• Title/Summary/Keyword: Underlying Disease

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A Case of Pachydermoperiostosis (경피골막증: 증례보고)

  • Nam, Young O;Ko, Sung Hoon;Oh, Suk Joon
    • Archives of Plastic Surgery
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    • v.35 no.3
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    • pp.316-320
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    • 2008
  • Purpose: Pachydermoperiostosis is a rare hereditary disease characterized by finger clubbing, periosteal reaction, and pachydermia. The underlying pathogenic mechanism of this disease remains unclear. This disease is known to be associated with a variety of diseases such as cranial suture defect, bone marrow failure, hypertrophic gastropathy, Crohn's disease, and female escuchen. Methods: A 50-year-old male had digital clubbing of both hands, coarse hypertrophic skin changes of face, progressive thickening and furrowing on the scalp(cutis verticis gyrata), persistent pain in the limbs and joints. Other cutaneous features include moderate blepharoptosis, pole-like lower legs and feet. Results: We performed surgical excision for hypertrophic skin change of scalp because of frequent eczematous skin change, severe itching sensation and cosmetic problem. Diagnosis is confirmed by bony proliferative periosteal reaction, pathologic findings, and characteristic clinical findings. Conclusion: Pachydermoperiostosis is manifested by finger clubbing, and hypertrophic skin changes causing coarse facial features with thickening and periosteal bone formation. We experienced a case of pachydermoperiostosis. Brief review of related literature is given.

2 Cases Report of Bullous Emphysema in Infancy. Treated by Surgery (영아에 발생(發生)한 거대(巨大) 기낭성폐기종(氣囊性肺氣腫) 2례(例) 보고(報告))

  • Kim, J.E.;Choi, S.S.;Lee, J.H.;Yoo, Y.S.;Yu, H.S.;Park, M.H.;Park, H.S.
    • Journal of Chest Surgery
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    • v.9 no.1
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    • pp.78-82
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    • 1976
  • Bullous emphysema is usually associated with extensive chronic obliterative pulmonary disease. It is the disease of old age but rare in children or infancy. The bulla in this disease is acquired one. In general the symptoms are due to not the mere presence of the bulla but the extent of underlying lung pathology as emphysema or bronchitis. Occassionally giant bulla of great size may cause symptoms and in this occassion it should be differentiated from other diseases. Especially in children or infancy pneumothorax, congenital pulmonary cyst of lobar obstructive-emphysema should be excluded. Recently we experienced 2 cases of bullous emphysema in infancy with severe respiratory symptoms because of bullae of great size. We felt difficulties in differentiating with other conditions. The purpose of this report is to review our cases thoroughly and enhance considerations of this disease.

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Acute myocardial infarction with a giant left main aneurysm in atypical Kawasaki disease (비전형적인 가와사키 병 환자에서 발생한 좌주간지 거대 동맥류를 동반한 급성심근경색)

  • Kim, Min Wook;Kim, Hyun Soo;Lee, Myung Dong;Jung, Hyun Sook;Yoon, Seong-Bo;Kim, Young Woo
    • Journal of Yeungnam Medical Science
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    • v.34 no.1
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    • pp.106-110
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    • 2017
  • Kawasaki disease (KD) is an acute vasculitis of small and medium sized arteries. Even many years after onset, aneurysms and stenosis in coronary arteries may lead to an acute myocardial infarction, which is described as atypical or missed KD in childhood. KD is an underlying disease of young adults with acute myocardial infarction. We report on a rare case involving a total occlusion in the proximal left anterior descending coronary artery combined with a giant left main aneurysm in a young adult patient with acute myocardial infarction ascribed to antecedent KD that is undefined but almost certain.

Branched N-glycans and their implications for cell adhesion, signaling and clinical applications for cancer biomarkers and in therapeutics

  • Taniguchi, Naoyuki;Korekane, Hiroaki
    • BMB Reports
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    • v.44 no.12
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    • pp.772-781
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    • 2011
  • Branched N-glycans are produced by a series of glycosyltransferases including N-acetylglucosaminyltransferases and fucosyltransferases and their corresponding genes. Glycans on specific glycoproteins, which are attached via the action of glycosyltransferases, play key roles in cell adhesion and signaling. Examples of this are adhesion molecules or signaling molecules such as integrin and E-cadherin, as well as membrane receptors such as the EGF and TGF-${\beta}$ receptors. These molecules also play pivotal roles in the underlying mechanism of a variety of disease such as cancer metastasis, diabetes, and chronic obstructive pulmonary disease (COPD). Alterations in the structures of branched N-glycans are also hall marks and are useful for cancer biomarkers and therapeutics against cancer. This mini-review describes some of our recent studies on a functional glycomics approach to the study of branched N-glycans produced by N-acetylglucosaminyltransferases III, IV, V and IX (Vb) (GnT-III, GnT-IV, V and IX (Vb)) and fucosyltransferase 8 (Fut8) and their pathophysiological significance, with emphasis on the importance of a systems glycobiology approach as a future perspective for glycobiology.

Clinical Analysis of Cervical Necrotizing Fasciitis (경부 괴사성 근막염의 임상적 고찰)

  • Park, Byung Kuhn;Lee, Do-Joon;Lee, Sang Joon
    • Korean Journal of Bronchoesophagology
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    • v.17 no.1
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    • pp.46-49
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    • 2011
  • Background and Objectives Cervical necrotizing fasciitis is a fulminant disease associated with necrosis of connective tissue, spread along the fascial plane, and high mortality. We analyzed the clinical characteristics and treatment outcome of this rare fatal disease. Materials and Methods We retrospectively reviewed the medical records of 19 patients treated for cervical necrotizing fasciitis from January 1999 to January 2009. Mean age was 53.7 years. Results The most common predisposing illness was tonsillitis (36.8%), followed by odontogenic infection (15.7%). Diabetes mellitus was most common underlying disease. Liver cirrhosis and chronic renal failure were found in 2 patients each. All patients were treated with combination of parenteral antibiotics and wide surgical debridement by transcervical and/or thoracotomy approach. Multiple surgical debridements were performed in 7 patients. Tracheotomy was performed in most of the patients (88.8%). Period of total hospitalization and Intensive care unit was 23 days and 10.1 days. Two patients died of disease and overall survival rate was 89.4%. Conclusion Early surgical management and care in intensive care unit are essential for cervical necrotizing fasciitis. Possible complications such as respiratory failure, mediastinitis or sepsis should be carefully evaluated.

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Co-occurrence of apocrine adenocarcinoma and invasive mammary-type ductal carcinoma in extramammary Paget disease of the axilla

  • Jang, Seung Bin;Kim, Sung-Eun;Kim, Young Ah;Choi, Hye Ryeon
    • Archives of Plastic Surgery
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    • v.47 no.1
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    • pp.83-87
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    • 2020
  • Extramammary Paget disease (EMPD) is an uncommon malignancy that occurs in apocrine gland-rich areas of the body. EMPD of the axilla is rare, but a few cases have been reported. Some cases of EMPD have been reported with underlying apocrine adenocarcinoma; rarely, mammary-type ductal carcinoma can accompany EMPD. Here, we report a very rare case of EMPD with apocrine adenocarcinoma and invasive mammary-type ductal carcinoma. A 55-year-old woman was referred with a brownish pigmented plaque accompanying an area of ulceration in the left axilla. A preoperative biopsy indicated Paget disease, and an additional evaluation was performed to determine whether it was of primary or secondary origin. A wide excision was made, and the axilla was reconstructed using a thoracodorsal artery perforator flap. The biopsy showed apocrine adenocarcinoma and invasive mammary-type ductal carcinoma with pagetoid spreading. The patient had no evidence of recurrence or other postoperative complications.

Hemorrhagic Moyamoya Disease : A Recent Update

  • Fujimura, Miki;Tominaga, Teiji
    • Journal of Korean Neurosurgical Society
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    • v.62 no.2
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    • pp.136-143
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    • 2019
  • Moyamoya disease (MMD) is a progressive cerebrovascular disease with unknown etiology, characterized by bilateral steno-occlusive changes at the terminal portion of the internal carotid artery and an abnormal vascular network formation at the base of the brain. MMD has an intrinsic nature to convert the vascular supply for the brain from internal carotid (IC) system to the external carotid (EC) system, as indicated by Suzuki's angiographic staging. Insufficiency of this 'IC-EC conversion system' could result not only in cerebral ischemia, but also in intracranial hemorrhage from inadequate collateral anastomosis, both of which represent the clinical manifestation of MMD. Surgical revascularization prevents cerebral ischemic attack by improving cerebral blood flow, and recent evidence further suggests that extracranial-intracranial bypass could powerfully reduce the risk of re-bleeding in MMD patients with posterior hemorrhage, who were known to have extremely high re-bleeding risk. Although the exact mechanism underlying the hemorrhagic presentation in MMD is undetermined, most recent angiographic analysis revealed the characteristic angio-architecture related to high re-bleeding risk, such as the extension and dilatation of choroidal collaterals and posterior cerebral artery involvement. We sought to update the current management strategy for hemorrhagic MMD, including the outcome of surgical revascularization for hemorrhagic MMD in our institute. Further investigations will clarify the optimal surgical strategy to prevent hemorrhagic manifestation in patients with MMD.

Phytobiome as a Potential Factor in Nitrogen-Induced Susceptibility to the Rice Blast Disease

  • Jeon, Junhyun
    • Research in Plant Disease
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    • v.25 no.3
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    • pp.103-107
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    • 2019
  • Roles of nutrients in controlling plant diseases have been documented for a long time. Among the nutrients having impact on susceptibility/resistance to crop diseases, nitrogen is one of the most important nutrients for plant growth and development. In rice plants, excess nitrogen via fertilization in agricultural systems is known to increase susceptibility to the rice blast disease. Mechanisms underlying such phenomenon, despite its implication in yield and sustainable agriculture, have not been fully elucidated yet. A few research efforts attempted to link nitrogen-induced susceptibility to concomitant changes in rice plant and rice blast fungus in response to excess nitrogen. However, recent studies focusing on phytobiome are offering new insights into effects of nitrogen on interaction between plants and pathogens. In this review, I will first briefly describe importance of nitrogen as a key nutrient for plants and what changes excess nitrogen can bring about in rice and the fungal pathogen. Next, I will highlight some of the recent phytobiome studies relevant to nitrogen utilization and immunity of plants. Finally, I propose the hypothesis that changes in phytobiome upon excessive nitrogen fertilization contribute to nitrogen-induced susceptibility, and discuss empirical evidences that are needed to support the hypothesis.

Cognitive dysfunctions in individuals with diabetes mellitus

  • Kim, Hye-Geum
    • Journal of Yeungnam Medical Science
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    • v.36 no.3
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    • pp.183-191
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    • 2019
  • Some patients with type 1 and type 2 diabetes mellitus (DM) present with cognitive dysfunctions. The pathophysiology underlying this complication is not well understood. Type 1 DM has been associated with a decrease in the speed of information processing, psychomotor efficiency, attention, mental flexibility, and visual perception. Longitudinal epidemiological studies of type 1 DM have indicated that chronic hyperglycemia and microvascular disease, rather than repeated severe hypoglycemia, are associated with the pathogenesis of DM-related cognitive dysfunction. However, severe hypoglycemic episodes may contribute to cognitive dysfunction in high-risk patients with DM. Type 2 DM has been associated with memory deficits, decreased psychomotor speed, and reduced frontal lobe/executive function. In type 2 DM, chronic hyperglycemia, long duration of DM, presence of vascular risk factors (e.g., hypertension and obesity), and microvascular and macrovascular complications are associated with the increased risk of developing cognitive dysfunction. The pathophysiology of cognitive dysfunction in individuals with DM include the following: (1) role of hyperglycemia, (2) role of vascular disease, (3) role of hypoglycemia, and (4) role of insulin resistance and amyloid. Recently, some investigators have proposed that type 3 DM is correlated to sporadic Alzheimer's disease. The molecular and biochemical consequences of insulin and insulin-like growth factor resistance in the brain compromise neuronal survival, energy production, gene expression, plasticity, and white matter integrity. If patients claim that their performance is worsening or if they ask about the effects of DM on functioning, screening and assessment are recommended.

A Study on Brain Disease Research Trends and Need to Conduct Mechanism Studies on Temporomandibular Joint Disorder Related Cerebrovascular Diseases (뇌질환 연구 동향 조사 및 턱관절 장애 관련 뇌질환 기전 연구의 필요성)

  • Lee, Se-Eun;Lee, Min Ji;Lee, Byoungho;Lim, Sehyun;Cho, Suin
    • Journal of TMJ Balancing Medicine
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    • v.8 no.1
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    • pp.11-15
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    • 2018
  • Recently, clinical efficacies of the intraoral balancing appliance therapy have been reported by several researchers, and it has been found that there are various kinds of diseases that can be effectively applied. However, studies on cerebrovascular disease, one of the main diseases with a high mortality rate, are still poorly reported, and studies of temporomandibular joint disorder (TMD)-induced changes in brain function suggest that cerebrovascular disease is more appropriate as an adaptive disorder of the temporomandibular joint (TMJ) balancing device. In the developed countries, the importance of research on the structure and function of the brain has been recognized and spurred on the related research. In Korea, the research on brain function and cognitive disorders should have promoted more massively. In order to regain its former reputation in the Korean medicine in the field of cerebrovascular disease, it should be spurred on basic research and clinical case studies. In addition, extensive and in-depth studies including animal studies are needed to establish the basis of underlying mechanisms of the TMJ balancing therapies.

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