Assessment of Skeletal and Sexual Maturity: Theoretical and Practical Aspects

Total Page:16

File Type:pdf, Size:1020Kb

Assessment of Skeletal and Sexual Maturity: Theoretical and Practical Aspects Clin Pediatr Endocrinol 1993; (Suppl 3):15-33 Copyright (C)1993 by The Japanese Society for Pediatric Endocrinology Assessment of Skeletal and Sexual Maturity: Theoretical and Practical Aspects Milo Zachmann Department of Pediatrics, University of Zurich, Kinderspital, Ziirich, Switzerland Abstract. Physical changes of sexual maturation and their clinical relevance are discussed in the first part, and the timetable of development in Swiss girls and boys as established from the Zurich longitudinal study is presented. The second part deals with changes in body size and shape and aspects of skeletal maturation. Prepubertal height is nearly identical in both sexes. During puberty important sex differences become evident and maturation is faster in girls by about 2 years. Puberty bigins at the same stage of skeletal maturation (sesamoid bone) in both sexes, and milestones (e.g. peak height velocity) are reached at the same degree of maturation. For bone age estimation, the Greulich and Pyle method is frequently used, but depending on the situation, that of Tanner et al. may be more useful. For height prediction, the methods of Bayley and Pinneau, Roche et al. and Tanner et al. are most widely used. None of them is always the most accurate one, but depending on the condition and relation between bone and chronologic age, the criteria for selection are discussed. Sex hormones modulate body proportions but have no influence on adult height. Adult height and velocity of maturation are independent multifactorial variables. The sex difference in adult height (12.5cm in Switzerland) is due to several components. Puberty is also characterized by changes in body composition. Lean body mass increases early in boys and girls. In girls, it peaks at menarche and then diminishes. The amount and distribution of water increases in boys and decreases in girls secondary to the greater accumulation of fat in girls. Hormonal changes during puberty are discussed in the last part. Basal gonadotropin levels increase, FSH more in girls than in boys, LH about equally in both sexes. More important is the advent of rhythmic, pulsatile gonadotropin secretion. The most obvious changes are the increas- ing secretion rates of sex steroids. To avoid extreme variation of normal values, estradiol and testoserone should be related to bone age and/or pubertal stages rather than to chronologic age. The role of DHEA in puberty is unclear. It increases before puberty at adrenarche. Also growth hormone, its binding protein, and IGF I and its binding protein 3 increase. Finally, synergistic metabolic effects between GH and sex hormones with examples using the stable isotope 15N are discussed. Key words: puberty, growth, bone age, height prediction Puberty is, apart from the fetal period, the time during human life where the most Correspondence: Milo Zachmann, M.D. Professor of Pediatric Endocrinology Department of Pediatrics University of Zurich Kinderspital Steinwiesstr. 75 CH-8032 Ztirich Switzerland 15 16 ZACHMANN dramatic somatic changes take place. If one only occurs before menarche. It can now be looks at the simplest thing, the weight of easily studied by echography. different organs, it can be seen how tremen- One of the first signs of puberty in girls is dous these changes are. Compared with the the onset of breast development. The physical age of 10 years, the body weight, the anterior characteristics of the stages of breast develop- pituitary and the pancreatic islets about dou- ment have been outlined by Tanner and his ble during this period, the thyroid triples, the group [5,6]. Many clinicians combine breast ovaries increase five-, and the testes even and pubic hair standards into one staging; about 20-fold. however, it seems to be important to stage In the following, first a short account will breast and pubic hair development separately be given of the physical changes of sexual to be able to determine if there is a discrep- maturation and their clinical relevancel. [1]. ancy between the two stages, as can be the In the second part, a short review of aspects of case e.g. in excessive adrenal androgen secre- growth and skeletal maturation will be given tion such as in late-onset congenital adrenal and finally, hormonal changes that take place hyperplasia. Breast development is mainly during pubery will be briefly discussed. regulated by ovarian estrogen secretion, but pubic hair development is stimulated by adrenal androgen secretion. The stages of Female secondary sex characteristics breast development are individually variable: In girls, secondary sexual development some adult women never progress beyond includes the enlargement of the ovaries, stage IV, and some girls progress directly from uterus, vagina, labia, and breasts and the stage III to stage V. Unilateral breast develp- growth of pubic hair [2,3]. The vagina begins ment is normal in early puberty and may to increase in length before the appearance of persist for several months before the other secondary sex characteristics and continues to breast bud appears. Marked or prolonged enlarge until menarche. The cytologic developmental asymmetry, however, may changes of the vagina occur as a result of reflect estrogen insensitivity of the breast tis- estrogen stimulation. The vaginal epithelium sue, prior trauma, or underdevelpment of the undergoes a transformation from parabasal to underlying pectoral muscle. Adolescent girls about 50% intermediate cells. At approximate- are often concerned about breast size, think- ly one year before menarche, the vaginal pH ing their breasts might be either too small or converts from neutral to an acidic environ- too large, but there are no normal standards ment secondary to the presence of lactobacilli. for size. Height, weight, level of estrogen and With puberty the size of the mons pubis progesterone once in the normal adult range, increases by fat accumulation and the labia or breast size of other women in the family majora become larger. The surfaces of the does not have a high correlation with amount labia develop fine wrinkles. With great indi- of breast tissue, but overall non-gladular vidual variation, the clitoris also increases in breast size is somewhat influenced by weight. size [4]. Concerning the uterus, it has no flex- Experience with tall girls treated with ion during childhood. In the prepubertal high doses of estrogens to reduce final adult period, the corpus increases only slightly, height also shows that breast size is not direct- accounting for about one third of the com- ly related to the quantity of circulating bined weight of cervix and corpus. The estrogens, because in these girls breast size is growth of the uterus during early puberty is not larger than in normal untreated girls [7]. primarily caused by the enlargement of the The age at which each stage of breast myometrium. The endometrium does not development occurs has been determined in begin to develop until after the onset of secon- longitudinal studies in American, British and dary sexual characteristics, and rapid increase Swiss girls [3,6,8]. The complete process of 17 Skeletal and Sexual Maturation breast development, from the appearance of cycles in 12- to 14- year-old girls are anovular, stage II to the attainment of stage V, takes an whereas at the age of 21 years, 60% to 80% of average of 4 years, with a range of 1.5 to 9.0 the cycles are ovular. years. As far as pubic hair is concerned, the Some voice change occurs in pubertal sequence in girls generally begins with the girls, but unlike in boys, may consist more of appearance of breast buds and linear growth an alteration in quality than in pitch [9]. A acceleration but a number of normal girls time plan in girls has been established from develop pubic hair before breast enlargement the Swiss study by Largo and Prader [8]. has occurred. The age at which each stage of pubic hair development occurs has also been determined in longituidinal studies. Marshall Male secondary sex characteristics and Tanner [6] used photographs to study Secondary sexual development in boys pubic hair growth so the ages at which sub- includes genital development, testicular vol- jects were said to reach stage II are somewhat ume, pubic and axillary hair growth and later than in other studies. The mean duration change of voice. The sequence of pubertal of time to progress from stage II to stage V development of the external genitalia in most pubic hair is 2.5 years, with a range of 1.4 to 3. normal boys begins with testicular enlarge- 1 years. There is a high correlation between ment. The changes at puberty in boys are breast and pubic hair stages for black and primarily the result of androgen secretion by white American girls [3]. Black girls are more the testes. Adrenal androgens account, if at advanced than whites for each chronologic all, for a small part of very early pubic hair age and girls in Western regions of the United only. Again, it is better to describe the genital States lagged slightly behind girls from other stage separately from the pubic hair stage. regions in pubertal development. Cross-sectional data of testicular volume Axillary hair occurs at a mean age of 13.1 are not very valuable, because they show a years in American girls, 12.5 years in British very wide distribition. A testicular volume of girls, and somewhere in beween in Swiss girls. about 8ml may still be normal. In this context, Axillary hair takes approximately 15 months it has to be said that the inexperienced exam- from first appearance to adult distribution. iner using Prader's orchiometer generally The apocrine glands of the axilla and vulva obtains too high volumes. Although measure- start to function at about the same time as ment of tesicular volume with this instrument hair appears in these regions.
Recommended publications
  • Module 3 Basics of Growing Up- Understanding Adolescence
    Module 3 Basics of Growing up- Understanding Adolescence Module 3: Basics of Growing up – Understanding Adolescence FLOW CHART Content Flow at A Glance Module 3: Basics of Growing Up – Understanding Adolescence Subject/topic/activity Objective Page No. Exercise on understanding the To understand the physical 3-3 to 3-5 physical changes of changes that take place during adolescence. adolescence and to know the reasons for them. Exercise on cognitive and To know and become aware of 3-6 to 3-8 emotional changes during the emotional and cognitive adolescence. cha nges that take place during adolescence. Exercise on understanding To become aware of sex and 3-9 to 3-10 ‘Sex’ and ‘Sexuality’. sexuality. Exercise on sexual maturity. To identify the sexual organs in 3-11 to 3 -13 the body Exercise on sexual maturity. To understand the prevalent 3-14 to 3 -17 beliefs on sex and sexuality among the participants. Exercises on nutrition during To understand the eating habits 3-18 to 3 -22 adolescence. of young people. To become aware of the consequences of unhealthy eating. To become aware of the food that is healthy and nutritious. 3-1 Module 3: Basics of Growing up – Understanding Adolescence Module 3 Basics of Growing Up – Understanding Adolescence “I – I hardly know sir, just at present at least I know who I was when I got up this morning, but I think I must have been changed several times since….” Alice in Wonderland I Introduction s one grows up, one experiences many changes. There are changes in the body; in the way one behaves and the way others expect one to be.
    [Show full text]
  • Understanding the Physical Changes of Puberty Maria R
    G1701 Understanding the Physical Changes of Puberty Maria R. de Guzman, Extension Adolescent Specialist 1) Maturation of secondary sex characteristics. Secondary Understanding the Physical Changes of Puberty sex characteristics are those that are related to, but not explains what puberty is and why it occurs, how a directly involved in reproduction. For instance, young body changes and strategies to help youth • As early as age 8, girls experience “breast budding” or through puberty. the emergence of breasts. Hair growth under the arms and pubic areas also begin. Physical changes during the adolescent years are dra- • As early as age 11, boys experience growth in the testicular matic — with teens seemingly growing inches overnight, area, and start facial, underarm and pubic hair growth. their bodies re-shaping and voices changing all at once. This 2) The growth spurt. During puberty, hormones signal the transformation is complex, sometimes confusing, and even body to grow faster. This hastened growth is called “the anxiety provoking. Understanding this process of change, growth spurt.” Some things to note: and knowing what to expect can help prepare youth and their • The growth spurt starts at around age 11 for girls and parents for the transitions ahead. This NebGuide provides read- 13 for boys. Thus, girls become generally taller than ers with basic facts about the changes that teens go through boys during these years, though boys often catch up during puberty. It also provides parents with suggestions on and generally end up taller. how to help their developing adolescent through this period • At the peak of the spurt, boys can increase their height of physical transitions.
    [Show full text]
  • Ancestor`S Tale (Dawkins).Pdf
    THE ANCESTOR'S TALE By the same author: The Selfish Gene The Extended Phenotype The Blind Watchmaker River Out of Eden Climbing Mount Improbable Unweaving the Rainbow A Devil's Chaplain THE ANCESTOR'S TALE A PILGRIMAGE TO THE DAWN OF LIFE RICHARD DAWKINS with additional research by YAN WONG WEIDENFELD & NICOLSON John Maynard Smith (1920-2004) He saw a draft and graciously accepted the dedication, which now, sadly, must become In Memoriam 'Never mind the lectures or the "workshops"; be Mowed to the motor coach excursions to local beauty spots; forget your fancy visual aids and radio microphones; the only thing that really matters at a conference is that John Maynard Smith must be in residence and there must be a spacious, convivial bar. If he can't manage the dates you have in mind, you must just reschedule the conference.. .He will charm and amuse the young research workers, listen to their stories, inspire them, rekindle enthusiasms that might be flagging, and send them back to their laboratories or their muddy fields, enlivened and invigorated, eager to try out the new ideas he has generously shared with them.' It isn't only conferences that will never be the same again. ACKNOWLEDGEMENTS I was persuaded to write this book by Anthony Cheetham, founder of Orion Books. The fact that he had moved on before the book was published reflects my unconscionable delay in finishing it. Michael Dover tolerated that delay with humour and fortitude, and always encouraged me by his swift and intelligent understanding of what I was trying to do.
    [Show full text]
  • Sex Education As Viewed by Islam Education
    European Journal of Scientific Research ISSN 1450-216X Vol. 95 No 1 January, 2013, pp.5-16 © EuroJournals Publishing, Inc. 2012 http://www.europeanjournalofscientificresearch.com Sex Education as Viewed by Islam Education Mamdouh M. Ashraah Faculty of Educational Sciences / Hashemite University Ibrahim Gmaian Faculty of Educational Sciences / Hashemite University Sadeq Al-shudaifat Faculty of Educational Sciences / Hashemite University Abstract This study aimed to study sex education in light of Islamic Education views. It sought to answer the following questions: 1) How does Islam Education view toward sex education? 2) Is the concept of sex education in Islam Education different from that in western cultures? 3) How does Islam view sex in different development stages? 4) What are sex education's main aspects as seen by Islam Education? The study concludes that Islam Education did not neglect sex education. On the contrary, much attention and direction has been provided for in accordance with its teaching and regulations. The study also concludes that Islam Education viewed libido as being coherent with human nature, indicated ways in which it can be achieved and explored that are different from those in western cultures, and provided precautionary and remedial plans to deal with sexual problems that child and adolescent face. In addition, the study indicates that the main aspects of sex education in Islam Education are developmental, humanistic and integrated in nature. It recommends that further comprehensive studies for curricula be carried out, and the inclusion of sex education concepts, in curricula, that are in accordance with Islamic Education and regulations. Keywords: Sex Education, Child, Islamic Education.
    [Show full text]
  • Physical Activity and Adolescent Sexual Maturity: a Systematic Review
    DOI: 10.1590/1413-81232021265.17622019 1823 R Atividade física na adolescência e maturidade sexual: EVISÃO uma revisão sistemática R Physical activity and adolescent sexual maturity: EVIEW a systematic review Cezenário Gonçalves Campos (https://orcid.org/0000-0001-5650-0096) 1 Fabiangelo de Moura Carlos (https://orcid.org/0000-0003-1392-2875) 2 Luciene Aparecida Muniz (https://orcid.org/0000-0003-2185-1595) 2 Wendell Costa Bila (https://orcid.org/0000-0003-3048-5953) 1 Vinícius de Oliveira Damasceno (https://orcid.org/0000-0003-0577-9204) 3 Márcia Christina Caetano Romano (https://orcid.org/0000-0002-1819-4689) 2 Joel Alves Lamounier (https://orcid.org/0000-0002-8386-4140) 4 Abstract The objective was to ve rify the asso- Resumo O objetivo é verificar a associação entre ciation between sexual maturation and physical maturação sexual e atividade física na adolescên- activity during adolescence. A systematic review cia. Trata-se de um estudo de revisão sistemáti- of articles published between 2008 and 2018 ca elaborado a partir de artigos publicados entre was conducted using the following databases: 2008 a 2018 nas bases de dados Medline-PubMed, PubMed/Medline, SciELO, Web of Science, Sco- SciELO, Web of Science, Scopus, Lilacs e Adolec pus, Lilacs, and BVS Adolec Brasil. The following BVS. Utilizou-se os descritores e palavras-chave descriptors and keywords were used in English adolescente, maturação sexual, inquérito, ques- and Portuguese: adolescent, sexual maturation, tionário e atividade física, no idioma português e survey, questionnaire, and physical activity. The sua equivalência na língua inglesa e foram identi- literature search retrieved 806 articles.
    [Show full text]
  • Reproductive Biology Spawning
    Malison, J. A,, and J. A. Held. 1996. Reproductive biology and spawning. Pages 11-18 in R. C. Summerfelt, editor. Walleye culture manual. NCRAC Culture Series 101. North Central Regional Aquaculture Center Publications Office, Iowa State University, Ames. Reproductive Biology and Spawning Jeffrey A. Malison and James A. Held, University of Wisconsin-MadisonAquaculture Program, Department of Food Science, 123 Babcock Hall, Madison, WI 53706 An understanding of the basic mechanisms that control February in the southern extremes of the walleye's reproduction is an important prerequisite for the range, and end as late as July in the northern extremes efficient commercial culture of any species. The case (Hokanson 1977). Peak spawning activity occurs at studies that accompany ths chapter deal primarily with water temperatures of 42-50°F (5.6-10°C; Becker techniques to artificially propagate walleye. In order to 1983), and generally lasts for 1-2 weeks at any one furnish the reader with the underlying rationale for the location. The level and duration of peak spawning development of these specific techniques, we have activity can be affected by rapid changes in the weather. provided a brief description of the reproductive biology of walleye in the first half of this chapter. The second Courtshp behavior has been described by Ellis and half summarizes walleye propagation techniques and Giles (1965). In brief, it is characterized by approaches highlights some of the hfferent spawning, incubation, and contacts, with no continued relationshp between and hatching protocols used by walleye culturists. any particular pair of fish. The spawning act culminates in a rush to the surface by a female and two flanking males, with other males in close pursuit (Priegel 1970), Walleye reproduction and a turning or pushng of the female onto its side Sexual maturity in walleye is dependent on both the which indicates spawning has occurred.
    [Show full text]
  • Endocrine Disruptor Screening and Testing Advisory Committee Final
    EDSTAC Final Report Chapter Five Appendices August 1998 Appendix K Brief Overviews of Assays Considered for Tier 1 Screening Table of Contents I. Estrogen and Anti-estrogen - Intrinsic Activity .................................1 A. Rat (and other non-human mammalian and avian) ER Binding Assay ................ 1 B. hER Binding From MCF-7 Cell Lysate ...................................... 2 C. Estrogen Competitor (Binding) Screening Assay (A Receptor/Ligand Assay, PanVera) .. 2 II. Estrogen and Anti-estrogen - In Vitro ........................................3 A. MCF-7 Proliferation Assay (ESCREEN) ..................................... 3 B. YES-Yeast Estrogen Screen .............................................. 4 C. MVLN Assay. Stably Transfected Reporter Gene Assay in Mammalian Cells ......... 5 D. Cotransfected Reporter Gene Assay in Mammalian Cells (e.g., CV-1 or COS Cells) .... 6 E. Stably Transfected Reporter Gene Assay in Mammalian Cells (e.g., MCF-7 Cells) ...... 6 III. Estrogen and Anti-estrogen - In Vivo ........................................7 A. Uterine Peroxidase Assay ................................................ 7 B. Developmental Uterotrophic Assay ......................................... 8 C. Uterine Weight Bioassay in Juvenile or Adult Ovariectomized Female Rats .......... 10 D. Vaginal Smears (Mucification and Cornification) ............................. 11 E. Puberty. Age at Vaginal Opening (First Estrus, Onset of Cyclicity) ................ 12 F. Induction of Female Sex Behavior (Proceptive and Receptive Behaviors)
    [Show full text]
  • Primary Gonadal Failure
    Best Practice & Research Clinical Endocrinology & Metabolism 33 (2019) 101295 Contents lists available at ScienceDirect Best Practice & Research Clinical Endocrinology & Metabolism journal homepage: www.elsevier.com/locate/beem Primary gonadal failure Asmahane Ladjouze, MD, Maître de Conferences en Pediatrie a, 1, Malcolm Donaldson, MB ChB MD (Bristol) FRCP FRCPCH * DCH, Honorary Senior Research Fellow b, a FacultedeMedecine d’Alger, Service de Pediatrie, Centre Hospitalo-Universitaire Bad El Oued, 1 Boulevard Said Touati, Algiers, Algeria b Section of Child Health, School of Medicine, Queen Elizabeth University Hospital, Govan Road, Glasgow, G51 4TF, United Kingdom article info The term primary gonadal failure encompasses not only testicular fi fi Article history: insuf ciency in 46,XY males and ovarian insuf ciency in 46,XX Available online 12 July 2019 females, but also those disorders of sex development (DSD) which result in gender assignment that is at variance with the genotype Keywords: and gonadal type. In boys, causes of gonadal failure include Kli- 46,XX nefelter and other aneuploidy syndromes, bilateral cryptorchidism, 46,XY testicular torsion, and forms of 46,XY DSD such as partial androgen Klinefelter syndrome insensitivity. Causes in girls include Turner syndrome and other Turner syndrome aneuploidies, galactosemia, and autoimmune ovarian failure. Iat- late effects of childhood cancer rogenic causes in both boys and girls include the late effects of fertility preservation childhood cancer treatment, total body irradiation prior to bone marrow transplantation, and iron overload in transfusion- dependent thalassaemia. In this paper, a brief description of the physiology of testicular and ovarian development is followed by a section on the causes and practical management of gonadal impairment in boys and girls.
    [Show full text]
  • Science Perspectives on Psychological
    Perspectives on Psychological Science http://pps.sagepub.com/ What Do Sex, Twins, Spotted Hyenas, ADHD, and Sexual Orientation Have in Common? Dennis McFadden Perspectives on Psychological Science 2008 3: 309 DOI: 10.1111/j.1745-6924.2008.00082.x The online version of this article can be found at: http://pps.sagepub.com/content/3/4/309 Published by: http://www.sagepublications.com On behalf of: Association For Psychological Science Additional services and information for Perspectives on Psychological Science can be found at: Email Alerts: http://pps.sagepub.com/cgi/alerts Subscriptions: http://pps.sagepub.com/subscriptions Reprints: http://www.sagepub.com/journalsReprints.nav Permissions: http://www.sagepub.com/journalsPermissions.nav Downloaded from pps.sagepub.com at University of Texas Libraries on November 5, 2014 PERSPECTIVES ON PSYCHOLOGICAL SCIENCE What Do Sex, Twins, Spotted Hyenas, ADHD, and Sexual Orientation Have in Common? Dennis McFadden Department of Psychology and Center for Perceptual Systems, University of Texas ABSTRACT—The otoacoustic emissions (OAEs) measured in measures may exist. They are unquestionably imperfect, but we a collection of special populations of humans and certain now have measures that seem to be sensitive to the degree of nonhuman species suggest that OAEs may provide a win- exposure to androgens a developing fetus receives. Some read- dow into some processes of human prenatal development ers surely will find it bizarre that those measures come from the and sexual differentiation. For reasons that are unclear, auditory system. Even more bizarre, some of the measures in- OAEs appear to be highly sensitive to events occurring volve sounds that come out of the ear.
    [Show full text]
  • Male Pubertal Development: Are Endocrine-Disrupting Compounds Shifting the Norms?
    W ZAWATSKI and M M LEE Puberty and EDCs 218:2 R1–R12 Review Male pubertal development: are endocrine-disrupting compounds shifting the norms? Correspondence 1 William Zawatski and Mary M Lee should be addressed to M M Lee Pediatric Endocrine Division, 1Department of Pediatrics, University of Massachusetts Medical School, 55 Lake Email Avenue North, Worcester, Massachusetts 01655, USA [email protected] Abstract Endocrine-disrupting compounds (EDCs) are synthetic or natural compounds that interfere Key Words with endogenous endocrine action. The frequent use of chemicals with endocrine active " testosterone properties in household products and contamination of soil, water, and food sources by " development persistent chemical pollutants result in ubiquitous exposures. Wildlife observations and " puberty animal toxicological studies reveal adverse effects of EDCs on reproductive health. " endocrine-disrupting In humans, a growing number of epidemiological studies report an association with compounds altered pubertal timing and progression. While these data are primarily reported in females, " sexual development this review will focus on the small number of studies performed in males that report an association of polychlorinated biphenyls with earlier sexual maturity rating and confirm subtle effects of lead, dioxins, and endosulfan on delaying pubertal onset and progression in boys. Recent studies have also demonstrated that EDC exposure may affect pubertal Journal of Endocrinology testosterone production without having a noticeable effect on sexual maturity rating. A limitation to understand the effects of EDCs in humans is the potential for confounding due to the long temporal lag from early-life exposures to adult outcomes. The complex interplay of multiple environmental exposures over time also complicates the interpretation of human studies.
    [Show full text]
  • Evaluation and Management of Amenorrhea Related to Congenital Sex Hormonal Disorders
    Review article https://doi.org/10.6065/apem.2019.24.3.149 Ann Pediatr Endocrinol Metab 2019;24:149­157 Evaluation and management of amenorrhea related to congenital sex hormonal disorders Ju Young Yoon, MD, Primary amenorrhea is a symptom with a substantial list of underlying etiologies Chong Kun Cheon, MD, PhD which presents in adolescence, although some conditions are diagnosed in childhood. Primary amenorrhea is defined as not having menarche until 15 years Department of Pediatrics, Pusan Na­ of age (or 13 years with secondary sex characteristics). Various etiologies of primary tional University Children's Hospital, amenorrhea include outflow tract obstructions, gonadal dysgenesis, abnormalities Yangsan, Korea of the central nervous system, various endocrine diseases, chronic illnesses, psychologic problems, and constitutional delay of puberty. The management of primary amenorrhea may vary considerably depending on the patient and the specific diagnosis. In this article, the various causes, evaluation, and management of primary amenorrhea are reviewed with special emphasis on congenital sex hormonal disorders. Keywords: Primary amenorrhea, Evaluation, Management, Adolescent Introduction Amenorrhea is defined by the transient or permanent absence of menstrual flow. Amenorrhea can be divided into primary and secondary presentations. Primary amenorrhea is diagnosed when there is no menarche by 13 years of age in those without development of secondary sexual characteristics, or by 15 years of age in those with secondary sexual characteristics.1) Secondary amenorrhea is defined as discontinuation of previously regular menses for 3 months or previously irregular menses for 6 months.2) The prevalence of primary amenorrhea is less than 0.1%, and secondary amenorrhea is more common with an incidence of about 4.0%.3,4) Primary amenorrhea can result from many different underlying conditions.
    [Show full text]