5β-PREGNAN-3α-OL-20-ONE IN URINE

1962 ◽  
Vol 41 (2) ◽  
pp. 247-258 ◽  
Author(s):  
H. J. van der Molen ◽  
U. W. Kleef

ABSTRACT The occurrence, metabolic formation and degradation of 5β-pregnan-3α-ol-20-one are reviewed. Estimation of 5β-pregnan-3α-ol-20-one in urine has shown that: under acid conditions 5β-pregnane-3α,17α,20α-triol is destroyed with partial formation of pregnanolone; however, the amounts of pregnanolone that are formed, do not interfere with the estimation of pregnanolone in urine of normal adults after acid hydrolysis and offer no explanation for the high levels of pregnanolone in urine of patients with adrenal hyperplasia, as observed by some investigators. Excretion of pregnanolone found in urine during pregnancy (1–15 mg/day) is considerably below the levels reported in the literature so far. Normal adults excrete pregnanolone in urine in amounts within a range of 0.05–0.70 mg/day. There is a positive rank-correlation between the excretion of pregnanolone and pregnanediol in urine of normal males and females and of pregnant females. After administration of ACTH to adults an increase in the excretion of pregnanolone in urine could not always be demonstrated.

1964 ◽  
Vol 45 (1) ◽  
pp. 55-67 ◽  
Author(s):  
Beryl M. A. Davies

ABSTRACT An oxycellulose adsorption-HC1 elution procedure has been used to concentrate corticotrophin (ACTH) from about 1000 ml normal pooled human blood or 500 ml blood from patients with Cushing's syndrome. The concentrates were bioassayed by measuring their adrenal ascorbic acid depleting action in cortisol-treated rats. Satisfactory recovery figures were obtained for the concentration of 2nd International Standard for ACTH added to out-of-date blood-bank blood or to blood from a hypophysectomized patient. Normal blood ACTH levels are reported from 8 estimations each on pooled blood from 2–3 individuals. Mean normal ACTH was 0.7 mU per 100 ml blood (range 0.60–1.2 mU). Eleven patients with Cushing's syndrome were investigated. These included non-treated cases with adrenal hyperplasia and post-adrenalectomy cases with recurrence of Cushingoid symptoms in the presence of a pituitary tumour. In both groups, mean blood ACTH values were three to four times normal.


1968 ◽  
Vol 58 (4) ◽  
pp. 600-612 ◽  
Author(s):  
Robert Boyd ◽  
Donald C. Johnson

ABSTRACT The effects of various doses of testosterone propionate (TP) upon the release of luteinizing hormone (LH or ICSH) from the hypophysis of a gonadectomized male or female rat were compared. Prostate weight in hypophysectomized male parabiotic partners was used to evaluate the quantity of circulating LH. Hypophyseal LH was measured by the ovarian ascorbic acid depletion method. Males castrated when 45 days old secreted significantly more LH and had three times the amount of pituitary LH as ovariectomized females. Administration of 25 μg TP daily reduced the amount of LH in the plasma, and increased the amount in the pituitary gland, in both sexes. Treatment with 50 μg caused a further reduction in plasma LH in males, but not in females, while pituitary levels in both were equal to that of their respective controls. LH fell to the same low level in partners of males or females receiving 100 μg TP. When gonadectomized at 39 days, males and females had the same amount of plasma LH, but males had more stored hormone. Pituitary levels were unchanged from controls following treatment with 12.5, 25 or 50 μg TP daily, but plasma values dropped an equal amount in both sexes with the latter two doses. Androgenized males or females, gonadectomized when 39 days old, were very sensitive to the effects of TP and plasma LH was significantly reduced with 12.5 μg daily. Pituitary LH in androgenized males was higher than that of normal males but was reduced to normal by small amounts of TP. The amount of stored LH in androgenized females was not different from that of normal females and it was unchanged by any dose of TP tested. Results are consistent with the conclusion that the male hypothalamic-hypophyseal axis is at least as sensitive as the female axis to the negative feedback effects of TP. Androgenization increases the sensitivity to TP in both males and females.


1960 ◽  
Vol XXXIII (II) ◽  
pp. 230-250 ◽  
Author(s):  
Eileen E. Hill

ABSTRACT A method for the fractionation of the urinary 17-ketogenic steroids with no oxygen grouping at C11 and those oxygenated at C11, is applied to the clinical problems of congenital adrenal hyperplasia. In normal children the mean ratio of the non-oxygenated to oxygenated steroids is 0.24. In childrern with congenital adrenal hyperplasia the ratio is 2.3. The reason for this difference in ratio is discussed. The changes in ratio found under stimulation of the adrenal gland with exogenous or endogenous corticotrophin and the suppression with cortisone therapy are studied. This test can be applied to isolated samples of urine, a major advantage in paediatric practice, and can be carried out in routine laboratories. It is found to be reliable in the diagnosis and sensitive in the control of congenital adrenal hyperplasia.


1960 ◽  
Vol XXXIII (III) ◽  
pp. 388-400 ◽  
Author(s):  
L. G. Huis in 't Veld ◽  
B. Louwerens ◽  
P. A. F. van der Spek

ABSTRACT In two male patients and two castrated males, the influence of corticotrophin (ACTH) on the urinary excretion of neutral 17-ketosteroids and 17-hydroxycorticosteroids was determined before and during a period in which patients were treated with 5 mg 17α-methyl-19-nortestosterone (MNT) daily. In two castrated males, moreover, the influence of chorionic gonadotrophin and ACTH + chorionic gonadotrophin on the urinary excretion of 17-ketosteroids and 17-hydroxycorticosteroids was determined before and during a period of treatment with 5 mg MNT daily. Prolonged administration of MNT causes a decrease in the urinary excretion of neutral 17-ketosteroids and 17-hydroxycorticosteroids both in the normal males and in the male castrates. ACTH caused an increase in the urinary excretion of 17-ketosteroids and 17-hydroxycorticosteroids before and during MNT administration. During MNT administration this increase (expressed in mg/24 hours) was ≤ the increase produced by the same dose of ACTH prior to MNT administration. In two male castrates treated with MNT, chorionic gonadotrophin caused no increase in the urinary excretion of 17-ketosteroids and 17-hydroxycorticosteroids. The effect obtained before and during MNT administration by administration of ACTH + chorionic gonadotrophin did not exceed the effect obtained by the same dose of ACTH alone. Our conclusion is that the effect of MNT on the excretion of adrenocortical steroids is not due to the inhibition of the ACTH secretion. The possibility of a direct effect of MNT on the adrenal cortex has not been excluded with complete certainty. A change in the corticosteroid metabolism due to the influence of MNT, however, must also be taken into consideration.


2019 ◽  
Vol 70 (4) ◽  
pp. 211-216
Author(s):  
Jim Pateman ◽  
Peter Russell

Two Euphydryas aurinea beckeri females from Sierra Blanca, Malaga, Spain were captured and returned to the U.K.; each laid a single egg batch on Succisa pratensis. Both batches hatched and the resulting larvae were reared through to adulthood. One group of larvae produced butterflies with three different mutations: pupae with spines, legs lacking distal tarsals and claws, and with appendages attached to the genitalia which protruded though the rear of the abdomens in both males and females. The other group of larvae produced normal males and females.


Author(s):  
Sophie Comte-Perret ◽  
Anne Zanchi ◽  
Fulgencio Gomez

Summary Medical therapy for Cushing's syndrome due to bilateral macronodular adrenal hyperplasia (BMAH) is generally administered for a limited time before surgery. Aberrant receptors antagonists show inconsistent efficacy in the long run to prevent adrenalectomy. We present a patient with BMAH, treated for 10 years with low doses of ketoconazole to control cortisol secretion. A 48-year-old woman presented with headaches and hypertension. Investigations showed the following: no clinical signs of Cushing's syndrome; enlarged lobulated adrenals; normal creatinine, potassium, and aldosterone; normal urinary aldosterone and metanephrines; elevated urinary free cortisol and steroid metabolites; and suppressed plasma renin activity and ACTH. A screening protocol for aberrant adrenal receptors failed to show any illegitimate hormone dependence. Ketoconazole caused rapid normalisation of cortisol and ACTH that persists over 10 years on treatment, while adrenals show no change in shape or size. Ketoconazole decreases cortisol in patients with Cushing's syndrome, and may prevent adrenal overgrowth. Steroid secretion in BMAH is inefficient as compared with normal adrenals or secreting tumours and can be controlled with low, well-tolerated doses of ketoconazole, as an alternative to surgery. Learning points Enlarged, macronodular adrenals are often incidentally found during the investigation of hypertension in patients harboring BMAH. Although laboratory findings include low ACTH and elevated cortisol, the majority of patients do not display cushingoid features. Bilateral adrenalectomy, followed by life-long steroid replacement, is the usual treatment of this benign condition, and alternative medical therapy is sought. Therapy based on aberrant adrenal receptors gives disappointing results, and inhibitors of steroidogenesis are not always well tolerated. However, ketoconazole at low, well-tolerated doses appeared appropriate to control adrenal steroid secretion indefinitely, while preventing adrenal overgrowth. This treatment probably constitutes the most convenient long-term alternative to surgery.


Author(s):  
Geetanjali Kale ◽  
Elaine M Pelley ◽  
Dawn Belt Davis

Summary Myelolipomas have been reported in patients with congenital adrenal hyperplasia (CAH). ACTH excess, as seen with non-adherence to glucocorticoid therapy, may be responsible for tumor development. We report a case of a 51-year-old man with classic salt-wasting CAH managed on prednisone 7.5 mg daily and fludrocortisone who presented with chronic back pain and was found to have giant bilateral retroperitoneal masses. On computed tomography (CT) imaging, the masses were heterogeneous, but contained predominantly low-density fat attenuation. The tumors were resected due to concern for malignancy and mass symptoms. Pathologic examination identified both retroperitoneal masses as myelolipomas. The left tumor was 34×20×13 cm and weighed 4.7 kg and the right tumor was 20 cm in the largest dimension. Adrenal tissue was present in the specimen. The patient reported long-term compliance with glucocorticoid treatment. However, no biochemical monitoring of ACTH levels had occurred. Therefore, it is unclear if ACTH excess contributed to the development of these large tumors in this patient. It was presumed that both adrenal glands were inadvertently removed during surgery and the patient was treated with physiologic replacement doses of hydrocortisone and fludrocortisone postoperatively. In this case, the bilateral adrenalectomy was inadvertent. However, adrenalectomy can be considered as a treatment option in patients with classical CAH under certain circumstances to avoid complications of glucocorticoid excess. Learning points Myelolipomas should be considered in the differential diagnosis of adrenal or retroperitoneal masses in patients with CAH. On CT imaging, myelolipomas are seen as heterogeneous masses with low-density mature fat interspersed with more dense myeloid tissue. Myelolipomas are usually unilateral and measure <4 cm; however, very large and bilateral tumors have been reported. Treatment of CAH typically involves using supraphysiologic doses of glucocorticoid to suppress adrenal hyperandrogenism. Bilateral adrenalectomy is an alternative treatment option in patients with CAH. There is an association between ACTH excess and increased incidence of adrenal myelolipoma but the direct causal link remains to be established.


Author(s):  
Tina Kienitz ◽  
Jörg Schwander ◽  
Ulrich Bogner ◽  
Michael Schwabe ◽  
Thomas Steinmüller ◽  
...  

Summary Apart from adrenal myelolipomas, adrenal lipomatous tumors are rare and only seldom described in the literature. We present the case of a 50-year-old man, with a classical form of congenital adrenal hyperplasia (CAH), which was well treated with prednisolone and fludrocortisone. The patient presented with pollakisuria and shortness of breath while bending over. On MRI, fat-equivalent masses were found in the abdomen (14 × 19 × 11 cm on the right side and 10 × 11 × 6 cm on the left side). The right adrenal mass was resected during open laparotomy and the pathohistological examination revealed the diagnosis of an adrenal lipoma. Symptoms were subdued totally postoperatively. This is the first report of a bilateral adrenal lipoma in a patient with CAH that we are aware of. Learning points Macronodular hyperplasia is common in patients with congenital adrenal hyperplasia (CAH). Solitary adrenal tumors appear in approximately 10% of adult CAH patients and are often benign myelolipomas. The Endocrine Society Clinical Practice Guideline does not recommend routine adrenal imaging in adult CAH patients. Adrenal imaging should be performed in CAH patients with clinical signs for an adrenal or abdominal mass. Adrenal lipoma is rare and histopathological examinations should rule out a differentiated liposarcoma.


1958 ◽  
Vol 36 (1) ◽  
pp. 869-881 ◽  
Author(s):  
Wojciech Nowaczynski ◽  
Thomas Sandor ◽  
Erich Koiw ◽  
R. Norman Jones ◽  
Jacques Genest

The isolation and the partial chemical and physicochemical characterization of a chloroform-soluble substance obtained from human urine and citrous fruit juices are presented. The urinary compound, designated as compound III, is present mostly as a conjugate hydrolyzed by animal β-glucuronidase whereas it is in free form in fruit juices. It is present in the urine of normal males and females and in a number of pathological conditions. Its urinary excretion is significantly increased as a result of diets rich in potassium (250 meq./day). In various paper chromatographic systems, the compound has a mobility similar to that of aldosterone. Detailed study of its chemical and physicochemical characteristics, including oxidative degradation and infrared and elementary analysis, suggests a steroidal or sesquiterpinoidal structure. However, at the present time, the data obtained do not permit the final elucidation of the chemical formula of compound III.


2019 ◽  
Vol 7 (1) ◽  
Author(s):  
T R Robeck ◽  
R S Amaral ◽  
V M F da Silva ◽  
A R Martin ◽  
G A Montano ◽  
...  

AbstractThis study was conducted to characterize immunoreactive thyroid hormone concentrations in wild Amazon river dolphins, also called boto (Inia geoffrensis) by age group, sex, pregnancy and lactation status, and to determine if thyroid hormone concentration differences could be detected between pregnant females with and without successful parturition outcomes. Radioimmunoassays were used to analyse total T3 and total T4 in 182 serum samples collected from 172 botos living in the Mamirauá Sustainable Development Reserve, in the Brazilian Amazon from 2003 through 2015. Age significantly affected tT3 and tT4 concentrations in males, with values in immature males and females being significantly lower than those in adult males, whereas no age effects were noted between immature females and adult non-pregnant, non-lactating females. Significant sex differences were noted in tT3 concentrations between immature males and females and in tT4 concentrations between adult males and females. These resulted in significant differences in the tT3:tT4 ratio between males and females within the immature and adult groups. Lactating and non-pregnant adult females had significantly higher tT3 concentrations than pregnant females, and this difference was primarily driven by a 12% drop in tT3 concentrations during the last two-thirds of pregnancy. No differences in thyroid hormone concentrations were detected between females diagnosed as pregnant and later found to have or not have a live calf. These results are the first to define thyroid hormone reference intervals and normal physiological variations in a wild population of river dolphins.


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