Foetal virilisation caused by overproduction of non-aromatisable 11-oxygenated C19 steroids in maternal adrenal tumour

2020 ◽  
Vol 35 (11) ◽  
pp. 2609-2612
Author(s):  
Keisuke Nagasaki ◽  
Kaoru Takase ◽  
Chikahiko Numakura ◽  
Keiko Homma ◽  
Tomonobu Hasegawa ◽  
...  
Keyword(s):  
Pregnant Women ◽  
External Genitalia ◽  
Serum Levels ◽  
Fundamental Question ◽  
Placental Barrier ◽  
Adrenal Tumour ◽  
Normal Range ◽  
Tumour Removal ◽  
Pass Through ◽  
Pathogenic Property

Abstract It is widely believed that adrenal tumours and ovarian luteomas in pregnant women cause virilisation of female foetuses through overproduction of testosterone and/or androstenedione. However, this notion raises a fundamental question as to how these classic androgens pass through the placenta without being converted by aromatase into oestrogens. Here, we report a case of maternal adrenal tumour, in which overproduction of 11-oxygenated C19 steroids (11ox C19s), newly characterised non-aromatisable androgens in humans, caused foetal virilisation. The female proband presented with severely virilised external genitalia at birth. The mother exhibited hirsutism, hyperglycaemia and hypertension and was diagnosed as having adrenal tumour. The mother was subjected to comprehensive steroid measurement. Serum levels of 11ox C19s were markedly elevated. In contrast, testosterone and androstenedione levels remained within the normal range, and levels of most other steroids in the conventional and backdoor androgenic pathways were normal or only mildly elevated. After tumour removal, levels of 11ox C19s were markedly reduced. These results provide the first evidence that 11ox C19s can be synthesised in adrenal adenomas and, due to their non-aromatisable nature, can pass through the placental barrier to cause foetal virilisation. These findings highlight a unique pathogenic property of these newly specified androgens in humans.

SERUM LEVELS OF HUMAN CHORIONIC SOMATOMAMMOTROPIN (HCS) IN NORMAL AND ABNORMAL PREGNANCIES

1974 ◽  
Vol 77 (3_Suppl) ◽  
pp. S35-S43 ◽  
Author(s):  
Paul E. Lebech ◽  
Birgit Borggaard
Keyword(s):  
Pregnant Women ◽  
Serum Levels ◽  
Normal Group ◽  
Placental Weight ◽  
Normal Range ◽  
Placental Function ◽  
Chorionic Somatomammotropin ◽  
Twin Pregnancies

ABSTRACT Serum levels of human placental chorionic somatomammotropin (HCS) were determined by radioimmunoassay (immunosorbent) in 800 pregnancies, normal as well as pathological. The normal group comprised 200 pregnant women (254 estimations). Correlation has been found to exist between the placental weight and the HCS levels as determined in the last few weeks before onset of labour. In connection with twin pregnancies HCS levels were found to be in the upper end or above the normal range. In cases of anencephaly HCS levels were found to be normal with low values of oestrogen excretion in urine. Other examples may be mentioned in support of the importance of the HCS estimation to the evaluation of the placental function.

STUDIES ON THE AROMATISATION OF NEUTRAL STEROIDS IN PREGNANT WOMEN

1964 ◽  
Vol 45 (4) ◽  
pp. 535-559 ◽  
Author(s):  
E. Bolté ◽  
S. Mancuso ◽  
G. Eriksson ◽  
N. Wiqvist ◽  
E. Diczfalusy
Keyword(s):  
Pregnant Women ◽  
Comparative Studies ◽  
Chorionic Gonadotrophin ◽  
Human Chorionic Gonadotrophin ◽  
Radioactive Material ◽  
Placental Barrier ◽  
Therapeutic Abortion ◽  
Limited Ability ◽  
Better Than

ABSTRACT In 15 cases of therapeutic abortion by laparotomy the placenta was disconnected from the foetus and perfused in situ with tracer amounts of radioactive dehydroepiandrosterone (DHA), dehydroepiandrosterone sulphate (DHAS), androst-4-ene-3,17-dione (A), testosterone (T) and 17β-oestradiol (OE2). Analysis of the placentas, perfusates and urine samples revealed an extensive aromatisation of DHA, A and T; more than 70% of the radioactive material recovered was phenolic, and at least 80 % of this phenolic material was identified as oestrone (OE1), 17β-oestradiol (OE2) and oestriol (OE3), the latter being detected only in the urine. Comparative studies indicated that A and T were aromatised somewhat better than DHA and that all three unconjugated steroids were aromatised to a much greater extent than DHAS. Radioactive OE1 and OE2 were isolated and identified in the placentas and perfusates, but no OE3, epimeric oestriols, or ring D ketols could be detected in these sources, not even when human chorionic gonadotrophin (HCG) was added to the blood prior to perfusion. Lack of placental 16-hydroxylation was also apparent when OE2 was perfused. Regardless of the precursor perfused, there was three times more OE2 than OE1 in the placenta and three times more OE1 than OE2 in the perfusate. This was also the case following perfusion with OE2. The results are interpreted as suggesting the existence in the pregnant human of a placental »barrier« limiting the passage of circulating androgen. The barrier consists of a) limited ability to transfer directly DHAS and b) an enzymic mechanism resulting in the rapid and extensive aromatisation of the important androgens DHA, A and T.

Recovery of thyroid function with a decreased titre of antimicrosomal antibody in a hypothyroid man with Hashimoto's thyroiditis

1983 ◽  
Vol 102 (4) ◽  
pp. 531-534 ◽  
Author(s):  
Makiko Yamamoto ◽  
Kazuro Kaise ◽  
Hirofumi Kitaoka ◽  
Katsumi Yoshida ◽  
Nobuko Kaise ◽  
...  
Keyword(s):  
Hashimoto’S Thyroiditis ◽  
Serum Levels ◽  
Hashimoto's Thyroiditis ◽  
Thyroid Peroxidase ◽  
Normal Range ◽  
Surgical Biopsy ◽  
Low Levels ◽  
Serum Thyroid Hormones ◽  
Antimicrosomal Antibody ◽  
Serum Tsh

Abstract. A 36 year old man with a diffuse goitre, signs of mild hypothyroidism, strikingly low levels of T4 (0.9 μg/dl) and T3 (24 ng/dl), elevated TSH (140 μU/ml) and elevated microsomal haemagglutination antibody (MCHA, 1:409 600), subsequently became non-goitrous and euthyroid with a decreased titre of antimicrosomal antibody without any medication. At the time of surgical biopsy, serum levels of T4 and T3 had risen to the normal range (4.6 μg/dl and 73 ng/dl, respectively), serum TSH had decreased to 30 μU/ml and the titre of MCHA to 1:25 600. Thyroid specimens showed Hashimoto's thyroiditis. The activity of thyroid peroxidase (TPO) was normal. The latest examination, 1 year and 3 months after initial evaluation, showed that the patient remained euthyroid with no goitre, that serum thyroid hormones were within the normal range (T4 7.7 μg/dl and T3 97 ng/dl), and that TSH was not detectable. The titre of MCHA decreased strikingly to 1:400.

Dietary and serum vitamin D and preeclampsia risk in Chinese pregnant women: a matched case–control study

2021 ◽  
pp. 1-26
Author(s):  
Xue-min Huang ◽  
Yan-hua Liu ◽  
Han Zhang ◽  
Yuan Cao ◽  
Wei-feng Dou ◽  
...  
Keyword(s):  
Vitamin D ◽  
Dietary Intake ◽  
Pregnant Women ◽  
Case Control Study ◽  
Serum Levels ◽  
Case Control ◽  
Serum Concentrations ◽  
Matched Case ◽  
Matched Case Control Study ◽  
Control Study

Abstract The effect of vitamin D (VD) on the risk of preeclampsia (PE) is uncertain. Few of previous studies focused on the relationship between dietary VD intake and PE risk. Therefore, we conducted this 1:1 matched case-control study to explore the association of dietary VD intake and serum VD concentrations with PE risk in Chinese pregnant women. A total of 440 pairs of participants were recruited during March 2016 to June 2019. Dietary information was obtained using a 78-item semi-quantitative food frequency questionnaire. Serum concentrations of 25(OH)D2 and 25(OH)D3 were measured by liquid chromatography–tandem mass spectrometry. Multivariate conditional logistic regression was used to estimate odds ratios (ORs) and 95% confidence intervals (CIs). Restricted cubic splines (RCS) were plotted to evaluate the dose-response relationship of dietary VD intake and serum VD concentrations with PE risk. Compared with the lowest quartile, the ORs of the highest quartile were 0.45 (95%CI: 0.29-0.71, Ptrend = 0.001) for VD dietary intake and 0.26 (95%CI: 0.11-0.60, Ptrend = 0.003) for serum levels after adjusting for confounders. In addition, the RCS analysis suggested a reverse J-shaped relationship between dietary VD intake and PE risk (P-nonlinearity = 0.02). A similar association was also found between serum concentrations of total 25(OH)D and PE risk (P-nonlinearity = 0.02). In conclusion, this study provides evidence that higher dietary intake and serum levels of VD are associated with the lower risk of PE in Chinese pregnant women.

Serum levels of perfluorooctanoic acid and perfluorooctane sulfonic acid in pregnant women: Maternal predictors and associations with birth outcomes in the PIPA Project

2021 ◽  
Author(s):  
Aline de Souza Espindola Santos ◽  
Armando Meyer ◽  
Vanessa Emídio Dabkiewicz ◽  
Volney de Magalhães Câmara ◽  
Carmen Ildes Rodrigues Froes Asmus
Keyword(s):  
Pregnant Women ◽  
Birth Outcomes ◽  
Sulfonic Acid ◽  
Serum Levels ◽  
Perfluorooctanoic Acid ◽  
Maternal Predictors

scholarly journals Dehydroepiandrosterone (DHEA) Serum Levels Indicate Cerebrospinal Fluid Levels of DHEA and Estradiol (E2) in Women at Term Pregnancy

2021 ◽  
Author(s):  
Pardes Habib ◽  
Joseph Neulen ◽  
Shahin Habib ◽  
Benjamin Rösing
Keyword(s):  
Pregnant Women ◽  
Strong Correlation ◽  
Serum Levels ◽  
Neuroactive Steroids ◽  
Term Pregnancy ◽  
Serum Samples ◽  
Functional Changes ◽  
The Central Nervous System ◽  
Csf Levels ◽  
Fluid Levels

AbstractNeuroactive steroids such as dehydroepiandrosterone (DHEA), estradiol (E2), and progesterone (P4) are associated with structural and functional changes in the central nervous system (CNS). Measurement of steroid levels in the CNS compartments is restricted in accessibility. Consequently, there is only limited human data on the distributional equilibrium for steroid levels between peripheral and central compartments. While some neuroactive steroids including DHEA and E2 have been reported to convey excitatory and proconvulsant properties, the opposite was demonstrated for P4. We aimed to elucidate the correlation between peripheral and central DHEA, E2, and P4 levels in women at term pregnancy. CSF and serum samples of 27 healthy pregnant women (22–39 years) at term pregnancy were collected simultaneously under combined spinal and epidural anesthesia and used for DHEA ELISA and E2, and P4 ECLIA. All three neuroactive steroids were detected at markedly lower levels in CSF compared to their corresponding serum concentrations (decrease, mean ± SD, 97.66 ± 0.83%). We found a strong correlation for DHEA between its serum and the corresponding CSF levels (r = 0.65, p = 0.003). Serum and CSF levels of E2 (r = 0.31, p = 0.12) appeared not to correlate in the investigated cohort. DHEA serum concentration correlated significantly with E2 (r = 0.58, p = 0.0016) in CSF. In addition, a strong correlation was found between DHEA and E2, both measured in CSF (r = 0.65, p = 0.0002). Peripheral DHEA levels might serve as an indicator for central nervous levels of the neuroactive steroids DHEA and E2 in pregnant women.

ENDOCRINE STUDIES AND SUCCESSFUL TREATMENT IN A PATIENT WITH TRUE HERMAPHRODITISM

1979 ◽  
Vol 91 (1) ◽  
pp. 184-192
Author(s):  
Evangelina Valdés ◽  
Carlos Fernández del Castillo ◽  
Raul Gutiérrez ◽  
Fernando Larrea ◽  
Martha Medina ◽  
...  
Keyword(s):  
Chromosome Complement ◽  
Reproductive Function ◽  
External Genitalia ◽  
Serum Testosterone ◽  
Serum Levels ◽  
Normal Children ◽  
Serum Lh ◽  
Genital Ambiguity ◽  
And Gender ◽  
Lh Rh

ABSTRACT A 12-year old, 46 XX true hermaphrodite born with genital ambiguity was studied and successfully treated. The serum LH and FSH profile resembled that of a pubertal normal individual, and LH-RH administration induced a normal LH response. Baseline testosterone serum levels were within the range for normal children. Exogenous HCG stimulation induced a significant serum testosterone increase up to values similar to those observed in normal post-pubertal males. Surgical examination disclosed the presence of bilateral ovotestis, normal Mullerian derivatives, epididymis, and vas deferens. A complete ovotestis with testicular predominance and the testicular portion of the contralateral ovotestis as well as the Wolffian derivatives, were removed. A further HCG stimulation 3 months after surgery, failed to induce serum testosterone increase. Spontaneous menarche was observed 6 months after surgery and ovulation was well documented. At present the patient has several characteristics of female sex including those of chromosome complement, gonad, internal and external genitalia, hormone levels and gender identity, thus demonstrating that treatment was successful and that reproductive function could be obtained. The finding of spontaneous ovulation following removal of the testicular portion suggests normal cyclic gonadotrophic release implying a difference between animal models and man in regard to hypothalamic virilization.

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