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Journal of Pediatric and Adolescent... Dec 2022Infants with genital development considered atypical for assigned female sex may undergo feminizing genitoplasty (clitoroplasty and/or vaginoplasty) in early life. We... (Observational Study)
Observational Study
STUDY OBJECTIVE
Infants with genital development considered atypical for assigned female sex may undergo feminizing genitoplasty (clitoroplasty and/or vaginoplasty) in early life. We sought to identify factors associated with parent/caregiver decisions regarding genitoplasty for their children with genital virilization.
DESIGN
Longitudinal, observational study SETTING: Twelve pediatric centers in the United States with multidisciplinary differences/disorders of sex development clinics, 2015-2020 PARTICIPANTS: Children under 2 years old with genital appearance atypical for female sex of rearing and their parents/caregivers INTERVENTIONS/OUTCOME MEASURES: Data on the child's diagnosis and anatomic characteristics before surgery were extracted from the medical record. Parents/caregivers completed questionnaires on psychosocial distress, experience of uncertainty, cosmetic appearance of their child's genitalia, and demographic characteristics. Urologists rated cosmetic appearance. For 58 patients from the study cohort with genital virilization being raised as girls or gender-neutral, we compared these data across 3 groups based on the child's subsequent surgical intervention: (i) no surgery (n = 5), (ii) vaginoplasty without clitoroplasty (V-only) (n = 15), and (iii) vaginoplasty and clitoroplasty (V+C) (n = 38).
RESULTS
Fathers' and urologists' ratings of genital appearance were more favorable in the no-surgery group than in the V-only and V+C groups. Clitorophallic length was greater in the V+C group compared with the V-only group, with substantial overlap between groups. Mothers' depressive and anxious symptoms were lower in the no-surgery group compared with the V-only and V+C groups.
CONCLUSIONS
Surgical decisions were associated with fathers' and urologists' ratings of genital appearance, the child's anatomic characteristics, and mothers' depressive and anxious symptoms. Further research on surgical decision-making is needed to inform counseling practices.
Topics: Child; Female; Humans; Infant; Adrenal Hyperplasia, Congenital; Gynecologic Surgical Procedures; Parents; Plastic Surgery Procedures; Sexual Development; Virilism; Longitudinal Studies
PubMed: 35948206
DOI: 10.1016/j.jpag.2022.08.004 -
Revista Da Associacao Medica Brasileira... 2022In this study, the hormone profile, lipid profile, and inflammatory parameters of patients with polycystic ovary syndrome were compared with those of non-polycystic...
OBJECTIVE
In this study, the hormone profile, lipid profile, and inflammatory parameters of patients with polycystic ovary syndrome were compared with those of non-polycystic ovary syndrome patients to determine predictive markers in young polycystic ovary syndrome patients who have not yet had children.
METHODS
Patients' age, height, weight, body mass index, waist circumference, degree of hirsutism, and ultrasound findings were recorded. Hormone profile, lipid levels, ratio of complete blood count parameters, monocyte/high-density lipoprotein ratio, and total cholesterol/high-density lipoprotein ratio were compared between groups.
RESULTS
No statistically significant differences were found between groups in terms of age, weight, waist circumference, body mass index, and dysmenorrhea (p>0.05). A significant relationship was found between the height and the degree of hirsutism in the groups (p<0.05). It was found that prolactin, total testosterone, and dehydroepiandrosterone sulfate levels were statistically significantly higher in the polycystic ovary syndrome group compared to the control group. The polycystic ovary syndrome group had hemoglobin, leukocytes, lymphocytes, neutrophils, platelets, and statistically higher ratios of total cholesterol/high-density lipoprotein, Low-density lipoprotein/high-density lipoprotein, and triglycerides/high-density lipoprotein. No statistically significant relationships were found between homeostatic model assessment insulin resistance, neutrophil-to-lymphocyte ratio, and monocyte-to-high-density lipoprotein ratio between the groups (p<0.05). There was no significant difference in systemic immune inflammation index values between the groups.
CONCLUSION
Polycystic ovary syndrome patients are at risk for short- and long-term complications, and the use of the total cholesterol/high-density lipoprotein, Low-density lipoprotein/high-density lipoprotein, and triglycerides/high-density lipoprotein ratios in clinical practice during the follow-up of these patients may allow easy follow-up for patients. The health status of polycystic ovary syndrome patients can be objectively determined by tracking these outcomes at regular intervals.
Topics: Child; Female; Humans; Lipoproteins, HDL; Hirsutism; Polycystic Ovary Syndrome; Lipoproteins, LDL; Triglycerides; Testosterone; Cholesterol
PubMed: 36449764
DOI: 10.1590/1806-9282.20220854 -
Annales de Biologie Clinique Aug 2016Androstenedione is an androgen hormone and a precursor for testosterone and estrone synthesis. It is produced in both gonadal (testes and ovaries) and extragonadal...
Androstenedione is an androgen hormone and a precursor for testosterone and estrone synthesis. It is produced in both gonadal (testes and ovaries) and extragonadal levels (peripheral conversion of dehydroepiandrosterone). This hormone is useful in the assessment of virilism and hirsutism in women. In this paper, after a brief physiological reminder, the author presents the preanalytical and analytical considerations and the clinical utility of this hormone.
Topics: Adult; Androstenedione; Blood Chemical Analysis; Child; Female; Hirsutism; Humans; Immunoassay; Male; Polycystic Ovary Syndrome; Research Design
PubMed: 27492704
DOI: 10.1684/abc.2016.1171 -
Journal of Medical Case Reports Sep 2016Tel Hashomer camptodactyly syndrome is a rare disease and only a few cases have been reported. Dermatoglyphics potentially provide relevant phenotypic biomarkers that... (Review)
Review
INTRODUCTION
Tel Hashomer camptodactyly syndrome is a rare disease and only a few cases have been reported. Dermatoglyphics potentially provide relevant phenotypic biomarkers that were initially noted as a vital clinical feature of this disease. Dermatoglyphics possibly can indicate growth disturbances that took place during early fetal development at the time when epidermal ridges were being formed into discernable patterns. Consequently, these intrauterine effects might well have occurred in association with the expression of the Tel Hashomer camptodactyly syndrome. Therefore, this review was undertaken to provide, as far as we know, the first attempt to broadly assess dermatoglyphic features that are connected with the Tel Hashomer camptodactyly syndrome. If a developmental association between dermatoglyphics and Tel Hashomer camptodactyly can be firmly established, this would probably document that Tel Hashomer camptodactyly disease has its origins during the early fetal period.
METHODS
A systematic literature search was conducted using articles from PubMed (Medline), POPLINE, Trip Database, Cochrane Library, and gray literature up to 31 March 2015. The review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement.
RESULTS
Fourteen relevant publications were included in the review. There were 23 cases of patients with Tel Hashomer camptodactyly syndrome that were described in these published articles. We reviewed the dermatoglyphics of 21 available cases out of all of the published and electronically available cases of Tel Hashomer camptodactyly. Eight cases reported whorls to be the most common digital pattern with an expected rise of ridge count. Two cases show significantly high frequencies of arch patterns. Further, there were increased numbers of palmar creases, along with abnormal flexion creases or other palmar dermatoglyphic abnormalities reported in all cases.
CONCLUSION
This review highlighted the desirability of thoroughly observing and recording dermatoglyphic features when reporting on future patients with Tel Hashomer camptodactyly syndrome, in conjunction with carrying out modern molecular methods.
Topics: Abnormalities, Multiple; Biomarkers; Bone Diseases, Developmental; Dermatoglyphics; Fingers; Hand Deformities, Congenital; Heart Septal Defects, Atrial; Hirsutism; Humans; Muscular Diseases
PubMed: 27650795
DOI: 10.1186/s13256-016-1048-7 -
The Journal of Clinical Endocrinology... May 2022The clinical use of dexamethasone (DEX) prenatally to reduce virilization of external genitalia in female fetuses with congenital adrenal hyperplasia (CAH) is efficient...
CONTEXT
The clinical use of dexamethasone (DEX) prenatally to reduce virilization of external genitalia in female fetuses with congenital adrenal hyperplasia (CAH) is efficient but still controversial. It remains challenging to prevent the excessive exposure of DEX in unborn healthy babies during the first trimester of pregnancy.
OBJECTIVE
Since endogenous glucocorticoids contribute to the maintenance of blood pressure (BP) and since events during fetal life may program the fetus and affect future metabolic health, the aim of this study was to analyze ambulatory BP measurements in CAH-unaffected children and adults that were prenatally exposed to DEX treatment.
METHODS
Ambulatory BP measurements were analyzed in 33 (16 female) DEX-treated participants aged 5.1 to 26.3 years (19 participants aged ≤ 18 years) and in 54 (28 female) age- and sex-matched apparently healthy controls aged 5.5 to 25.3 years (27 participants aged ≤ 18 years) with ambulatory normotension.
RESULTS
Participants' age, height, weight, and body mass index were similar between the DEX-treated group and the control group. Heart rate, 24-hour BP, pulse pressure, and nighttime dipping did not statistically significantly differ between DEX-treated participants and controls.
CONCLUSION
Our study suggests that prenatal DEX treatment in CAH-unaffected children and adults does not appear to adversely affect ambulatory BP later in life. Our observations need to be confirmed in larger studies.
Topics: Adrenal Hyperplasia, Congenital; Adult; Blood Pressure; Blood Pressure Monitoring, Ambulatory; Child; Dexamethasone; Female; Glucocorticoids; Humans; Pregnancy; Prenatal Exposure Delayed Effects; Virilism
PubMed: 35148399
DOI: 10.1210/clinem/dgac081 -
Cleveland Clinic Journal of Medicine 1990The most common signs of androgen excess in women are acne, alopecia, and hirsutism. Less common manifestations include android obesity, virilization, and acanthosis... (Review)
Review
The most common signs of androgen excess in women are acne, alopecia, and hirsutism. Less common manifestations include android obesity, virilization, and acanthosis nigricans. These changes appear to be the result of excessive androgen production or increased target organ sensitivity. To evaluate excessive androgen production, an androgen screening protocol is recommended that includes measurement of dehydroepiandrosterone sulfate, testosterone, androstenedione, prolactin, follicular stimulating hormone, and luteinizing hormone. When androgen excess is confirmed, dexamethasone suppression is recommended to determine the source of the androgen(s). Once excessive androgen production is confirmed, more specific therapies can be administered.
Topics: Acne Vulgaris; Alopecia; Androgens; Clinical Protocols; Endocrine System Diseases; Female; Hirsutism; Humans
PubMed: 2142637
DOI: 10.3949/ccjm.57.5.423 -
Acta Dermatovenerologica Alpina,... Sep 2008Hirsutism is an important medical problem affecting about 8% of women. Despite an extensive amount of published work, some aspects of hirsutism are still controversial... (Review)
Review
Hirsutism is an important medical problem affecting about 8% of women. Despite an extensive amount of published work, some aspects of hirsutism are still controversial or understudied. This paper reviews the current data that have been published in recent years on this subject.
Topics: Female; Gonadal Steroid Hormones; Hair Removal; Hirsutism; Hormone Antagonists; Humans
PubMed: 18853073
DOI: No ID Found -
Journal of Neuroendocrinology Dec 2022Polycystic ovary syndrome (PCOS) is the most common endocrinopathy to affect women of reproductive-age world-wide. Hyperandrogenism is both a hallmark feature of PCOS,...
Polycystic ovary syndrome (PCOS) is the most common endocrinopathy to affect women of reproductive-age world-wide. Hyperandrogenism is both a hallmark feature of PCOS, and is hypothesised to be an underlying mechanism driving the development of the condition in utero. With circulating hormones known to profoundly influence maternal responses in females, we aimed to determine whether maternal behaviour is altered in a well-described prenatally androgenised (PNA) mouse model of PCOS. Mouse dams were administered with dihydrotestosterone or vehicle on days 16, 17 and 18 of pregnancy. Maternal responses were assessed in both the dihydrotestosterone-injected dams following parturition and in their adult female PNA offspring. Exposure of dams to excess androgens during late pregnancy had no detrimental effects on pregnancy outcomes, including gestation length, pup survival and gestational weight gain, or on subsequent maternal behaviour following parturition. By contrast, PNA virgin females, modelling PCOS, exhibited enhanced maternal behaviour when tested in an anxiogenic novel cage environment, with females rapidly retrieving pups and nesting with them. In comparison, most control virgin females failed to complete this retrieval task in the anxiogenic environment. Assessment of progesterone receptor and oestrogen receptor α immunoreactivity in the brains of virgin PNA and control females revealed increased numbers of oestrogen receptor α positive cells in the brains of PNA females in regions well known to be important for maternal behaviour. This suggests that increased oestrogenic signalling in the neural circuit that underlies maternal behaviour may be a possible mechanism by which maternal behaviour is enhanced in PNA female mice.
Topics: Animals; Female; Mice; Pregnancy; Androgens; Dihydrotestosterone; Estrogen Receptor alpha; Polycystic Ovary Syndrome; Reproduction; Virilism; Maternal Behavior
PubMed: 36416198
DOI: 10.1111/jne.13206 -
Life Sciences Jun 2015Androgen excess predisposes the organism to develop metabolic-endocrine and reproductive dysfunctions, among them the development of a phenotype resembling that of human...
AIM
Androgen excess predisposes the organism to develop metabolic-endocrine and reproductive dysfunctions, among them the development of a phenotype resembling that of human Polycystic Ovary Syndrome (PCOS).
METHODS
We analyzed the impact of a single neonatal (5day-old) testosterone propionate (TP; s.c. 1.25mg/female pup) dose on: a) several metabolic-endocrine activities and b) ovarian steroidogenic and granulosa cell (GC) functions and also follicular population in juvenile and adult TP and control (CT) rats.
KEY FINDINGS
Compared to CT rats, TP animals were characterized by: a) accelerated growth, hyperadiposity and hyperleptinemia, b) very early (pre-weaning age) vaginal opening, c) hyperinsulinemia in adult life, d) dysfunctional ovarian steroidogenesis, e) conserved GC functionality in both juveniles (in vitro) and adults (in vivo), and f) estrous cycles arrested at estrus. Finally, histological studies of the ovaries indicated that in TP (vs. CT) rats: i) primary and antral follicle frequencies were 3- and 15-fold higher and lower, respectively, in juveniles and ii) secondary and atretic follicle frequencies were 3- and 5-fold lower and higher, respectively, in adults. Large cystic images without corpus luteum were observed in the ovaries from adult TP rats only.
SIGNIFICANCE
Our results strongly suggest that transient neonatal hyperandrogenemia induced early misprogramming of metabolic-endocrine and ovarian (steroidogenesis/folliculogenesis) functions. Conversely, TP rats preserved their ovary GC endocrine function. Our results further support the high risk of developing ovarian hyperstimulation syndrome for infertile women with transient/chronic hyperandrogenemia (PCOS) subjected to assisted reproductive technologies.
Topics: Animals; Animals, Newborn; Disease Models, Animal; Estrous Cycle; Female; Granulosa Cells; Hyperinsulinism; Ovarian Hyperstimulation Syndrome; Ovary; Polycystic Ovary Syndrome; Rats; Rats, Sprague-Dawley; Testosterone Propionate; Virilism
PubMed: 25818182
DOI: 10.1016/j.lfs.2015.03.008 -
Hormones and Behavior May 2009Phoenix et al. (Phoenix, C., Goy, R., Gerall, A., Young, W., 1959. Organizing actions of prenatally administered testosterone propionate on the tissues mediating mating... (Review)
Review
Phoenix et al. (Phoenix, C., Goy, R., Gerall, A., Young, W., 1959. Organizing actions of prenatally administered testosterone propionate on the tissues mediating mating behavior in the female guinea pig. Endocrinology 65, 369-382.) were the first to propose an essential role of fetal testosterone exposure in the sexual differentiation of the capacity of mammals to display male-typical mating behavior. In one experiment control male and female guinea pigs as well as females given fetal testosterone actually showed equivalent levels of mounting behavior when gonadectomized and given ovarian steroids prior to adult tests with a stimulus female. This finding is discussed in the context of a recent, high-profile paper by Kimchi et al. (Kimchi, T., Xu, J., Dulac, C., 2007. A functional circuit underlying male sexual behaviour in the female mouse brain. Nature 448, 1009-1014.) arguing that female rodents possess the circuits that control the expression of male-typical mating behavior and that their function is normally suppressed in this sex by pheromonal inputs that are processed via the vomeronasal organ (VNO)-accessory olfactory nervous system. In another Phoenix et al. experiment, significantly more mounting behavior was observed in male guinea pigs and in females given fetal testosterone than in control females following adult gonadectomy and treatment with testosterone. Literature is reviewed that attempts to link sex differences in the anatomy and function of the accessory versus the main olfactory projections to the amygdala and hypothalamus to parallel sex differences in courtship behaviors, including sex partner preference, as well as the capacity to display mounting behavior.
Topics: Androgens; Animals; Brain; Female; Guinea Pigs; Humans; Male; Mice; Olfactory Pathways; Sex Differentiation; Sexual Behavior, Animal; Virilism; Vomeronasal Organ
PubMed: 19446074
DOI: 10.1016/j.yhbeh.2009.02.008