Primary Empty Sella with Partial Hypopituitarism and Autoimmune Thyroiditis
Philippine Journals Online
View Archive Info| Field | Value | |
| Title |
Primary Empty Sella with Partial Hypopituitarism and Autoimmune Thyroiditis
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| Creator |
Karla Kristine Silverio-Fernando; Fellow, Section of Endocrinology, Diabetes and Metabolism, University of the Philippines-Philippine General Hospital
Frances Lina Lantion-Ang; Training Officer, Section of Endocrinology, Diabetes and Metabolism, University of the Philippines-Philippine General Hospital Cecilia A Jimeno; Consultant, Section of Endocrinology, Diabetes and Metabolism, University of the Philippines-Philippine General Hospital |
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| Subject |
Endocrinology
Hypopituitarism; autoimmune thyroiditis; primary empty sella; primary amenorrhea |
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| Description |
Clinical Presentation: A 21-year-old female presented with primary amenorrhea, delayed puberty, continuous linear growth, and torticollis. She noted impaired memory and frequent respiratory infection. Physical Findings: Patient presented with eunuchoidal habitus, torticollis, facial asymmetry, micrognathia, and hyperelastosis. Thyroid gland was normal in size. Breast and pubic hair were Tanner Stage 2. Neurologic exam was normal. Laboratory Work-up: LH and FSH were low with estradiol at pre-pubertal level, suggesting hypogonadotropic hypogonadism. Prolactin was slightly elevated. Twenty four-hour urinary free cortisol and 8AM serum cortisol were low with low normal plasma ACTH, indicating secondary adrenal insufficiency. TSH was elevated with depressed FT4 and elevated antithyroglobulin, suggesting autoimmune thyroiditis. The following tests were normal: growth hormone, FBS and 2-hour OGTT. Patient had delayed bone age by the Greulich-Pyle method. Bone densitometry revealed osteoporosis. Patient had 46XX karyotype. Pelvic ultrasound revealed infantile uterus. Sellar MRI showed empty sella. Diagnosis: The patient was diagnosed with Primary Empty Sella based on Cranial MRI findings of fluid-filled sella with no history of pituitary adenoma or surgery; with hypogonadotropic hypogonadism, secondary adrenal insufficiency, and autoimmune thyroiditis. Treatment/Outcome: Sex hormone replacement was started, with improvement in breast size and commencement of regular menstrual cycles. Levothyroxine was started, with normalization of the TSH. Prednisone was given at 5mg/day. Alendronate was added for osteoporosis. Significance: PES is rare, with 5.5% occurrence rate. Previous studies have reported preserved pituitary function in most cases. However, partial or total hypopituitarism has been described in 25% of patients, and hyperprolactinemia in 10%. The coexistence of autoimmune thyroiditis may support the possibility of an autoimmune mechanism behind the development of hypopituitarism in PES. Recommendations: Full endocrine panel should be done in patients presenting with primary empty sella to screen for hormone deficiencies. Presence of autoantibodies should be determined since this syndrome may be linked to autoimmunity. Keywords: Hypopituitarism; autoimmune thyroiditis; primary empty sella; primary amenorrhea DOI: 10.3860/pjim.v47i2.1647 Phil. J. Internal Medicine, 47: 83-87, March-April, 2009
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| Publisher |
The Philippine College of Physicians
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| Date |
2010-07-23
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| Type |
Peer-Reviewed Item
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| Format |
application/pdf
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| Identifier |
http://www.philjol.info/index.php/PJIM/article/view/1647
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| Source |
Philippine Journal of Internal Medicine; Vol 47, No 2 (2009); 83-87
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| Language |
en
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| Coverage |
Philippines
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