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Letters to the Editor

Indian Pediatrics 1999; 36:416-417

Serum Control Following Replacement Therapy in Triple A Syndrome

We read with interest the recent communication on this subject(1). Triple A syndrome is characterized by selective defect in glucocorticoid production. Therefore, plasma cortisol is low and it does not rise after stimulation with ACTH. Plasma ACTH levels are elevated suggesting a primary unresponsive- ness of adrenal cortex to ACTH(2).

In the case described by the authors, low serum cortisol was consistent with the diagnosis of isolated glucocorticoid deficiency. However, normalization of serum cortisol following replacement with prednisolone as reported by the authors(1) is not explainable. One would expect serum cortisol to remain low or even become lower during prednisolone therapy because of adrenocorticoid suppression induced by exogenous prednisolone. In other words, because of feedback inhibition of ACTH production from pituitary by exogenous prednisolone, the production of endogenous cortisol which is already low despite maximum stimulation of adrenal cortex by ACTH, would fall further. Moreover, monitoring plasma cortisol levels is not areliable indicator of adequate glucocorticoid replacement therapy in adrenocorticoid hypofunction. In such cases adequacy of replacement therapy is 'better judged by clinical parameters(2,3).

Ratna Pori,
Jatinder S. Goraya,

Department of Pediatrics,
Government Medical College and Hospital,
32, Chandigarh 160047.



1. Shivananda, Premalatha R, Raheela, Gayathri P. Triple-A syndrome. Indian Pediatr 1998; 35: 1131-1135.

2. Forest MG. Adrenal steroid deficiency states. In: Clinical Pediatric Endocrinology, 3rd edn.

3. Ed Brooke CGD. London, Blackwell Science, 1995; pp 453-498.

4. Findling JW, Aron JC, Tyrrell JB. Glucocorticoids and adrenal androgens. In: Basic and Clinical Endocrinology,. 5th edn. Eds. Greenspan FS, Strewler GJ. London, Prentice- Hall International Inc, 1997; pp 317 -358.


We agree that therapy with prednisolone has no effect on serum cortisol level. The normal serum cortisol levels observed after the therapy could be due to fluctuating endogenous secretion. The patient is even now being monitored by clinical parameters. At present with 2.5 mg of alternate-day prednisolone therapy, she is free from symptoms.

Current analysis indicated ACTH level of 328 pg/ml (normal value is 0-37 pg/ml) and cortisol level of 30 Ilg/dl (normal-68-223 Ilg/ dl). This indicates end organ unresponsive- ness to ACTH, which is a feature of Triple-A syndrome.

Premalatha R.,
Department of Pediatrics,
Vanivilas Children's Hospital,
Bangalore, India.



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