• Maxillofacial Plastic and Reconstructive Surgery
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• v.16 no.2
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• pp.186-195
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• 1994

Fibromatosis is benign fibroblastic proliferative lesion with abundant collagenous neo-formation located principally in the abdominal wall and in the upper and lower extremities (Masson & Soule, 1966). Wilkins and Waldron, in 1975, suggested that the title aggressive fibromatosis was a more appropriate term, reflecting the invasive characteristics of the disease. Synonyms listed were extra-abdominal desmoid, juvenile fibromatosis, aggressive infantile fibromatosis and congenital fibrosarcoma. A total of 12% of all fibromatosis arise in head and neck. Fibromatosis of the oral cavity is uncommon and is even more rare when in involve the mandibule. It is a locally aggressive fibrous tissue tumor, generally does not metastasize, but may cause considerable morbility and even death due to local infiltration. The degree of microscopic cellularity is variable, not only from tumor to tumor but also from area to area in the same tumor. Some tumors present with proliferation of mature fibroblasts and a dominating collagenous component : others may show a lack of the tumor in both types. The common histologic denominator appears to be cellular interlacing bundles of elongated fibroblasts, showing little or no mitotic activity and no pleomorphism. Mitosis are not a consistent index of malignancy when found in younger age groups. Fibromatosis still posses difficult problems of diagnosis and treatment. It is frequently recurrent and infliltrates neighbouring tissues. These lesion infliltrate widely and replace muscle, fat, and even bone with fibrous tissue of varying cellularity. Lesion representing fibromatosis in the oral cavity must be carefully evaulated by both surgeon and pathologists to ensure proper diagnosis and treatment planning. When these lesions involve bone, surgeon must be aware of the lesion's potential to perforate the cortex and expand while remaining hidden from the surgeon's view. Careful and precise clinical correlation with histologic appearance is essential to preclude misdiagnosis of fibrosarcoma yet provide surgical treatment plan that provides adequate local excision and long-term follow up. As regards cause, little is known. It is attributed to trauma or alteration in the sex hormone(Carlos, et al, 1986). Clinially, the lesion is reported to be not painful in most cases, but capable of rapid growth. The treatment is essentially surgical excision with wide margin of adjacent uninvolved tissue. Radiotherapy, hormone treatment or chemotherapy are of no use (WIkins et al, 1975 ; Majumudar and Winiarkl, 1978). We report a case of aggressive fibromatosis of 15-year-old with a lesion in the soft tissue of the parotid area that invaded the underlying bone of the mandibular body.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.29 no.5
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• pp.394-404
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• 2007

Oral squamous cell carcinoma is the most prevalent oral cancer, which is characterized by its high metastasis and recurrent rates and poor prognosis. Taxol is an anticancer agent which is microbial products extracted from jew tree. It combines with the tubulin and induces apoptosis by inhibiting mitosis of cell with microtubule stabilization. Recently, it was reported to be effective in various solid tumors, but only very slight effect has been seen in oral squamous cell carcinomas due to its cell-specific potencies. Cyclosporin A is used as immune suppressant and is being applied in anticancer therapy as its mechanism of induction of change of apoptotic process in various cells have been known. In this study, oral squamous cell carcinoma HN22 cell line was used for in vitro experiment and as for the experimental group taxol and cyclosporin A were applied alone and to observe the synergistic effect of apoptosis, Taxol and cyclosporin A were coadministered with different concentration of taxol for comparison. The results were obtained as follow: 1. There was no difference in Bcl-2, Bax, caspase 3, 8, 9 mRNA expression when cyclosprin A or taxol was applied alone to HN 22 cell line. 2. Caspase 3, 9 mRNA expression was prominently increased when cyclosprin A and taxol were applied together to cancer cell. 3. No significant difference was observed when cyclosporin A and taxol($1{\mu}g/ml$ and $3{\mu}g/ml$) were applied together to cancer cell line. 4. No significant difference was seen in Bcl-2, Bax, and caspase 8 mRNA expression in all the groups of in vitro experiments. 5. When cyclosporin A was applied alone in vivo study on the nude mice, histopathologi cal findings was similar to those of the control group. Oral squamous cell carcinoma induced by inoculation of HN 22 cell line was not reduced after treatment of cyclosporin A. 6. When taxol was applied alone, the islands of squamous cell carcinoma still remained, which meant insignificant healing effect. There was a lesser volume increase compared with the cyclosporin A alone. 7. When taxol and cyclosporin A were applied together, the connective tissue and calcification were seen in the histopathologic findings. Oral squamous cell carcinoma was decreased and cancer cell was disappeared. In observing the tumor mass change with time, there was a gradual decreased size and healing features. As the results of the in vitro experiment, it could conclud that only when the two agents are applied together, mitochondria-mediated apoptosis occurred by considerable increase of caspase 3, 9 mRNA expression, irrespectable of the concentration of taxol. In vivo experiment, there was a discrete synergistic effect when the two agents were applied together. But single use of cyclosporin A was not effective in this study. Based on the results of this experiment, if further clinical studies are done, taxol and cyclosporin A could be effectively used in treatment of oral squamous cell carcinomas.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.31 no.5
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• pp.408-413
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• 2009

Odontogenic keratocyst (OKC) is a epithelial developmental cyst which were first described by Phillipsen in 1956. The frequency of OKC has been reported to vary from 3% to 11% of odontogenic cysts. The most characteristic clinical aspect of OKC is the high frequency of recurrence. The mechanism of recurrence is thought to be related to residues of cyst epithelium and an intrinsic growth potential following excision. And since the lining of the OKC is thin and friable, removal of the cyst in one piece may sometimes be difficult. Complete removal of the cyst lining without leaving behind remnants attached to the soft tissue or bone is necessary to avoid recurrence. Therapeutic approaches vary in different studies from marsupialization and enucleation, which may be combined with adjuvant therapy such as cryotherapy or Carnoy's solution, to marginal or radical resection. The recurrent rate varies from approximately 20% to 62%. And OKC in the angle-ramus region of the mandible had a higher tendency to recur, because of the difficulty in accessing and removing OKC from the ramus. By employing a sagittal splitting of the mandible a good surgical access was provided and cyst could be removed completely. We present an illustrative case of a small, lobulated OKC that involved ramus on mandible, and a review of the contemporary literature.

• Tuberculosis and Respiratory Diseases
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• v.40 no.2
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• pp.165-170
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• 1993

Background: Massive hemoptysis that may induce acute asphyxia can be a fatal problem. Bronchial arteries and other nonbronchial systemic arteries of lungs must be searched by angiography, because they are main source of hemoptysis. Arterial embolization is a well-accepted and widely used for management of massive hemoptysis. This study was designed to evaluate the effectiveness of this method. Method: Prospective analysis was done in 23 cases, that underwent arterial embolization from June 1990 to July 1992. Hemorrhaged arteries were embolized with Gelfoam particles. In cases with severe broad hemorrhagic findings, Coils were added to Gelfoam particles. And they were observed for 6 months at least. Results: Immediate cessation of hemoptysis was achieved in all cases. Recurrent hemoptysis was observed in 7 cases (30%). The patients with nonbronchial artery hemoptysis had increased tendency of recurrence (6/13) than only bronchial artery hemotysis (1/10). The 7 cases treated with Coils had not any recurrence. Conclusion: Arterial embolization in massive hemoptysis is a useful and safe procedure for immediate control. But, the patients with this procedure had a potentiality of recurrence. So diagnostic and therapeutic efforts for underlying causes should be performed.

• Tuberculosis and Respiratory Diseases
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• v.52 no.1
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• pp.86-91
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• 2002

A bronchial artery aneurysm(BAA) is rare, and has an unclear etiology. However, it may be caused by congenital abnormalities and acquired diseases like as bronchiectasis, tuberculosis, and other infections. The pathogenesis of a bronchial artery dilatation and the formation of an aneurysm results in an increase in the systemic blood flow to the chronic inflammatory pathologic lungs such as bronchiectasis or tuberculosis. It can be divided into the mediastinal and intrapulmonary BAA according to their location. The most common symptom is hemoptysis. Chest computed tomography and bronchial artery angiography may be used for a diagnosis. Treatment is mainly by a surgical resection of the aneurysmal artery. However, when patient is unstable due to massive hemoptysis or recurrent hemoptysis, bronchial artery embolization is useful. Here, we experienced a case of a bronchial artery aneurysm presenting as a massive hemoptysis.

• Journal of the Korean Chemical Society
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• v.50 no.2
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• pp.123-128
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• 2006

Behcet's disease (BD) is a systemic vasculitis characterized by recurrent oral and genital ulcers, and ocular inflammation, and which may involve the joints, skin, central nervous system and gastrointestinal tract. Although the exact pathogenesis for BD is not completely understood, it has been suggested that the disease is triggered in genetically susceptible individuals by environmental factors, such as microbial agents. It is noted that multiple genes, including MHC (major histocompatibility complex) and non-MHC genes, are implicated in the pathogenesis of BD. This study tries to determine whether IL-6 gene polymorphisms are associated with susceptibility to Behcet's disease in Koreans. Gene polymorphisms were typed by VNTR (variable number of tandem repeat), RFLP (restriction fragment length polymorphism), DHPLC (denaturing high performance liquid chromatography).There were no evidences for genetic association conferred by the IL-6prom polymorphism. However, significant differences in the IL-6vntr genotype and allele frequencies were found between patients with BD and controls. The IL-6vntr*C allele appeared to be an additional susceptibility gene to Korean BD. Further studies in other populations and gene are required to confirm these results.

• Korean Journal of Bronchoesophagology
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• v.3 no.2
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• pp.293-301
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• 1997

The tonsillitis has long been one of the most common disease in the otolaryngologic field. Peritonsillar abscess occurs when bacterial infection of the tonsil spreads to the potential peritonsillar space deep behind the tonsil, and it usually occurs in patients with recurrent tonsillitis or in those with tonsillitis who have been inadequately treated. We studied retrospectively 71 patients who had been diagnosed as acute tonsillitis and 82 patients who had been diagnosed as peritonsillar abscess and had admitted in our department of the Ulsan Dong Kang Hospital from January, 1995 to September, 1997. Especially in the bacteriologic studies, we compared acute tonsillitis and peritonsillar abscess with chronic tonsillitis. The following results were obtained： 1) The sex distributions of acute tonsillitis were 47 males(66%) and 24 females(34%) cases, but 57 males(70%) and 25 females(30%) in cases of peritonsillar abscess. There were predominant in male and frequently affected in second and third decades in 53 cases(76%) of acute tonsillitis and 56 cases(68%) of peritonsillar abscess. 2) It was same found in each season. 3) The duration from onset of symptom to visit in our department was 3.92 days in cases of acute tonsillitis and 5.95 days in cases of peritonsillar abscess in average 4) The major symptoms were sore throat, swallowing difficult. And others were fever, fatigability, dysarthria, trismus, headache, otalgia. 5) Among the 71 cases of acute tonsillitis and 82 cases of peritonsillar abscess, most temperature of patients at visit were 36.6-37.5 $^{\circ}C$ in each 36 cases(51%), 57 cases(70%). 6) In each disease, 35 cases(47%), 45 cases(75%) consisted of single infection and 39 cases(53%), 15 cases(25%) consisted of mixed infection. In acute tonsillitis, 111 strains were isolated from 74 cases, the most common strain was 69 strains(62.2%) of $\alpha$-hemolytic streptococci. In the peritonsillar abscess, 77 strains were isolated from 60 cases, the most common strain was 49 strains(63.6%) of $\alpha$-hemolytic streptococci. In chronic tonsillitis, 563 strains were isolated from 382 cases, the most common strain was 334 strains(50.3%) of $\alpha$-hemolytic streptococci. 7) In acute tonsillitis and peritonsillar abscess, the most common leukocyte levels were reported with 10, 000-15, 000/$\mu$L in 23 cases(32%). The CRP levels were reported with abnormal findings in 61 cases(97%), 63 cases(95%) above 0.3 in each cases.

• Tuberculosis and Respiratory Diseases
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• v.54 no.6
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• pp.628-634
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• 2003

Bronchiectasis is defined as an abnormal, irreversible dilatation of the bronchi, which may result from a number of possible causes, and the recognition of these causes may lead to a specific management strategy. Immunodeficiency is known as one of the conditions associated with bronchiectasis. X-linked agammaglobulinemia is a rare inheritable immunodeficiency disorder, caused by a differentiation block, leading almost to the complete absence of B lymphocytes and plasma cells. The affected protein is a cytoplasmic protein tyrosine kinase, Bruton's tyrosine kinase. The early detection and treatment with immunoglobulin replacement are most important for the management of recurrent infections and for reducing severe complications. We report a 20-year-old male patient, with X-linked agammaglobulinemia associated with bilateral bronchiectasis, carrying a missense mutation(R520P) in the BTK gene.

• Tuberculosis and Respiratory Diseases
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• v.63 no.1
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• pp.72-77
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• 2007

Antiphospholipid syndrome (APS) causes recurrent thromboses and morbidity during pregnancy, including fetal loss. This malady is associated with the persistent presence of anticardiolipin antibody or lupus anticoagulant. The pulmonary manifestations of antiphospholipid syndrome include pulmonary thromboembolism, pulmonary hypertension, acute respiratory distress syndrome, etc. Pulmonary thromboembolism is often the initial manifestation of antiphospholipid syndrome and a timely diagnosis is critical due to the high mortality rate. We herein report on a 19-year-old man with pulmonary thromboembolism that was caused by primary antiphospholipid syndrome. He presented with blood-tinged sputum, fever and epigastric pain, and his chest computerized tomography showed pulmonary thromboembolism. The other possible causes of pulmonary thromboembolism were excluded and the diagnosis of primary antiphospholipid syndrome was confirmed by the lupus anticoagulant that was present on two occasions six weeks apart. We also discuss the nature and management of antiphospholipid syndrome, along with a brief review of the relevant literatures.

• Journal of Genetic Medicine
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• v.7 no.1
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• pp.37-44
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• 2010

Purpose : Kabuki syndrome is a multiple congenital malformation syndrome with mental retardation. It was named after its characteristic appearance, a face resembling that of an actor in a Kabuki play. To date, six Korean cases of Kabuki syndrome have ever been reported. Here, we present the phenotypic and genetic characteristics of six patients with Kabuki syndrome. Materials and Methods : Between 2003 and 2009, six Korean girls have been diagnosed and followed up as Kabuki syndrome at Center for Genetic Diseases of Ajou University Hospital. Their clinical and laboratory data were collected and analyzed by the retrospective review of medical records. Results : All six patients showed the characteristic facial dysmorphism and developmental delay. Persistent fingertip pads were also found in all patients. Most patients showed postnatal growth retardation (83.3%) and hypotonia (83.3%). Opthalmologic problems were common, particularly for strabismus (83.3%). Congenital heart defects were present in three patients (50%). Skeletal abnormalities including 5th finger shortening (83.3%), clinodactyly (50%), joint hypermobility (50%) and hip dislocation (16.7%) were also observed. There was no patient who had positive family history for Kabuki syndrome. Cytogenetic and molecular cytogenetic analyses including karyotyping and array CGH could not reveal any underlying genetic cause of Kabuki syndrome. Conclusion : Korean patients with Kabuki syndrome showed a broad spectrum of clinical features affecting multiple organ systems. Although clinical manifestations of Kabuki syndrome have been well established, our results failed to detect recurrent chromosome aberrations which could cause Kabuki syndrome. Its natural history and genetic background remains to be further studied for providing appropriate management and genetic counseling.