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Primary hypothyroidism associated with secondary adrenocortical insufficiency.

A 50 year old man is described in whom primary hypothyroidism and isolated ACTH deficiency leading to adrenocortical insufficiency occurred simultaneously. Low thyroid hormone levels, elevated serum TSH values and high titers of antithyroid antibodies (ATA) were consistent with primary hypothyroidism. The diagnosis of secondary adrenocortical insufficiency was based on low cortisol and ACTH morning levels, low urinary steroids, a significant increase of cortisol levels after 0.25 mg tetracosactide iv, an absence of ACTH and cortisol release after insulin-induced hypoglycemia and a similar absence of ACTH and 11-deoxycortisol increase after metyrapone administration. After one year treatment and five days withdrawal of cortisol administration, the insulin-induced hypoglycemia still failed to elicit secretory responses of ACTH and cortisol, despite the normalization of thyroid hormone levels. The etiopathogenesis of these two coexisting endocrine deficiencies is discussed.

Adrenal Cortex Diseases↗

Possible adrenocortical insufficiency in postmature neonates.

Total plasma cortisol in cord and neonatal blood was measured by a radioassay method in neonates resulting from (1) term vaginal delivery following uncomplicated labor (control group), (2) term vaginal delivery following fetal distress during labor, (3) postterm, postmature vaginal delivery following fetal distress during labor, and (4) postterm, postmature emergency cesarean section performed because of signs of severe fetal distress during labor. Comparison of the mean peripheral plasma cortisol values showed that whereas the mean level (plus or minus S.E.) of the distressed term neonates (22.2 plus or minus 5.3 mug per 100 ml.) use 180 per cent of that of the control group (12.3 plus or minus 1.1 mug per 100 ml.; P smaller than 0.01) the mean level for the vaginally delivered postmature group (7.5 plus or minus 1.8 mug per 100 ml.) was only 61 per cent of that of the control group (P smaller than 0.05). Furthermore, in the postmature group with sufficient intrapartum distress to warrant emergency cesarean section the mean level (4.6 plus or minus 1.5 mug per 100 ml.) was found to be only 37 per cent of that of the control group. No differences were observed among the cord plasma cortisol values. These results are strongly suggestive of a relative adrenocortical insufficiency in postmature neonates. Such insufficiency could result from a defect in any portion of the adrenal-pituitary-hypothalamic axis. Effort is under way to further define such a defect.

Adrenal Cortex Diseases↗

Primary adrenocortical micronodular dysplasia: enzyme histochemical and ultrastructural studies of two cases with a review of the literature.

The adrenal glands from two patients with primary adrenocortical micronodular dysplasia (PAMD) were studied (no. 1, a 23-year-old man with cardiac myxomas and sarcoidosis; no. 2, a 16-year-old girl). The PAMD cells showed intense activity of 3 beta-hydroxysteroid dehydrogenase (3 beta DH), succinate DH, glucose-6-phosphate DH, alkaline phosphatase (AlPase), and other DHs and lysosomal hydrolases, giving a characteristic staining pattern. The staining patterns correlated well with ultrastructural findings. The larger adrenals (no. 1: 16.6 g) were associated with larger black nodules and internodular cortex (INC) with fairly well-retained enzyme activities, and contained cell clusters and single cells with intense AlPase activity with the appearance of PAMD nodules in the buds. The smaller adrenals (no. 2: 5.4 g) were associated with smaller black/yellow nodules, and had INC with weaker enzyme activity. Analysis of descriptions of INC in 25 previously reported cases revealed that the larger adrenals (more than 10 g) had less atrophic INC than the smaller ones. The characteristic enzyme pattern in PAMD cells explains the paradox that PAMD adrenals smaller than normal can cause Cushing's syndrome, and may be useful for investigating neoplastic and non-neoplastic counterparts in other adrenal lesions. The relationship between PAMD cells and INC is not simply one of autonomy versus atrophy, and both cell types may be stimulated by a certain common trophic factor. Thus, PAMD belongs to the category of hyperplasia.

3-Hydroxysteroid Dehydrogenases↗

The role of parmacologic manipulation in adrenal cortical scintigraphy.

Adrenal scintigraphy is a unique nuclear medicine procedure that provides noninvasive, functional information concerning the status of the adrenal cortex. The uptake of iodocholesterol, in addition to localizing adrenal cortical abnormalities, can be manipulate to focus on adrenal cortical dysfunction in either the aldosterone, cortisol, or androgenproducing portions of the adrenal cortex. The uptake of iodocholesterol correlates significantly with the abnormal secretion of cortisol in Cushing's syndrome, aldosterone in the model of adrenal zona glomerulosa function, and adrenal androgen secretion in hyperandorgenism. For these reasons, adrenal scintigraphy is an important diagnostic modality in the evaluation of adrenal cortical function.

Adrenal Cortex↗