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Long-term urologic and gynecologic follow-up in anorectal anomalies: The keys to success
Published in Alejandra Vilanova-Sánchez, Marc A. Levitt, Pediatric Colorectal and Pelvic Reconstructive Surgery, 2020
Geri Hewitt, Daniel G. DaJusta, Christina B. Ching
A 16-year-old patient with previously repaired imperforate anus with rectovestibular fistula presents to the emergency department with abdominal pain. She has not yet had her first period and has never been sexually active. She denies any urinary or GI symptoms. She has regular bowel movements without soiling. The patient underwent thelarche and adrenarche approximately 3 years ago. Her mother notes increasing lower abdominal pain over the past couple of months that appears to be cyclic in nature. She is afebrile and has normal vital signs. Her abdomen is soft, with normal bowel sounds, with fullness and tenderness in the lower abdomen.
Pubertal Development and Menarche
Published in Jane M. Ussher, Joan C. Chrisler, Janette Perz, Routledge International Handbook of Women’s Sexual and Reproductive Health, 2019
Another pubertal process is the activation of the hypothalamic-pituitary-adrenal (HPA) axis. This process, known as adrenarche, involves the maturation of the adrenal glands and their production of certain androgens (Dorn & Biro, 2011). Adrenarche begins in children aged 6–8, although no outward signs of the process appear until later in puberty when increased androgen levels result in observable pubic hair growth (i.e., pubarche), other ancillary body hair, body odor, and perhaps acne (Abrue & Kaiser, 2016; Dorn & Biro, 2011). Typically, pubarche occurs before thelarche, and menarche occurs last. However, variations in this sequence also occur, such as thelarche before pubarche, or pubarche and thelarche appearing together (Biro, Huang, Daniels, & Lucky, 2008). In studying peripubertal girls, Biro et al. (2014) found that hormonal changes related to adrenarche occurred before those associated with gonadarche, suggesting that two pathways may represent the onset of puberty.
Pubertal abnormalitiesPrecocious and delayed
Published in Joseph S. Sanfilippo, Eduardo Lara-Torre, Veronica Gomez-Lobo, Sanfilippo's Textbook of Pediatric and Adolescent GynecologySecond Edition, 2019
Adrenarche refers to the process of androgenic maturation, beginning with increasing production of the adrenal hormones, androstenedione and DHEA-S, and culminating with pubarche, the appearance of axillary and pubic hair, as well as increased oiliness of the scalp and skin, apocrine body odor, and potential for acne. Pubarche usually but not always occurs after thelarche. In contrast to adrenarche and pubarche, virilization describes abnormal androgen production leading to clitoromegaly, muscle enlargement, and hirsutism. Normal female puberty does not include hirsutism, clitoromegaly, deep voice, and/or muscular hypertrophy. A cause for virilization should always be sought, considering exogenous androgen exposure, congenital adrenal hyperplasia, mixed gonadal dysgenesis, other genetic disorders previously unrecognized in the child's life (such as 5-α reductase deficiency) or ovarian, adrenal, or rare hepatic tumors. Virilization can decrease reproductive potential.
The First Ejaculation: A Male Pubertal Milestone Comparable to Menarche?
Published in The Journal of Sex Research, 2020
Physiological events that occur at puberty are typically triggered by hormonal patterns. For instance, the events of adrenarche are triggered by androgens released from the adrenal cortex (Parker, 1991), gonadarche is triggered by pituitary gonadotropins (Abreu & Kaiser, 2016), and menarche is triggered by a series of hormonal processes ultimately leading to the shedding of the uterine lining (Karapanou & Papadimitriou, 2010). Does thorarche also have an explicit biological trigger? Hormones do affect the onset of ejaculation, and treating adolescent boys with testosterone has been found to increase the occurrence of nocturnal emissions (Finkelstein et al., 1998). However, hormones are not a direct trigger of thorarche, as boys can voluntarily choose to induce a first ejaculation themselves, pursuant to Tanner’s notion that culture can influence its timing. Nonetheless, thorarche does still occur in the absence of voluntary ejaculation, typically through a nocturnal emission, and some boys have reportedly been surprised to accidentally experience a first ejaculation while awake without the intention of masturbation (Kinsey, 1948). Thus, there is evidently some sort of innate biological mechanism to ensure ejaculation occurs in adolescence even in the absence of cultural influences.
Drug design strategies for Cushing’s syndrome
Published in Expert Opinion on Drug Discovery, 2019
S. A. Usanov, A. V. Kliuchenovich, N. V. Strushkevich
Steroid biosynthesis is an essential pathway with different tissues and a vast array of structurally similar compounds involved; therefore, the consequences of affecting a certain target might be unpredictable. Rational drug design based on crystal structures is the only strategy for the development of effective GR-antagonists and CYP11B1-inhibitors. The most promising choice is to explore the alternative/allosteric site of the receptor (alternative to cortisol binding site) or the steroidogenic enzyme (alternative to the active site of CYP11B1). Due to structure similarity of glucocorticoids and other steroid hormones, it is also important to pay attention to the potential adverse effects due to off-target activity. These similarities can also lead to undesirable metabolism, which is why non-steroidal compounds area more appropriate for GRs antagonists or CYP11B1 inhibitors. On a pre-clinical stage, differences in steroidogenesis as well as with the function of certain hormones in humans and animals also needs to be considered. Specifically, there is a significant difference in the catalytic activity profile of CYP11Bs and CYP17 in animals [68,69]. Moreover, human primates appear to be unique amongst mammals in that they have an adrenarche – an endocrine developmental process associated with age-dependent changes in adrenal steroidogenesis [67]. Therefore, the careful selection of any experimental in vivo model is extremely important to achieve success when translating to humans.
Increased symptoms of anxiety and depression in prepubertal girls, but not boys, with premature adrenarche: associations with serum DHEAS and daily salivary cortisol concentrations
Published in Stress, 2018
Chrisanthi Marakaki, Panagiota Pervanidou, Ioannis Papassotiriou, George Mastorakos, Ze’ev Hochberg, George Chrousos, Anastasios Papadimitriou
Premature adrenarche (PA) is defined as the early appearance (before the age of 8 years in girls and 9 years in boys) of clinical signs of androgen activity (pubic and/or axillary hair, adult-type body odor, oily hair, and acne) and it may be accompanied by an increase in serum adrenal androgen levels, mainly DHEAS. The etiology of PA is multifactoral. Environmental factors (such as intrauterine growth restriction and obesity) and genetic factors (genes encoding steroidogenic enzymes, insulin-IGF signaling, and androgen receptor sensitivity) have been associated with PA. Pituitary ACTH is essential for adrenal androgen production, as evidenced by the absence of adrenarche in children with hypopituitarism and in patients with ACTH receptor defect (Voutilainen & Jaaskelainen, 2015). Exaggerated reactivity of HPA axis has been proposed to account in part for the development of adrenal hyperandrogenism in PA girls (Cizza et al., 2001). However, ACTH is not considered to be the trigger for adrenarche and its initiating mechanisms remains at least partly obscure.