ISSN:1040-8703    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

Imaging investigation of the pediatric central nervous system may be considered in terms of structural imaging and functional imaging. Established structural imaging modalities include ultrasonography, radiography, computed tomography, magnetic resonance imaging (MRI), cerebrospinal fluid imaging (eg, myelography), and angio-graphy. Functional imaging technologies include single photon emission computed tomography (SPECT), positron emission tomography, magnetic resonance spectroscopy, and magnetic resonance perfusion imaging. Furthermore, more recent advances in computer software now allow the superimposed display of separate imaging modalities using image fusion techniques. This technique allows the superimposition of structural imaging data with functional imaging data, eg, SPECT and MRI, or the fusion of structural imaging modalities, eg, computed tomography and MRI. In this respect, current practical and investigative applications of structural and functional imaging of the pediatric central nervous system are reviewed.

ISSN:1040-8703    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

During this “Decade of the Brain” there have been hopeful developments in childhood seizures. 1993, in particular has been an exciting time for physicians caring for patients with epilepsy. Three promising new antiepileptic drugs will be introduced and several more are undergoing clinical trials. Epilepsy surgery is becoming a more frequent option in children with medically intractable seizures, particularly those with catastrophic epileptic syndromes. Improved anatomic and functional neuroimaging has improved our ability both to detect brain anomalies responsible for seizures and to localize the epileptic focus. The increasing availability of new antiepileptic drugs coupled with the increasing success of epilepsy surgery has dramatically improved the life of many children with epilepsy.

ISSN:1040-8703    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

Our ability to identify cerebrovascular lesions in children has improved in recent years and with it our willingness to consider the diagnosis of cerebrovascular disease in children. Magnetic resonance angiography and other tests have made diagnosis easier and safer, particularly in very young children and in patients with sickle cell disease. Transcranial Doppler examination makes it possible to accurately predict without angiography which patients with sickle cell disease are at most risk frr stroke. Many patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS) syndrome have a specific mutation of the mitochondrial DNA, and soon the recently devised diagnostic criteria for MELAS syndrome may be replaced by molecular markers. There are many causes of cerebrovascular disease in children, and a thorough evaluation will discover a likely cause in about two thirds of the patients. Recent attention has focused on the HLA-B51 antigen and chicken pox as possible risk factors. Magnetic resonance imaging is also helpful in assessing the prognosis of children with cerebrovascular disease or head trauma.

ISSN:1040-8703    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

This review covers three areas. First, dramatic progress in the discovery of abnormal loci on certain chromosomes associated with several motor unit diseases eventually will obviate the need for more invasive testing. Second, new information about the natural course of spinal muscular atrophy is beginning to clarify the nature of this disease. Third, changes in the treatment of the acute and chronic polyneuropathies has shortened hospital stays.

ISSN:1040-8703    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

Cognitive disorders affect thinking and perceptual processes and the acquisition of knowledge and new information. They have an enormous societal impact because special educational resources are required, and independent living often cannot be achieved. Learning problems may lead to behavioral disorders in the home and community. The pathogenesis of most mild and moderate cognitive disorders is poorly understood. Severe cognitive impairment is usually accompanied by somatic abnormalities, and an etiology can be identified in many cases. Specific treatments are available for disorders such as congenital hypothyroidism, some metabolic acidurias, and congenital toxoplasmosis. Other disorders affecting cognition such as fetal alcohol syndrome, maternal cocaine and heroin exposure, HIV encephalopathy, and prematurity require aggressive prevention and education to reduce their occurrence. The recent advances in molecular genetics offer a faster and better method of diagnosing fragile X syndrome, now recognized as the most common inheritable cause of mental retardation. In the future, DNA analysis may elucidate the basis of many other cognitive disorders.

ISSN:1040-8703    

出版商:OVID    

年代:1993

数据来源: OVID

ISSN:1040-8703    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

The molecular basis for three well-defined X-linked diseases has recently been identified. In X-linked agammaglobulinemia, the gene encoding a novel cytoplasmic tyrosine kinase (btk) expressed by B cells is defective. This B-cell-specific kinase belongs to a new subfamily of tyrosine kinases. The molecular defect in X-linked hyper IgM affects the gene encoding the CD40 ligand (CD40L, gp39) on T cells. This protein binds to its natural receptor, CD40, expressed constitutively by B cells. The ligand-receptor interaction initiates B-cell proliferation and isotype switching. The molecular defect in X-linked severe combined immunodeficiency disease has been assigned to the gene encoding the y chain of the interleukin-2 receptor (IL-2Rγ), which is constitutively expressed by T cells and is involved in the formation of high and intermediate affinity IL-2R complexes. IL-2R-γ is responsible for the failure of X-linked severe combined immunodeficiency disease T and B lymphocytes to respond to IL-2–dependent signals.

ISSN:1040-8703    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

Allergen avoidance should be considered as a first line of defense for all types of allergic disease. For some allergens, such as the common pollens, significant avoidance may be difficult or even impossible to accomplish without major alterations in lifestyle. For others, however, particularly the common indoor allergens, avoidance is a very real option that can be achieved through practical environmental control measures. In addition to the inherent logic of allergen avoidance, there are substantial data to support its efficacy in the treatment of patients with atopic disease, particularly asthma. Further, there is an emerging body of evidence to support its role in the prevention of allergic disease in infants and children. In this article, recent research advances in the areas of indoor allergens, environmental control, and allergen avoidance are reviewed.

ISSN:1040-8703    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

IgG subclass deficiency has been noted in association with a variety of clinical conditions. It's relative importance as a contributing factor to these conditions is unclear. Despite reservations about the importance of partial IgG subclass deficiency, evidence is beginning to emerge that suggests that in select patients with recurrent infection and subclass deficiency, gamma globulin therapy may be beneficial.

ISSN:1040-8703    

出版商:OVID    

年代:1993

数据来源: OVID