J Korean Soc Endocrinol.  2005 Feb;20(1):90-95. 10.3803/jkes.2005.20.1.90.

A Case of Protein-losing Enteropathy with an Abnormal Cortisol Response to ACTH Stimulation

• Kim HI
• Koo BK
• Lee YJ
• Lee EJ
• Kwak SH
• Cho SW
• Choi HJ
• Cho YM
• Kim SY

• KMID: 2100458
• DOI: http://doi.org/10.3803/jkes.2005.20.1.90

Abstract

We hereby report a case of a 62-year-old male patient who was misdiagnosed with adrenal insufficiency during the course of protein-losing enteropathy caused by superior mesenteric arterial thrombosis. The patient was suspected to have adrenal insufficiency due to hyponatremia and severe weakness. The cortisol responses to the initial challenge of 250microgram ACTH were inadequate (maximum serum cortisol level after ACTH challenge was 10.9microgram/dL), while the serum albumin concentration was 1.9g/dL. Subsequently, intravenous steroid therapy was given to the patient. However, after bowel resection, the serum albumin level increased to 3.4g/dL and the cortisol response to the follow-up rapid ACTH stimulation was completely normal. Accordingly, we discontinued steroid replacement and discharged the patient without any problem. In conclusion, measuring total serum cortisol in a patient with hypo-pro-teinemia may lead to misdiagnosis of adrenal insufficiency. In such cases, caution should be exercised in interpreting the results in terms of total serum cortisol level or measurement of serum free cortisol levels should be considered.

MeSH Terms

Adrenal Insufficiency
Adrenocorticotropic Hormone*
Diagnostic Errors
Follow-Up Studies
Humans
Hydrocortisone*
Hyponatremia
Male
Middle Aged
Protein-Losing Enteropathies*
Serum Albumin
Thrombosis
Adrenocorticotropic Hormone
Hydrocortisone
Serum Albumin

Figure

• 

  Fig. 1 Computed tomography of abdomen. Appearance of both adrenal glands was normal.

• 

  Fig. 2 Gross finding of resected bowel. Mucosal folds were lost in the small intestine 10~40 cm from proximal resection margin. Neither hemorrhage nor necrosis were found in the mucosal surface.

Reference

1. Wolfgang Oelkers. Adrenal Insufficiency. N Engl J Med. 1996. 335:1206–1212.

2. Hamrahian Amir H, Oseni Tawakalitu S, Arafah Baha M. Measurements of Serum Free Cortisol in Critically Ill Patients. N Engl J Med. 2004. 350:1629–1638.

3. Le Roux CW, Chapman GA, Kong WM, Dhillo WS, Jones J, Alaghband-Zadeh J. Free cortisol index is better than serum total cortisol in determining hypothalamic-pituitary-adrenal status in patients undergoing surgery. J Clin Endocrinol Metab. 2003. 88(5):2045–2048.

4. Dhillo WS, Kong WM, Le Roux CW, Alaghband-Zadeh J, Jones J, Carter G, Mendoza N, Meeran K, O'Shea D. Cortisol-binding globulin is important in the interpretation of dynamic tests of the hypothalamic--pituitary--adrenal axis. Eur J Endocrinol. 2002. 146(2):231–235.

5. Yamamoto M, Ariyoshi Y, Matsui N. The serum concentrations of unbound, transcortin bound and albumin bound cortisol in patients with dysproteinemia. Endocrinol Jpn. 1982. 29(5):639–646.

6. Wilke TJ, Utley DJ. Total testosterone, freeandrogen index, calculated free testosterone, and free testosterone by analog RIA compared in hirsute women and in otherwise-normal women with altered binding of sex-hormonebinding globulin. Clin Chem. 1987. 33(8):1372–1375.

7. Hashimoto T, Kawai K, Nishibu M, Fujita S, Horita H. Clinical evaluation of accuracy in determining serum free thyroxine and free triiodothyronine in patients with non-thyroidal illness: immunoglobulin effect on T3/TBG ratio and T4/TBG ratio. Endocrinol Jpn. 1991. 38(6):633–639.

8. Vogesera Michael, Briegelb Josef, Zachoval Reinhart. Dialyzable Free Cortisol after Stimulation with Synacthen. Clinical Biochemistry. 2002. 35:539–543.

9. Brennan Aoife, O'Connor Kieran A, Plant William D, O'Halloran Domhnaill J. Nephrotic syndrome: cause of an abnormal response to the rapid ACTH stimulation test. Nephrol Dial Transplant. 2004. 19:477–478.

10. Zietza Bettina, Locka Guntram, Placha Barbara, Drobnikb Wolfgang, Grossmanna Johannes, Schölmericha Jürgen. Dysfunction of the hypothalamic-pituitary-glandular axes and relation to ChildPugh classification in male patients with alcoholic and virus-related cirrhosis. Eur J Gastroenterol Hepatol. 2003. 15:495–501.

11. Reinard T, Jacobsen HJ. An inexpensive small volume equilibrium dialysis system for proteinligand binding assays. Anal Biochem. 1989. 176:157–160.

12. Clerico A, Del Chicca MG, Zucchelli G, Bartolomei C, Riccioni N. Free-cortisol assay by immunoextraction: comparison with an equilibrium dialysis procedure. Clin Chem. 1982. 28(6):1343–1345.

13. Annane Djillali, Sébille Véronique, Charpentier Claire, Bollaert Pierre-Edouard, François Bruno, Korach Jean-Michel, Capellier Gilles, Cohen Yves, Azoulay Elie, Troché Gilles, Chaumet-Riffaut Philippe, Bellissant Eric. Effect of Treatment With Low Doses of Hydrocortisone and Fludrocortisone on Mortality in Patients With Septic Shock. JAMA. 2002. 288:862–871.

14. Marik PE, Zaloga GP. Adrenal insufficiency during septic shock. Crit Care Med. 2003. 31:141–145.

15. Beishuizen A, Thijs LG. Relative adrenal failure in intensive care: an identifiable problem requiring treatment? Best Pract Res Clin Endocrinol Metab. 2001. 15(4):513–531.