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In a boy suffering from adrenoleukodystrophy (ALD) the function of the adrenal cortex and pituitary gland was followed prospectively. At the onset of the disease an autonomic hyperfunction of the adre

Summary An instance of precocious puberty in a 7-year-old boy has been presented. After extensive study to exclude adrenal dysfunction, testicular tumor, and teratoma elsewhere in the body, a tumor of the third ventricle was found. The tumor was a teratoma and contained a secreting chorionepithelioma. Although it has been suggested that brain tumors may themselves secrete a hormone which influences sexual development, no proof of this has been found in the literature and we believe in this respect our case is unique. *

The clinical value of sweat electrolyte determinations is limited at present to the diagnosis of cystic fibrosis of the pancreas in children, but the function of the eccrine sweat gland in various systemic diseases has aroused considerable interest in recent years. Abnormalities of sweat electrolytes have been found in a number of systemic diseases. In some the alterations are explicable on the basis of adrenal dysfunction. Attempts have been made to discover an adult form of cystic fibrosis, but findings are inconclusive.

SummaryAdult rats fed the drug DDD rapidly develop signs of some adrenal dysfunction; decreased eosinophil response, decreased uric acid/creatinine ratio, increased insulin sensitivity and decreased response to stress of cold. The urinary and plasma Na and K are not affected.

The C-20 α and β epimers of Δ5-pregnene-3β,17,20-triol-7α-3H were injected intravenously in human subjects, and radioactive steroids in urine were isolated and measured. The results obtained were compared with previous studies of the fate of 3β, 17-dihydroxy-Δ5-pregnene-20-one. All 3 compounds were shown to be interconverted, and all 3, to varying extents, served as precursors for 5β-pregnane-3α,17,20α-triol and Δ5-pregnene-3β,17,20α-triol. The results obtained were compatible with the formation of 5β-pregnane-3α,17,20α-triol from 3β,17-dihydroxy-Δ5-pregnene-20-one both by initial reduction of the C-20 ketone (route A) and by initial oxidation of the 3β-hydroxyl group (route B). The findings contribute to an understanding of the precursors of pregneneand pregnanetriols in adrenal dysfunction and are in conformity with a previously reported peripheral locus of action of the drug, 2,2-bis (2-chlorophenyl, 4-chlorophenyl) 1,1-dichloroethane, trivial designation o,p′DDD.

A relatively simple technique is reported for the separation and measurement of individual Zimmermann-staining and blue tetrazolium-reducing steroids in adult and infant urine. When combined with a similar previously reported technique for the assay of 3β-hydroxy δ5-steroids and pregnanetriol, a fairly comprehensive profile of the neutral urinary steroids can be obtained. After enzyme hydrolysis and solvolysis, the steroids are extracted and separated on thin-layer silica-gel chromatograms. Assay is by direct reflectance densitometry of the coloured bands developed on the chromatograms by spraying with Zimmermann and blue tetrazolium reagents. One reasonably skilled technician can obtain a quantitative profile on 6 urines during one normal working week. For most clinical purposes visual assessment of the stained plates can replace quantitative scanning with considerable reduction of the work involved. Normal ranges for adults are given together with examples of assays on patients with various types of adrenal dysfunction.

DESOXYCORTICOSTERONE. ACETATE (D.C.A.) has been shown to be effective not only in maintaining the life of bilaterally adrenalectomized animals but as a protective agent when normal and adrenalectomized animals are subjected to excessive amounts of water by mouth. This phenomenon of water intoxication has been described by Rowntree (1926) and it is now generally agreed that the cause of convulsions and death resulting from large amounts of water is due to excessive hydration of tissue cells with marked depletion of plasma electrolytes. Failure of adrenalectomized animals to excrete water normally when large amounts of water are ingested has been associated with adrenal dysfunction (Rigler 1935; Swingle et al. 1937; Gaunt et al. 1937; Shipley 1945; Stein and Wertheimer 1944; Hays and Mathieson 1945) but the mechanism is still not clear.

Summary Evidence suggesting that the adrenal cortex is involved in the Stein-Leventhal syndrome is examined. These patients show abnormalities on fractionation of total 17-ketosteroids and pregnanetriolone has been detected in the urine of 16 consecutive cases. As this steroid has been previously detected only in patients with gross adrenal dysfunction it is felt that this provides additional support for the thesis that the adrenal cortex plays a role in the Stein-Leventhal syndrome.

Autoantibodies to the adrenal cortex (AA) were sought by indirect immunofluorescence using unfixed human adrenal tissue in 1675 patients with insulin-dependent diabetes (IDD), 2032 relatives of patients with IDD, and 2543 normal subjects. The frequencies of AA were significantly greater in patients with IDD (1.8%) and their relatives (1.4%) than in normal subjects (0.6%; P less than 0.013). Women more frequently had AA than men (P less than 0.011). There were no differences in the frequencies of AA between caucasoid and black individuals in all three patient groups. Patients with AA had higher frequencies of thyroid microsomal and gastric parietal cell autoantibodies than age-, sex-, and race-matched normal subjects (P less than 0.01). Adrenal function was studied in 30 asymptomatic patients (13 with AA, including 5 with IDD, and 17 subjects with no AA, including 8 with IDD). The mean plasma levels of ACTH at 0600 and 2000 h were significantly higher in those with AA than in matched subjects with no AA (P less than 0.01). The mean PRA levels (both recumbent and upright) were also significantly higher in those with AA than in subjects without AA (P less than 0.01). However, serum cortisol and aldosterone concentrations or 24-h urinary cortisol and aldosterone excretion were no different between the groups. These patients, therefore, appear to have compensated adrenal hypofunction, with the compensation maintained by increased ACTH and renin secretion. Whether these patients will remain in this compensated state of adrenal dysfunction or whether they will develop overt adrenal insufficiency requires longer follow-up.

This chapter focuses on the management of adrenal diseases in pregnancy. Data concerning adrenal disease in the nonpregnant patient are presented first as background for interpreting the available information concerning the effect of adrenal disease on the pregnant patient. Then the normal physiological changes of pregnancy are compared with the alterations induced by specific adrenal dysfunction. The effects of maternal disease and its treatment on the fetus are considered.

We have recently demonstrated that hypophysectomy enhances the potency of β-endorphin or morphine. This effect was not solely a result of adrenal dysfunction following extirpation of the pituitary (Holaday et al. 1977a). While conducting those studies, we observed unique behavioral effects of i.v.t. injections of β-endorphin that were never observed with equi-antinociceptive doses of morphine. Wet-dog shakes were seen within the first 5 min of drug injection, a phenomenon previously described by Bloom et al. (1976). When these wet-dog shakes ceased, we occasionally observed a clonic, seizure-like state accompanied by copious salivation. This effect was never observed in hypophysectomized rats (Holaday et al,1977b; Holaday et al. 1978a). Both wet-dog shakes and these ‘sialogic-seizures’ were potentiated by heat exposure.

The best way to detect Cushing's syndrome is to measure urinary free cortisol and to conduct a 1-mg dexamethasone suppression test. The best way to distinguish among the varieties of Cushing's syndrome is to measure plasma adrenocorticotropic hormone (ACTH). Urinary 17-ketosteroid and plasma testosterone determinations are the most useful tests for finding the cause of virilization. Short ACTH stimulation is the test of choice to detect adrenal insufficiency.

The availability of highly specific radioimmunoassays for measurement of practically every steroid synthesized by the adrenal cortex has greatly enhanced and simplified evaluation of adrenal function. The next section of this chapter focuses on the basal tests available to evaluate adequacy of adrenocortical function. These tests employ collection of a single sample of blood or urine. The assays in the plasma or urine available to the clinician and the limitations of these tests are pointed out. The following section of this chapter deals with dynamic tests that are employed in the evaluation of adrenal dysfunction.

The clinical signs and endocrinologic abnormalities that characterize horses with pituitary, thyroid, and adrenal dysfunction are described. Laboratory tests and hormonal assays useful for evaluating horses with suspected endocr-nopathies are discussed.

ELEVATED serum potassium levels usually result from deranged metabolic processes in renal or adrenal dysfunction whose detection often involves extensive laboratory investigation. Clinical and elec...

Summary Four patients with bronchopulmonary aspergillosis were treated with clotrimazole in a dose of 100 mg/kg/day. To assess the effect of treatment on the amount of fungus present in sputum, the numbers of colonies of Aspergillus fumigatus cultured from 24-hour specimens of sputum, collected before and at regular intervals during clotrimazole administration were recorded. Chest radiographs and tomographs were examined at fortnightly intervals and serum levels of clotrimazole were measured. One patient was treated with clotrimazole for 3 months, 2 for 2 months and in 1 treatment had to be withdrawn after a few days because of unwanted effects. There was no mycological or radiographic evidence of improvement in any patient. Serum levels of clotrimazole were found to be considerably lower than the minimum inhibitory concentration of the organisms in all patients treated, and serum levels estimated after a single dose of clotrimazole given to 4 patients not suffering from aspergillosis were also low. No evidence of haematological, liver, renal or adrenal dysfunction was observed.

Cushing's syndrome, primary hyperaldo-steronism, congenital adrenal hyperplasia and Addison's disease are adrenal dysfunctional diseases usually associated with abnormal blood pressure levels. Adrenal regeneration hypertension and desoxycorticosterone hypertension represent experimentally induced adrenal dysfunction states in which hypertension occurs. Renal-adrenal relationships may result in the production of hypertension and secondary hyperaldosteronism in some patients. Adrenalectomy and subdiaphragmatic sympathectomy performed in 171 severely hypertensive patients have resulted in an excellent or good blood pressure response in 82 per cent of 105 survivors observed from 3 to 11 years after treatment.

SUMMARY Pregn-5-ene-3α, 16α,20α-triol in amounts from 50 to 220 μg/24 h was found in the urine of normal adult men and women. Excretion within this range was also found in five adult women of whom two had congenital adrenal hyperplasia, one had Cushing's disease, one was hirsute and infertile and one was obese. Except for one patient, who excreted amounts within the normal range, subjects with adrenal carcinoma excreted up to 15 mg of this steroid/24 h.

THE increasing clinical investigative interest in adrenocortical function and awareness of therapeutic aspects of adrenal dysfunction have increased the need for specific methods of urinary corticoid assay which are adaptable to use in the hospital laboratory. Certain problems are involved in applying any of the published methods (1–7) to clinical investigations. In attempting to choose the most appropriate method for our institution, three important functions were deemed desirable: 1) the method should be conveniently applicable to a sufficiently large number of specimens; 2) it should detect the greatest possible number of the known adrenocortical steroid metabolites of the C19 and C21 series with only a minimum number of compounds to be analyzed per specimen; and 3) the method should have high chemical specificity. It is the purpose of this communication to describe a new method of analyzing urinary corticoids, which embodies the advantage of measuring in one step a pair of isomeric oxidation products ...

The SU test remains the best available in the assessment of the activity of the pituitary-adrenal axis. However, in the most usual procedures, using oral SU, it is an expensive test due to the 2 to 5 days hospitalization. We have evaluated a protocole using IV.SU in 25 normal infants and children (age 9m to 14.5 y) and 9 patients (9 to 18 y) with pituitary adrenal dysfunction at tested by the classical oral SU test. SU (1g/m2) was infused in 300 ml/m2 of saline for 2hours (NB : the infusion was painful with smaller saline diluent volume). Plasma cortisol (F), 11 deoxycortisol (S) and ACTH were determined by competitive protein binding assays and radioimmunoassay at 0,2,3 and 4h. The main hormonal changes were observed at 3 and 4h : F (μg/100ml) decrea sed from mean ± SD 15.3 ± 5.6 to 3.1 ± 1.8 ; S increased from mostly undetectable values (