Gastrointestinal manifestations in Williams syndrome: A prospective analysis of an adult and pediatric cohort.

Williams syndrome (WS) is a multi-system condition caused by the deletion of 25-27 coding genes on human chromosome 7. Irritability, gastrointestinal (GI) reflux and slow growth are commonly reported in infants with WS, but less data exist regarding GI concerns in older children and adults with the condition. This study evaluates 62 individuals with WS (31 children aged 3-17, and 31 adults aged 18-62) as well as 36 pediatric and adult controls to assess current and historical rates of common GI symptoms. Data were evaluated using a regression model including age, sex, self-reported race, and diagnosis. Symptoms including food intolerance, reflux, dysphagia, choking/gagging, vomiting, constipation, bloating, diarrhea, hematochezia, rectal prolapse, abdominal pain, and weight loss are more common in those with WS relative to controls. In addition, people with WS utilize more GI medications, specialty care, procedures, and supplemental feeds. Among those with WS, symptoms were present at similar rates in children and adults, except for diverticular disease, which was not noted until adulthood. GI symptoms are frequent in people with WS and serve as a significant source of morbidity.

Acid Suppression and Antibiotics Administered during Infancy Are Associated with Celiac Disease.

OBJECTIVE: To investigate why certain at-risk individuals develop celiac disease (CD), we examined the association of proton pump inhibitors (PPI), histamine-2 receptor antagonists (H2RAs), and antibiotic prescriptions in the first 6 months of life with an early childhood diagnosis of CD. STUDY DESIGN: A retrospective cohort study was performed using the Military Healthcare System database. Children with a birth record from October 1, 2001, to September 30, 2013, were identified. Outpatient prescription records were queried for antibiotic, PPI, and H2RA prescriptions in the first 6 months of life. Cox proportional hazards regression was used to calculate the hazard ratio (HR) of developing CD based on medication exposure. International Classification of Diseases, Ninth Revision, Clinical Modification codes identified children with an outpatient visit for CD. RESULTS: There were 968 524 children who met the inclusion criteria with 1704 cases of CD in this group. The median follow-up for the cohort was approximately 4.5 years. PPIs (HR, 2.23; 95% CI, 1.76-2.83), H2RAs (HR, 1.94; 95% CI, 1.67-2.26), and antibiotics (HR, 1.14; 95% CI, 1.02-1.28) were all associated with an increased hazard of CD. CONCLUSIONS: There is an increased risk of developing CD if antibiotics, PPIs and H2RAs are prescribed in the first 6 months of life. Our study highlights modifiable factors, such as medication stewardship, that may change the childhood risk of CD.

Public health response to an anthrax attack: an evaluation of vaccination policy options.

A discrete-time, deterministic, compartmental model was developed and analyzed to provide insight into how the use of anthrax vaccine before or after a large-scale attack can reduce casualties. The model accounts for important response and protection factors such as antibiotic and vaccine efficacy, the protective effects of buildings, the timing of emergency response, and antibiotic adherence and vaccine coverage in the population prior to the attack. The relative benefit of pre- versus post-exposure vaccination is influenced by the timing of the post-exposure antibiotic distribution campaign as well as assumptions of antibiotic adherence. The results indicate that, regardless of which vaccination policy is adopted, a rapid and effective post-attack medical response has a large impact on the number of lives that can be saved by a post-exposure prophylaxis (PEP) campaign. A sensitivity analysis of the model indicates that uncertainty in medical efficacy and the time to initiate a PEP campaign are the model parameters that have the greatest impact on the number of predicted deaths. It is shown that for each day that a mass prophylaxis campaign is delayed, more casualties and deaths result than for each day that the completion of the campaign is delayed.

Mass prophylaxis dispensing concerns: traffic and public access to PODs.

The ability to quickly dispense postexposure prophylaxis (PEP) using multiple points of dispensing (PODs) following a bioterrorism event could potentially save a large proportion of those who were exposed, while failure in PEP dispensing could have dire public health consequences. A Monte Carlo simulation was developed to explore the traffic flow and parking around PODs under different arrival rates and how these factors might affect the utilization rate of POD workers. The results demonstrate that the public can reasonably access the PODs under ideal conditions assuming a stationary (uniform) arrival rate. For the 5 nonstationary arrival rates tested, however, the available parking spaces quickly become filled, causing long traffic queues and resulting in total processing times that range from 1 hour to over 6 hours. Basic planning considerations should include the use of physical barriers, signage, and traffic control officers to help direct vehicular and pedestrian access to the PODs. Furthermore, the parking and traffic surrounding PODs creates long queues of people waiting to access the PODs. Thus, POD staff are fully used approximately 90% of the time, which can lead to worker fatigue and burn out.