摘要:

Advances are slowly being made in the understanding and treatment of primary childhood brain tumors. The addition of chemotherapy has resulted in better survival for children with medulloblastoma. For other tumor types, therapeutic advances have been slower. High-dose chemotherapy is increasingly being employed to treat malignant childhood tumors, with variable results. Chemotherapy has been effective in low-grade glial tumors.

ISSN:1040-8703    

出版商:OVID    

年代:1997

数据来源: OVID

ISSN:1040-8703    

出版商:OVID    

年代:1997

数据来源: OVID

ISSN:1040-8703    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Over the past decade, a number of important advances have been made in the molecular characterization and the treatment of the primary immunodeficiency disorders. These advances include identification of the abnormal genes responsible for such syndromes as X-linked severe combined immune deficiency, several forms of autosomal severe combined immune deficiency, X-linked and autosomal agammaglobulinemia, Wiskott-Aldrich syndrome, and other primary immunodeficiency disorders. In the past year, the biologic functions of the abnormal gene products responsible for these syndromes have been better defined, and new molecular defects that lead to primary immunodeficiency disorders have also been reported. This better understanding of the molecular basis of the primary immunodeficiency disorders has led to improvement of established therapies, such as gene product replacement and stem cell transplants, and to new treatment strategies, such as gene therapy.

ISSN:1040-8703    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Atopic dermatitis is the most common chronic skin disease of young children and is frequently associated with asthma and allergies. Th2-type cytokine secreting T cells expressing the cutaneous lymphocyte-associated antigen play a central role in the induction of local IgE responses and recruitment of eosinophils in this disease. Chronic inflammation in atopic dermatitis likely involves a number of interdependent factors, including repeated or persistent exposure to allergens, which can lead to Th2-cell expansion. In addition, exotoxins secreted byStaphylococcus aureusacting as both superantigens and allergens can contribute to persistent inflammation or exacerbations of atopic dermatitis. Treatment of atopic dermatitis with topical agents such as tacrolimus ointment and phospho-diesterase-4 inhibitors offers new approaches directed at correction of the immune dysfunction associated with this disease.

ISSN:1040-8703    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Immunotherapy has been shown to prevent rhinitis symptoms in seasonal allergic rhinitis, and to prevent anaphylaxis to hymenoptera and fire ant stings. The evidence supporting its effectiveness in the treatment of asthma is much more limited; it appears that clinical trials, until the past year, have only tested therapy for single allergens such as house dust mite, cats, pollen, and mold. In the past year, a double-blind placebo-controlled clinical trial was completed that tested immunotherapy with a mixture of multiple allergen extracts in 121 perennial asthmatic children. This was the first test of treatment with allergen mixtures. The trial showed that over 30% of children underwent a complete or partial remission during the 2.5 years of therapy, but the changes were almost identical in both treatment and placebo control groups. Other trials were reported that examined injection therapy using synthetic fragments of the major allergen responsible for allergic reactions to cats. In this trial, acute rhinitis and asthma during exposure to naturally occurring airborne particles of cat allergen were reduced significantly.

ISSN:1040-8703    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Although discussed for many years, systemic adverse effects from inhaled corticosteroid therapy remains a complicated and controversial topic. It is by now well-known that inhaled corticosteroids may affect growth, bone, and adrenal function, yet the clinical relevance and the risks for such adverse effects are poorly defined. Earlier intervention, use of higher doses, differing potency claims, and new inhalers also affect the potential for systemic effects following inhaled administration. Until further research better characterizes these risks and identifies appropriate monitoring, caution in the use of this highly effective therapy is advised.

ISSN:1040-8703    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Adherence to clinical regimens is paramount in determining their effectiveness. Asthma is a chronic illness that requires consistent, anti-inflammatory therapy. A “high risk” profile for fatal or near-fatal asthma has emerged that includes suboptimal pharmacotherapy in conjunction with severe disease, psychologic dysfunction, black race or Hispanic-American ethnicity, and residence in an urban area with a high concentration of poverty. Collaboration between physicians and patients is necessary to allow individuals to make choices informed by medical facts and the physician's experience. Objective monitoring of adherence and pulmonary function and acceptance by the clinicians of the National Asthma Education and Prevention Program guidelines to control airway inflammation are essential in order to reduce the unacceptable rates of morbidity and mortality associated with asthma.

ISSN:1040-8703    

出版商:OVID    

年代:1997

数据来源: OVID

ISSN:1040-8703    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Most of the genes involved in the pathogenesis of the DNA replication and repair syndromes have now been cloned, and our understanding of the basis for the pleiotropic phenotype associated with many of these syndromes has rapidly and dramatically expanded. The elucidation of the specific interactions between proteins that comprise the transcription factor complex TFIIH raises the possibility that nucleotide excision repair, RNA polymerase II transcription, and cell cycle control are connected. Defects in theXPB, XPD, andXPGgenes can result in three different syndromes, xeroderma pigmentosum, Cockayne syndrome, or trichothiodystrophy, depending on the specific mutation involved. The recent cloning of the genes involved in Bloom syndrome (BLM) and Werner syndrome (WRN) show that both are DNA and RNA helicases with homology to each other and to other DExH box helicases, yet the mechanism by which defects in these genes cause such different phenotypes is not yet understood. The ataxia-telangiectasia gene (ATM) is involved in a variety of signal transduction pathways that regulate the cellular response to normal proliferative stimuli as well as the response to DNA damage, and the disruption of these signal transduction pathways provides an explanation for ataxia-telangiectasia characteristics such as ionizing radiation sensitivity, immunodeficiency, and infertility. Although the first Fanconi anemia gene (FAC) was cloned over 5 years ago, and a second Fanconi anemia gene (FAA) was cloned in 1996, the biochemical function of Fanconi anemia proteins largely remains a mystery. The recent construction of mutant mouse strains for several of these diseases should help unlock the difficult puzzle of the pathogenesis of these syndromes.

ISSN:1040-8703    

出版商:OVID    

年代:1997

数据来源: OVID