- Histomorphometry of Osteogenesis Imperfecta I.
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Seong Bin Hong, Suk Myun Ko, Yong Koo Park, Young Joo Park, Yoon Juo Oh, Young Wan Kim, Sung Ki Kim, Moon Suk Nam, Yong Seong Kim
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J Korean Endocr Soc. 2002;17(1):117-123. Published online February 1, 2002
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 Abstract
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- Osteoporosis imperfecta (OI) is a genetic disorder characterized by fragility of bone, deafness, blue sclerae; and laxity of joints. Four types of OI are distinguished by clinical findings. Although mutations affecting collagen I are responsible for the disease in the most patients, the mechanism by which the genetic defects cause abnormal bone development has not been well established. Therefore we evaluated static and dynamic bone histomorphometry of type I OI in the case study of a 15 year old boy with OI who had blue sclerae, a history of frequent fracture and a familial history of blue sclerae. Biopsy of the ilium showed loss of connection between the cortical bone and trabecular bones. The Harversian system in the cortical bone was poorly developed. In the trabecular bones, the lamellar pattern was poorly developed. Mineral apposition rate of the cortical bone was 1.0 m/day and of the trabecular bone was 0.79 m/day. Thus OI might be regard as a disease whereby abnormal collagen synthesis interferes with bone strength by multiple mechanisms.
- A Case of Normal Full Term Delivery after Afrenalectomy for Cushing's Syndrome in a Pregnant Patient.
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Sung Sik Yang, Yong Seong Kim, Yong Bum Cho, Young Wan Kim, Seong Bin Hong, Yeo Joo Kim, Mi Rim Kim, Moon Suk Nam, Sei Joong Kim, Suk Hwan Shin, Byoung Ick Lee
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J Korean Endocr Soc. 1999;14(3):562-567. Published online January 1, 2001
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Abstract
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- We experienced a case of Cushings syndrome due to adrenal adenoma associated with pregnancy 23th week in a 26 year old female patient. On physical findings, hypertension, moon face, hirsutism, abdominal striae and buffalo hump were noticed. Laboratory findings showed increased serum cortisol, increased 24 hours-urinary cortisol, and decreased plasma ACTH. Abdominal MRI demonstrated 4 cm sized in long diameter, well marginated, and ovoid-shape right adrenal mass. Unilateral total adrenalectomy was performed at the 23th week of pregnancy and the mass it was confirmed to benign adrenal adenoma. Steroid replacement therapy was continued with 7.5 mg prednisolone during remaining pregnancy. At the 38th week of pregnancy, labor was developed and she delivered healthy female weighing 2.5 kg through normal vaginal delivery without complication. Pregnancy rarely occurs in patients with Cushings syndrome. We think she was the first case in Korea who had normal 38th week transvaginal delivery after adrenalectomy in adrenal Cushings syndrome during pregnancy.
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