Premature adrenarche

Pubertet je životno razdoblje na prijelazu iz djetinjstva u odraslu dob. Glavne su karakteristike puberteta pojava sekundarnih spolnih oznaka, ubrzani tjelesni rast i razvoj te postizanje spolne i reprodukcijske zrelosti. Prve spolne oznake u djevojčica pojavljuju se oko 10. godine, a u dječaka oko 12. godine života. Pojava sekundarnih spolnih oznaka prije 8. godine u djevojčica i 9. godine u dječaka označava pojavu preuranjenog puberteta. Preuranjeni pubertet može biti pravi, lažni ili nepotpuni oblik preuranjenoga spolnog razvoja. Pravi preuranjeni pubertet većinom se javlja u djevojčica u kojih je najčešće idiopatski. U više od 50% dječaka uzroci pravoga preuranjenog puberteta su organski. Lažni preuranjeni pubertet uzrokuju razni tumori gonada i nadbubrežnih žlijezda, virilizirajući oblik kongenitalne androgene hiperplazije, uporaba estrogena ili androgena te druge bolesti kao McCune-Albrightov sindrom i testotoksikoza. Lažni preuranjeni pubertet može biti izoseksualan ili heteroseksualan. U nepotpune oblike preuranjenoga spolnog razvoja ubrajaju se prematurna telarha, prematurna menarha i prematurna adrenarha. Prematurna adrenarha je preuranjena pojava pubične i aksilarne dlakavosti i ostalih znakova pojačanoga androgenog djelovanja kao što su odrasli tip tjelesnog mirisa, masna kosa, akne te ubrzani rast i koštano dozrijevanje. Javlja se prije 8. godine u djevojčica, u kojih je češća pojava, i prije 9. godine u dječaka. Etiologija prematurne adrenarhe nije u potpunosti razjašnjena, a uglavnom se objašnjava preuranjenim sazrijevanjem zone retikularis nadbubrežne žlijezde koja dovodi do povećanog lučenja adrenalnih androgena. Dijagnoza se postavlja isključenjem drugih uzroka. U laboratorijskim nalazima karakteristične su povišene koncentracije prekursora adrenalnih androgena DHEA, DHEAS i androstendiona. Klinička slika izraženija je u djevojčica jer je u njih veća periferna konverzija androgenih prekursora u potentnije androgene. Djeca s prematurnom adrenarhom često su pretila i viša od vršnjaka. Prematurna adrenarha povezuje se u neke djece s povećanim rizikom razvoja hiperinzulinizma, dislipidemije, metaboličkog sindroma, kardiovaskularnih bolesti, funkcionalnoga ovarijskog hiperandrogenizma i sindroma policističnih ovarija. Budući da je ovaj poremećaj benignog tijeka, nije potrebna specifična terapija, no potrebno je praćenje i pravovremena prevencija mogućih metaboličkih poremećaja.Puberty is a transitional period of life from childhood to adulthood. The main characteristics of puberty are the appearance of secondary sex characteristics, accelerated physical growth and development, accompanied by sexual maturation and reproductive ability. The first sex characteristics appear around the age of 10 in girls, and around the age of 12 in boys. The appearance of secondary sexual characteristics before the age of 8 in girls and the age of 9 in boys indicates the onset of precocious puberty. Precocious puberty can be classified as central, peripheral and as an incomplete form of precocious pubertal development. The central precocious puberty mostly occurs in girls among whom the etiology is mainly idiopathic. Among boys, in more than 50%, the causes of central precocious puberty are pathological. Peripheral precocious puberty is caused by various tumours of gonads and adrenal glands, the virilizing form of congenital adrenal hyperplasia, use of estrogen or androgen and other rare disorders such as McCune-Albright syndrome and testotoxicosis. Peripheral precocious puberty can be isosexual or heterosexual. Premature thelarche, premature menarche and premature adrenarche are incomplete forms of precocious pubertal development. Premature adrenarche is a premature appearance of pubic and axillary hair and other signs of increased androgenic action such as adult-type body odour, oily hair, acne, accelerated growth and bone maturation. It occurs before the age of 8 in girls, in whom it is more common, and before the age of 9 in boys. The etiology of premature adrenarche is not fully understood and is mainly explained by premature maturation of the adrenal reticular zone, which leads to increased secretion of adrenal androgens. Diagnosis of premature adrenarche requires exclusion of other causes of androgen excess secretion. Levels of adrenal androgen precursors DHEA, DHEAS and androstenedione are elevated. The symptoms are found more often among girls due to a higher peripheral conversion of the androgenic precursors to more potent androgens. Children with premature adrenarche are often obese and taller than their peers. In some children, it can lead to development of hyperinsulinism, dyslipidemia, metabolic syndrome, cardiovascular disease, functional ovarian hyperandrogenism and polycystic ovarian syndrome. Because of the benign nature of this disorder, no specific therapy is required, but children should be followed-up in order to prevent the development of metabolic complications