Diagnostic et prise en charge d’une aménorrhée chez l’adolescente

Amenorrhea in adolescents can be primary, with or without breast development, or secondary. Whether amenorrhea is primary or secondary, height, body mass index, food intake, the level of physical activity per week, the presence of hirsutism or galactorrhea, pelvic pain and past history of intercourse need to be investigated. Initially, blood tests should include hCG, FSH, estradiol, testosterone and prolactin serum levels. This screening will discriminate between hypogonadotropic hypogonadism and amenorrhea from primary ovarian insufficiency (POI). In case of primary amenorrhea, hypogonadism may be due to congenital hypogonadotropic hypogonadism (HH) or more rarely acquired HH. If FSH is elevated, amenorrhea is due to primary ovarian failure, mainly related to Turner syndrome. If pubertal development is normal, a pelvic ultrasound should be performed. It may visualize a hindering of menses output or less frequently an absence of uterus, as in Rokitansky syndrome or androgen insentivity syndrome. The most frequent etiologies of secondary amenorrhea are polycystic ovarian syndrome (PCOS), functional hypothalamic amenorrhea and less frequently POI and hyperprolactinemia. The differential diagnoses of PCOS are late-onset 21-hydroxylase deficiency and very rare ovarian or adrenal tumors. When contraception is not necessary, hormonal replacement therapy, including estrogen and progestins should be administered in order to avoid hypoestrogenism. In case of PCOS, sequential progestins can be prescribed. A contraceptive pill can be considered when contraception is needed and/or when hyperandrogenism needs to be treated.     

Neuroendocrine aspects of amenorrhea related to stress

Reproductive functions are controlled by a very sophisticated system that is composed by the perfect syncronization of neuronal end endocrinological functions. Hypothalamus is the neuroendocrine gland that discharges GnRH that stimulates LH and FSH secretion from pituitary thus promoting ovarian function. Whatever affects the hypothalamic activity might be able to interfere with the reproductive axis. In fact changes in such sophisticated stimulatory system is able to negatively affect the periodical function of the ovary and may induce amenorrhea. Stressant situations, due to physical, psychologic or metabolic stressors, are able to negatively modulate the hypothalamus-pituitary-ovarian axis (HPO) mainly acting through an impairment of the many neuromodulators and neuropeptides that are produced inside the brain and in the hypothalamic areas. Stress induced amenorrhea is usually called hypothalamic amenorrhea (HA) and affect a consistent percentage of women, independently from the age. Nevertheless, quite often HA is diagnosed in adolescents or in girls below 20 years of age. Undernutrition, excess of training, psychological stress, are able to induce HA and then hypo-estrogenism due to the reduced ovarian activity. When sports or exaggerated training is performed before or during the onset of menarche, a specific delay of menarche might occur, as direct consequence of the unbalance between feeding, energy consumption and psychological stress. Osteopenia and osteoporosis are possible consequence of such situation. Clinicians have to pay great attention to all kind of delay of occurrence of meanarche or of amenorrhea, especially if related to lost of weight, since these might mask adverse environmental situations that might induce HA through an exaggerated stress-induced neuroendocrine response.     

Institutionalized disabled adolescents: gynecologic care. The pediatrician's role

Severely handicapped and institutionalized teenage girls have a variety of gynecologic problems that can be readily addressed by a physician with an interest in adolescent medicine who makes "home visits" to the institutions. Disabled teens are a unique group of adolescents whose needs intersect with those of normal teens. Educating and listening to the caretakers, who can then prepare the patients, is essential. Key topics include menses and menstrual calendars, adolescent development, the pelvic examination, and contraception. The clinical problems most frequently encountered are vaginal discharge, menstrual cycle dysfunction, and oligomenorrhea. Basic on-site management is described. In this population, severe chronic illness and subnormal weights for age are not necessarily associated with secondary amenorrhea. Patients, parents, and staff are satisfied when gynecologic needs are met in an empathetic and thorough fashion. A list of educational resources is appended.     

Anorexia nervosa, athletics, and amenorrhea

We examined menstrual function in two groups of patients meeting the DSM III criteria for anorexia nervosa who differed only in their physical activity. Sixteen athletes with anorexia nervosa were compared with eight sedentary patients who had anorexia nervosa. Athletic patients with anorexia were found to have lower gonadotropin levels, a longer period of amenorrhea both before significant weight loss and after weight rehabilitation, and a higher weight at the time of resumption of menses than patients with anorexia who were sedentary. However, both groups were markedly undernourished, had amenorrhea before significant weight loss and after weight rehabilitation, and had lower gonadotropin levels than normal subjects. These data suggest that the increased physical activity often seen in patients with anorexia nervosa worsens, but does not cause, menstrual dysfunction.     

Adolescent girls,the menstrual cycle,and bone health

In adolescent girls, amenorrhea is sometimes viewed as a variant of normal; in fact, however, during the first gynecologic year, the 95th percentile for cycle length is 90 days. Although early menstrual cycles are frequently anovulatory and may be somewhat irregular, girls with menses coming less frequently than every 90 days may have significant pathology associated with hypoestrogenism. Hypoestrogenism is a known risk factor for the development of osteoporosis. Causes of oligomenorrhea and amenorrhea include the relatively common conditions of hyperandrogenism, eating disorders, and exercise-induced amenorrhea, as well as uncommon conditions such as pituitary tumor, gonadal dysgenesis, and premature ovarian failure. Even functional hypothalamic oligomenorrhea has been linked to reduced bone density. Attention to menstrual irregularity and the earlier diagnosis of conditions causing it may lead to interventions that will benefit life-long bone health.     

Concussions: assessment, management, and recommendations for return to activity

Concussions in children and adolescents are a common presenting complaint seen in all emergency departments. Concussions range from a brief period of neural dysfunction to a prolonged period of unconsciousness with retrograde amnesia. It is imperative that the emergency physician be familiar with the appropriate assessment of the child or adolescent who has sustained a concussion, methods for grading their severity, current acute management, and recommendations for returning to activity.     

A case of Langerhans cell histiocytosis with thyroid involvement

Thyroid involvement with Langerhans cell histiocytosis (LCH) is very rare. We report here the case of a 15-year-old female patient with LCH affecting the thyroid gland. She was referred to the department of pediatric endocrinology for secondary amenorrhea. Prior to the diagnosis of LCH, the patient had symptoms of diabetes insipidus (DI) and amenorrhea. The mean time from symptom onset to diagnosis was 2 years. On physical examination the patient had grade 2 goiter, and ultrasound showed bilateral multiple hypoechoic nodules and thyroid heterogeneity. Biochemical analysis indicated central diabetes insipidus and panhypopituitarism. Magnetic resonance imaging (MRI) demonstrated a mass lesion involving the hypothalamus, which appeared iso- to hypo-intense on T2-weighted images and had an intense postcontrast enhancement on T1-weighted images. Nodular goiter coinciding with a hypothalamic mass suggested LCH, and an excisional biopsy was performed. Histological evaluation of the thyroid gland revealed extensive involvement by LCH, and this was confirmed by immunohistochemical analysis showing S-100 protein and CD1a positive Langerhans cells that were weakly positive for CD68. LCH should be considered in the differential diagnosis of a diffusely enlarged firm and irregular thyroid gland and posterior or anterior pituitary dysfunction.     

Bacterial sexually transmitted infections in gay, lesbian, and bisexual adolescents: medical and public health perspectives

Gay, lesbian, and bisexual adolescents, like all adolescents who engage in high-risk sexual behaviors, are at elevated risk for acquiring sexually transmitted infections (STIs). Personal sexual risk factors and issues related to access to care complicate the lives of youth who engage in same-gender sexual activity and who may or may not self-identify as gay, lesbian, or bisexual. Whereas epidemiologic rates of gonorrhea, chlamydia, and syphilis generally have trended downward in adolescents as a whole during the past 15 years, rates for these common reportable bacterial STIs have increased overall during recent years among men who have sex with men. This article focuses on bacterial STIs in youth with same-gender sexual activity. An understanding of trends among gay, lesbian, and bisexual youth remains incomplete, given the absence of consistent and uniform mechanisms for collecting data on sexual behaviors in adolescents and difficulties in associating these behaviors with reportable STIs. Special attention should be given to the screening, diagnosis, and treatment of bacterial STIs in those who engage in same-sex behavior, as new recommendations from the Centers for Disease Control and Prevention have been made available. It is critical that healthcare providers who work with adolescents be aware of the assortment of specific healthcare needs of gay, lesbian, and bisexual youth and address them appropriately in the clinical setting. Medical providers may be one of few true advocates for this often-marginalized adolescent population and have the power to have a positive influence on health promotion and education efforts.     

Thalamic and hypothalamic tumors of childhood: endocrine late effects

Children who have received chemotherapy and radiation therapy for treatment of thalamic/hypothalamic tumors are at risk for late effects, specifically endocrine dysfunction. Evaluation of growth and pubertal development, thyroid function and integrity of the hypothalamic-pituitary-adrenal axis should be undertaken in a prospective manner. Issues of metabolic disturbances such as obesity, altered body composition/bone density as well as ultimate fertility also need to be addressed by ongoing prospective evaluations.     

Stress management techniques in childhood and adolescence. Relaxation training, meditation, hypnosis, and biofeedback: appropriate clinical applications

Many childhood and adolescent stress-related symptoms have a psychophysiological component that involves muscular tension and/or autonomic nervous system dysfunction. Examples of this include recurrent headache, chest pain, abdominal pain, syncope, and dizziness. After a careful medical and psychosocial evaluation, the clinician may identify many patients who are appropriate for the application of stress reduction techniques such as progressive muscular relaxation, meditation, biofeedback, and relaxation/mental imagery (self-hypnosis). This review describes these techniques and their application with selected children and adolescents.     

Adolescents and contraception. What should the pediatrician know? (Part one)]

Many adolescents are sexually active and reproductive health is an important aspect of adolescent medicine. However, pediatricians are often uncomfortable with the issues of sexuality and contraception, for which they have not been particularly trained. The general purpose of this article is to increase pediatricians' sense of competence with adolescents, particularly when having to deal with or counsel on such a sensitive issue as contraception. This first of two parts is an updated review of the relevant contraceptive methods available, mainly condoms and various contraceptive pills, but also less-prescribed method like long-term progestins, as well as the recently debated emergency contraception. Each of these methods is described in its context of use at adolescence.     

Bowel function, mental health, and psychosocial function in adolescents with Hirschsprung's disease

Congenital intestinal malformations are uncommon and may pose lasting somatic difficulties. Patients with anorectal anomalies have a high frequency of persistent faecal dysfunction and psychosocial problems. This study examined whether adolescents with Hirschsprung's disease have more psychosocial problems than their healthy peers. Nineteen adolescents (mean age 15.7 years) with Hirschsprung's disease were assessed for bowel function, anorectal physiology, mental health, and psychosocial functioning by physical examinations, semistructured interview, and standardised questionnaires. The adolescents were compared with controls. The parents of 13 adolescents with Hirschsprung's disease were interviewed and completed questionnaires. Thirty two per cent of the adolescents with Hirschsprung's disease had significant impairment of continence, but no more psychopathology (16%) nor psychosocial dysfunction as a group than their healthy peers. Faecal incontinence was associated with poorer psychosocial functioning and parental criticism. The fact that a significant number of patients with Hirschsprung's disease have incontinence into adulthood indicates the need for parental counselling, encouraging realistic expectations about continence.     

Activity-induced asthma

Exercise is the most common trigger of persistent childhood asthma. The history for EIA can be complicated by the lack of perception of significant airway obstruction during exercise. One must carefully identify those children with EIA from the group of children who report low level of activity because of lack of interest or because they are out of shape. Baseline spirometry of children with persistent asthma is frequently normal. Spirometry is important to identify those children with EIA who underrecognize their disease, but normal results should not be used as evidence of absence of disease. Formal exercise testing should be considered when the diagnosis is unclear or if there seems to be a lack of bronchoprotection with inhaled albuterol. The goal of treatment of EIA should be the attainment of a normal activity level for children and adolescents. Identification of the limits imposed by EIA and establishment of goals of therapy with the child and family should be the initial action. Inactivity or reduced exertion, in the presence of this diagnosis. should not be accepted. Therapy for EIA starts with control of the underlying persistent asthma. Inhaled corticosteroids are the most effective initial treatment of both EIA and persistent asthma in children and adolescents. Exercise-induced asthma is a common aspect of a prevalent disease that warrants proper diagnosis and treatment. With appropriate therapy, children with EIA should be able to participate in sports and maintain normal activity. They should strive to compete in the same playing field as their peers and have the same goals as those children and athletes who do not have exercise-induced asthma.     

Association of empty sella and neuroendocrine disorders in childhood

Empty sella syndrome (ESS) is a multicausal entity. The incidence of primary empty sella syndrome (PESS) in children with neuroendocrine dysfunction is not known. In the pediatric age group, frequency seems to have been underestimated. A total of 117 cases of neuroendocrine disorders, including complete growth hormone deficiency, primary hypothyroidism with pituitary resistance to thyroid hormone, obesity, central precocious puberty, hypothalamic hypogonadism and central diabetes insipidus, have been studied with computed tomography and/or magnetic resonance imaging of sellar region for etiologic evaluation. Twenty-one patients were found to have PESS. We noted a high incidence of PESS in children with neuroendocrine dysfunction (17.9%). Children with neuroendocrine dysfunction should be investigated with respect to PESS, and children with PESS recognized coincidentally should be studied with the particular consideration of subclinical neuroendocrine dysfunction.     

Gynecological concerns in young and adolescent physically active girls

Young girls should be advised to develop the regular exercise habit and to maintain it throughout life. Prepubertal athletes are more likely than their sedentary friends to experience menarcheal delay. Postmenarcheal athletes have increased susceptibility to oligomenorrhea and amenorrhea. Athletes with delayed puberty deserve examination and possibly further evaluation. Adolescent athletes with oligomenorrhea or amenorrhea deserve examination and hormonal evaluation. Hormonal replacement therapy is unnecessary prior to age 16, is optional between ages 16 and 18, and is recommended after age 18.     

Gonadal function of young patients with beta-thalassemia following bone marrow transplantation

Bone marrow transplantation (BMT) can induce short- and long-term impairment of gonadal function. Patients with beta-thalassemia represent a special group, as their primary diagnosis and its treatment modalities are responsible for gonadal dysfunction. To address the effect of BMT on puberty and gonadal function, we investigated 25 patients (12 males) with thalassemia who received allogenic BMT during childhood or adolescence and at the post-transplant evaluation were at an age that the pubertal process should have started. Pubertal stage by Tanner of breast and pubic hair, as well as testicular volume were assessed pre-BMT once and post-BMT at least twice. Menstrual history was recorded. FSH, LH, testosterone and estradiol levels were also determined. The impact of BMT appears to be different in the two sexes. Males seem to have higher tolerance, as all males who were pubertal at the time of BMT had normal testosterone, and all but one normal gonadotropin levels. From those who were prepubertal at BMT, 62% proceeded to normal pubertal development. Post-menarcheal females seem to be an extremely sensitive group to the deleterious effect of the transplantation process, as 100% of the post-menarcheal females exhibited amenorrhea and elevated gonadotropin levels. These findings are important for pre- and post-BMT counseling.     

Update of nutrition guidelines for the teen: trends and concerns

Sociocultural influences are known to affect adolescent eating patterns and behaviors. Some teens reject a meat-based diet to become vegetarians; others take up dieting to lose weight or develop an eating disorder. Teens require increased nutrients to provide for the accelerated growth that takes place during these years. Nutritional deficiencies in adolescence can lead to loss of height, osteoporosis, and delayed sexual maturation. Sports also play an important role in many teens' lives. The desire to excel can lead to increased training and prolonged periods of dieting. Disordered eating, amenorrhea, and osteoporosis describe the "female athlete triad." Although more frequently seen in girls, boys who participate in sports that have an emphasis on size and weight such as wrestling and body building are also at risk for subclinical eating disorders. This paper discusses issues surrounding restrictive and nonrestrictive food patterns of adolescents and their nutritional consequences.     

Angiotensin-converting enzyme inhibitor therapy for ventricular dysfunction in infants, children and adolescents: a review

Angiotensin-converting enzyme (ACE) inhibitors have become an important part of the pharmacologic armamentarium in the battle against treatment of ventricular dysfunction. There have been a number of large controlled, randomized trials in adults with both asymptomatic and symptomatic ventricular dysfunction, which confirm the safety and efficacy of this category of drugs for the treatment of this potentially lethal condition. ACE inhibitors may be used to treat infants, children and adolescents with asymptomatic and symptomatic ventricular dysfunction as well. The data supporting their use in children is less complete than that concerning the treatment of adults. We review here the various causes of ventricular dysfunction and congestive heart failure (CHF) in infants, children, and adolescents; the data available regarding treatment of these conditions with ACE inhibitors, and the safety and efficacy of these drugs for the various conditions. The pharmacokinetics and proposed mechanisms of action of ACE inhibitors in children are reviewed, as are speculated long-term results of ACE inhibitor use in cohorts of growing children. Recommendations are made for future studies.     

Abnormal vaginal bleeding in adolescents

The evaluation and management of abnormal vaginal bleeding should be tailored to the individual case. Pediatricians caring for adolescents need to be skilled in the evaluation and management of this common adolescent problem. Physicians who do not feel comfortable with their skills in the performance of a pelvic exam should obtain consultation from an adolescent specialist or a gynecologist who has experience with adolescents.     

Meningococcal conjugate vaccines policy update: booster dose recommendations

The Advisory Committee on Immunization Practices of the Centers for Disease Control and Prevention and the American Academy of Pediatrics approved updated recommendations for the use of quadravalent (serogroups A, C, W-135, and Y) meningococcal conjugate vaccines (Menactra [Sanofi Pasteur, Swiftwater, PA] and Menveo [Novartis, Basel, Switzerland]) in adolescents and in people at persistent high risk of meningococcal disease. The recommendations supplement previous Advisory Committee on Immunization Practices and American Academy of Pediatrics recommendations for meningococcal vaccinations. Data were reviewed pertaining to immunogenicity in high-risk groups, bactericidal antibody persistence after immunization, current epidemiology of meningococcal disease, meningococcal conjugate vaccine effectiveness, and cost-effectiveness of different strategies for vaccination of adolescents. This review prompted the following recommendations: (1) adolescents should be routinely immunized at 11 through 12 years of age and given a booster dose at 16 years of age; (2) adolescents who received their first dose at age 13 through 15 years should receive a booster at age 16 through 18 years or up to 5 years after their first dose; (3) adolescents who receive their first dose of meningococcal conjugate vaccine at or after 16 years of age do not need a booster dose; (4) a 2-dose primary series should be administered 2 months apart for those who are at increased risk of invasive meningococcal disease because of persistent complement component (eg, C5-C9, properdin, factor H, or factor D) deficiency (9 months through 54 years of age) or functional or anatomic asplenia (2-54 years of age) and for adolescents with HIV infection; and (5) a booster dose should be given 3 years after the primary series if the primary 2-dose series was given from 2 through 6 years of age and every 5 years for persons whose 2-dose primary series or booster dose was given at 7 years of age or older who are at risk of invasive meningococcal disease because of persistent component (eg, C5-C9, properdin, factor H, or factor D) deficiency or functional or anatomic asplenia.