Neuronal intranuclear inclusion disease: Two cases of dopa‐responsive juvenile parkinsonism with drug‐induced dyskinesia

There are very few conditions that present with dopa‐responsive juvenile parkinsonism. We present two such children with neuronal intranuclear inclusion disease (NIID) who had an initial good levodopa response that was soon complicated by disabling dopa‐induced dyskinesia. One child was diagnosed by rectal biopsy in life, and the other diagnosis was confirmed at postmortem. In this patient, dopamine transporter imaging showed severely decreased binding of the radiotracer in the striatum on both sides. Bilateral subthalamic deep brain stimulation in this patient produced initial improvement, but this was not sustained. Both patients died within 10 years of symptom onset. As well as levodopa responsiveness with rapid onset of dyskinesia, clues to the diagnosis of NIID in patients presenting with parkinsonism include the presence of gaze‐evoked nystagmus, early onset dysarthria and dysphagia and oculogyric crises. Differential diagnosis of clinical symptoms and neuropathological findings are discussed including the approach to rectal biopsy for early diagnosis. © 2010 Movement Disorder Society     

Work-related exacerbation of asthma

Adults with asthma who had been enrolled in an HMO for at least a year were requested to complete a questionnaire about their health status. Approximately 25% of the 1,461 participants responded positively to "Does your current work environment make your asthma worse?" and were classified as having workplace exacerbation of asthma. Those with workplace exacerbation were more likely to have never attended college, be current or former smokers, have a history of other respiratory diseases, have missed work or usual activities at least one day in the past for weeks, and report their asthma was moderate, severe, or very severe. Percentages with workplace exacerbation of asthma were highest for mining and construction (36%), wholesale and retail trade (33%), and public administration (33%), and lowest for educational services (22%), finance, insurance, and real estate (22%), and non-medical and non-educational services (18%). Future studies are needed for objective validation of self-reported workplace exacerbation, and to follow subjects prospectively to clarify the temporal sequence of workplace exacerbation and asthma severity, and how other respiratory conditions and smoking might contribute to work-related worsening of asthma.     

Adherence to CDC STD guideline recommendations for the treatment of Chlamydia trachomatis infection in two managed care organizations

BACKGROUND: The extent of adherence to the Centers for Disease Control and Prevention (CDC) STD guidelines by clinicians practicing in managed care settings is unknown.GOAL The goal was to assess adherence to the CDC guideline recommendations for the treatment of genital chlamydial infection, by clinicians at two group model managed care organizations. DESIGN: Retrospective cohort study of men and women with laboratory-confirmed chlamydial infection. Patients were members of either the Kaiser Permanente Foundation Health Plan of Colorado or HealthPartners of Minneapolis/St. Paul who had tested positive for cervical or urethral chlamydial infection during the period from January 1, 1998, through June 30, 1999. RESULTS: During the study period, 1,078 patients with positive tests for genital Chlamydia trachomatis were identified. More than 97% of men and nonpregnant women and more than 98% of pregnant women were prescribed treatment, consistent with current CDC guidelines. CONCLUSION: Adherence to CDC-recommended therapy was high for patients with genital chlamydial infections at these two managed care organizations.     

The prognostic value of QT dispersion in patients presenting with acute neurological events

QT dispersion (QTD) reflects heterogeneity of myocardial repolarization, which is modulated by the central nervous system. Previous studies have shown increased QTD to be a predictor of adverse outcomes in various cardiac states. The objective of this study was to determine the significance of QTD in patients hospitalized with cerebrovascular accidents (CVA) and transient ischemic accidents (TIA). We studied 140 consecutive patients (72 years old, 48% male) admitted to our institution with neurologic events from January 1998 to April 1998. QTD was calculated from admission electrocardiogram as the difference between maximum and minimum QT intervals in at least 11 of 12 leads. Three separate instruments (NIH Stroke Scale, Barthel Index and Modified Rankin Scale) were used to assess functional status on discharge. QTD was higher in patients with intercerebral hemorrhage as compared to CVA and TIA (70 15 msec versus 53 27 msec versus 48 31 msec, respectively; p = 0.03). Increasing QTD was associated with lower functional outcomes on all 3 scales (all p < 0.05) and with higher mortality (p = 0.02). QTD was higher in patients with congestive heart failure (80 43 msec versus 47 24 msec; p = 0.006) and with carotid disease (59 32 msec versus 46 27 msec; p = 0.045) as compared to those without. On multivariate analysis, other independent predictors of worse outcome were QTD (odds ratio, 1.35; 95% confidence interval, 1.08 1.68) and a trend toward age (odds ratio, 1.07; 95% confidence interval, 0.99 1.16). On age-adjusted logistic regression, mortality increased by odds ratio of 1.28 and 95% confidence interval of 1.02 1.61 for every 10 msec increase in QTD. QTD is an independent predictor of functional outcome and mortality following acute neurological events. In this setting, QTD reflects neurological injury as well as underlying heart disease.     

Analyses of apoptotic regulators CASP9 and DFFA at 1P36.2, reveal rare allele variants in human neuroblastoma tumours

The genes encoding Caspase-9 and DFF45 have both recently been mapped to chromosome region 1p36.2, that is a region alleged to involve one or several tumour suppressor genes in neuroblastoma tumours. This study presents an update contig of the 'Smallest Region of Overlap of deletions' in Scandinavian neuroblastoma tumours and suggests that DFF45 is localized in the region. The genomic organization of the human DFF45 gene, deduced by in-silico comparisons of DNA sequences, is described for the first time in this paper. In the present study 44 primary tumours were screened for mutation by analysis of the genomic sequences of the genes. In two out of the 44 tumours this detected in the DFFA gene one rare allele variant that caused a non-polar to a polar amino acid exchange in a preserved hydrophobic patch of DFF45. One case was hemizygous due to deletion of the more common allele of this polymorphism. Out of 194 normal control alleles only one was found to carry this variant allele, so in respect of it, no healthy control individual out of 97 was homozygous. Moreover, our RT-PCR expression studies showed that DFF45 is preferably expressed in low-stage neuroblastoma tumours and to a lesser degree in high-stage neuroblastomas. We conclude that although coding mutations of Caspase-9 and DFF45 are infrequent in neuroblastoma tumours, our discovery of a rare allele in two neuroblastoma cases should be taken to warrant further studies of the role of DFF45 in neuroblastoma genetics.     

Lack of an association between medicare supplemental insurance and delay in seeking emergency care for patients with myocardial infarction

STUDY OBJECTIVE: Previous studies have shown that Medicare patients without supplemental insurance are less likely to receive preventive services, such as mammography. The effect of supplemental insurance on the appropriate use of emergency services is unknown. We sought to determine whether the absence of supplemental Medicare coverage is associated with increased delay in seeking care for acute myocardial infarction. METHODS: In this retrospective cohort study, we compared the time from symptom onset to hospital arrival (the time-delay interval) in Medicare patients with and without supplemental insurance coverage who presented with an acute myocardial infarction to 1 of 19 hospitals in King County, WA, from 1989 to 1993. There were 1,373 patients with Medicare-only coverage and 2,050 patients with Medicare plus supplemental insurance coverage. RESULTS: The age-, sex-, and race-adjusted median time delay was 135 minutes for the Medicare-only group and 130 minutes for the Medicare plus supplemental insurance group (P =.34; 95% confidence interval for median time-delay difference in minutes -5 to 10). There was no significant association between the presence of Medicare supplemental insurance coverage and time delay in Cox regression models, which also adjusted for event year, income, education, past cardiac history, and clinical symptoms. CONCLUSION: For this cohort of Medicare patients, the absence of supplemental insurance coverage did not lead to significantly increased delays in seeking care for myocardial infarction. Lack of supplemental insurance for Medicare patients might not have as great an effect on the use of emergency services as it has on other health care services.     

Major congenital malformations and residential proximity to a regional industrial park including a national toxic waste site: An ecological study

Background  

Public concern about exposure to emissions from the regional industrial park (IP), including 17 chemical plants and the national industrial toxic waste site, initiated this study of the possible association between major congenital malformations (MCM) and residence near the IP in Israel's Southern District.     

Decreasing prevalence of salivary lactobacilli in Swedish schoolchildren 1987-1998

The prevalence and number of salivary lactobacilli was determined in all schoolchildren starting the 7th grade (12-13 yr; n = 1,578) in comprehensive school located in Kumla, Sweden between 1987 and 1998. Whole saliva samples were collected and transferred to dip-slides (Dentocult-LB) and incubated in room temperature for 7 d. Caries data were collected from the dental records and from bitewing radiographs. Both the number of lactobacilli and the prevalence of manifest caries and restorations exhibited a decreasing tendency and were significantly lower in 1998 than in 1987. The mean caries prevalence (DFS) declined from 5.2 to 1.8. In 1987, 45% of the children harboured high or very high lactobacilli counts ( > or =10(5) CFU/ml) in their saliva compared to approximately 10-15% at the later examinations. The proportion of children with no detectable counts varied between 4-29% during the study period, while children with low counts (10(3)-10(4) CFU/ml saliva) constituted a clear majority at the recent samplings. With such low levels in the population, the use of lactobacilli counts as a didactic tool in dietary counselling must be called into question.     

Good nurse, bad nurse…

The construction of the nursing subject is discussed The paper takes a historical perspective, arguing that the range of speaking positions available to the nurse is limited by gender, class and education It evaluates the position of nursing in the university, showing how this also has the propensity to limit the development of the nursing profession