- A Korean Pedigree of Paget Bone Disease.
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Young Kee Shong, Joong Yeol Park, Ki Up Lee, Ghi Su Kim, Suhn Hee Kim, Jae Kun Cho, You Sook Cho, Hong Ja Kim, Myung Jin Shin
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J Korean Endocr Soc. 1995;10(4):451-455. Published online November 7, 2019
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Abstract
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- Paget bone disease(PBD) is usually focal, but can be wide spread disorder of the skeletal remodeling characterized by greatly increased osteoclast size and activity. It has extremely variable prevalence worldwide, being common in England and northern European countries and areas populated by their descendants, but strikingly uncommon in Asia, the middle east, Africa and Scandinavia. It's occurrence also shows familial clustering, some postulates autosomal dominant inheritance. Many studies have shown that paramyxoviruses may play a critical role in the etiology of this disorder. However, the precise etiology of PBD remains unknown.We describe a kindred with PBD in 3 successive generations. The propositus, a 55-year-old man, has panostotic PBD and giant cell reparative granuloma of pagets disease involving his head, mandible, abdomen and ileum, rare tumorous complication of Paget's disease. Bowed limbs were first noticed at age 25 years, and progressed for 20 years. Giant cell reparative granuloma began manifesting at age 45 years, and responded dramatically to high-dose dexamethasone therapy. His pretreatment biochemical finding were remarkable for elevated serum ALP, 765(normal 66-220 u/L) and osteocalcin, 154(normal 6.3-30.7 mg/ml), but normal serum calcium, phosphorous, 250HD and PTH. A nondecalcified iliac crest specimen demonstrated classic histopathologic 25OHD and PTH. A nondecalcified iliac crest specimen demonstrated classic histopathologic changes of PBD on light microscopy. His decreased father had a similar degree of bony deformities beginning at age 20 years, but had not been examined. His two asymptomatic daughters, 20 and 24-year-old, were both found to be affected with widespread PBD by bone scan, radiographic study, and their serum ALP levels, 939 and 435U/L, respectively. This is the first report of familial occurance of PBD and a case of giant cell reparative granuloma of Paget's disease in Korea, where PBD is very rare.
- A Case of Primary Thyroid Lymphoma Involving Bone Marrow (Stage IVE) Assoeiated with Hashimoto's Thyroiditis.
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Young Kee Shong, Joong Yeol Park, Ghi Su Kim, Jae Kun Cho, Yun Ho Chu, Wan Sik Eom, Sang Hee Kim, Hyun Sook Chi, Gyung Yub Gong, Ki Up Lee
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J Korean Endocr Soc. 1994;9(4):390-394. Published online November 6, 2019
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Abstract
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- Primary lymphoma of the thyroid is a relatively rare malignant tumor of the thyroid. It is known to be frequently associated with Hashimoto's thyroiditis. In Korea, a few cases of primary lymphoma of the thyroid have been reported and most of these cases were in the stage I E or II E. Recently, we experienced a case of the primary thyroid lymphoma, stage IV E associated with Hashimoto's thyroiditis in a 70-year-old woman who presented with dysphagia and dyspnea due to rapidly enlarging neck mass.She was treated with combination chemotherapy(cyclophosphamide, adriamycin, vincristine and methylprednisolone) and local radiotherapy and achieved partial response with resolution of dysphagia and dyspnea.
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