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 epidemiology of asthma in childhood is undergoing changes in many parts of the world. Prevalence, rates of hospitalization and mortality are all on the rise. In the United States, urban dwellers and minorities have experienced the greatest acceleration in rates. Elsewhere, some countries have experienced large increases in asthma morbidity and mortality, while others have not. Inconsistency in defining asthma and variations in methodology complicate epidemiologic investigation, but recent studies confirm the disturbing trend toward increased morbidity and mortality from childhood asthma and offer some insights into possible contributing factors.
Tissue complications of radiation therapy depend on the interplay of therapy, patient and tumor factors. Acute local effects result from parenchymal cell hypoplasia. Chronic damage is caused by both injury to parenchymal cells and to the underlying vasculature. No body tissue or system is immune to damage from radiation therapy, but many effects are dose-dependent. Many of the toxicities can currently be avoided by the optimal use of this important cancer therapy.
Congenital vascular birthmarks, classified as either hemangioma or vascular malformations (portwine stains), can now be cleared successfully using certain lasers. Since their introduction into medicine in the mid-1960s, several types of lasers including the argon, carbon dioxide, neodymium yttrium aluminum garnet (Nd:YAG) and pulsed tunable dye lasers have been used to treat benign cutaneous vascular lesions such as portwine stains. After extensive research examining the effects of specific laser parameters on tissue, a set of parameters was identified which together selectively destroyed blood vessels in the skin. Application of this laser technique for the treatment of benign cutaneous vascular lesions, such as portwine stains, meant that it was possible to destroy only the abnormally ectatic vessels which are inherent in these birthmarks. Because of the specificity of the laser injury, only the abnormal blood vessels are destroyed and healthy adjacent structures such as epidermal pigment and dermal collagen are left intact. Thus, unwanted adverse effects such as scar formation and pigmentary abnormalities at the laser-exposed site have been minimized. Skin treated using this combination of laser parameters normalizes in color, texture and its markings, and adnexae are maintained at the treated site. Not only has this new laser treatment provided a way of removing these disfiguring birthmarks with the return of normal-appearing skin at the treated site, but the lack of adverse effects following this treatment has meant that not only adults but children of all ages can now be treated using this technique.(ABSTRACT TRUNCATED AT 250 WORDS)
Vascular nevi occur in up to 70% of infants. The term includes hemangiomas, of which the majority involute, and vascular malformations, such as port wine stains which tend to persist. The appearance, and in some cases rapid growth of the lesion, may be alarming to the parents and may cause great distress to the patient. In rare cases systemic involvement and associated abnormalities may be life threatening or permanently disabling. Correct diagnosis of the type of lesion is vital to know the prognosis of the lesion, the likelihood of complications and whether investigations and treatment are indicated.
Intelligence and academic achievement testing of long-term survivors of childhood cancer reveal a high incidence of memory deficits, visual-spatial skill impairment, and attention deficit disorders. While the results of various studies must be interpreted carefully, the data available identify CNS irradiation and the toxic synergism of CNS irradiation and intrathecal chemotherapy as primary etiologic factors in the neuropsychologic sequelae of curative therapy. Early education intervention is mandatory to identify survivors of childhood cancer who require assistance in overcoming intellectual disabilities.
Survivors of childhood cancer who received anthracycline treatment have a high incidence of abnormal cardiac function. Cardiac decompensation and death can appear many years after completion of chemotherapy. These survivors require periodic evaluation of cardiac function and rhythm.
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.
When broader aspects of health are considered especially when assessing the life of the disabled it becomes necessary to reach beyond physical measurements to more dynamic aspects including the individual's other resources and demands. In such a context issues of quality of life are important. Quality of life is here defined as a term describing the total existence of an individual or a group and is operationalized in three life spheres: external conditions, interpersonal conditions and personal psychological conditions. National samples of youths aged 12-18 years with cystic fibrosis and myelomeningocele are compared to a reference group of normal youths in the five Nordic countries. The study was based on a mailed questionnaire. The results show that the disabled groups had equal external conditions as their peers while they rated lower on the interpersonal and personal levels. This indicates that the Nordic countries have succeeded in providing a good material support for families with handicapped youths. The fact that these families are also more satisfied with the different life spheres means that both objective and subjective needs are met. The social networks and personal psychological conditions, though, proved to be less sufficient and there is still a lot to be done to give handicapped youngsters a full life.
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.
Advances in the long-term survival and possible cure of the child with cancer have challenged pediatric professionals to no longer view this child as terminally ill, but rather as a developing person with a future. The time of initial diagnosis presents the family with coping tasks which, once mastered, lay the ground work for future adjustment and, hopefully, the child's eventual return to good health. A multidimensional psychosocial assessment can be used to identify strengths and difficulties. A range of psychosocial interventions helps children and families with the coping tasks of this difficult but challenging experience.
Hospitalization due to acute severe asthma represents a failure in the preventive, long-term as well as home care of asthma. Recognition of danger signs and prompt treatment can prevent the risk of morbidity and mortality of an acute asthma episode. The principle pharmacological management is use of inhaled beta2 sympathomemetrics and systemic steroids given with monitoring of respiratory status with the help of clinical parameters and pulmonary function tests. In patients non responsive to routine management, there is a role of inhaled cholinergic compounds, intravenous magnesium sulphate, and beta2 sympathomemetic infusion. Patients in respiratory failure need intensive care. Carefully managed prognosis of an acute attack of asthma is good.
The concept of the truly cured child, denoting a child on par with his or her peers in development, maturation, achievement, and aspirations, was introduced in 1977. 'Cure' is the norm in pediatric oncology. However, the cure of a disease and the consequences of that disease are complex concepts. Cure has at least three components: a biological cure, a psychological cure, and a social cure. Biological and psychological cures have been realized, but the social cure is yet to be achieved. The concept of the truly cured child is widely accepted. School reintegration is the primary method by which psychosocial cure is approached. The characteristics of psychosocial cure and the obstacles that hinder uniformly achieving the goal should be recognized so that the truly cured child can be a realistic goal in pediatric oncology.
Late sequelae of bone marrow transplantation (BMT) include growth impairment, gonadal failure, cataract formation, neurological and pulmonary complications, nephropathy, hepatic dysfunction, development of secondary malignancies, chronic graft-vs.-host disease, and psychosocial stress. Lifelong follow-up after BMT is required to detect late sequelae.
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.
As the treatment of childhood cancer continues to improve, the number of survivors at risk for late effects rises. One such late effect is the risk of second malignant neoplasms. Large multicenter registries have been established to accumulate data on the incidence of second cancers. Relative risks and cumulative risks can now be calculated for retinoblastoma, Wilm's tumor and Hodgkin's disease. Early data are now available for leukemia, sarcomas and central nervous system tumors. Genetic cancer syndromes, radiation therapy and treatment with chemotherapeutic agents are known risk factors for second malignant neoplasms in survivors of childhood cancer.
Linear growth and final adult stature in survivors of childhood cancer may be affected adversely by the disease itself as well as by the treatments utilized. The endocrine causes of impaired growth, which include growth hormone deficiency, primary thyroid dysfunction, and premature sexual maturation, are generally the consequence of radiation therapy. Growth failure is most prominent following craniospinal irradiation for brain tumors and total body irradiation for bone marrow transplantation. Pubertal development in both males and females is generally unaffected by chemotherapy. Leydig cell failure is seen most often after direct testicular irradiation with doses greater than 20 Gy. Ovarian failure is seen commonly following abdominal, craniospinal and total body irradiation.
The aggressive use of multiple therapeutic modalities has led to a significant increase in the number of survivors of childhood malignancy. These forms of cancer therapy have important effects on multiple organ systems. This review article evaluates the long-term effect of therapy on the reproductive potential of both boys and girls. While alkylating agents have been shown to cause a 50% reduction in the fertility potential of boys, they have almost no adverse effect in girls. Other chemotherapeutic agents and combinations of chemotherapeutic agents have also been shown to cause a greater reduction in the reproductive potential of girls than boys. Radiation produces severe dose-related gonadal damage in both boys and girls. The effect of Hodgkin's disease, leukemia and their therapies are evaluated. Despite the known mutagenic potential of some forms of cancer therapy there has not been an increased frequency of congenital abnormalities in the offspring of survivors of childhood cancer. The use of oophoropexy and other forms of prophylactic therapy to limit toxicity are also considered.
With improved medical treatment for childhood cancer, many patients are enjoying long disease-free remission or cure. It is important to address the psychosocial adjustment of the survivor's life. There are two approaches to the study of psychosocial adjustment: study of psychiatric disturbances and assessment of quality of life. Incidences of psychiatric disturbances were reviewed with the most commonly reported difficulties being depression, anxiety and chemical dependency for older survivors and school attendance problems and learning difficulties for school age survivors. The assessment of quality of life focused on school performance, social adjustment, employment status, independent living and marital status. In addition, family coping has received increasing emphasis with regard to the effects on marital relationship and financial difficulties, although there is no consistent evidence to suggest an increased divorce rate in these families. Healthy siblings of cancer survivors are also subject to vulnerability. However, there is evidence to suggest most siblings will resolve their feelings of jealousy, fear of abandonment and establish a normal sibling relationship with the survivor.
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.
