The Diagnostic Challenges in ACTH Dependent Cushing’s Disease
Journal Title: International Journal of Medical and Pharmaceutical Case Reports - Year 2015, Vol 3, Issue 1
Abstract
Objective: To discuss the difficulties in the diagnostic workup and management of ACTH dependent Cushing’s syndrome. Methods: We report the case of a patient with ACTH dependent Cushing’s syndrome, in whom the identification of the source of ACTH production was a challenge. Results: A 26-year-old man presented with a weight gain of 20kg, hypertension (250/160 mmHg), hypokalemia, purple stretch marks and severe proximal muscle weakness 6 months of evolution. He also had 4 episodes of pneumonia requiring hospitalization. ACTH-dependent hypercortisolism (cortisol 32ng/dl, ACTH 135pg/ml) was diagnosed. The severe clinical presentation suggested the possibility of ectopic ACTH production. The dexamethasone suppression test (DST) showed no inhibition of cortisol. Whole body octreotide scintigraphy and gallium-68 PET-CT did not show any evidence of ectopic tumor. A pituitary MRI was reported as normal. Bilateral inferior petrosal sinus sampling was carried out and was essential in establishing a diagnosis of Cushing’s disease. In a second MRI, a pituitary microadenoma on the left side was identified. The patient underwent transsphenoidal resection. Postoperatively, hypercortisolism persisted, and since the patients clinical condition deteriorated further, bilateral adrenalectomy was successfully performed, providing a definitive cure for the life threatening hypercortisolism. Conclusion: The diagnosis and management of ACTH dependent Cushing´s syndrome remains a challenge. The results of dexamethasone suppression tests should be evaluated in the context of the patient. These tests have a high negative predictive value; however in the context of critical illness (pneumonia) may alter the hypothalamic-pituitary-adrenal axis response. This case confirms the need for a bilateral IPS sampling in order to establish diagnosis.
Authors and Affiliations
Enedina T Cuatecontzi Xochitiotzi, Mariana Quintanar Martínez, Tomás Herrera Arzola, René E Lizola Crespo, Roopa Mehta
Attenuated Phosphorus and Sodium Sulfate in Diabetes Mediated Vasculitis Treatment: 3 Case Studies
Vasculitis, a combination of disorders which affects and disrupts the blood vessels due to inflammation and abnormal immune system, may be predisposed to increased glucose in blood circulation commonly observed in a skin...
Spiradenoma: A Case Report with Evidence Reinforcing the Thickened Nerve Fibers in the Tumor Capsule
Aim: Spiradenoma is a rare tumor. Spontaneous pain and tenderness on palpation is a frequent symptom of the disease. Although the cause of pain in spiradenoma is not clarified yet, thickened nerve fibers in the capsule o...
Amniotic Band Sequence: A Case Report
Background: Amniotic band sequence (ABS) is a rare condition in which parts of a fetus get entangled by the amniotic bands attached to the fetal surfaces and structures. This can lead to grotesque structural fetal anomal...
A Case Report of Community Acquired Pneumonia Due to Multi-drug Resistance Pseudomonas aeruginosa Treated with Elores
Community-acquired pneumonia (CAP) is a common and potentially serious illness associated with considerable morbidity and mortality, particularly in patients infected with Multidrug resistance (MDR) Gram-negative bacilli...
The Diagnostic Challenges in ACTH Dependent Cushing’s Disease
Objective: To discuss the difficulties in the diagnostic workup and management of ACTH dependent Cushing’s syndrome. Methods: We report the case of a patient with ACTH dependent Cushing’s syndrome, in whom the identific...