• Title/Summary/Keyword: Clinical Feature

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DENTAL MANAGEMENT OF THE RUSSELL-SILVER SYNDROME: CASE REPORT (Russell-Silver Syndrome 환아의 치과적 관리: 증례 보고)

  • Kim, Jun-Hhewk;Sohn, Hyung-Kyu;Kim, Seung-Hye;Choi, Hyung-Jun;Lee, Jae-Ho
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.6 no.2
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    • pp.99-104
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    • 2010
  • Russell-Silver syndrome is a genetic disorder characterized by intrauterine and/or postnatal growth restriction and typical facies. The clinical feature is various due to heterogeneous genetic characters. Their common findings are short stature without catch-up growth, normal head size for age, a distinctive triangular face with prominent forehead and a pointed chin, low set ears and clinodactyly of the fifth fingers. Intraoral features of the syndrome are microdontia, delayed tooth eruption, hypodontia, and crowding. More than 400 case have been reported in the literature, and estimated incidence is from 1 in 3000 to 1 in 100,000. In this case we performed caries treatment under the general anesthesia for the patient with Russell-Silver syndrome. Dentist have to consider microstomia for the management of patients with Russell-Silver syndrome.

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CAROTID-CAVERNOUS SINUS FISTULA ACCOMPANYING FACIAL BONE FRACTURE : Report of a Case (안면골 골절과 동반된 경동맥해면동루의 증례보고)

  • Park, No-Bu;Seo, Yeon-Ho;Moon, Seon-Hye;Lee, Yong-Oh
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.15 no.2
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    • pp.100-104
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    • 1993
  • Carotid-cavernous sinus fistula(CCSF) is an abnormal arterio-venous communication between the cavernous sinus and the internal carotid artery. It is usually caused by craniofacial trauma and a very rarely encountered complication, but it may also occur spontaneously. The most common cause of traumatic CCSF is blunt trauma, which usually associated with a skull base, frontal or midfacial fracture. The common clinical feature of CCSF are orbital bruit, headache, exophthalmos, chemosis, diplopia, visual disturbance and others. This dramatic ocular-orbital symptoms are principally due to orbital venous hypertension. The symptoms occured within a few hours to a maximum of a year after injury, usually within several weeks. The patient, 33-year-old female, developed a carotid-cavernous sinus fistula after only minimal closed trauma We present a rare case of CCSF associated facial bone fracture that was successfully treated by detachable balloon embolization with a review of the literature.

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A Case of Paraneoplastic Limbic Encephalitis Associated with Primary Adenocarcinoma of Lung (비소세포 폐암과 동반된 부수종양성 변연계뇌염 1예)

  • Shin, Hyun Jong;Kim, Hyun Soo;Lim, Keum Nam;Noh, U Seok;Choi, Jung Hye;Kim, In Soon;Lee, Young Yeul;Park, Byeong Bae;Park, Dong Woo
    • Tuberculosis and Respiratory Diseases
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    • v.63 no.4
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    • pp.382-386
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    • 2007
  • Paraneoplastic limbic encephalitis is a rare disorder that is characterized by personality changes, irritability, depression, seizures, memory loss and dementia, and is commonly associated with small cell lung cancer. The cause is unknown but it is believed to be an autoimmune disorder that develops secondary to a carcinomatous process. We report a patient with the clinical feature consistent with limbic encephalitis. A 64-year-old women developed disorientation, memory loss and general weakness. She was diagnosed with NSCLC (adenocarcinoma) with a brain metastasis 1 year earlier and was treated with radiation and chemotherapy. Although the lung mass and brain metastatic lesions had improved, the brain T2-weighted MRI showed high signal intensity in the right temporal region. This lesion consisted of with limbic encephalitis and was negative to the other viral and immune markers. The patient's symptoms did not improve after steroid treatment. Our case demonstrated that a NSCLC (adenocarcinoma) also can be associated with paraneoplastic limbic encephalitis.

A Case of Bronchiolitis Interstitial Pneumonitis (Bronchiolitis Interstitial Pneumonitis 1예)

  • Chi, Su Young;Ryu, Kyoung Ho;Lim, Dae Hun;Shin, Hong-Joon;Ban, Hee Jung;Oh, In-Jae;Kwon, Yong Soo;Kim, Kyu-Sik;Lim, Sung-Chul;Kim, Young-Chul;Choi, Yoo-Duk;Song, Sang-Yun;Seon, Hyun Ju
    • Tuberculosis and Respiratory Diseases
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    • v.67 no.4
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    • pp.364-368
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    • 2009
  • Bronchiolitis interstitial pneumonitis (BIP), an unclassified and newly described interstitial pneumonia, has a combined feature of prominent bronchiolitis, interstitial inflammation, and fibrosis. It is distinct from bronchiolitis obliterans or bronchiolitis obliterans organizing pneumonia (BOOP). BIP has a better prognosis than common cases of interstitial pneumonia. However, BIP has a poorer prognosis than BOOP. BIP's response to corticosteroids is not as successful as BOOP's response to this treatment. We encountered the case of a 31-year-old woman with BIP with an initial presentation of dyspnea and a cough that had lasted for 3 months. The patient's chest CT scan demonstrated patchy ground glass opacities and multiple ill-defined centrilobular nodules in both lungs, suggesting military tuberculosis or nontuberculous mycobacterial infection. A video-assisted thoracoscopic lung biopsy resulted in the diagnosis of BIP. Clinical symptoms, pulmonary lesions, and pulmonary function tests were improved after oral glucocorticoid therapy.

ORAL SELF-MUTILATION IN THE LESCH-NYHAN SYNDROME : CASE REPORT (Lesch-Nyhan syndrome 환아의 oral self-mutilation에 대한 증례보고)

  • Jeon, Jin-Yong;Lee, Jae-Ho;Choi, Hyung-Jun;Choi, Byung-Jai
    • Journal of the korean academy of Pediatric Dentistry
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    • v.26 no.1
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    • pp.151-156
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    • 1999
  • Self-injurious behavior is defined as deliberate harm to one's own body without suicidal intent. It usually occurs as head banging or hitting, skin cutting, or finger biting and includes ocular, genital and oral self-mutilation. Lesch-Nyhan syndrome is a rare X-linked recessively inherited disorder, caused by complete absence of hypoxanthin-guanine phosphoribosyl transferase(HPRT) activity. Clinical presentation is characterized by mental retardation, chorea, athetosis, hyperuricemia, uricosuria and self-mutilating behavior. In these patients, the most typical feature is loss of tissue from biting themselves, even though they are not insensitive to pain. The dental management of self-mutilation includes treatment with appliances such as soft mouthguard or lip bumper, extraction of all the teeth, and orthognathic surgery. We report a 25-month-old boy who was a known case of Lesch-Nyhan syndrome and presented with severe self-mutilation wound on his lower lip. Vital pulpectomy and coronal resection was done as a more conservative approach than extracting all primary anterior teeth. Due to maintaining the root portion of the teeth in the bone, it is expected that the normal growth of the alveolar bone will be achieved.

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Treatment Results for Adenoid Cystic Carcinoma of the Major Salivary Gland (주타액선에서 발생한 선양낭세포암종의 방사선치료성적)

  • Lee Sang-Wook;Kim Gwi-Eon;Park Cheong-Soo;Choi Eun-Chang;Lee Kang-Kyoo;Park Won;Suh Chang-Ok
    • Korean Journal of Head & Neck Oncology
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    • v.18 no.2
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    • pp.197-202
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    • 2002
  • Purpose: This study was to investigate clinical feature of adenoid cystic carcinoma (ACC) in the major salivary gland. Materials and Methods: A retrospective analysis was undertaken for 23 patients with adenoid cystic carcinoma of the major salivary gland, who completed postoperative radiotherapy at the Yonsei Cancer Center, Yonsei University Hospital between May 1981 and December 1999. The male to female ratio was 1.1:1. Median age was 50. Follow up periods were 12-180 months with the median follow-up time of 59 months. Results: Parotid gland, submandibular gland and sublingual gland were 17, 5 and 1 respectively. Overall failure rate was 26%. Local failure was observed in two patients with parotid gland origin. Five cases showed distant failure. Overall and disease free survival rate were 68% and 72% respectively. Conclusion: Major pattern of failure was distant failure. Resection margin status had not prognostic significance. Postoperative radiation treatment is an effective to control in the adenoid cystic carcinoma of the major salivary gland.

A PRESERVATIVE APPROACH TO PREVENTION OF SELF-MUTILATION IN PATIENT WITH LESCH-NYHAN SYNDROME ; A CASE REPORT (Lesch-Nyhan 증후군 환아의 자해 예방을 위한 보존적 접근 : 증례보고)

  • Kang, Dong-Gyun;Kim, Tae-Wan;Kim, Young-Jin
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.2 no.2
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    • pp.147-152
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    • 2006
  • Lesch-Nyhan syndrome is rare X-linked genetic disorder involving absence of the enzyme hypoxanthine guanine phosphoribosyl transferase (HGPRT) related to purine metabolism. The deficiency of HGPRT activity leads to an excesscive uric acid production and consequent hyperuricemia. It occurs almost exclusively in males, and the incidence is estimate to be 1/100,000~380,000. Clinical presentation is characterized by developmental delay, mental retardation, choreoathetosis, spastic cerebral palsy, nephrolithiasis, obstructive nephropathy and acute gouty arthritis. A characteristic feature of Lesch-Nyhan syndrome is the appearance of intractable self-mutilation behavior. Self-mutilation behavior is complicated by secondary infection and tissue loss as well as pain. The dental management of self-mutilation includes presertive methods of using appliances such as lip bumper or soft mouthguard and radical methods such as extraction of all teeth or orthognathic surgery. A case of Lesch-Nyhan syndrome patient with self-mutilation and severe lower lip injury is presented. He was treated successfully with soft mouthguard.

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Cytologic Findings of Chordoma in Fine Needle Aspiration Cytology (척삭종의 세침흡인 세포학적 소견)

  • Ryu, Han-Suk;Kim, Min-Suk;Ha, Hwa-Jung;Kim, Jung-Soon;Shin, Myung-Soon;Park, Sun-Hoo;Chung, Jin-Haeng;Koh, Jae-Soo;Lee, Seung-Sook
    • The Korean Journal of Cytopathology
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    • v.15 no.1
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    • pp.45-51
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    • 2004
  • It is important to recognize the pathognomonic cytologic findings of chordoma, because of overlapping cytologic features between chordoma, chondrshdosarcoma, myxofilbrillary ependymoma, and metastatic adenocarcinoma. We reviewed the cytomorphologic features of 5 cases of chordoma diagnosed by fine needle aspiration cytology at Korean Cancer Center Hospital from 1987 to 2003. Clinical and radiographic findings of each case were reviewed. Four males and one female (29-54 years) had tumors involving the sacrum. Pain was the presenting symptom in 4 cases. The three cases showed moderate to high cellularity. In all cases, typical physaliferous cells with or without cytoplasmic processes were noted. In two cases, the background was myxoid with single scattered cells. Cell clusters showing cord-like arrangement were occasionally seen. The single or clustered cells showed mild cellular pleomorphism with slightly increased nuclear/cytoplasmic ratio. Mitotic figures were not seen. In our review, the recognition of physaliferous cells is the most important feature to diagnose chordoma and to differentiate it from other lesions mimicking chordoma.

A Study on Nobel SUNBLOCK Materials Containing an Active Ingredient Extracted from Symbiodinium (심바이오디니움으로부터 추출된 유효성분을 함유하는 신규 SUN BLOCK 물질에 관한 연구)

  • Kim, Dong-Myong;Jung, Ju-Yeong;Lee, Hyung-Kon;Park, Sang-Keun;Kwon, Yong-Sung;Yang, Seung-Koo;Han, In-Suk
    • Journal of Marine Bioscience and Biotechnology
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    • v.12 no.1
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    • pp.20-28
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    • 2020
  • Symbiodinium is a dinoflagellate genus that coexists with coral reefs and is known to provide ultraviolet (UV) protection in nature through the synthesis of mycosporin-like amino acids (MAA). In order to develop a natural and ecofriendly sunblock for use in summer resorts, the possibility of using a Symbiodinium microbiome extract or an MAA was investigated. Two sunblocks, one containing 7% Symbiodinium extract and the other containing MAA were prepared to be tested on hairless mice and human skin. In this clinical study, the sun protection factor (SPF) and Protection factor of UV A (PFA) values of the sunblock containing either Symbiodinium extract or MAA were determined. The SPF values of the sunblock containing Symbiodinium extract and MAA were 10.43 and 10.83, respectively, and the PFA values were 3.42 and 3.39, respectively. Based on their PFA values of ≥2, the UV A protection can be graded as PA+ (low) for both the sunblocks. In addition, the UV-blocking extract of Symbiodinium has a low phototoxicity and cytotoxicity, reducing the possibility of a heavy feeling to the skin or a foreign-body sensation caused by residue build-up. The low toxicity feature of the major sunblock component will also prevent side effects, such as allergic reactions. Although Symbiodinium extract or MAA alone do not provide effectiveprotection against UV irradiation, their protection capacity can be complemented by the addition of inorganic UV-protecting compounds.

Implementation of the Classification using Neural Network in Diagnosis of Liver Cirrhosis (간 경변 진단시 신경망을 이용한 분류기 구현)

  • Park, Byung-Rae
    • Journal of Intelligence and Information Systems
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    • v.11 no.1
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    • pp.17-33
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    • 2005
  • This paper presents the proposed a classifier of liver cirrhotic step using MR(magnetic resonance) imaging and hierarchical neural network. The data sets for classification of each stage, which were normal, 1type, 2type and 3type, were analysis in the number of data was 231. We extracted liver region and nodule region from T1-weight MR liver image. Then objective interpretation classifier of liver cirrhotic steps. Liver cirrhosis classifier implemented using hierarchical neural network which gray-level analysis and texture feature descriptors to distinguish normal liver and 3 types of liver cirrhosis. Then proposed Neural network classifier learned through error back-propagation algorithm. A classifying result shows that recognition rate of normal is $100\%$, 1type is $82.8\%$, 2type is $87.1\%$, 3type is $84.2\%$. The recognition ratio very high, when compared between the result of obtained quantified data to that of doctors decision data and neural network classifier value. If enough data is offered and other parameter is considered this paper according to we expected that neural network as well as human experts and could be useful as clinical decision support tool for liver cirrhosis patients.

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