Relative adrenal insufficiency in post-transplant lymphoproliferative disorder.

Post-transplant lymphoproliferative disorder is treated with rapid decrement of immunosuppressive therapy. This cannot be achieved with ease in patients on long-term glucocorticoid therapy, as chronically suppressed adrenal glands may not be capable of mounting adequate response to stress. A 52-year-old Caucasian male presented with fever, orthostatic hypotension, lymphadenopathy and hyponatraemia. Serum cortisol levels were within normal levels with a sub optimal response to stimulation by ACTH. Hyponatraemia and orthostasis responded poorly to fluid restriction, saline and salt repletion but corrected after increasing the steroid dose. The normal baseline cortisol levels represented a stimulated adrenal gland, however, the ACTH stimulation had inadequate response. This sub optimal stimulation and a good response to increased steroids suggest the presence of relative or occult adrenal insufficiency. Relative adrenal insufficiency must be considered in patients who have received prolonged glucocorticoid therapy and have symptoms such as hypotension and/or hyponatraemia.

P450scc deficiency (congenital lipoid adrenal hyperplasia): first reported case in Thailand and literature review.

A male infant presented with hyponatremia, hyperkalemia, generalized skin hyperpigmentation, and female type external genitalia. These clinical findings were compatible with mineralocorticoid, glucocorticoid and androgen insufficiency. Serum cortisol, progesterone and testosterone levels were extremely low after ACTH stimulation test, suggestive of defect in all of the adrenal steroidogenesis. Computed tomography demonstrated enlarged adrenal glands. The diagnosis of P450scc deficiency or lipoid congenital adrenal hyperplasia was based on all these characteristics. Physiologic replacement therapy with hydrocortisone and 9 alpha-fluorocortisol were effective and the patient achieved normal growth. The clinical characteristics, differential diagnoses, and prenatal diagnosis are discussed and reviewed.

Incidence of Nelson's syndrome and residual adrenocortical function in patients of Cushing's disease after bilateral adrenalectomy.

Eighteen patients of Cushing's Disease, who had undergone bilateral "total" adrenalectomy 2 to 10 years back, were evaluated for residual adrenocortical function and for any evidence of Nelson's Syndrome. Surprisingly, all patients were discovered to have measurable plasma cortisol, albeit in the subnormal range. The standard criteria for accepting "completeness of adrenalectomy" were fulfilled in 16 patients. It was possible to wean one patient off replacement therapy. Thus, the dose of replacement steroids needs to be regulated according to the blood steroid levels in order to avoid unnecessary hypercortisolism. Radiological evidence of pituitary tumor diagnosed Nelson's Syndrome in 2 patients. Nelson's Syndrome was further suspected in 8 others who showed pigmentation. One of these had an enlarged sella and an erosion of the dorsum sellae, but had a normal CT scan. Another patient had evidence of incidental pituitary pathology (incidentaloma) which resolved spontaneously. Contrast enhanced CT scans of the sella are necessary for early detection of Nelson's Syndrome.

Adreno-cortical reserve in pulmonary tuberculosis.

Serum cortisol in response to ACTH was assessed in 28 patients with moderately severe to far advanced pulmonary tuberculosis. Ten healthy individuals served as controls. Whereas the basal values were comparable in the two groups the mean delta peak, mean and area under the response curve were significantly lower in the tubercular patients (p < 0.05 to 0.001). Six patients had negligible cortisol response to ACTH stimulation while five others exhibited an inadequate response (rise between 200-300 nmol/L0. None of the subjects had any clinical evidence of adrenocortical insufficiency. The data thus revealed a functional impairment of adrenal cortical reserve in patients of pulmonary tuberculosis.