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Nevus comedonicus (NC) is a rare type of organoid epidermal nevus with comedones and inflammatory cysts 1 . It may be associated with extracutaneous manifestations (cataract, neurological and skeletal anomalies), defining NC syndrome 2 . Acne nevus, a clinically similar mosaic skin condition, is related to FGFR2, whereas postzygotic NEK9 mutations have been identified in four NC patients so far, including only one with NC syndrome 3 4 . Here we report on novel clinical findings, fibrous dysplasia and premature puberty in a case of NC syndrome associated with a NEK9 mutation.

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.

Sekularne promjene određuju rast i razvoj. Pokazatelji zdravstvenog stanja jedinke i cjelokupne populacije su rast i razvoj. S godinama ljudska vrsta postaje sve viša. Porast visine i tjelesne mase, odnosno ranije sazrijevanje djece prateći nekoliko generacija označavaju pozitivne sekularne promjene, te se javlja u razvijenim zemljama. Uzroci sekularnog trenda su brojni, među kojima su najvažniji bolja ishrana, rjeđa obolijevanja, bolja zdravstvena zaštita i bolji životni uvjeti. Visina odraslih osoba veća je za oko 10 cm nego prije jednog stoljeća, i sama djeca su u svim uzrasnim grupama viša, teža i zrelija u odnosu na svoje vršnjake prije 20 i više godina. Početkom dvadesetog stoljeća se raslo do 25 godine, dok se sada rastenje u visinu završava s 18-20 godina. Ostaje pitanje, jesu li i u kojoj mjeri današnje generacije uspjele da postignu svoj genetski maksimum rasta i razvoja, te kakvi se trendovi mogu očekivati u budućnosti. Odličan pokazatelj zdravstvenog stanja pojedinca je primjeren rast i razvoj, ali i cjelokupne promatrane populacije. Rast i razvoj određeni su djelovanjem nasljednih faktora i brojnih faktora okoline. Sekularne promjene rasta uglavnom su pozitivne, ali i mogu biti i negativne ovisno o djelovanju nepovoljnih uvjeta u kojima dijete odrasta. Sekularne promjene rasta zahtijevaju kompleksno proučavanje pojedinih segmenata linearnog rasta, mehanizama njihove kontrole. Također tijekom posljednjih desetljeća pozornost privlači i sekularni porast učestalosti pretilosti već u dječjoj i adolescentnoj dobi. Sekularne promjene mogu biti dobar temelj za razvoj psihosocijalnih poremećaja, metaboličkog sindroma, dijabetesa tipa 2, kardiovaskularnih bolesti, karcinoma dojke, a otvaraju i pitanje redefiniranja prijevremenog puberteta, te ih je zbog toga potrebno pratiti., Secular changes determine growth and development. Indicators of the health status of individuals and the overall population are growth and development. With years the human beings becomes ever higher. Increase in height and body mass, or premature childhood upbringing of several generations that indicate positive secular changes, and occurs in developed countries. The causes of the secular trend are numerous, among which the most important are better nutrition, rivers disease, better health care and better living conditions. The height of the adult was greater by about 10 cm before it was one part, and the children themselves in all ages were taller, heavier and more mature than theirs 20 years ago. At the beginning of the twentieth century it grew to 25 years, while now growing to height ends with 18-20 years. The question remains whether the current generations have succeeded in achieving their genetic maximum growth and development and what trends can be expected in the future. An excellent indicator of the health status of individuals is appropriate growth and prosperity, but also the overall population observed. Growth and development are determined by hereditary factors and numerous environmental factors. Secular changes are growing mostly positive, but they may also have a negative dependence on adverse conditions. Secular growth changes require complex study of individual linear growth segments, mechanisms of their control. Over the last decades, attention has also been drawn to the secular increase in obesity rates in children and adolescents. Secular changes can be a good basis for the development of psychosocial disorders, metabolic syndrome, type 2 diabetes, cardiovascular diseases, breast cancer, and the question of redefining premature puberty is also being introduced and should therefore be monitored.

The prevalence of obesity in female children in the Unites States has increased from 5% to 17.1% from 1965 to 2015 and the prevalence of premature puberty has increased as well. Increased weight is correlated with premature puberty in females and interestingly, different studies have shown that there has been a gradual decline of age for onset of puberty. Early sexual maturation in female children are associated with many negative consequences such as increased risk of adult obesity, type 2 diabetes, increased aggression, increased adolescent risk taking behaviors, increased sexual activity, and breast cancer. The target population for this study is obese female children in the United States in the past 5 years. Using the primary data from the National Health and Nutrition Examination Survey (NHANES), the specific study population of obese females aged 5 through 17 years in the United States recorded during the period January 2013 - December 2014. The results of this study also showed a mean BMI of 21.1 which was higher than the 2015 average of 17.1 while a mean age of puberty was found to be 11.3 which is higher than the 2015 average of 9. The strength of correlation was found with Spearman’s Rho. In this study, the results have shown a contrasting view and have shown a negative weak association between the two variables with a rho value of -.089. Efforts towards a reduction of obesity is expected to yield a lowered incidence of premature puberty.&nbsp

Estrogens are a group of sex hormones. The entry of this hormone into the human body causes premature puberty, breast, and uterus cancer, etc. Due to sustainability, harmful effects, and its skin absorption, the identification of this hormone in swimming pools is a must. The carrying out of this research was based on estrogen tracking from a collection of four swimming pools in various days of the week: exclusively masculine, exclusively feminine, and those used by both men and women alternatively. Estrogen and the remained chlorine of raw water of the pool inlet as well as water inside the pool were measured during hot and cold seasons of the year. The measurements of the samples were done using electrochemiluminescence apparatus. The sampling results show a significant increase in the pools relative to filling water. Also, the results verify that the average increase in estrogen concentrations in the feminine pool was 17.95 pg/ml, while the increase in the masculine was 8.96 pg/ml. The maximum estrogen of the feminine pools at 30.8 pg/ml occurred in hot seasons due to more usage. According to the obtained results, swimming pools have considerable level of estrogens. It needs more studies about the health risks of these levels of estrogen in swimming pools.

Puberty is an important process that providers of health care to children and adolescents should be comfortable discussing. The normal process of puberty is complex and involves many different hormonal pathways. A clear understanding of these pathways will help providers counsel patients on what to expect as they anticipate and progress through puberty as well as be alerted when puberty is not progressing normally. Both early and late puberty can have physical and psychological implications for the pediatric population. Being familiar with the common causes and initial testing of abnormal puberty will allow the primary care provider to monitor appropriately and initiate further investigation if warranted. This article reviews both the typical pubertal pathway as well as delayed and premature puberty and their common causes. [ Pediatr Ann . 2019;48(4):e141–e145.]

Studies suggest a relationship between exposure to endocrine disrupters, such as mercury (Hg), and premature puberty. Hg exposure from Thimerosal-containing hepatitis B vaccine, administered at specific intervals within the first six months of life, and the child&rsquo, s long-term risk of being diagnosed with premature puberty (ICD-9 code: 259.1), was retrospectively examined, using a hypothesis-testing, longitudinal case-control design on prospectively collected data, in the Vaccine Safety Datalink (VSD). Cases diagnosed with premature puberty were significantly more likely to have received increased exposure to Hg from hepatitis B vaccines preserved with Thimerosal given in the first month after birth (odds ratio (OR) = 1.803), first two months after birth (OR = 1.768), and first six months after birth (OR = 2.0955), compared to control subjects. When the data were separated by gender, the effects remained among females but not males. Female cases, as compared to female controls, were significantly more likely in a dose-dependent manner to have received a greater exposure to Hg from hepatitis B vaccines preserved with Thimerosal, given in the first six months after birth (OR = 1.0281 per &micro, g Hg). The results of this study show a dose-dependent association between increasing organic Hg exposure from Thimerosal-containing hepatitis B vaccines administered within the first six months of life and the long-term risk of the child being diagnosed with premature puberty.

Government officials in China have tested samples of a locally-produced baby formula for hormone levels after a child of 4 months old was reported to have grown breasts.

Last year we reported observations on the treatment of undescended testes with an anterior pituitary-like principle.2 We were able to produce descent in only 19 per cent of the cases, in contrast to an average of 70 per cent of successful results of other workers. However, we did observe that this material, by stimulating genital growth, seemed to make operative procedures less difficult, and therefore appeared to be valuable preceding surgery, whether it produced descent or not. These observations have now been extended and confirmed. Our incidence of successful results has remained about the same, being now 22 per cent instead of 19 per cent. However, an important development has taken place. Premature puberty has been produced in young boys by the prolonged administration of this material. This marked stimulation of genital growth has led to the application of the APL principle to the treatment of hypogenitalism in the male with very satisfactory results.

In recent years numerous reports concerning the treatment of cryptorchidism and hypogenitalism with the use of various gonadotropic hormones have appeared in the literature. Most reports have dealt with the use of gonadotropic hormones either from pregnancy urine, placenta, or beef pituitary and have been used chiefly for the treatment of the afore-mentioned conditions in young males. Recently, Thompson (1) and his associates have reported on the production of precocious puberty and hypertrophy of the genitalia in males receiving gonadotropic hormone for the treatment of cryptorchidism. In their cases, however, these conditions were produced only after large doses of hormone had been administered over long periods. In our own experience (2) covering a period of over 6 years, during which time we have treated a considerable number of males with hypogenitalism, some with and some without cryptorchidism, we have not observed the development of premature or precocious puberty or abnormal hypertrophy of the ge...

In order to accelerate epiphysial maturation, 7 prepubertal girls who were of “excessive” height and whose parents were 178 cm or taller were given estradiol valerate, 10–20 mg 3 times monthly, over a period of one year or longer. Growth at the end of the first year averaged 10.4 cm, weight gain averaged 11.04 kg, and the bone age advanced 30 months. The average growth from the end of the first year of treatment until complete epiphysial closure was 6.3 cm. These data indicate that the treatment of excessive height in girls by the induction of premature puberty with estradiol valerate is of value. They also indicate that endogenous estrogens are responsible for the prepubertal growth and weight spurts, and at this time are, therefore, powerful anabolic agents.

We report the isolation and characterization of a steroid, 1,4-androstadiene-3,17-dione from the urine of a 15-yr-old boy with a history of epileptic convulsions and premature puberty. To our knowledge, this is the first report of detection of this steroid in human urine. A substance from the urine of an epileptic boy, under prolonged treatment with diphenylhydantoin and phenobarbital, was characterized and shown to be 1,4-androstadien-3,17-dione. This characterization depended preliminarily on examining the behaviour of the substance and its derivatives on thin-layer, paper and gas Chromatographie systems, and conclusively by physico-chemical analysis of its spectra in U.V., I.R., circular dichroism, nuclear magnetic resonance, and gas chromatogram mass spectrometer. The question of whether the appearance of this substance in urine was caused by the therapeutic handling or whether this steroid is a true intermediate during aromatization of testosterone and androstenedione is discussed.

Early puberty in 28 children (23 girls, five boys) treated for acute lymphoblastic leukaemia (ALL) at a mean age of 4.0 years (range 1.4-7.8) is described. All but one had received prophylactic cranial irradiation (1800-2400 cGy) and three children had received additional cranial or craniospinal irradiation as treatment for relapse of their leukaemia. Mean age for the onset of puberty was 8.8 (SD 0.8) years in the girls and 9.3 (0.8) years in the boys; this is greater than two standard deviations from the mean for normal girls and boys. Five children (three girls, two boys) had precocious puberty. The onset of puberty occurred at greater than two standard deviations from the mean for normal girls and boys in 14(13%) girls and 4(3%) boys treated at less than eight years of age between 1970 and 1985. In a group of 55 girls treated for ALL who had survived in first remission for six years or more from diagnosis, there was a relation between young age at onset of treatment and early menarche. We suggest that premature activation of the hypothalamic-pituitary-gonadal axis occurs as a consequence of hypothalamic dysfunction due to cranial irradiation. Precocious and premature puberty in children treated for ALL may be an important factor in contributing to short stature.

In a recent communication toThe Journalwe1demonstrated that the anterior pituitary-like principle from the urine of pregnant women may produce such marked stimulation of genital growth in young boys that a syndrome resembling premature puberty results. The present paper is concerned with the influence of this material on undescended testes. Since Schapiro's2report eight years ago, the anterior pituitary-like principle has been used extensively in the treatment of this condition. In spite of numerous observations in other clinics showing a high incidence of successful results, we have consistently been unable to produce descent in most instances of true cryptorchidism.3In order to explain this discrepancy a critical survey of the problem is desirable. An analysis of the results of other observers4is given in table 1. It may be seen that there were 860 undescended testes in 579 patients. The condition was bilateral in

Rapidly accumulating evidence clearly shows that the anterior pituitary-like principle from the urine of pregnant women exerts a profound influence on the growth, and function of the male genitalia. It may produce an increase in the size of the penis, scrotum and prostate in both man1and animals2before the age of puberty. The consensus is that in the testis it influences primarily the interstitial cells,3although there is some evidence that the seminiferous tubules may also be affected.4Some increase in the size of the testes has been produced in rats5and monkeys.6In the course of treating patients for undescended testes we have been struck more by its influence on the growth of the genitalia than by its influence on the descent of the testis.7It will be shown that this stimulation of genital growth may be so marked that changes simulating premature puberty are produced.

Considerable attention has been focussed on the presence and possible significance of the estrogenic factor in certain ovarian and other tumors,1 in adenomata of the breast,2 and in fibroids of the uterus.3In previous publications we have emphasized the presence of the estrogenic factor in the follicle fluid, in certain cysts of the ovary, in malignant tumors of children in which premature puberty had occurred.In none of these reports have control tissues been examined except the liver. We have now assayed the psoas muscle of 4 normal cyclical females and have there found the presence of large amounts of estrogenic factor varying in the different cases from 800 to 8000 M.U. kg. when calculated to a dry weight as Lewis and Geschickter have done. In one case, where the material was obtained at autopsy shortly after death from embolism, the psoas muscle titered 2000 M.U. kg., the normal uterus, 8000 M.U. kg. (intermenstrual).Therefore it appears unwarranted to base general conclusions on the mere presence of...

In April 1937 Albright and his co-workers in Boston reported 5 cases in which were presented the bizarre concurrent features of osteitis fibrosa cystica, areas of pigmentation of the skin and endocrine dysfunction, with premature puberty in the female patients. Although 12 similar cases had previously been reported in the literature under a variety of titles, beginning with Weil's report in 1922, the paper of Albright and his associates was the first to establish this peculiar syndrome as a clinical entity distinctly separate from, rather than an aberrant type of, one of the other known diseases affecting bone. For this reason and because there is no simple term to encompass all the prerequisite elements, in the "Standard Classified Nomenclature of Disease" the term "Osteitis Fibrosa Cystica (Albright)" has been adopted to identify this particular disease picture. CHARACTER OF THE SYNDROME The osseous disorder in this group of cases is one

The effect of supernormal size and strength on the age at which the onset of walking occurs has often been discussed, but no factual evidence relative to the question has ever been adduced. In that connection, the cases of most extreme development are probably the most important, since the greater in excess of normal is the bodily development of the child the greater might be expected to be its effect on the age at which walking begins. The instances of most advanced physical growth in the first and second years of life are undoubtedly those rare cases in which puberty is attained during this period. It has been known since the time of Geoffroy-Saint-Hilaire that premature puberty is preceded and accompanied by an acceleration of skeletal development and of ossification. This is amply shown by the literature on precocious maturity. Smith, in 1822, reported the case of a boy who