Determination of Urinary Tetrahydro-11-Deoxycortisol and Tetrahydro-11-Deoxycorticosterone by Gas Chromatography

Koepp, P; Völlmin, J. A.; Zachmann, M.; Curtius, Hans−Christoph

doi:10.1530/acta.0.0660756

Cited by 12 publications

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“…Urinary THE, THSlTHDOC and pregnenetriol were determined by gas chromatographic methods on glass capillary columns developed in our laboratory (Vollmin, 1970;Koepp et al, 1971). These methods were also used to confirm the quantitative results obtained on packed columns.…”

Section: A T E R I a L S A N D M E T H O D S Urinary Steroid Determmentioning

confidence: 99%

Steroid 17, 20‐desmolase Deficiency: A New Cause of Male Pseudohermaphroditism

Zachmann

Völlmin

Hamilton

et al. 1972

Clinical Endocrinology

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158

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SUMMARY In a child with male pseudohermaphroditism (ambiguous external genitalia, XY sex chromosomal constitution and normal adrenocortical function), incubations of testicular tissue with pregnenolone/progesterone, 17α‐hydroxy‐pregnenolone/17α‐hydroxyprogesterone and androstenedione/dehydroepiandrosterone showed that testosterone could be formed from androstenedione and dehydroepiandrosterone only, but not from other substrates. In urine, testosterone did not increase after HCG, but small amounts of pregnanetriolone were found, which increased after HCG and ACTH. There was no DHA increment after ACTH. It is concluded that this patient, as well as a first cousin and a gonadectomized maternal 'aunt' with the same clinical and urinary steroid findings have testicular and adrenal steroid 17,20‐desmolase deficiency, causing a defect of androgen biosynthesis, which has not previously been described. The heredity of this condition seems to be autosomal or X‐chromosomal.

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Section: A T E R I a L S A N D M E T H O D S Urinary Steroid Determmentioning

confidence: 99%

Steroid 17, 20‐desmolase Deficiency: A New Cause of Male Pseudohermaphroditism

Zachmann

Völlmin

Hamilton

et al. 1972

Clinical Endocrinology

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158

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“…The results of steroid determinations in 8 urine samples collected between the age of 2 days and 17 months are summarized in Table 1. 3 shows a gas chromatogram on a glass capillary column (14,15) from a urine of the untreated patient (sample VII, Table 1) in comparison with the chromatogram from the urine of a normal subject of the same age (13 months, see also Table 4). Sample VIII was obtained 3 days after treatment had been interrupted.…”

Section: Resultsmentioning

confidence: 99%

“…Determination of urinary testosterone glucuronide was carried out by a gas-chromatographic method also published from our laboratory (13). Temperatures: column 220 C, injector block 260 C, detector 260 C. Urinary tetrahydro-11-deoxycortisol (THS) and tetrahydro-11-deoxycorticosterone (THDOC) determinations were done by gas chromatography of methoxime derivatives on glass capillary columns (14) by a method from our laboratory (15). Stationary phase was XE 60.…”

Section: Methodsmentioning

confidence: 99%

Congenital Adrenal Hyperplasia due to Deficiency of 11β-Hydroxylation of 17α-Hydroxylated Steroids¹

Zachmann¹,

Völlmin²,

New³

et al. 1971

The Journal of Clinical Endocrinology & Metabolism

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The case of an infant girl with congenital adrenal hyperplasia due to 11/3-hydroxylase deficiency is described. The diagnosis was made at the age of 1 week and the steroid excretion of the untreated patient was followed during the first year of life. Urinary THS reached very high levels only after 16a-hydroxypregnenolone had disappeared from the urine, suggesting an influence of the physiologically reduced 3/3-hydroxysteroid dehydrogenase activity of infancy on the steroid pattern induced by the 11/3-hydroxylase deficiency. The secretion rate of S was extremely high and that of F was low normal, while the secretion rates of DOC and B and the aldosterone excretion were within normal limits. This indicates an impairment of the 11/3hydroxylation of 17a-hydroxylated compounds only and is indirect evidence for the existence of two 11/3-hydroxylating systems in the human adrenal cortex. {J Clin Endocr 33: 501, 1971)

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“…Urinary THS (after single dose metyrapone, 35) and THE (before and after synthetic depot-ACTH 1 mg/m2 i.m.) were analyzed by gas chromatography on a glass capillary column (15,32).…”

Section: Methodsmentioning

confidence: 99%

Low Urinary Estriol During Pregnancy Caused by Isolated Fetal Acth‐deficiency

Zachmann¹,

Girard²,

Duc³

et al. 1979

Acta Paediatrica

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Zachmann, M., Girard, J., Duc, G., Illig, R. and Prader, A. (Departments of Pediatrics, Universities of Zurich and Basel, Switzerland). Low urinary estriol during pregnancy caused by isolated fetal ACTH-deficiency. Acta Paediatr Scand, Suppl. 277: 26, 1979.-In a 34-year-old pregnant woman, serum HPL and urinary HCG were normal, but urinary estriol was repeatedly low. A normal boy was delivered after 38 weeks gestation. During the neonatal period, he had hypoglycemia, muscular hypotonia and transient hyperbilirubinemia. The ACTH-test was normal, hut the THS-response to metyrapone was low. Serum ACTH did not respond to insulin and metyrapone. Growth hormone, TSH and gonadotropin responses to stimuli were normal. Treatment with hydrocortisone resulted in disappearance of the symptoms. It is concluded that fetal ACTH-deficiency is one of the specific endocrine causes of low maternal estriol.KEY WORDS: ACTH-deficiency, urinary estriol, metyrapone test, placental sulphatase deficiency Determinations of estriol (E,) in urine of pregnant women are widely used to monitor fetal well-being. In general, low values indicate placental insufficiency and fetal distress. In few instances, however, pregnancy progresses normally. In this situation, specific endocrine defects of the fetus or the fetoplacental unit may be the cause of reduced E,.In the following, we present evidence suggesting that low maternal E, excretion may be caused by isolated ACTH-deficiency of the fetus. MATERIALS AND METHODSThe following trivial names and abbreviations are used: ACTH = adrenocorticotrophic hormone DHA = dehydroepiandrosterone = 3p-hydroxy-androst-E, = estriol = 1.3.5(10)-estratriene-3,16a,l7/?-triol F = cortisol = hydrocortisone = 17p,17a,21-trihydro-FSH = follicle stimulating hormone GH = growth hormone 170HP = 17-hydroxy-progesterone = l7a-hydroxy-pregn-ITT = insulin tolerance test LH = luteinizing hormone LHRH = luteinizing hormone releasing hormone PRL = prolactin 5-en-17-one xypregn-4-ene-3-,20-dione 4-ene-3,20-dione T, = thyroxine T, = triiodothyronine THE = tetrahydrocortisone = 3a, 17,2l-trihydroxy-SP-THS = tetrahydrocortexolone = 3a, 17,21-trihydroxy-5P-pregnan-20-one Urinary E, in pregnancy was determined according to Brown et al. (6), urinary Porter-Silber chromogens according to Peterson & Pierce (21). Urinary THS (after single dose metyrapone, 35) and THE (before and after synthetic depot-ACTH 1 mg/m2 i.m.) were analyzed by gas chromatography on a glass capillary column (15, 32).Urinary 16-hydroxylated steroids were also assessed by gas chromatography (25).The following hormones in plasma and serum were measured radioimmunologically: ACTH (8) before and after insulin (4 IU/m2 i.v.) and 4 hours after metyrapone (500 mg/m2 p.0.). Routine radioimmunoassay procedures were used for determinations of cortisol and 170H-progesterone before and after synthetic ACTH (250 pg/m2 i.v.), testosterone and DHA-sulphate under basal conditions, GH before and after insulin (4 IU/m2 i.v.) and arginine (0.5 g/kg i.v.), TSH before and after TRH (200...

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Determination of Urinary Tetrahydro-11-Deoxycortisol and Tetrahydro-11-Deoxycorticosterone by Gas Chromatography

Cited by 12 publications

References 0 publications

Steroid 17, 20‐desmolase Deficiency: A New Cause of Male Pseudohermaphroditism

Steroid 17, 20‐desmolase Deficiency: A New Cause of Male Pseudohermaphroditism

Congenital Adrenal Hyperplasia due to Deficiency of 11β-Hydroxylation of 17α-Hydroxylated Steroids¹

Low Urinary Estriol During Pregnancy Caused by Isolated Fetal Acth‐deficiency

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Determination of Urinary Tetrahydro-11-Deoxycortisol and Tetrahydro-11-Deoxycorticosterone by Gas Chromatography

Cited by 12 publications

References 0 publications

Steroid 17, 20‐desmolase Deficiency: A New Cause of Male Pseudohermaphroditism

Steroid 17, 20‐desmolase Deficiency: A New Cause of Male Pseudohermaphroditism

Congenital Adrenal Hyperplasia due to Deficiency of 11β-Hydroxylation of 17α-Hydroxylated Steroids1

Low Urinary Estriol During Pregnancy Caused by Isolated Fetal Acth‐deficiency

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Congenital Adrenal Hyperplasia due to Deficiency of 11β-Hydroxylation of 17α-Hydroxylated Steroids¹