Adolescent medicine (Philadelphia, Pa.)最新文献

Leukemia remains the most common cancer in childhood, and while great strides have been made in increasing event-free survival in the past 20 years, patients with high-risk features still pose a challenge for successful disease-free survival. Older children and adolescents are included in that high-risk group. Approximately 80-85% of cases of leukemia in the pediatric population are of the lymphocytic subtype. Overall disease-free survival rates for acute lymphocytic leukemia have increased to 80% for those with standard or low-risk disease and 65-70% for those with high-risk disease. This is a product of both a better understanding of the molecular pathophysiology of ALL and the development of better treatment strategies based on risk. In acute myelogenous leukemia, we have not achieved such success, and disease-free survival rates are in the 30-40% range. This article discusses the diagnosis of leukemia in the adolescent population with attention to pathogenesis, prognostic risk factors, therapy, outcome, and late effects of acute lymphocytic leukemia.

Adolescent patients with immune-mediated hematologic disorders present a challenging clinical picture. The combination of a careful history, physical examination, and appropriate laboratory tests will help establish the correct diagnosis. Thoughtful consideration of therapeutic options will result in the best treatment outcomes.

Myelodysplastic syndromes (MDS) are a group of acquired blood diseases that are the result of abnormal bone marrow function. The ineffective production of red cells, platelets, and white blood cells can lead to symptomatic anemia, bruising, infections, and the likelihood of evolution into acute myelogenous leukemia. While MDS is uncommon in the adolescent patient, a surprising number of affected individuals are also affected with a predisposing constitutional syndrome. The treatment of MDS in the adolescent patient is in part determined by symptoms and also by the historical outcomes associated with each of five morphologic categories of presentation. Improved supportive care has allowed for increasingly more children with MDS to survive into the second decade of life. The management of MDS in affected adolescents presents a number of interesting and worthwhile challenges to health care professionals.

Malignant bone tumors in the adolescent population are rare but serious problems that are both life- and limb-threatening. Most of these tumors originate in the extremities, hip girdle, or pelvic girdle and require complete surgical resection for adequate therapy. The greatest majority of these tumors are diagnosed as osteogenic sarcomas. In the past, limb ablation was the only effective therapeutic option available to surgical oncologists in adolescent osteosarcoma patients. However, today, after two decades of advances in chemotherapy protocols and reconstructive surgical techniques, limb-salvage surgery has become an accepted treatment standard. Because skeletal immaturity and future bone growth is generally not a major reconstructive consideration in adolescents, 90% of the patients in this age group are today treated with limb-sparing surgery. The most significant question regarding the successful use of limb-salvage surgery is whether it adversely affects long-term outcome compared with standard amputations. The principal studies, both single- and multi-institutional, that compared the risk of local tumor recurrence and overall disease-free survival rate of the two types of procedures, demonstrated no significant difference in disease-free survival rates between the two groups. Similarly, multivariant analyses have shown no survival benefit for choice of surgical procedure in osteosarcoma patients. As a result, limb-sparing surgery for osteosarcoma patients has now been firmly established as a safe, effective, and successful oncology procedure compared with limb ablation.

For adolescents, negotiating developmental tasks while living with hemophilia can be difficult. Affected adolescents must somehow negotiate tasks inherent within normal adolescent development, along with the added complications of hemophilia. Complications may include changes in physical appearance due to a history of bleeding episodes and the need for adolescents to think about the consequences of neglected treatment before they are developmentally ready. However, current strategies employed in the treatment of hemophilia have provided adolescents, their families, and their caregivers with opportunities to minimize the impact of hemophilia on adolescent development. Self-infusion minimizes the social impact by allowing adolescents to quietly leave the classroom, treat themselves, and return to class without notice. Prophylactic care maintains a factor level that allows adolescents to participate in many more activities. A multidisciplinary treatment center model for care promotes focused care, education, and anticipatory guidance to minimize the impact and help families adjust to chronic illness. Strong state and national leadership provides research advocacy and funding support for adolescent programming.

Adolescents need reproductive and sexuality-related health care. State and federal laws affect consent, confidentiality, and payment for services. State minor consent laws allow minors to consent based on the minor's status or the services sought. State and federal laws affect confidentiality of health information generally as well as programs for specific services, such as family planning or drug and alcohol abuse treatment. Medicaid, the new State Children's Health Insurance Program, and categorical funding programs are important funding sources. Adolescents' ability to use these sources for confidential reproductive and sexuality-related services is evolving. However, possible changes in federal law and the transition to managed care in both public and private insurance present significant challenges they may face in the future.

All types of ovarian tumors seen in the adult population can also be seen in children and adolescents. However, the relative incidence of each entity varies considerably with age. Functional non-neoplastic cysts and masses are much more frequent in the adolescent. Furthermore, in the adolescent age group, neoplasms are more likely to be germ cell rather than epithelial in origin. Fortunately, in children and adolescents only 10% of ovarian tumors are malignant. In diagnostic evaluation of ovarian masses, a complete history and physical examination are of utmost importance. Whenever an ovarian mass, be it physiologic or neoplastic, benign or malignant, is diagnosed in an adolescent female or prepubertal child, every effort must be made to preserve the reproductive function in that female in order to ensure future childbearing.

This article reviews numerous studies of parent-adolescent communication about sexuality and 30 studies of sexuality and HIV education programs. Although parent-adolescent communication about sexuality has increased during the last few decades, both the occurrence and the quality of this communication could still be greatly improved. There does not exist a clear simple relationship between greater parent-adolescent communication and less adolescent risk-taking behavior, but both adults and youth believe it is important anyway. Sexuality and HIV education programs do not increase any measure of sexual activity, but some of them with ten common characteristics do reduce sexual risk-taking, either by delaying or reducing sexual behavior or increasing condom use. Thus, these programs can be an effective component in a larger initiative to reduce HIV, other STDs, and unplanned pregnancy in adolescence.

Bacterial and fungal vulvitis, dermatitis, inflammatory dermatoses, secondary drug reactions, viral infections, and a variety of vulvar tumors can all present as primary vulvar problems in adolescents. In addition, systemic disease can present with vulvar involvement. These disorders can be extremely anxiety-provoking in adolescent females who are dealing with issues surrounding self-image, physical maturation, and sexuality. A detailed history and physical exam can provide many clues to the underlying problem, but sometimes a biopsy is necessary to establish a diagnosis. Consultations from other services, such as dermatology or infectious diseases, can be very helpful. The best approach involves a combination of empathetic reassurance, careful diagnosis, and successful treatment. In order to facilitate optimal care of adolescents with vulvar disorders, a comprehensive review of the literature is presented.

Many adolescents present with hirsutism and irregular menses. The challenge for the clinician is to distinguish physiologic anovulatory cycles from true menstrual disorders such as PCOS, and to differentiate PCOS from other causes of hyperandrogenism in hirsute adolescents. Common clinical features seen in adolescents with PCOS include hirsutism, acne, menstrual irregularity, and obesity. Biochemical abnormalities include hyperandrogenism, acyclic estrogen production, LH hypersecretion, decreased levels of SHBG, and hyperinsulinemia. Management strategies for a patient with PCOS include treatment of features which may cause distress to the adolescent, such as hirsutism, acne, and irregular menses, and prevention of long-term sequelae. Oral contraceptive pills, antiandrogens, and cosmetic treatments are used to treat hirsutism, acne, and menstrual irregularity. Oral contraceptive pills or medroxyprogesterone acetate are given to prevent endometrial hyperplasia and carcinoma. Counseling about weight loss and nutrition are essential, as weight loss may improve signs of hyperandrogenism and menstrual irregularity and may prevent NIDDM and cardiovascular disease. Insulin-sensitizing agents show promise in terms of decreasing hyperandrogenism, restoring ovulatory cycles, treating infertility, and preventing long-term sequelae. Finally, it is important to recognize that adolescents with PCOS may experience psychological distress because of the clinical manifestations of hyperandrogenism or when confronted with the information that they have a chronic illness. Psychological support should be available for these young women. Future research is likely to further elucidate the pathophysiology of PCOS, identify candidate genes, and clarify which adolescents are at risk for long-term sequelae. Prospective studies are needed to identify which therapies could potentially reduce the risk of infertility, diabetes, cardiovascular disease, and endometrial carcinoma in young women with PCOS.