A Case of Pseudopseudohypoparathyroidism with Partial Empty Sella.
- Author:
Jang Hyun KOH
1
;
Yeon LEE
;
Joung Wook CHOI
;
Tae Won HONG
;
Mi Jin KIM
;
Young Goo SHIN
;
Choon Hee CHUNG
Author Information
1. Department of Internal Medicine, Yonsei University Wonju College of Medicine, Wonju, Korea.
- Publication Type:Case Report
- Keywords:
Pseudopseudohypoparathyroidism;
Empty sella
- MeSH:
Abdominal Pain;
Brachydactyly;
Calcium;
Female;
Humans;
Hypoparathyroidism;
Hysterectomy;
Phosphorus;
Pseudohypoparathyroidism;
Pseudopseudohypoparathyroidism*;
Tomography, X-Ray Computed;
Uterine Hemorrhage
- From:Journal of Korean Society of Endocrinology
2004;19(4):433-438
- CountryRepublic of Korea
- Language:Korean
-
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
