Pediatrician最新文献

Asthma is the most common chronic lower respiratory tract condition in children. Thus, it is imperative that physicians caring for asthmatic patients understand the pathophysiology of asthma and its implications for optimal therapeutic management. A precise understanding of asthma pathophysiology has been impeded due to the fact that a universally acceptable definition for asthma has been difficult to formulate. Clinicians treating asthmatic patients should be aware that the airway obstruction present in these individuals is the result of multiple interrelated factors. Although bronchial smooth muscle spasm can be effectively treated producing rapid symptomatic relief, other factors contributing to airway obstruction, such as airway inflammation and edema, need to be a major focus of therapeutic strategies for more chronic management. While the concepts of reversibility and hyperresponsiveness have been appreciated for some time, the idea that asthma should be viewed as an inflammatory lung disease (or syndrome) has only recently received appropriate emphasis. In this regard, the late phase asthmatic response has provided a convenient model to study the biochemical and cellular interactions that contribute to the pathogenesis of asthma. Further, these responses will aid in the analysis of the potential beneficial effects of various pharmaceuticals as they undergo development and testing for use in asthmatic patients.

The principal goal of treatment of the acute exacerbation of asthma is the rapid reversal of the airway obstruction which is best accomplished by the frequent administration of inhaled beta 2-agonists. In addition, the early addition of systemic corticosteroids improves the response in patients who incompletely respond to beta 2-agonists. If present, hypoxemia should be corrected with administration of supplemental oxygen. Close monitoring of the patient's response to treatment is essential and if the patient is over 4 years of age and can cooperate, this should include peak expiratory flow rate (PEFR) measurement.

In many industrialized nations of the world it is estimated that nearly 10% of young people have chronic conditions; nearly one third have limitations of functioning. The most common conditions include hay fever, chronic sinusitis, acne, and asthma. Dramatic strides in medical management of chronic conditions in industrialized nations have resulted in nearly 85% of children born today with a congenital anomaly living at least until the age of 20. Not only are children with previously fatal conditions living until adulthood, but more of them are increasingly being mainstreamed into community settings. There is a diverse set of issue of which the clinician needs to be cognizant to successfully work with youth with chronic illness and disabilities as well as their parents.

In adolescents with chronic illness or disability, the progress of puberty may be abnormal. Growth or sexual maturation may be delayed. In some cases, adulthood may be characterized by short stature or sexual immaturity. Genetic endowment, nutritional deficiency, or concomitants of treatment all may contribute to disordered growth; frequently the basis of abnormal physical development in adolescence is multifactorial. This paper reviews the major mechanisms by which chronic conditions may interfere with puberty and discusses the effects of common chronic conditions on the pubertal process.

In this paper, a family systems approach is advocated as an optimal model for managing the health care of youth with disabilities. The impact of the youth's disability on family functioning and, reciprocally, the impact of the family system on the course of the disability and the youth's development are reviewed. These two effects are viewed as mutually causal and thus call for a nonlinear approach to treatment--one where both adolescent and family outcomes are simultaneously considered. The implications for pediatric practice of adopting a family system perspective in working with youth with disability is emphasized.

Specific developmental issues and long-term psychosocial implications are associated with a diagnosis of cancer during the period of adolescence. Effects on the adolescent's developing independence, sexual identity, and social and psychological maturity must be considered. Repercussions of these disruptions emphasize the need for creative treatment approaches which incorporate both immediate and long-term preventive and rehabilitative strategies.

As increasing numbers of children are surviving beyond the 1st decade after marrow transplantation and increasing numbers of children are receiving marrow transplants each year, the delayed effects related to the transplant procedure itself, the original disease, and/or the transplant preparative regimen are becoming apparent. Late effects related to the transplant procedure include those of engraftment stability, the chronic immunosuppression of chronic graft-versus-host disease and delayed immunologic recovery. Recurrent disease is the major late effect related to the patient's original disease. The late effects which may be related to previous therapy administered and/or the transplant preparative regimen include abnormalities of neuroendocrine function, ocular problems, dental developmental abnormalities in young children, central nervous system dysfunction and the development of secondary malignancies. To improve the quality of life of marrow transplant recipients and to prevent some of the growth and development abnormalities which may occur, an awareness of the problems encountered to date is needed.

Inherited epidermolysis bullosa (EB) comprises some 23 different phenotypic disorders which have as common features the presence of mechanically fragile skin and the tendency to develop blisters, erosions, and in some cases, scarring of the skin. While some forms of this disease are relatively mild, other rarer variants may prove to be life-threatening. In the past 15 years, a wealth of new information has elucidated new clinical and laboratory characteristics of specific EB subsets. In this brief review, the most important aspects of inherited EB are summarized to update the practicing pediatrician about the ever-increasing complexities being discovered about this disease.

Bronchial hyperresponsiveness (BHR) is a major feature of asthma that is determined by both genetic and environmental factors. The assessment of BHR can be a valuable means of assessing asthma severity and also response to therapy. BHR can be measured with a variety of provocative agents including exercise, methacholine, histamine, hypotonic and hypertonic saline and cold air. Methacholine and histamine challenges have been well studied and standardized. They are extremely sensitive indicators of BHR, but this is not synonymous with asthma. This requires that the physician interprets the clinical significance of test results with caution.

Acne is a skin disorder of the sebaceous follicles that commonly occurs in adolescence and young adulthood. The pathogenesis involves abnormal follicular hyperkeratosis and obstruction of the follicle, stimulation of sebaceous gland secretion by androgens, and proliferation of Propionibacterium acnes, which promotes inflammation. Treatment regimens should be designed based upon an understanding of the multifactorial basis of pathogenesis. Both topical and systemic agents may be employed to normalize keratinization, decrease sebaceous gland activity, decrease the follicular P. acnes population, and minimize inflammation.