IN HYPOPHOSPHATASIA (HPP), DEFICIENT ALP can ruin bones, bodies, and lives. Alexion Endocrinology, Diabetes & Metabolism Case Reports

ISSN 1470-3947 (print)
ISSN 1479-6848 (online)

Searchable abstracts of presentations at key conferences in endocrinology

Published by BioScientifica
Endocrine Abstracts (2010) 22 P555 

Negative magnetic resonance imaging in Cushing's disease

Milina Tancic-Gajic, Miomira Ivovic, Milos Stojanovic, Ljiljana Marina, Ana Nenezic, Zorana Arizanovic, Marija Barac & Svetlana Vujovic

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Introduction: Cushing’s syndrome (CS) results from inappropriate exposure to excessive glucocorticoids. Untreated, it has significant morbidity and mortality.

Case report: A 38-year-old woman with a typical appearance of CS was admitted for further evaluation of hypercortisolism. The serum cortisol level was elevated without diurnal rhythm, with no adequate supression of cortisol after 1 mg dexamethasone suppresion test. Twenty-four-hour urinary free cortisol level was elevated. Differential diagnostic testing was showed ACTH-dependent lesion (ACTH=17.4/95.6 ng/l), of the pituitary origin: suppression of cortisol level to 83% of baseline in 2 days 8 mg dexamethasone suppression test and a rise in the cortisol value of 23% above basal level along with a rise in ACTH value of 85% above basal level in CRH stimulation test. Pituitary abnormalities were not observed during repeated MRI scanning. Inferior petrosal sinus (PS) sampling was performed: i) baseline ratio ACTH PS/peripferal was <2; ii) CRH stimulated ratio ACTH PS/peripheral was <3; iii) baseline intersinus ratio of ACTH, was <1.4; iv) increase in central and peripheral ACTH of more than 50% above basal level after CRH; v) Baseline ratio ACTH v. jugularis inf./peripheral >1.7. Transsphenoidal exploration and removal of the tumor was performed with hypopithyitarism as the complication. Postoperativly morning serum cortisol levels are <50 nmol/l on adequate replacement therapy with hydrocortisone, thyroxine and estro-progesterone.

Conclusions: The Cushing’s disease remains a challenge for diagnose and clinical management.

No single test provides an absolute distinction, but the combined results of several tests generally provide a correct diagnose.

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