Increased total daily energy expenditure in mild to moderate ALS: greater contribution from physical activity energy expenditure than hyper-metabolism.

: It is unknown whether the relative contribution to energy imbalance in amyotrophic lateral sclerosis (ALS) is due to decreased energy intake, or increased energy expenditure from hyper-metabolism and/or physical activity, or both. : We studied 10 free-living sporadic ALS subjects with mild to moderate disease and 10 matched healthy controls to address this question. We estimated energy intake by 24-h recall in ALS subjects and three-day food diary in all.

Body composition in amyotrophic lateral sclerosis subjects and its effect on disease progression and survival.

Background: Motor neuron degeneration and malnutrition alter body composition in amyotrophic lateral sclerosis (ALS). Resulting losses of weight, fat mass (FM), and fat-free mass (FFM) shorten survival. Nutritional management relies on body weight or BMI; neither reliably indicates malnutrition nor differentiates body compartments.

Effect of riluzole on weight in short-term and long-term survivors of amyotrophic lateral sclerosis.

Riluzole is the first disease-modifying therapy for amyotrophic lateral sclerosis (ALS) approved in 1995 by the Food and Drug Administration in the USA, and is now available worldwide. It delays time to tracheostomy or death and prolongs survival. The precise mechanism of the survival prolonging effect is unknown.

Thymectomy may not be associated with clinical improvement in MuSK myasthenia gravis.

Introduction: A randomized trial demonstrated benefit from thymectomy in nonthymomatous acetylcholine receptor (AChR)-antibody positive myasthenia gravis (MG). Uncontrolled observational and histologic studies suggest thymectomy may not be efficacious in anti-muscle-specific kinase (MuSK)-MG.

Methods: The therapeutic impact of thymectomy was evaluated from data collected for a multicenter, retrospective blinded review of rituximab in MuSK-MG.

Hydration measured by doubly labeled water in ALS and its effects on survival.

We present a study of hydration in ALS patients and its effects on survival. This was a multicenter study over 48 weeks in 80 ALS patients who underwent 250 individual measurements using doubly labeled water (DLW). Total body water (TBW) and water turnover (a surrogate for water intake) were 3.

Rituximab as treatment for anti-MuSK myasthenia gravis: Multicenter blinded prospective review.

Objective: To evaluate the efficacy of rituximab in treatment of anti-muscle-specific kinase (MuSK) myasthenia gravis (MG).

Methods: This was a multicenter, blinded, prospective review, comparing anti-MuSK-positive patients with MG treated with rituximab to those not treated with rituximab. The primary clinical endpoint was the Myasthenia Gravis Status and Treatment Intensity (MGSTI), a novel outcome that combines the Myasthenia Gravis Foundation of America (MGFA) postintervention status (PIS) and the number and dosages of other immunosuppressant therapies used.

Rituximab treatment of myasthenia gravis: A systematic review.

Rituximab is a chimeric mouse/human anti-CD20 monoclonal immunoglobulin. We reviewed the efficacy and safety of rituximab in 169 myasthenia gravis (MG) patients from case reports and series. Antibodies to the acetylcholine receptor (AChR) were present in 59% and muscle-specific tyrosine kinase (MuSK) in 34%.

The contemporary spectrum of multiple sclerosis misdiagnosis: A multicenter study.

Objective: To characterize patients misdiagnosed with multiple sclerosis (MS).

Methods: Neurologists at 4 academic MS centers submitted data on patients determined to have been misdiagnosed with MS.

Results: Of 110 misdiagnosed patients, 51 (46%) were classified as "definite" and 59 (54%) "probable" misdiagnoses according to study definitions.

"Central vessel sign" on 3T FLAIR* MRI for the differentiation of multiple sclerosis from migraine.

Objective: The diagnosis of multiple sclerosis (MS) presently relies on radiographic assessments of imperfect specificity. Recent data using T2* methodology for the detection of the "central vessel sign" (CVS) in MS lesions suggests this novel MRI technique may distinguish MS from other disorders. Our aim was to determine if evaluation for CVS on 3T FLAIR* MRI differentiates MS from migraine.

Mining and Visualizing Family History Associations in the Electronic Health Record: A Case Study for Pediatric Asthma.

Asthma is the most common chronic childhood disease and has seen increasing prevalence worldwide. While there is existing evidence of familial and other risk factors for pediatric asthma, there is a need for further studies to explore and understand interactions among these risk factors. The goal of this study was to develop an approach for mining, visualizing, and evaluating association rules representing pairwise interactions among potential familial risk factors based on information documented as part of a patient's family history in the electronic health record.

Cognitive outcome after spinal anesthesia and surgery during infancy.

Background: Observational studies on pediatric anesthesia neurotoxicity have been unable to distinguish long-term effects of general anesthesia (GA) from factors associated with the need for surgery. A recent study on elementary school children who had received a single GA during the first year of life demonstrated an association in otherwise healthy children between the duration of anesthesia and diminished test scores and also revealed a subgroup of children with "very poor academic achievement" (VPAA), scoring below the fifth percentile on standardized testing. Analysis of postoperative cognitive function in a similar cohort of children anesthetized with an alternative to GA may help to begin to separate the effects of anesthesia from other confounders.

Utility of spine bone mineral density in fracture prediction within FRAX.

Predicting individuals at risk for fracturing and modifying that risk are important in preventative health. Our aim was to quantify the impact of spine bone mineral density (BMD) on fracture risk prediction and determine the positive predictive value of fracture prediction using the lowest BMD value at the femoral neck, total hip, or lumbar spine. A retrospective cross-sectional analysis of 15,033 women was performed, assessing the contribution of age, body mass index, number of clinical risk factors, T-score, and osteoporosis category to the presence of fracture.

Neurodevelopmental outcome of infants resuscitated with air or 100% oxygen: a systematic review and meta-analysis.

Background: The use of air for the initial resuscitation of newborn infants has been shown to reduce neonatal mortality. However, a precise estimate of the neurodevelopmental status upon follow-up of infants resuscitated in air is lacking.

Objective: To perform a meta-analysis of all studies reporting resuscitation of newborn infants with air or 100% oxygen that included follow-up data.

Development of a substance abuse program for opioid-dependent nonurban pregnant women improves outcome.

Background: The goal of this study was to determine whether improved access to medication assisted therapy in the general population, with improved coordination of ancillary services for pregnant women, improved perinatal outcomes in a nonurban area.

Methods: The cohort of women treated for opioid dependence during pregnancy with medication-assisted therapy and delivered at a single institution between 2000 and 2006 were retrospectively identified (n = 149 women; n = 151 neonates). Access to opioid agonist therapy for the general population was determined as the combined number of available treatment positions for medication-assisted therapy.

Randomized trial comparing 3 approaches to the initial respiratory management of preterm neonates.

Objective: We designed a multicenter randomized trial to compare 3 approaches to the initial respiratory management of preterm neonates: prophylactic surfactant followed by a period of mechanical ventilation (prophylactic surfactant [PS]); prophylactic surfactant with rapid extubation to bubble nasal continuous positive airway pressure (intubate-surfactant-extubate [ISX]) or initial management with bubble continuous positive airway pressure and selective surfactant treatment (nCPAP).

Design/methods: Neonates born at 26 0/7 to 29 6/7 weeks' gestation were enrolled at participating Vermont Oxford Network centers and randomly assigned to PS, ISX, or nCPAP groups before delivery. Primary outcome was the incidence of death or bronchopulmonary dysplasia (BPD) at 36 weeks' postmenstrual age.

Intrapartum and postpartum analgesia for women maintained on buprenorphine during pregnancy.

Objective: To determine whether buprenorphine maintenance alters intrapartum or postpartum pain or medication requirements.

Methods: Sixty three patients treated with buprenorphine for opioid dependence during pregnancy (vaginal n = 44; cesarean n = 19) were matched retrospectively to control women. Analgesic medication and pain scores (0-10) were extracted from the medical record.

Neurodevelopmental outcome of extremely low birth weight infants from the Vermont Oxford network: 1998-2003.

Background: Physicians and parents face significant uncertainties when making care decisions for extremely low birth weight (ELBW) infants. Many published estimates of death and developmental outcome are from well-funded university programs and may not reflect outcomes of infants from a variety of settings. The best estimates of the probabilities of death and severe disability combine local experience and published data.

Intrapartum and postpartum analgesia for women maintained on methadone during pregnancy.

Objective: To determine whether methadone maintenance alters intrapartum or postpartum pain or medication requirements.

Methods: Sixty-eight patients treated with methadone for opiate dependence during pregnancy (vaginal n=35; cesarean n=33) were matched retrospectively to control women. Analgesic medication and pain scores (0-10) were extracted from the medical record.

Ensuring accurate knowledge of prematurity outcomes for prenatal counseling.

Objectives: To determine the accuracy of knowledge of different health care providers regarding survival and long-term morbidity rates for very premature infants and to examine whether a focused educational intervention improves the accuracy of this knowledge and influences health care decisions.

Methods: Using hypothetical case scenarios with infants at < or =28 weeks of gestation, we surveyed a variety of caregivers involved in perinatal communication and decision-making processes at a tertiary center that provides intensive care for neonates. We asked physicians from the pediatrics and obstetrics services and nurses and nurse practitioners from the NICU and obstetrics ward for their best estimates of survival and major long-term disability rates and for their opinions regarding the appropriateness of resuscitation and life support at each week of gestation of <29 weeks.

Outpatient misoprostol compared with dinoprostone gel for preinduction cervical ripening: a randomized controlled trial.

Objective: To determine whether a single outpatient dose of intravaginal misoprostol (versus intracervical dinoprostone gel) reduces the oxytocin use for induction. Despite the numerous trials examining misoprostol for induction, the efficacy of a single outpatient dose of misoprostol followed by oxytocin induction is unknown.

Methods: Patients with a term, vertex, singleton pregnancy and a Bishop score of 6 or less were randomly assigned to receive misoprostol (n = 42, 0.

An Update Survey of Bulk Tank Milk Quality in Vermont.

Quality of Vermont bulk tank milk was first surveyed in 1985 as part of a statewide milk quality enhancement program. In a second survey conducted in 1990, bulk tank milk from 1,971 farms was sampled and tested for standard plate count, bacterial type and species distribution, and somatic cell count. Test results from 1,203 duplicate bulk tank milk samples were compared between five Vermont milk processors and the University of Vermont Quality Milk Research Laboratory.

Comparison of Infectious Dose of Listeria monocytogenes F5817 as Determined for Normal Versus Compromised C57B1/6J Mice.

The infectious dose of Listeria monocytogenes F5817, a serotype 4b human patient isolate, was determined following oral challenge in normal and compromised C57B1/6J mice. In an attempt to mimic human populations previously shown to be at risk to ingestion of L. monocytogenes , groups of mice used in this study consisted of the following: mice pretreated with hydrocortisone acetate or cimetidine; pregnant mice (12-14 d gestation); or beige mutants of C57B1/6J mice (deficient in lysosome production within monocytes and granulocytes).