors such as top dreasing. Light intersity etc. and interrelationship between minerals were investigated. Top dressing was not effective due to low minera1 requorement Physiological characteristics on tempelature light and water were well elucidated and applied to assess traditional cultivation method and its inovation. Photosyrnthetic pigments. light harvest proteins and activity of related enzymes were studied. In nitrogen metabolism arginine, praline, ammonium, threonine appeared to have important role in re growth of shoot Saponin metabolism was studied in relation to growth and new ginsenosides were found but physiological role of saponin was not clearly elucidated yet Endogenous growth regulators were reported and various erogenous growth regulators were studied for growth stimulation. short stem and seed pruning etc. Various physiological diseases were investigated for cause and control measures were established. Water culture was little studied Forest culture was studied but not retched the recommendable stage Drip irrigation straw mulching. seasonal shading and soil preparation method including soil fertility adjustment were established for practical application. Shading materials completely changed to polyethylene net and materials of polymers The research on ginseng cultivation in paddy field opened the way to establish the permanent ginseng cultivation plantation Ginseng harvester and seeder were developed in the late 1950s. Transplanted and many other machines were developed in the early 1990s. In ginseng breeding only pure line selection was of practical significance several verities were at the stage of seed propagation at ginseng plantations. Mutation breeding (${\gamma}$-ray. X-ray chemicals) was not successful. The research on plantlet formation through tissue culture was a little progressed but still far behind to vegetative propagation. Disease control research was concentrated in the isolation and identification of pathogans. their ecological charactelistics and biological control and soil humigation. Potato root rot nematodes was found and control method was established. Insect and small animal control research was greatly progresses in identification, ecological investigation, and ecological and physical control. Weed control was less important due to the development of mulching method of ridge and ditch. Quality factors of raw ginseng in relation to red ginseng process were extensively studied. Traditional quality measures were elucidated in accordance with modern analytical chemistry resulting in the importance of peptides in the centrat part rather than ginsenosides For large root production growth promoting rootzone micrcorganisms (PGPRM) were isolated and active compounds were identified. Field test on PGPRM was on going. Varictus methods formality improvement through cultivation were developed. Management research of ginseng production was rare Extension was active throuch official and private organizations and through workshop for the extension specialists, and direct lectures to grower's. Extension services made the researcher to understand the existing problems at grower's fields. Research environment for ginseng production was in prime time only for three years when Korea Ginseng Research Institute was established then gradually aggravated.

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