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  • 1
    Electronic Resource
    Electronic Resource
    s.l. : American Chemical Society
    Journal of agricultural and food chemistry 34 (1986), S. 96-97 
    ISSN: 1520-5118
    Source: ACS Legacy Archives
    Topics: Agriculture, Forestry, Horticulture, Fishery, Domestic Science, Nutrition , Process Engineering, Biotechnology, Nutrition Technology
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1440
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary An improved technique in testing adrenocortical function will be described, allowing to demonstrate the immediate, short-time response of the adrenal cortex in infants and children. After i.v. stimulation with 25 E of a synthetic ACTH-preparation 11-hydroxycorticoid (Cortisol) determination in an 8-hour-urine specimen is done by a fluorescence method. Values are compared with basal dates without stimulation. Normally, there is a 4–20 fold increase of the cortisol excretion. In adrenocortical insufficiency no increase is observed. In hypopituitarism, a decreased reactivity indicates an adrenocorticotrophic insufficiency.
    Notes: Zusammenfassung Es wird über eine verbesserte Technik der NNR-Funktionsdiagnostik berichtet, welche erlaubt, bei Säuglingen und Kindern im Harn von 8 Std nach i.v. Stimulation mit synthetischem ACTH mit bisher nicht erreichter Präzision und Einfachheit die kurzfristige Funktionsbereitschaft der NNR zu erfassen. Als biochemisches Kriterium wird eine vonDörner angegebene fluorometrische Methode der 11-Hydroxycorticoid-(Cortisol-)Bestimmung im Harn eingesetzt. Die Methode gestattet, neben der primären NNR-Insuffizienz die sekundäre NNR-Insuffizienz bei langfristiger Corticoidbehandlung und beim Panhypopituitarismus exakt zu erfassen. Die Methode ist technisch relativ einfach, wenig zeitaufwendig, erfordert jedoch ein Fluorometer.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1440
    Keywords: Pseudohermaphroditism ; Gynecomastia ; Androstenedione ; 17β-Hydroxysteroid-oxydoreductase deficiency ; Testosterone inborn error ; Pseudohermaphroditismus masculinus ; Gynäkomastie ; Androstendion ; 17β-Hydroxysteroid-oxydoreductase Defekt ; Testosteron-Synthesestörung
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Beschreibung eines Jungen mit Pseudohermaphroditismus maskulinus, mit normalem männlichen Karyotyp, welcher bei der Geburt als Mädchen angesehen worden war. Mit 12 Jahren Auftreten einer hochgradigen Gynäkomastie. Steroiduntersuchungen wurden mit Radiogaschromatographie und mit radioimmunologischen Methoden durchgeführt. Die Steroidbestimmungen ergaben im Plasma bei altersnormalen Testosteronspiegeln Androstendionwerte, welche die Norm um den Faktor 9–23 überstiegen. Die Plasmaoestrogene wurden insgesamt erhöht gefunden, Oestron weit mehr als Oestradiol. In Inkubationsversuchen vom bioptisch gewonnenen Hodengewebe des Jungen konnte gezeigt werden, daß zugesetztes markiertes Androstendion nur in geringem Maß zu Testosteron metabolisiert wurde, während normales Hodengewebe rund 60% zu Testosteron metabolisierte. Auf Grund dieser biochemischen Befunde wird eine Testosteron-Synthesestörung mit Defekt einer 17β-Hydroxy-steroid-Oxydoreduktase als Ursache des Pseudohermaphroditismus und der Gynäkomastie angenommen. Der Enzymdefekt ist bisher zweimal beschrieben, scheint aber nach entsprechender Untersuchung häufiger zu sein.
    Notes: Summary A boy with male pseuodhermaphroditism described here with normal male caryotype was at first raised as a girl. The error was detected within the first year of life. At the age of 12 years the boy developed a severe gynecomastia. At this time determinations of plasma and urinary steroids were undertaken. 17-ketosteroids and total 17-OHCS were determined by colorimetry. Plasma testosterone, androstenedione, 17α OH-progesterone, urinary pregnantriol and pregnantriolone were measured by GLC. Plasma estrone and estradiol were determined by radioimmunoassay (RIA). Investigations at 12 years of age and later revealed plasma testosterone in the lower normal range and androstenedione 9–23 times higher compared to normal boys. Plasma estrogens were found to be elevated, estrone much more than estradiol. Incubation studies with testicular tissue showed very little conversion of3H-androstenedione (15%) whereas normal testicular tissue converted up to 60% to3H-testosterone. On the basis of these data 17β-hydroxysteroid-oxydoreductase deficiency is suggested as the underlying cause of pseudohermaphroditism and gynecomastia in this boy. Up to the present this enzyme deficiency is described only two times but it would seem to be more frequent if one looked at the elevated androstenedione plasma level.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 65 (1987), S. 925-925 
    ISSN: 1432-1440
    Keywords: Fabry's disease ; Priapism ; Testosterone
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1440
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 63 (1985), S. 607-612 
    ISSN: 1432-1440
    Keywords: Cortisol secretion ; 11β-hydroxylation ; Ketoconazole
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We investigated basal and ACTH stimulated levels of cortisol, corticosterone, 17α-hydroxyprogesterone, 11-deoxycortisol and 11-deoxycorticosterone as well as plasma levels of ACTH before and during the oral administration of ketoconazole in five patients with Cushing's syndrome (3 with bilateral adrenal hyperplasia, 1 with adrenal adenoma and 1 with adrenal carcinoma) and in three controls. The influence of ketoconazole on the transformation of3H-17α-hydroxyprogesterone to3H-11-deoxycortisol and3H-cortisol and of3H-11-deoxycortisol to3H-cortisol as well as of3H-11-deoxycorticosterone to3H-corticosterone was also examined in slices or homogenates of normal and hyperplastic adrenal tissue from four patients. Ketoconazole induced a rise of 11-deoxycortisol and 11-deoxycorticosterone, but not of cortisol and inconsistantly of corticosterone which were increased by ACTH. Thus the ratio 11-deoxycortisol/cortisol rose more after ketoconazole than after ACTH and the ratio 11-deoxycorticosterone/corticosterone rose after ketoconazole but fell after ACTH. Plasma ACTH levels were stimulated 2–50 fold by ketoconazole. Incubation studies of adrenal tissue slices with3H-17α-hydroxyprogesterone showed that ketoconazole inhibited the transformation of3H-17α-hydroxyprogesterone to3H-cortisol but not to3H-11-deoxycortisol so that the ratio3H-11-deoxycortisol/3H-cortisol increased 15–80 fold. After incubation of adrenal slices with3H-11-deoxycortisol or3H-11-deoxycorticosterone and ketoconazole, a 2–260 fold increase of the ratios3H-11-deoxycortisol/3H-cortisol and3H-11-deoxycorticosterone/3H-corticosterone were also found. In conclusion, the in vivo data indicate and the in vitro data confirm that ketoconazole inhibits cortisol and corticosterone secretion by blocking adrenal 11β-hydroxylase activity in normal subjects as well as in patients with Cushing's syndrome, an effect which is compensated in vivo by high ACTH levels.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 63 (1985), S. 192-192 
    ISSN: 1432-1440
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 62 (1984), S. 1011-1013 
    ISSN: 1432-1440
    Keywords: Etomidate ; Adrenocortical suppression ; 17-Deoxysteroids ; 17-Hydroxysteroids
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary To investigate the adrenocortical suppression caused by the anesthetic etomidate, plasma levels of progesterone (P), 17-hydroxyprogesterone (17-OHP), 11-deoxycorticosterone (DOC), corticosterone (B), aldosterone (Aldo), 11-deoxycortisol (S), cortisol (F), and cortisone (E) were measured simultaneously before and after a short-term ACTH stimulation test in a 6.5-year-old boy whose convulsions could be kept under control only with constant etomidate infusions. During etomidate therapy, plasma levels of DOC and S were extremely elevated, the progestins P and 17-OHP were slightly elevated, whereas B and Aldo were in the lower normal range, and F and E were markedly decreased. A short-term ACTH stimulation test during etomidate infusion gave a blunted response of B, Aldo, F and E, whereas the level of DOC remained high and S even further increased. P and 17-OHP showed a positive response to ACTH. The ratios of B/DOC and F/S, which reflect adrenocortical 11β-hydroxylase activity, were extremely decreased during etomidate and did not change after ACTH stimulation. In contrast, the ratios of DOC/P and S/17-OHP, which relect 21-hydroxylase activity, were elevated and remained elevated after ACTH stimulation. After discontinuation of etomidate therapy, all the baseline steroid levels were somewhat elevated, but responded normally to ACTH. These results demonstrate that etomidate causes a specific and reversible blockade of the 11β-hydroxylation of adrenal steroid synthesis.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 45 (1967), S. 792-792 
    ISSN: 1432-1440
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary The diurnal rhythem of the excretion of cortisol in urine was investigated in 25 overweight children and 25 children of normal weight by a fluorometric method. The urine was collected in 10 portions. The high cortisol excretion in the morning lasts in normal children until 10 h a.m., in adipose children longer extending over 12 h noon. The elevation of the excretion of cortisol in the period 10–12 h a.m. in adipose children is high significant (P〈0,001).
    Notes: Zusammenfassung Es wurde mit einer fluorometrischen Methode der Tagesrhythmus der Cortisolausscheidung im Harn in zehn Einzelportionen bei 25 normalgewichtigen und 25 fettsüchtigen Kindern bestimmt. Die morgendliche, hohe Cortisolausscheidung währt bei Normalgewichtigen bis 10 Uhr, bei fettsüchtigen über 12 Uhr hinaus. Die Erhöhung der Cortisolausscheidung von 10–12 Uhr vormittags bei Fettsüchtigen ist mitP〈0,001 hochsignifikant.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 54 (1976), S. 467-473 
    ISSN: 1432-1440
    Keywords: Cryptorchidism ; Male fertility ; Human chorionic gonadotrophin (HCG) ; Sperm count ; Hodenhochstand ; Maldescensus testis ; Kryptorchismus ; Fertilität (männlich) ; Humanes Choriongonadotropin (HCG) ; Spermatogramm
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Von 212 Patienten, welche vor 7 – 12 Jahren im Schulalter vor Pubertätsbeginn wegen eines Maldescensus testis mit humanem Choriongonadotropin (HCG) behandelt worden waren, konnten mit 17 Jahren oder später 151 nachuntersucht werden. Bei 121 wurde ein Spermatogramm durchgeführt. 10 Patienten konnten wegen Hypogonadismus kein Ejakulat gewinnen. Im Gesamtkollektiv der spermatographisch untersuchten Patienten (n=121) was es bei 78 unter HCG zu einem ausreichenden Descensus gekommen. Bei 43 war die HCG-Behandlung erfolglos, es erfolgte anschließend die operative Verlagerung. Vom Gesamtkollektiv (n=121) zeigten nur 39% ein normales Spermatogramm. Nach unilateralem Maldescensus waren 18%, nach bilateralem Maldescensus 43% als wahrscheinlich infertil einzuordnen. Spermienzahlen unter 1 Mill./ml nach bilateralem Maldescensus fanden Maier und Spann bei primär operativer Behandlung bei 9 von 17 Patienten. Nach gleichen Kriterien wurden in dieser Studie nach bilateralem Maldescensus und alleiniger HCG-Behandlung bei 9 von 35 die Spermienzahl von 1 Mill./ml unterschritten. Plasma LH und FSH konnte bei 93 Patienten gemessen werden. Hypergonadotrope FSH-Werte wurden bei 6 Patienten, alle mit Spermienzahlen unter 10 Mill./ml, gefunden. Nachträglich wurden im Gesamtkollektiv 3 Klinefelter-Syndrome festgestellt. Der Grund des Maldescensus ist nicht einheitlich. Anatomische, hormonale und chromosomale Ursachen sind möglich.
    Notes: Summary 151 out of 212 adolescents having had been treated by human chorionic gonadotrophin (HCG) before puberty because of undescended testes, were re-examined 7 to 12 years later at an individual age of at least 17 years. In 121 of them a semen specimen for sperm counts could be obtained. In 10 patients, however, no ejaculate could be collected because of their hypogonadism. 78 of these 121 patients examined by sperm count had a sufficient testicular descensus after HCG treatment. In 43 adolescents HCG therapy failed, therefore subsequent orchidopexy was performed. A normal sperm count was seen in only 39 per cent of the 121 patients. After unilateral testicular maldescensus 18%, after bilateral maldescensus 43% of the patients had to be evaluated as prospectively infertile. Maier and Spann reported sperm counts below 106/ml in 9 out of 17 patients with bilateral maldescensus who had been treated by surgery alone. In the present study, however, only 9 out of 35 individuals treated for bilateral maldescensus by HCG alone showed sperm counts below 106/ml. Plasma LH and FSH concentrations were determined in 93 patients, 6 out of which showed hypergonadotrophic values of FSH and sperm counts below 107/ml. Subsequently, 3 cases of Klinefelter's syndrome could be diagnosed among the total group of 121 patients. The etiology of testicular maldescensus is by no means uniform since anatomical, endocrine and/or chromosomal factors are involved.
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