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- Case Report A Case of Pseudopseudohypoparathyroidism with Partial Empty Sella.
- Jang Hyun Koh, Yeon Lee, Joung Wook Choi, Tae Won Hong, Mi Jin Kim, Young Goo Shin, Choon Hee Chung
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Endocrinology and Metabolism 2004;19(4):433-438
Published online: August 1, 2004
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2Department of Internal Medicine, Konkuk University College of Medicine, Seoul, Korea.
Abstract
Pseudohypoparathyroidism (PHP) is a rare endocrine syndrome, which is characterized by Albright's hereditary osteodystrophy (AHO). Pseudopseudohypoparathyroidism (PPHP) presents with AHO features, but does not show clinical symptoms or the same laboratory finding as hypoparathyroidism. A 54 aged woman visited our department with lower abdominal pain. She had a history of a total hysterectomy due to persistent uterine bleeding 30 years previously. She showed abnormal physical findings, including a round face, short stature and brachydactyly. Her hormonal data were within normal limits, as follows: PTH 40.72 pg/mL, calcium 8.6 mg/dL, phosphorus 4.0 mg/dL and 24 hour urine cAMP 3.2 nmol/mg. On a pituitary challenge test, the basal levels and response rates of FSH and LH were low, and a sella CT scan showed partially empty sella. Herein is reported a case of PPHP, with partial empty sella, with a review of the literature
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