Factors associated with treatment delays in pediatric refractory convulsive status epilepticus.

OBJECTIVE: To identify factors associated with treatment delays in pediatric patients with convulsive refractory status epilepticus (rSE). METHODS: This prospective, observational study was performed from June 2011 to March 2017 on pediatric patients (1 month to 21 years of age) with rSE. We evaluated potential factors associated with increased treatment delays in a Cox proportional hazards model. RESULTS: We studied 219 patients (53% males) with a median (25th-75th percentiles [p25-p75]) age of 3.9 (1.2-9.5) years in whom rSE started out of hospital (141 [64.4%]) or in hospital (78 [35.6%]). The median (p25-p75) time from seizure onset to treatment was 16 (5-45) minutes to first benzodiazepine (BZD), 63 (33-146) minutes to first non-BZD antiepileptic drug (AED), and 170 (107-539) minutes to first continuous infusion. Factors associated with more delays to administration of the first BZD were intermittent rSE (hazard ratio [HR] 1.54, 95% confidence interval [CI] 1.14-2.09; p = 0.0467) and out-of-hospital rSE onset (HR 1.5, 95% CI 1.11-2.04; p = 0.0467). Factors associated with more delays to administration of the first non-BZD AED were intermittent rSE (HR 1.78, 95% CI 1.32-2.4; p = 0.001) and out-of-hospital rSE onset (HR 2.25, 95% CI 1.67-3.02; p < 0.0001). None of the studied factors were associated with a delayed administration of continuous infusion. CONCLUSION: Intermittent rSE and out-of-hospital rSE onset are independently associated with longer delays to administration of the first BZD and the first non-BZD AED in pediatric rSE. These factors identify potential targets for intervention to reduce time to treatment.

Determination of the effect of brand and product identification on consumer palatability ratings of ground beef patties.

The objective of this study was to determine the effect of brand and product identification on consumer palatability ratings of ground beef patties. Six treatments were used in the study: 90/10 Certified Angus Beef (CAB) ground sirloin, 90/10 ground beef, 80/20 CAB ground chuck, 80/20 ground chuck, 80/20 ground beef, and 73/27 CAB ground beef. Ground beef was processed into 151.2-g patties using a patty former with 2 consecutively formed patties assigned to blind consumer testing and the following 2 assigned to informed testing. Following cooking to 74°C, patties were cut into quarters and served to consumers. Consumers ( = 112) evaluated samples in 2 rounds for tenderness, juiciness, flavor liking, texture liking, and overall liking. Each trait was also rated as either acceptable or unacceptable. In the first round of testing, samples were blind evaluated, with no information about the treatments provided to consumers, but in the second round, product type and brand were disclosed prior to sample evaluation. Additionally, texture profile and shear force analyses were performed on patties from each treatment. Few differences were observed for palatability traits during blind consumer testing; however, during informed testing, 90/10 CAB ground sirloin was rated greatest ( < 0.05) for all palatability traits other than juiciness. Also, 90/10 CAB ground sirloin had increased ( < 0.05; (consumer informed score - consumer blind score)/consumer blind score) ratings for tenderness (17.4%), juiciness (36.5%), flavor liking (23.3%), texture liking (18.2%), and overall liking (24.7%) due to brand disclosure. Increased ( < 0.05) ratings were found for CAB products for multiple traits due to treatment disclosure, whereas the only non-CAB-branded product that received increased ( < 0.05) ratings during informed testing was 90/10 ground beef for tenderness and juiciness. Texture results indicated that decreased fat level increased hardness, cohesiveness, gumminess, and chewiness. These results indicate that when sampling ground beef without brand and product information, few consumers find differences in eating quality among ground beef treatments; however, when consumers are aware of the brand, fat level, and subprimal blend prior to sampling, these factors have a large impact on consumer eating satisfaction.

The effect of branding on consumer palatability ratings of beef strip loin steaks.

The objective of this study was to determine the influence of knowing the brand or USDA grade on consumer palatability ratings of beef strip loin steaks. Strip loins were selected to represent 5 USDA grades and brands, USDA Select, Choice, Prime, Certified Angus Beef (CAB; upper 2/3 Choice), and Select, from carcasses of cattle classified as Angus on the basis of phenotype. After 21 d of aging, 2.5-cm-thick steaks were cut, consecutively cut steaks were paired for consumer evaluation. Consumer panelists ( = 112) evaluated samples for tenderness, juiciness, flavor liking, and overall liking. Additionally, consumers rated each palatability trait as either acceptable or unacceptable. Samples were fed in 2 rounds on the same day: blind and informed testing. In the first round, blind testing, consumers were served 1 sample from each treatment, with no product information provided. In the second round, consumers were informed of the brand or quality grade prior to sampling. During blind testing, CAB rated similar ( > 0.05) to Choice for all palatability traits; however, CAB rated greater ( < 0.05) than Choice for all traits during informed testing. Additionally, Angus Select and Select were rated similar > 0.05) for all traits when tested blind, but Angus Select was rated greater ( < 0.05) than Select for flavor and overall liking when brand was declared. When comparing blind and informed ratings, Angus Select and CAB had greater ( < 0.05) ratings for juiciness, flavor liking, and overall liking, and Prime had increased ( < 0.05) ratings for flavor liking and overall liking because of brand disclosure. However, ratings for Choice and Select samples were unaffected ( > 0.05) when brand was disclosed. Brand knowledge increased ( < 0.05) the percentage of Prime samples rated as acceptable for flavor and the percentage of Angus Select samples rated as acceptable for flavor and overall liking. Conversely, there was no difference ( > 0.05) in the percentage of Choice and Select samples rated as acceptable for all palatability traits. These data indicate that Prime, CAB, and Angus Select steaks receive an increase in consumer palatability perception, or "brand lift," which does not occur for Choice and Select beef.

A severe case of dentatorubro-pallidoluysian atrophy (DRPLA) with microcephaly, very early onset of seizures, and cerebral white matter involvement.

We report a severe case of Dentatorubro-pallidoluysian atrophy (DRPLA) presenting with microcephaly, developmental delay, severe epilepsy, and progressive mental deterioration with a very early onset of disease. The case is notable for the early detection of white matter changes by brain MRI. Neuroradiological findings from the case were compared to those of previously reported patients with disease onset before 10 years of age.

Decremental motor responses to repetitive nerve stimulation in ALS.

Repetitive nerve stimulation (RNS) of the trapezius muscle at slow rates was performed on 192 patients with amyotrophic lateral sclerosis (ALS). Fifty-six patients (29%) showed classical neuromuscular decrement of 10-43% (mean 16.8%) while 44 patients (23%) had a borderline decrement of 5-9%. The trapezius was significantly more sensitive in revealing the defect than the distal hypothenar muscles. In 30 patients followed serially, the decremental response remained constant or increased with time. However, 25% of patients continued to show no decrement in spite of progression of disease. No statistical correlation was found between decrement and clinical severity, disease staging, or disease progression. The finding that at least 50% of ALS patients show some degree of decrement on RNS of the trapezius muscle suggests that functional alterations of the neuromuscular junction accompany this disease.

Cerebral aspergillosis: MR imaging and histopathologic correlation.

The authors report a case of intracranial aspergillosis secondary to immunosuppression. Signal intensity changes in the lesion on brain MR are compared with gross and histopathologic findings at autopsy. A peripheral ring of low signal intensity relates to a dense population of Aspergillus hyphal elements and small areas of hemorrhage. CT findings are included for comparison.

Myasthenia gravis in a child with sequelae of opsoclonus-myoclonus syndrome.

Immune-mediated myasthenia gravis occurred in a 13-month-old girl who had sequelae of an opsoclonus-myoclonus syndrome. She had an abnormal Raji cell assay along with elevated antiacetylcholine receptor and antithyroglobulin antibodies. No evidence of an associated malignancy was found with either condition. Clearly, the co-occurrence of two diseases within a single patient does not establish a common pathogenesis. Nevertheless, this is a provocative association and may point to an immunologically mediated process in the opsoclonus-myoclonus syndrome.

Opsoclonus-myoclonus with Beckwith-Wiedemann syndrome and hepatoblastoma.

The association between Beckwith-Wiedemann syndrome and hepatoblastoma is well established and relatively commonplace. The occurrence of opsoclonus-myoclonus syndrome in individuals with occult neoplasia is also well documented. However, the development of opsoclonus-myoclonus syndrome in an infant with Beckwith-Wiedemann syndrome and hepatoblastoma has not been reported previously. The list of underlying causes of opsoclonus-myoclonus syndrome should be expanded to include hepatoblastoma, particularly in any child with features suggestive of Beckwith-Wiedemann syndrome.

Pressor effects of systemic administration of methionine and leucine enkephalin in the conscious rat.

Experiments were designed using conscious Sprague-Dawley rats to determine the blood pressure (BP) and heart rate (HR) responses to intravenous doses of (1) the adrenal catecholamines noradrenaline (NA) and adrenaline (A), (2) adrenal pentapeptides methionine enkephalin (ME) and leucine enkephalin (LE), (3) combination (i.v.) injections of both ME or LE with NA or A that modulate the hemodynamic responses when the adrenal catecholamines were given alone, and (4) the possible receptor mechanisms mediating the resultant BP and HR response to i.v. pentapeptide administration. NA (0.48 and 2.4 nmol) and A (0.3 and 1.5 nmol) given i.v. evoked potent, dose-related pressor responses associated with reflex bradycardia. ME and LE (1.6 - 48 nmol) elicited transient (10-20 s) increases in mean arterial pressure (MAP), which was associated either with no change in mean heart rate (MHR), such as ME, or with slight bradycardia (i.e., LE). Combining ME or LE (16 nmol) with NA (2.4 nmol) or A (0.3 or 1.5 nmol) did not change MAP and MHR from when these respective doses of NA or A were given alone. However, 16 nmol of ME or LE with a low dose of NA (0.48 nmol) increased the pressor response compared with NA (0.48 nmol) given alone. Other experiments whereby specific receptor blockers (naloxone, diprenorphine, atropine, propranolol, phentolamine or guanethidine) were given i.v. 5 min before subsequent i.v. administration of LE or ME (16 nmol) indicated that only phentolamine or guanethidine could completely suppress the pressor responses of LE and ME. Naloxone and diprenorphine pretreatment attenuated the pressor response of LE but did not affect the BP response to ME.(ABSTRACT TRUNCATED AT 250 WORDS)

Lateral cerebral ventricle and preoptic-anterior hypothalamic area infusion and perfusion of beta-endorphin and ACTH to unrestrained rats: core and surface temperature responses.

Both beta-endorphin and ACTH have been found in high concentrations within the hypothalami of mammals and each neuropeptide has been proposed to play a physiological role in regulating body temperature. In an attempt to determine how these peptides may alter thermoregulation, small, microgram concentrations of beta-endorphin and ACTH were injected either into lateral cerebral ventricle (ICV) or directly into the preoptic-anterior hypothalamic area (POAH) or perfused into the POAH of unrestrained rats. Core (rectal) and surface (tail) temperatures were recorded before and after ICV and POAH injection of 1 microgram of beta-endorphin or ACTH or perfusion (10 ng/microL) of either neuropeptide. POAH perfusion of naloxone HCl following the neuropeptide perfusion was tested to determine the specificity of the temperature responses. Regardless of the route of central administration, beta-endorphin, in the concentrations used, consistently evoked a hyperthermic core temperature response, that could be antagonized by naloxone. Increased core temperatures may, in part, have been due to peripheral vasoconstriction, as suggested by the decreases seen in tail temperature. The same concentrations of ACTH failed to show any prominent core temperature changes. Results suggest that beta-endorphin is a more potent modulator than ACTH in altering core temperatures of unrestrained rats. Whether beta-endorphin and ACTH act physiologically in an antagonistic manner to maintain a constant body temperature remains to be proven.