• Tuberculosis and Respiratory Diseases
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• v.44 no.4
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• pp.935-941
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• 1997

Mediastinal fibrosis is pathologically characterized by chronic inflammation and fibrosis of mediastinal soft tissue. Mediastinal fibrosis is local expression of a family of systemic fibrosing syndroms. This can result in compression of adjacent mediastinal structures. Idiopathic fibrosing syndromes include retroperitoneal fibrosis, sclerosing cholangitis of the orbit and fibrosis of the thyroid gland(Riedel's struma). The cause of these disorders is obscure, in some instance there is an underlying malignancy, infection, history of drug ingestion, or trauma with retroperitoneal bleeding. Treatment of mediastinal fibrosis depends on structures involved by the fibrotic process. The disease is self limited in most case or improved by steroids uses. We experienced a case of idopathic sclerosing mediastinitis with orbital fibrous dysplasia of unknowm cause, which was confirmed by open lung biopsy, so reported it with a review of literature.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.14 no.1
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• pp.41-44
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• 2018

McCune-Albright syndrome (MAS) is a rare disease characterized by fibrous dysplasia (FD), Cafe-au-lait spots, and endocrine disorder. A 4-year-old girl with MAS visited the clinic with a chief complaint of facial asymmetry and bruxism without any pain. Facial asymmetry and many dental problems such as midline deviation, "ground glass appearance" on the entire jaw, thinned cortical bone, loss of lamina dura and ectopic germs were found. Because of severely displaced tooth germs and FD affected jaw, there is a high possibility of malocclusion during mixed/permanent dentition. It is necessary to observe the eruption pattern periodically. If there are clinical symptoms like an abnormal eruption pattern, facial asymmetry or high caries susceptibility, appropriate interventions of dentist are required.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.27 no.5
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• pp.478-481
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• 2005

Ossifying fibroma is a rare, benign, fibro-osseous tumor of mesenchymal origin. It develops mainly in the mandible, where it is usually slow growing and asymptomatic, whereas in the maxilla and paranasal sinus, it is more aggressive. The vast majority are located in the posterior region of the mandible; however, ossifying fibroma involving the maxillary sinus is uncommon. It may reach a very large size and cause facial asymmetry. It shows a female predilection, and most cases are seen in the third and fourth decades of life. Radiographically, ossifying fibroma is radiolucent or radiopaque depending on the amount of calcification. Histologically, the tumor consists of a cementum-like or bony mass. Ossifying fibroma shares many histopathologic features with fibrous dysplasia. Their radiographic features may help to separate these entities when pathological differentiation is uncertain. It is important to differentiate ossifying fibroma from fibrous dysplasia. The treatment of ossifying fibroma involves its complete removal using curettage, enucleation, excision, or en bloc resection. This case report presents an unusual case of ossifying fibroma of the maxillary sinus. We describe a Caldwell-Luc operation with a lateral window approach for excision of an ossifying fibroma of the maxillary sinus.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.18 no.1
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• pp.61-68
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• 1996

In Fibrous dysplasia(FD) of the jaws, the majority of cases can await the cessation of growth before surgical intervention, and it seems prudent to delay surgery whenever possible until growth has ceased. In craniofacial FD, however, the dangers of dystopia, dystopia and loss of vision may require early surgery to prevent or control cranio-orbital complications. Delaying surgery in those circumstances may be significantly detrimental to such patients. Conservative surgical management of FD is widely practised and we advocate an extension to this conservative treatment by combining surgical recontouring with appropriate osteotomies if indicated, to achieve an optimal esthetic and functional results in craniofacial FD. One case will be presented to illustrate the feasiblility of such combined treatment, to report the uneventful healing of osteotomies in the FD of the jaws, and to demonstrate the use of titanium miniplate fixation in dysplastic bone. The other case had expansile disease of the left facial and fronto-temporal bones and osteolytic change left mandible. This patient complained of severe spontaneous bleeding of left mandibular premolar area and it was suspected as central hemangioma of the left mandible and craniofacial FD. Angiogram disclosed generalized dilation of the external carotid artery and its branches, especially terminal branches of the left facial and inferior alveolar arteries. But no specific abnormalities, such as A-V shunt, venous lake, or early venous drainage, was seen. So it was diagnosed craniofacial FD with hypercellularity and generalized bony recontouring was performed via coronal and transoral approaches.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.13 no.2
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• pp.120-125
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• 2013

Purpose: McCune-Albright syndrome (MAS) is caused by activating mutations in the GNAS gene, resulting in peripheral precocious puberty, caf$\acute{e}$-au-lait spots, and polyostotic fibrous dysplasia (POFD). The aim of the present study was to describe the diverse clinical and endocrine characteristics of patients with MAS. Methods: Seven patients with MAS were included in this study and medical charts were reviewed retrospectively for following parameters: patient's sex and age at diagnosis, POFD, ovarian cysts, and precocious puberty. Results: The mean age at diagnosis was $5.8{\pm}4.2$ years. One patient was male (14%) and the other six patients were female (86%). Peripheral precocious puberty was associated with 6 patients (86%). Five patients manifested premature menarche as early as 2 to 5 years of age. Letrozole was administered to 4 patients, tamoxifen to one patient and GnRH agonist to one patient. Five females developed ovarian cysts. Thyroid function tests were performed in all patients and one patient showed hyperthyroidism (14%) and has been treated with methimazole. One patient presented with pseudohypoparathyroisdism, phosphaturia, calciuria suggesting hypophosphatemic rickets. Six patients (86%) revealed POFD. One patient had symptoms of optic nerve compression and secondary esotropia and 2 patients had bone pain. Conclusion: This study described clinical characteristics and endocrine complications of patients with MAS. Careful physical examinations with history taking and serial endocrine function tests should be needed to detect complications such as endocrinologic hyperfunction and POFD.

• The Journal of the Korean bone and joint tumor society
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• v.13 no.1
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• pp.22-30
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• 2007

Purpose: We describe clinical, radiographic, MRI and pathologic findings as well as final outcome after simple curettage and bone graft of cystic fibrous dysplasia (FD) in the long bone, which has been rarely documented in the literature. Materials and Methods: Clinical records, radiographs, MRI and histologic slides of 11 patients with cystic FD in the long bone were retrospectively analyzed. Results: Six patients complained pain for several months, 4 patients presented pain after trivial injury event, and 1 patient suffered pathologic fracture. The mode of involvement was monostotic in 10 patients and polyostotic in l patient. The femur was affected in 7 patients, the humerus in 3, and the radius in 1. Radiography showed prominent, expansive lysis associated with ground-glass density of FD. MRI revealed 2 different signals of FD and cyst. Microscopic examination revealed classic findings of FD and non-specific cystic degeneration. The final outcome was satisfactory in every patient. Local recurrence was not observed. Conclusion: Cystic FD in the long bone seems not as rare as the scarcity of reported cases would indicate. MRI features provide a basis for differential diagnosis between benign cystic change and malignant transformation. Cystic FD would be an indication for surgery and simple curettage with allo-chip-bone graft is effective.

• The Korean Journal of Nuclear Medicine
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• v.31 no.4
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• pp.452-458
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• 1997

To evaluate the pinhole scintigraphic findings and its significance, authors retrospectively compared the pinhole bone scintigrams and corresponding radiograms of 16 lesions in 14 patients with fibrous dysplasia. They were diagnosed pathologically in 10 lesions and radiologically in 6 lesions. The mean age of patients was 41.1 years. The mean interval between two studies was 1.1 days. Locations were ribs 7, pelvic bone 4, clavicle 1, long bones 4(femur 2, tibia 1, humerus 1). The radiographic findings were as follows : the central portions were radiolucent(n=9), ground-glass opacities(n=5) or sclerotic(n=2) and the peripheral appearance were sclerotic rim(n=5), septation(n=7), cortical perforation (n=10) and invisible cortical thinning(n=9). Pinhole scintigraphic findings were as follows; Central portions showed normal 1+ uptake in 6 cases(radiolucent 5, ground-glass opacity 1), slightly increased 2+ upta- ke in 7 cases(radiolucent 4, ground-glass opacity 3), and marked 3+ uptake in 3 cases(ground-glass opacity 1, sclerotic 2). The 15 of 16 lesions showed more intense uptake in the peripheral portion: slightly increased 2+ uptake corresponding to the sclerotic rim(5/5) and unvisible cortical thinning(1/9), and irregular foci of marked 3 + uptake corresponding to septation(7/7), cortical perforation(10/10) and invisible cortical thinning (8/9). One of 16 lesions showed homogeneous 2+ uptake. In conclusion, pinhole scintigram provides information on regional activity of the fibrous dysplasia, which would be helpful in diagnosis, prediction of prognosis and determination of treatment plan.

• Journal of the Korean Orthopaedic Association
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• v.54 no.6
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• pp.519-527
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• 2019

Purpose: To evaluate the treatment result in polyostotic fibrous dysplasia classified according to the involvement of the femoral head. Materials and Methods: Twenty-three patients from March 1987 to March 2014 were reviewed retrospectively. Patients with no involvement of the physeal scar in the femoral head were classified as Type I, and those with involvement of the physeal scar were classified as Type II. A plain radiograph was used to measure the femoral neck shaft angle, articulo-trochanteric distance (ATD), and anterior bowing through the lateral view. A teleoroentgenogram of the lower limb was used to measure the leg length discrepancy and lower extremity mechanical axis. The pre- and postoperative femoral neck-shaft angle and ATD were compared to assess the degree of correction of the deformity. Results: Among a total of 46 cases (23 patients), 28 cases (23 patients) had lesions in the proximal femur. Type I were 16/28 cases (15/23 patients) and Type II were 12/28 cases (9/23 patients). The preoperative proximal femoral neck-shaft angle was 116.8° in Type I and 95.3° in Type II. The ATD was 12.08 mm in Type I and -5.54 mm in Type II. The deformity correction showed significant improvement immediately after surgery, the deformity correction was lost in Type II (neck shaft angle Type I: 133.8°-130.8°, Type II: 128.6°-116.9°, and ATD Type I: 17.66-15.72 mm, Type II: 7.44-4.16 mm). The extent of anterior bowing was 12.74° in Type I and 20.19° in Type II. The mean differences of 12 mm between the 9 patients who showed a leg length discrepancy and the lower extremity mechanical axis showed 4 cases of lateral deviation and 7 cases of medial deviation. Conclusion: In polyostotic fibrous dysplasia, when the femur head is involved, the femur neck shaft angle, ATD, and anterior bowing of the femur had more deformity, and the postoperative correction of deformity was lost, suggesting that the involvement of the femoral head was an important factor in the prognosis of the disease.

• Journal of Chest Surgery
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• v.13 no.4
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• pp.486-489
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• 1980

16 cases of the chest wall tumors that had been treated at the dept. of thoracic & cardiovascular surgery, Chungnam National University Hospital, for 3.5 years from Jan. 1977 to Jun. 1980 were analyzed. The results were as follows; 1. Generally the chest wall tumors were most frequent in the thirties, the youngest age was 2 years, and the oldest 65 years. The incidence rate of male to female was 1.3:1. The malignant tumors were common in the fifties & sixties, the incidence rate of male to female 5:1. 2. The common disease entities were rib tuberculosis [43.7%] and metastatic tumor [25.0%], and the another chondrosarcoma, osteosarcoma, fibrous dysplasia, chronic osteomyelitis, and granuloma accompanying with acute osteomyelitis by Klebsiella infection were 6.3%, respectively. 3. The common manifestations were local swelling [100.0%] and local chest pain [43.8%].

• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.22 no.2
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• pp.195-201
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• 1992

The authors evaluated the distribution of the antral pathologic conditions and their radiographic features on the Waters' radiolgraphs of 151 children patients who had been radiographed at the department of Oral Radiology, Seoul National University Hospitals. The obtained results were as follows: 1. The most common pathologic condition was inflammatory change(58%). Percentage of cases showing cystic lesion and fibro-osseous lesion were 25% and 11% respectively. 2. In cases of inflammatory change, odontogenic origins were 12 cases(13%) and bilateral occurrences were 37 cases(39%). 3. The most common radiographic feature of the inflammatory conditions was various types of mucosal thickenkng(78%).Percentage of cases showing totally increased radiopacity was 18%. 4. Intrinsic cystic lesions were 26 cases(65%) and dentigerous cyst was the most common extrinsic cyst. 5. Most of the fibro-osseous lesions(15 from 17 cases) were fibrous dysplasia. 6. Maxillary sinus hypoplasia(5 cases) and Burkitt's lyphoma(4 cases) were also observed.