A Case of Pseudopseudohypoparathyroidism with Partial Empty Sella.

Koh, Jang Hyun; Lee, Yeon; Choi, Joung Wook; Hong, Tae Won; Kim, Mi Jin; Shin, Young Goo; Chung, Choon Hee

Case Report

Journal of Korean Endocrine Society 2004;19(4):433-438.
Published online August 1, 2004.

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

¹Department of Internal Medicine, Yonsei University Wonju College of Medicine, Wonju, Korea.
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

Key Words: Pseudopseudohypoparathyroidism, Empty sella