Following Catastrophic Epilepsy Patients from Childhood to Adulthood

Summary:  As patients with catastrophic epilepsies move from childhood to adulthood, evolving and innovative therapeutic regimens are often required. However, the goal of providing the best quality of life while minimizing both seizures and side effects remains the same. Clinicians can develop appropriate care plans by being aware of patients' changing needs. Clinical symptoms of the catastrophic epilepsies may change over time; by understanding the natural history of a patient's condition, clinicians can help ease the transition from childhood to adulthood. Additionally, as children with catastrophic epilepsies become adults, medical issues (e.g., medication side effects, tolerance, and dependence) and nonmedical issues (e.g., guardian/caretaker issue, group home applications, and respite care options) must be considered when developing strategies for patient care. Regular assessment of patients, the development of emergency plans, and maintenance of consistency in the delivery of care are also important issues to consider. Finally, a multidisciplinary care plan that incorporates resources from health-care practitioners, social service professionals, and community agencies can be valuable in optimizing treatment for patients with catastrophic epilepsies.     

Bell's palsy associated with IFN-alpha and ribavirin therapy for hepatitis C virus infection.

First-line therapy for hepatitis C virus (HCV) infection comprises interferon-alpha (IFN-alpha) and ribavirin for 6 or 12 months. Mild complications of therapy are common, but more serious complications are rare. Three patients with chronic HCV infection, acquired through injecting drug use, developed idiopathic facial paralysis (Bell's palsy) during therapy, with spontaneous resolution after withdrawal of treatment. Large-scale cohort studies reveal that IFNs are associated rarely with neurologic complications, and only one previous report has linked IFN-alpha therapy and Bell's palsy. We postulate that IFN-alpha therapy led to a breakdown of peripheral tolerance to myelin sheath antigens, leading to neuropathy, just as IFN-alpha therapy can cause autoimmune thyroiditis through breakdown of tolerance to native thyroid antigens.     

Cricoid split for subglottic stenosis in infancy

Historically, tracheostomy has been used for infants with airway obstruction caused by congenital or acquired subglottic stenosis. Postoperative morbidity and mortality with this provisional operation led Cotton, in 1980, to substitute anterior cricoid split as the primary definitive procedure. Within the past three years, anterior cricoid split has been performed in 4 infants, aged 3 to 9 months, with acquired (3 patients) or congenital (1 patient) subglottic stenosis requiring ventilation through an endotracheal tube. Following cricoid split, the trachea is stented for 12 to 14 days by a nasotracheal tube, with extubation and rigid bronchoscopy in the operating room with the patient under anesthesia to confirm healing and patency. During an 18- to 24-month follow-up in these 4 patients, morbidity has been minimal, patency has persisted, and stridor has not recurred. Accordingly, a conclusive operation, cricoid split, rather than a temporizing tracheostomy may be employed for certain obstructive tracheal lesions early in life.     

The metamorphosis of health planning in the Netherlands and the USA

The United States and the Netherlands are the focus for this comparative analysis of the evolutionary interaction between health planning and the political system, seen in the context of change in social and economic ideologies. While health planning in the USA started in 1946, it was the comprehensive health planning program in 1966 that created the form to be followed by Health Systems Agency effort in 1974: local, voluntary planning, coordinated by state agencies, supported by federal funding. Health planning in the Netherlands has moved through four distinct periods: a hospital construction period, starting during the post-war recovery; a hospital regionalization period, from 1971 through the late 1970s; a transition period from the late 1970s to 1982, during which several planning approaches were considered; and, the current comprehensive health and social services planning period. Today, federal support for health planning in the US has been eliminated as part of the current de-regulatory, competitive health care strategy. Health planning in the US is now an institutional activity, with less focus on community needs. Advocated changes in the Dutch planning approach incorporate ideas similar to past approaches in the US; but, a failed approach in one nation may work in another, if the underlying cultural and organizational characteristics are sufficiently different.     

Adult outcomes of attention deficit hyperactivity disorder and conduct disorder: are the risks independent or additive?

METHODS: Data were obtained from a longitudinal study sample of 754 adoptees and categorized based on review of the available adoption agency, medical, and psychiatric records of the biological parents. Categorical data were analyzed using chi2 or Fisher's exact tests, as appropriate. Logistic regression analyses were used to assess the relative contribution of variables. RESULTS: There was not a statistically significant difference in the frequency or type of self-reported adult disruptive behavior, arrests, jail stays, felony arrests, or frequency of conduct disorder (CD) when inattentiveness, impulsivity, and hyperactivity were analyzed individually. The contributions of attention deficit hyperactivity disorder (ADHD) were independent and no additional increased risk for future illegal behavior was conferred by the combination of the disorders. While the effect of CD on illegal behavior was correlated with substance abuse and dependence, ADHD continued to be a significant contributor after controlling for substance abuse and dependence. CONCLUSIONS: Data indicated that ADHD and CD are related but different disorders conferring risk for adult illegal behavior or arrest. In this sample, inattention was the most common domain impaired among those with ADHD, followed closely by hyperactivity, with impulsivity reported least often among those endorsing symptoms of ADHD.     

Blood pressure related separately to parenchymal fibrosis and vasculopathy of the kidney.

The term nephrosclerosis is customarily used to designate a pathological entity that tends to characterize subjects with high blood pressure; it refers to a condition of diffuse fibrous replacement of renal substance secondary to ischemia from hypertension-related vascular injury. The features of parenchymal fibrosis can be distinguished from those of vasculopathies in tissue sections, parenchymal fibrosis being measured by assessing the degree of interstitial fibrosis and by counting obsolete glomeruli, while vasculopathies are measured by determining arterial intimal fibroplasia and by counting hyalinized arterioles. A series of 166 autopsies in subjects aged 25 to 92 years, selected because ample documentation of blood pressure was available, was assessed. One form of vasculopathy, arterial fibroplasia, is a better correlate of high blood pressure than is parenchymal fibrosis in this body of data. Cases with much vasculopathy and little parenchymal fibrosis occurred frequently, and these subjects were usually hypertensive. Cases with little vasculopathy and much parenchymal fibrosis were also encountered, but these subjects were usually not hypertensive. The suggested conclusion is that blood pressure relates less to the renoprival state of nephron loss than it does to renal ischemia in patients with nephrosclerosis.     

Examining Equivalence of Concepts and Measures in Diverse Samples

While there is growing awareness for the need to examine the etiology of problem behaviors across cultural, racial, socioeconomic, and gender groups, much research tends to assume that constructs are equivalent and that the measures developed within one group equally assess constructs across groups. The meaning of constructs, however, may differ across groups or, if similar in meaning, measures developed for a given construct in one particular group may not be assessing the same construct or may not be assessing the construct in the same manner in other groups. The aims of this paper were to demonstrate a process of testing several forms of equivalence including conceptual, functional, item, and scalar using different methods. Data were from the Cross-Cultural Families Project, a study examining factors that promote the healthy development and adjustment of children among immigrant Cambodian and Vietnamese families. The process described in this paper can be implemented in other prevention studies interested in diverse groups. Demonstrating equivalence of constructs and measures prior to group comparisons is necessary in order to lend support of our interpretation of issues such as ethnic group differences and similarities.     

Heterogeneity of human bone

Matched samples of bone from the lumbar spine and tibia were obtained at autopsy from three adult males who had no known evidence of metabolic bone disease at the time of their demise. The soluble noncollagenous bone proteins were quantitatively extracted from these samples and assayed for the relative content of two bone-associated proteins, osteocalcin and osteonectin. When compared to trabecular bone, cortical bone had higher levels of osteocalcin and much lower levels of osteonectin. When concentration is expressed per gram of dried bone, the osteocalcin excess in cortical bone ranged from 30- to 32-fold, and the osteonectin excess in trabecular bone ranged from 21- to 47-fold. These differences were significant (P less than 0.01) using analysis of variance. We conclude that the human skeleton is not homogeneous with regard to these biochemical markers and that cortical and trabecular bone are biochemically quite distinct. This implies that these two types of bone may be subject to distinct regulatory mechanisms and that global assessments of skeletal function and bone quality based upon soluble markers should be applied with caution. The data also imply that a differential assessment of skeletal performance may be possible using biochemical serum markers.