• Journal of Chest Surgery
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• 제33권2호
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• pp.179-182
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• 2000

Background: A pleural effusion is not a disease entity but a clincial sign of systemic or pleural disease. Although the diagnosis of pleural effusion can often be done by toracentesis and pleural needle biopsy the yeild of positive diagnosis is low.

• Tuberculosis and Respiratory Diseases
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• 제85권4호
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• pp.302-312
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• 2022

Chronic obstructive pulmonary disease (COPD) is a complex and heterogeneous disease. Not all patients with COPD respond to available drugs. Identifying respondents to therapy is critical to delivering the most appropriate treatment and avoiding unnecessary medication. Recognition of individual patients' dominant characteristics by phenotype is a useful tool to better understand their disease and tailor treatment accordingly. To look for a suitable phenotype, it is important to understand what makes COPD complex and heterogeneous. The pathology of COPD includes small airway disease and/or emphysema. Thus, COPD is not a single disease entity. In addition, there are two types (panlobular and centrilobular) of emphysema in COPD. The coexistence of different pathological subtypes could be the reason for the complexity and heterogeneity of COPD. Thus, it is necessary to look for the phenotype based on the difference in the underlying pathology. Review of the literature has shown that clinical manifestation and therapeutic response to pharmacological therapy are different depending on the presence of computed tomography-defined airway wall thickening in COPD patients. Defining the phenotype of COPD based on the underlying pathology is encouraging as most clinical manifestations can be distinguished by the presence of increased airway wall thickness. Pharmacological therapy has shown significant effect on COPD with airway wall thickening. However, it has limited use in COPD without an airway disease. The phenotype of COPD based on the underlying pathology can be a useful tool to better understand the disease and adjust treatment accordingly.

• Journal of Genetic Medicine
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• 제4권1호
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• pp.22-37
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• 2007

The autosomal dominant spinocerebellar ataxias (SCAs) are a group of neurodegenerative diseases, clinically and genetically heterogeneous, characterized by degeneration of spinocerebellar pathways with variable involvement of other neural systems. At present, 27 distinct genetic forms of SCAs are known: SCA1-8, SCA10-21, SCA23, SCA25-28, DRPLA (dentatorubral-pallidoluysian atrophy), and 16q-liked ADCA (autosomal dominant cerebellar ataxia). Epidemiological data about the prevalence of SCAs are restricted to a few studies of isolated geographical regions, and most do not reflect the real occurrence of the disease. In general a prevalence of about 0.3-2 cases per 100,000 people is assumed. As SCA are highly heterogeneous, the prevalence of specific subtypes varies between different ethnic and continental populations. Most recent data suggest that SCA3 is the commonest subtype worldwide; SCA1, SCA2, SCA6, SCA7, and SCA8 have a prevalence of over 2%, and the remaining SCAs are thought to be rare (prevalence <1%). In this review, we highlight and discuss the SCA7. The hallmark of SCA7 is the association of hereditary ataxia and visual loss caused by pigmentary macular degeneration. Visual failure is progressive, bilateral and symmetrical, and leads irreversibly to blindness. This association represents a distinct disease entity classified as autosomal dominant cerebellar ataxia (ADCA) type II by Harding. The disease affectsprimarily the cerebellum and the retina by the moderate to severe neuronal loss and gliosis, but also many other central nervous system structures as the disease progresses. SCA7 is caused by expansion of an unstable trinucleotide CAG repeat in the ATXN7 gene encoding a polyglutamine (polyQ) tract in the corresponding protein, ataxin-7. Normal ATXN7 alleles contain 4-35 CAG repeats, whereas pathological alleles contain from 36->450 CAG repeats. Immunoblott analysis demonstrated that ataxin-7 is widely expressed but that expression levels vary among tissues. Instability of expanded repeats is more pronounced in SCA7 than in other SCA subtypes and can cause substantial lowering of age at onset in successive generations termed ‘anticipation’ so that children may become diseased even before their parents develop symptoms. The strong anticipation in SCA7 and the rarity of contractions should have led to its extinction within a few generations. There is no specific drug therapy for this neurodegenerative disorder. Currently, therapy remains purely symptomatic. Cellular models and SCA7 transgenic mice have been generated which constitute valuable resources for studying the disease mechanism. Understanding the pathogenetic mechanisms of neurodegeneration in SCAs should lead to the identification of potential therapeutic targets and ultimately facilitate drug discovery. Here we summarize the clinical, pathological, and genetic aspects of SCA7, and review the current understanding of the pathogenesis of this disorder. Further, we also review the potential therapeutic strategies that are currently being explored in polyglutamine diseases.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제34권3호
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• pp.383-387
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• 2008

The calcifying odontogenic cyst(COC) is considered to occupy a position between a cyst and an odontogenic tumor-having charateristics of both. Gorlin and col. described the COC for first time as an own pathological entity in 1962. Clinically, the COC represents 1% of the odontogenic lesion. It is possible to be found from the first decade to the eight decade but is more frequent during the second decade. It affects in same proportion the maxilla and jaw, being the most in tooth-bearing area of the jaw. This case of COC associated with an unerupted tooth which appeared in the right mandible of 22-year-old woman, was reported. This case report is to present a review of the literature relates to this case of COC and its treatment, discuss clinical, radiographic, histological and therapeutic aspects.

• Tuberculosis and Respiratory Diseases
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• 제39권3호
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• pp.271-277
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• 1992

Diffuse panbronchiollitis (DPB), a rare progressive disorder, has lately been receiving increasing attention. DPB is a disease of obscure etiology, characterized by chronic inflammation localized mainly in the region of respiratory bronchiole just distal to the terminal bronchioles. In 1983, Homma and coworkers reported 82 cases of a new clinicopathological entity, DPB, in Japan. Also DPB is a disease largely restricted geographically to Japan but the prevalence in other countries is extremely low. Histoloically, it is characterized by a suppurative bronchiolitis involving primarily the respiratory and terminal bronchioles with subsequent progression to bronchiectasis. The disease progresses rapidly and results in respiratory failure due to repeated respiratory infections. We experienced a cases of DPB accompanied with chronic maxillary sinusitis in both sinuses. Diagnosis of DPB was comfirmed by pathological results from thoracoscopic lung biopsy, typical radiological findings, clinical symptoms and pulmonary function test. After treatment with erythromycin for 6 months, the patient's condition and the typical micronodular densities on the chest radiography improved. A few case of DPB was reported in Korea. We report a case of DPB through thoracoscopic lung biopsy.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제35권1호
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• pp.66-71
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• 2013

Dentinogenic ghost cell tumor (DGCT) is a rare epithelial odontogenic neoplasm, representing 1.9% to 2.1% of all odontogenic tumors. It is the neoplastic counterpart of the calcifying odontogenic cyst (COC), and characteristic islands of odontogenic epithelical cells contain numerous ghost cells and dysplastic dentin, and also have many common histological features with ameloblastoma. The 2005 World Health Organization (WHO) Classification of Odontogenic Tumours re-named this entity as calcifying cystic odontogenic tumor (CCOT) and defined the clinico-pathological features of the ghost cell odontogenic tumours, CCOT, DGCT and ghost cell odontogenic carcinoma (GCOC). We report a rare case of central DGCT in the posterior maxilla of a 31-year-old female with literature review, for the emphasis of Oral and Maxillofacial surgeon's role.

• Journal of Gastric Cancer
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• 제13권2호
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• pp.126-128
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• 2013

Pyogenic liver abscess in patients with malignant disease is a fatal state and is easily diagnosed. We presented a rare case of sudden fatal septicemia following anticancer treatment for recurred gastric cancer due to multiple liver abscesses which could not be diagnosed. A 72-year-old male with recurred gastric cancer received anticancer agents. He had a history of distal gastrectomy with right hepatic lobectomy for hepatic metastasis. He received anticancer treatment in the outpatient's service center periodically, and his performance status was preserved with nothing in particular. After administrating docetaxel, he suddenly developed septicemia and multiple organ failure and died 5 days after strong medical supports. Pathological autopsy revealed that multiple minute abscesses of the liver which could not be detected macroscopically were the causes of fatal septicemia. The etiology, therapies and prognosis of rare entity are being discussed.

• 대한두개안면성형외과학회지
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• 제18권2호
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• pp.141-144
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• 2017

An odontogenic cutaneous fistula is a pathological communication between the outer skin surface of the face and the oral cavity. Facial cutaneous fistula is a complication of odontogenic infection that is often misdiagnosed with skin infection. We report a rare case, which was diagnosed as basal cell carcinoma based on the biopsy of skin lesions in the patient who had been diagnosed with odontogenic cutaneous fistula. A 64-year-old male patient presented with a cutaneous odontogenic fistula. The patient had undergone surgical extraction of fistula tract and loose tooth before dermatology or plastic surgery consultation. With the biopsy and computed tomography, it was confirmed that fistula and basal cell carcinoma. However, the connection between the fistula and skin cancer was not clear. Positron emission tomography-computed tomography scan was performed and was not detected as other local or distant metastasis. After that, wide excision of the skin lesion was performed. Although skin cancer is not commonly observed, it is necessary to rule out this disease entity by performing biopsy of skin lesions.

• Archives of Plastic Surgery
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• 제35권1호
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• pp.104-106
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• 2008

Purpose: Fewer than 100 cases of calcifying aponeurotic fibroma have been reported in the literature since this entity was initially described by Keasbey in 1953 who called it calcifying juvenile aponeurotic fibroma. The tumor is a slowly growing, painless mass. In most cases the mass is poorly circumscribed and causes neither discomfort nor limitation of movement. Most lesions occur in children, with a peak incidence ages of 8-14 years. There is no evidence of any increased familial prevalence. Predilection sites are palm, finger, toe, but it also occurs in the wrist, forearm, elbow, upper arm, neck, abdominal wall, lumbar paravertebral area, leg and ankle. We herein describe a rare case of calcifying aponeurotic fibroma occurring on the chin with review of the literature. Methods: A 14-year-old male had painless, slowly growing mass(${\phi}2.5cm$) on a chin for a year. The tumor was excised elliptically under local anesthesia and the excisional site was repaired directly. Due pathological examination was processed. Results: Histological examination revealed an illdefined fibrous growth that extends with multiple processes into the surrounding tissue with centrally located foci of calcification. The tumor is composed of short spindled plump fibroblasts with round or ovoid nuclei separated by collagenous stroma, showing vaguely palisading pattern. Diagnosis of calcifying aponeurotic fibroma was conferred. Postoperatively, the patient did well, and the lesion had not recurred. Conclusion: Fewer than 100 cases of calcifying aponeurotic fibroma have been reported in the literature. The most common occurring sites are palm, finger & toe, but it has been reported in the wrist, forearm, elbow, upper arm, neck, abdominal wall, lumbar paravertebral area, leg and ankle. Two cases of calcifying aponeurotic fibroma occurring on the neck have been reported in the literature. To the authors knowledge, our case of calcifying aponeurotic fibroma occurring on the chin is the first to be reported.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제14권3호
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• pp.185-193
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• 1992

The calcifying odontogenic cyst was identified as a pathological entity by Gorlin & his associates in 1962. This lesion is one of the rarest and most disputable cysts in the oral region. The calcifying odontogenic cyst has variable clinical and radiological features. We review the previous literatures and report 2 cases of calcifying odontogenic cyst at Department of Oral and Maxillofacial Surgery, Kyung-Hee University. The 1st case was as follows. The patient vas 22 year old female. The past dental history revealed extraction of prolonged retained #73 tooth about 15days ago. She complained a painful swelling on the lower anterior teeth area. There were chin and vestibular swelling on the lower anterior teeth area, tenderness and missing of #33 tooth. The radiograph revealed well-demarcated unilocular radiolucency containing radiopaque calcific flecks around impacted #33 tooth. The clinical diagnosis was COC, so surgical enucleation was done. There was no recurrence and COC was confirmed by pathologist. The second case was as follows. The patient was 72 year old male. The past history revealed inactive tuberculosis, bronchial asthma and denture construction. The chief complaint was rapidly growing mass on the lower left anterior edentulous area. The clinical findings were chin swelling protruding mass with surface ulceration, fluctuation and a few bloody fluid in aspiration. The radiograph revealed well-demarcated radiolucency mimiking the residual cyst. The biopsy result was COC. The surgical excision was done, but the lesion was recurred 10 months later. The treatment was surgical excision with aggressive peripheral bone grinding and FTSG form groin area. There was no problem during the postoperative period.