Development of a Regionalized,Comprehensive Care Network for Pediatric Sickle Cell Disease to Improve Access to Care in a Rural State

Sickle cell disease (SCD), an inherited group of blood disorders, is a major public health problem worldwide. Patients experience severe anemia, increased risk of life-threatening infections, painful crisis, and chronic organ damage. Access to comprehensive care for SCD is known to improve outcomes; however, it is only reported from large urban centers serving one metropolitan area. Alabama, US, is a largely rural state with a significant number of children born each year with SCD. Prior to the development of our regional clinic network, the Children and Youth Sickle Network (CYSN^SM), 50% of patients identified by newborn screening were not enrolled in comprehensive sickle cell care. The majority of non-enrolled patients lived in southern Alabama. Rural areas in this region are particularly plagued by poverty and poor access to healthcare. Life expectancy is equivalent to residents of Sri Lanka. This area has 15.7 doctors/10 000 residents compared with the statewide ratio of 41.9 doctors/10 000 residents.To improve access to care, a regional clinic network, the CYSN^SM, was established in 1995. This paper reviews the impact of the CYSN^SM on pediatrie SCD in Alabama over the first 5 years of implementation.Since its inception in 1995, the CYSN^SM has provided care for 923 patients compared with 450 prior to the development of the clinic network. Currently, 90% of all cases identified by newborn screening are enrolled compared with 50% pre-CYSN^SM. Prior to the network, the average age of patients at their first clinic visit was 21 months. In the post-CYSN^SM period, the average age at first clinic visit decreased substantially to 5.3 months. Prior to the CYSN^SM, patients traveled on average 90 miles to a comprehensive clinic. Post-CYSN^SM, this distance has been cut in half to an average of 45 miles. A total of 70% of patients now live within 30 miles of a clinic. Most importantly, the infection death rate has decreased from 5.71 deaths/100 patient years to 1.94 deaths/100 patient years.The development, implementation, and evaluation of the CYSN^SM show that comprehensive care delivery in a rural setting is feasible and improves outcomes in pediatric SCD.     

A Comparison of Visual Field and Optic Disc Appearance Depending on the Peak Intraocular Pressure in Patients with Normal-tension Glaucoma

Purpose To review the outcome of surgery for strabismus due to ethmoid sinus surgery.Cases and Methods The series comprised 13 cases, 1 of inferior rectus paresis, 1 of superior oblique paresis, 6 of medial rectus paresis, and 5 of medial rectus muscle palsy due to third nerve palsy. In the cases of paresis of the rectus muscle, resection of the rectus muscles was mainly performed. In the cases of palsy of the rectus muscle, transposition of the extraocular muscle with simultaneous recession of the lateral rectus muscle was performed. The major aim of surgery was to bring both eyes into alignment and to eliminate diplopia in the primary position.Results The mean preoperative horizontal deviation of 18.1 degrees of exotropia in the paresis cases was reduced to 1.4 degrees of exotropia after surgery. The mean preoperative vertical deviation of 3.8 degrees of hypertropia was reduced to 1.4 degrees of hypertropia postoperatively. The mean preoperative horizontal deviation of 35.6 degrees of exotropia in the palsy cases was reduced to 9.4 degrees of exotropia after surgery. The mean preoperative vertical deviation of 2.0 degrees of hypertropia was increased to 2.6 degrees of hypertropia postoperatively. Postoperatively, diplopia was absent in 11 cases with a slightly compensatory head posture.Conclusions Surgery for strabismus due to sinus surgery induces improvements in eye position and diplopia. Nippon Ganka Gakkai Zasshi (J Jpn Ophthalmol Soc 107:425–432, 2003)     

Estimating numbers of injecting drug users in metropolitan areas for structural analyses of community vulnerability and for assessing relative degrees of service provision for injecting drug users

This article estimates the population prevalence of current injection drug users (IDUs) in 96 large US metropolitan areas to facilitate structural analyses of its predictors and sequelae and assesses the extent to which drug abuse treatment and human immunodeficiency virus (HIV) counseling and testing are made available to drug injectors in each metropolitan area. We estimated the total number of current IDUs in the United States and then allocated the large metropolitan area total among large metropolitan areas using four different multiplier methods. Mean values were used as best estimates, and their validity and limitations were assessed. Prevalence of drug injectors per 10,000 population varied from 19 to 173 (median 60; interquartile range 42–87). Proportions of drug injectors in treatment varied from 1.0% to 39.3% (median 8.6%); and the ratio of HIV counseling and testing events to the estimated number of IDUs varied from 0.013 to 0.285 (median 0.082). Despite limitations in the accuracy of these estimates, they can be used for structural analyses of the correlates and predictors of the population density of drug injectors in metropolitan areas and for assessing the extent of service delivery to drug injectors. Although service provision levels varied considerably, few if any metropolitan areas seemed to be providing adequate levels of services.     

Capacity and Competence in Child and Adolescent Psychiatry

Capacity and competence in the field of child and adolescent psychiatry are complex issues, because of the many different influences that are involved in how children and adolescents make treatment decisions within the setting of mental health. This article will examine some of the influences which must be considered, namely: developmental aspects, the paradoxical relationship between the need for autonomy and participation and the capacity of children, family psychiatry, and the duty of care towards children and adolescents. The legal frameworks relevant to consideration of consent and competence will be briefly considered, as well as some studies of children's consent, participation and competence. A case vignette will be used as a focus to consider the complexity of the issue of competence in child and adolescent psychiatry, in the particular mental disorder of anorexia nervosa.     

Patient Capacity in Mental Health Care: Legal Overview

The discriminatory effects of categorizing psychiatric patients into competent and incompetent, have urged lawyers, philosophers and health care professionals to seek a functional approach to capacity assessment. Dutch and English law have produced some guidelines concerning this issue. So far, most legal systems under investigation have concentrated on alternatives for informed consent by the patient in case of mental incapacity, notably substitute decision-making, intervention of a judge and advance directives. It is hard to judge the way in which the law may further adapt to a more functional assessment of capacity, because the nature of law shows that legal reforms usually take place only when new methods have been accepted by the field. This is not yet the case today.     

Expression of apoptosis-related proteins in adenomyotic uteri treated with danazol and GnRH agonists.

The biologic properties of adenomyosis and the effects of therapeutic agents on adenomyosis were evaluated with immunohistochemistry, terminal deoxy-nucleotidyl transferase-mediated dUTP-biotin nick end-labeling (TUNEL) method, transmission electron microscopy, and analysis of genomic abnormality. In the adenomyotic endometrium, estrogen receptor (ER) expression was more intense than in the eutopic endometrium during the secretory phase, and bcl-2 was constantly expressed throughout the menstrual cycle. The expression of ER and bcl-2 was weaker in the adenomyotic endometrium treated with danazol than in that treated with gonadotro-pin-releasing hormone agonist (GnRHa), whereas bcl-2 phosphorylated on serine-87 was more intensely expressed in danazol-treated adenomyotic endometrium than in the GnRHa-treated one. The number of TUNEL-positive cells increased in the adenomyotic endometrium treated with danazol or GnRHa. Ultrastructurally, most of the adenomyotic endometrial cells treated with danazol underwent postapoptotic necrosis and formed a cluster of dead cells. In contrast, cells treated with GnRHa underwent typical apoptosis and were sparsely distributed in the adenomyotic endometrium. Analysis of several cancer-related genes showed no microsatellite instability or loss of heterozygosity in adenomyotic tissues. Therefore, we conclude that the occurrence of adenomyosis is correlated to bcl-2 expression regulated by estrogen and ER rather than genetic mutation.     

Three-dimensional analysis of benign paroxysmal positional nystagmus in a patient with anterior semicircular canal variant.

OBJECTIVE: To show the positional nystagmus in a patient who had suffered from benign paroxysmal positional vertigo (BPPV) that was thought to be caused by involvement of the anterior semicircular canal (ASCC) (A-BPPV). STUDY DESIGN: Retrospective case report. SETTING: City hospital. PATIENT: The present study reports a rare case of A-BPPV in a 41-year-old woman. CASE REPORT: The patient is 41-year-old woman who developed a positional vertigo after playing volleyball on March 22, 2005 and consulted our hospital the next day. When left Dix-Hallpike maneuver was performed, she showed a positional nystagmus of which fast phase direction of the torsional component was clockwise while that of the vertical component was downward. We plotted the slow phase eye velocity of the positional nystagmus during the left Dix-Hallpike maneuver on three-dimensional coordinates that showed the axis of the positional nystagmus to be perpendicular to the plane of the right ASCC. CONCLUSION: These results suggested that the patient was suffering from A-BPPV.