• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.36 no.5
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• pp.434-437
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• 2010

A melanotic neuroectodermal tumor of infancy (MNTI) is a uncommon osteolytic pigmented neoplasm that primarily affects the jaws of newborn infants. Most patients (> 90%) present with the tumor in the first year of life. Approximately 65% form in the maxilla, 11% in the mandible, 5% in the brain and elsewhere. MNTI is normally benign, but up to 15% may recur and a few have metastasized. Approximately 200 cases of MNTI have been reported but only 2 of them presented as multifocal. A case of MNTI in a 7 month old boy was encountered. The chief complaint was maxillary anterior ridge swelling. The incisional biopsy findings were MNTI. Two months after the first operation, mild swelling of another site was observed. The infant was examined periodically since undergoing two procedures with no recurrence. This case demonstrates the possibility of a multicentric MNTI. We report a multicentric MNTI with a review of the relevant literature.

• Clinical and Experimental Reproductive Medicine
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• v.30 no.2
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• pp.179-184
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• 2003

The Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome refers to a condition which presents as normal female secondary sex characteristics, normal external genitalia, congenital absence of the internal vagina, usually a rudimentary uterus in the form of bilateral noncanaliculated muscular buds, and normal tubes and ovaries with normal cytogenetic and endocrine evaluation, frequent association of renal, skeletal and other congenital anomalies. However, rarely, whole uterus or a segment of uterus may be present, but lacking a conduit to the introitus. If a partial endometial cavity is present in a segment of uterus, cyclic abdominal pain may be a complaint and furthermore endometiosis can be developed. Recently, we experienced a case of MRKH syndrome with the segments of uterus accompanying endometriosis in young woman. We present this case with a brief review of literatures.

• Journal of Chest Surgery
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• v.25 no.6
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• pp.598-604
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• 1992

Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood and acounts for 6% to 15% of all cases of childhood cancer, Rhabdomyosarcoma in seventh most common form of childhood neoplasms, following acute leukemia, tumors of the central nervous system, lymphoma neuroblastoma, Wilm`s tumor, bone tumor. Rhabdomyosarcoma can arise anywhere in the body, but primary site in the thorax is relatively rare. We experienced a case of aveolar rhabdomyosarcoma arising from intercostal muscle, A 12 year-old woman was suffered from the intermittent left chest pain radiating to the scapular area and dyspnea, On physical examination, pulmonary friction rub was heard on the left upper lobe area. Qn adimission, the chest simple radiography revealed a 7 x 6, 5cm sized radio-opaque mass with pleural effusion in the superior mediastinum and the CT showed a well difined radio-opaque mass including the destructed 2nd rib and pleural effusion. The percutaneous tra-nsthoracic needle aspiration biopsy was likely to show blastoma. After the chemotherapy[vincristine, actinomycin-D, cyclophosphamde] was done to treat blastoma, the pleural effussion was subsided and the mass was slightly decreased by 4.5x 4. 5cm. For treatment and diagnosis, we performed en-bloc resection and the defected chest was reconstucted with Gortex patch. Grossly, the specimen was colored graysh-white and arised in between two ribs The microscopic findings showed that the tumor cells were small round with scant pinkish cytoplasm on the H-E stain and the tumor cell nests were grouped by reticulum fibers and showed alveolar pattern on the silver stain The electromicroscopic finding presented that the cytoplasm contained tangled fibrillar and flocculent materials. The histopathologic findings were compatable with laveolar rhabdomyosarcoma. She was discharged without any complication. After discharge, she has been treated with radiation theraphy and chemotheraphy, and not recurred untill last follow-up We report a case of alveolar rhabdomyosarcoma arising to intercostal muscle, developed in 12 year-old waman, with brief review of literatures.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.35 no.2
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• pp.130-135
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• 2013

Primary intraosseous squamous cell carcinoma (PIOSCC) is a rare form arising within the jaws. PIOSCC is not related to the oral mucosa, presumably developing from remnants of the odontogenic epithelium. Because odontogenic cyst epithelium often transforms malignantly into PIOSCC, it could be misdiagnosed as odontogenic cyst based on a relatively ill-defined radiolucent lesion. Therefore, definite diagnosis is established from histological examination of biopsy samples taken during cyst enucleation in many cases. The present study is reported with a case of patient complaining of discomfort on his mandible. He was diagnosed as a putative dentigerous cyst and underwent a cyst enucleation treatment. After definite diagnosis as PIOSCC was established based on histologic findings, partial mandible resection and mandible reconstruction were performed. Up to the present, 10 months follow up of the patient showed satisfactory healing without recurrence and abnormal findings; thereby, we are reporting this case with literature review.

• Journal of Korean Society of Occupational and Environmental Hygiene
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• v.27 no.4
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• pp.257-268
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• 2017

Objectives: This study was conducted to review the Toxic UseReduction Act of Massachusetts, USA, which has been evaluated as a successfulcase of a chemical reduction policy, and to search for ways to introduce it inKorea. Methods: We analyzed the implementation of the TURA by attending TUR Planning Course of the Toxic Use Reduction Institute in Massachusetts and researching the related literature. Results: As TURA took effect, the use of chemicals in Massachusetts was reduced, and cost savings were achieved in workplaces. The success factors for the legislation are considered to be support form the federal and state governments and the active participation of business and civic group. Domestic efforts to reduce toxic substances have already begun, so if the process of TURA is appropriately applied to domestic legislation of chemicals control, it would be expected to produce visible results. Therefore, we reviewed the 'Act on Chemicals Registration and Assessment', 'Act on Chemical Control' and 'Act on the Integrated Control of Pollutant-Discharging Facilities' and sought solution for applying TURA to each piece of legislation. For the first case, 'Toxic or Hazardous Substance List' and 'Establishment of Toxic Use Fee' is applicable. For the second case, 'Annual Toxic or Hazardous Substance Reports' is applicable. For the third case, 'Toxic Reduction Plans' and 'Toxics Use Reduction Institute and 'Toxic Use Reduction Planners' is applicable. Conclusions: The government should take notice appropriateness for the reduction of toxic chemicals and provide financial support. Businesses should invest in technologies that build trust with local communities, improve productivity, and reduce costs. Finally, civic group should cooperate with government and businesses.

• Journal of the Korean Arthroscopy Society
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• v.13 no.3
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• pp.264-267
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• 2009

Pigmented villonodular synovitis (PVNS) is a benign proliferative disorder of the synovium of joints. It occurs most commonly in the knee joint. The disease is composed of 2 different forms: diffuse and localized. The localized form is less frequent than the diffuse one. Most cases of localized PVNS involve the anterior compartment of the knee and can be usually easily diagnosed and treated with arthroscopy. We experienced a patient of localized PVNS developed on the posterior cruciate ligament, whose main symptom was persistent pain after trauma. This case occurs rarely and complete removal of the lesion was performed arthroscopically, using posterior trans-septal portal. We report this case with review of literatures.

• Journal of Genetic Medicine
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• v.18 no.2
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• pp.110-116
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• 2021

Microdeletions of chromosome 22q11.2 are one of the most common microdeletions occurring in humans, and is known to be associated with a wide range of highly variable features. These deletions occur within a cluster of low copy repeats (LCRs) in 22q11.2, referred to as LCR22 A-H. DiGeorge (DGS)/velocardiofacial syndrome is the most prevalent form of a 22q11.2 deletions, caused by mainly proximal deletions between LCR22 A and D. As deletions of distal portion to the DGS deleted regions has been extensively studied, the recurrent distal 22q11.2 microdeletions distinct from DGS has been suggested as several clinical entities according to the various in size and position of the deletions on LCRs. We report a case of long-term follow-up of a female diagnosed with a 22q11.2 distal deletion syndrome, identified a deletion of 1.9 Mb at 22q11.21q11.23 (chr22: 21,798,906-23,653,963) using single nucleotide polymorphism array. This region was categorized as distal deletion type of 22q11.2, involving LCR22 D-F. She was born as a preterm, low birth weight to healthy non-consanguineous Korean parents. She showed developmental delay, growth retardation, dysmorphic facial features, and mild skeletal deformities. The patient underwent a growth hormone administration due to growth impairment without catch-up growth. While a height gain was noted, she had become overweight and was subsequently diagnosed with pre-diabetes. Our case could help broaden the genetic and clinical spectrum of 22q11.2 distal deletions.

• Journal of the Korean Society of Radiology
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• v.85 no.2
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• pp.409-414
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• 2024

Hepatoid adenocarcinoma (HAC) is a rare form of adenocarcinoma that is diagnosed based on immuno-histochemical findings reminiscent of hepatocellular carcinoma (HCC). The clinical characteristics of HAC include increased levels of serum alpha-fetoprotein and a poor prognosis due to early liver metastasis. In particular, diagnosing liver metastasis of HAC can be challenging owing to radiological findings similar to those of HCC. Although HAC can occur in various organs, the stomach is the most common site. We present the case of a 64-year-old femalewho presented with multiple tumors in the liver. During subsequent examination, rectal cancer was identified and diagnosed as HAC through a biopsy. Herein, we report this case along with a literature review.

• Proceedings of KOSOMES biannual meeting
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• 2003.05a
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• pp.41-58
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• 2003

A recent trend of global shipping industry adopting Port State Control (PSC) system is recognized as a proficient mechanism in preventing costal traffic accident and protecting marine environment. Disadvantages of Korean shipping companies in PSC inspection are unavoidable as Tokyo MOU imposed priority listed flag to Korea. Having stated above, appropriate research and prompt preparation in compliance with current PSC requirements for the Korean shipping companies have become an immediate need. The objectives of this study are : First, to review in understanding of PSC in overall and foundation of enforcement. Second. to study interaction of international treaties regarding PSC. Third, to compare practical compliance among the countries and to open up a case study on Korean shipping companies in adopting PSC. Last, to suggest direction to the Korean shipping companies the most proficient way in compliance with the current Tokyo MOU requirement. Korean flagged vessels have become prioritized target in PSC inspection as Tokyo MOU imposed priority listed flag to Korea due to high detention rate of its fleet. Disadvantage of a priority listed flag is a burden in its proficient fleet operation. This study suggests solutions to the disadvantages as below. : 1) Change shore management system, and provide continuous & quality education to crew members 2) Form a network in sharing PSC information among the Korean shipping companies 3) Form a centralized function in which government, Korean Shipping register and shipowners can treat problems in a prompt manner.

• THE INTERNATIONAL COMMERCE & LAW REVIEW
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• v.51
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• pp.213-239
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• 2011

This article examines two important issues of the demand for payment by the beneficiary and the payment by the guarantor to the beneficiary under the revised Uniform Rules for Demand Guarantee (URDG) published by ICC, which are called URDG 758 and effected on July 1, 2010. Here, after first briefly defining the concept and nature of the demand for payment, this article discusses various issues surrounding the demand: By whom, where and how the demand has to be made; which documents are required in demanding the payment; how much amount can be demanded and paid; when and where the payment has to be made and which currency has to be used for the payment. The demand for payment has to be made by the beneficiary to the guarantor on or before expiry of the guarantee at the place of issuance of the guarantee unless any other place is specified in the guarantee. The demand has to be made in paper form unless the guarantee requires an electronic form. Unless otherwise expressly stipulated in the guarantee, the demand must be supported by a statement by the beneficiary indicating the applicant is in breach of the underlying contract. Also the demand must identify the guarantee under which it is made, and the time for examination by the guarantor starts on the date of identification. The demand cannot be for more than the amount available under the guarantee. When the demand is complying the guarantor must pay the amount demanded. The payment has to be made at the branch or office of the guarantor that issued the guarantee unless any other place is indicated in the guarantee. The payment has to be made in the currency specified in the guarantee, unless the guarantor is unable to make payment in that currency due to an impediment beyond its control or any illegality under the law of the place for payment. In case of "extend or pay" or "pay or extend" demands, the demand is deemed to be withdrawn if the extension is granted. But if not, the demand has to be paid without any further demand by the beneficiary.