ED Management of Status Epilepticus in Pediatric Patient with Dravet Syndrome

Case Presentation: Pediatric Patient With Rare Early-Onset Epilepsy Syndrome Presenting in Status Epilepticus ED Management of refractory status epilepticus in Pediatric Patient with Dravet Syndrome. 21 month old female with past medical history of Dravet Syndrome presenting to the ED via EMS for status epilepticus. Patients mother reported 20 minutes of seizure like activity at home described as focal right upper extremity twitching and fixed right gaze. She was given Diastat x 2 without improvement. Her mother reported compliance with prescribed Keppra at home. Denied recent illness, fevers, travel history, or head trauma. On arrival to the ED patient was demonstrating continued seizure like activity. Initial vital signs significant for tachycardia with bradypnea and was initially on supplemental oxygen via blow-by nasal cannula. POC glucose remained within normal limits. Labs obtained revealed no electrolyte derangements, signs of infection. Initial treatment included Ativan 1mg x2 without response. Patient required intubation with Rocuronium due to developing hypoxia. Due to continued subclinical seizure activity despite use of Keppra 20cc/kg, 1mg Versed x 3, a continuous Versed drip was then initiated. Patient had termination of seizures at that time. She was transferred to a tertiary center for escalation of care. Dravet Syndrome (DS), previously termed myoclonic epilepsy of infancy, is a rare syndrome of pediatric epilepsy that is notoriously difficulty to manage. It is typically caused by mutations in the alpha-1 subunit of the voltage-gated sodium channel gene (SCN1A) on chromosome 2q24. Majority of these mutations occur de novo, with approximately 10% attributed to familial mutations (1). Disease course typically occurs within the first year of life, without precipitating event for majority of patients. The characteristics of seizures associated with Dravet Syndrome are unique in that they have a multitude of seizure types, meaning a patient can have both focal and generalized seizure patterns. Other diagnostic criteria include refractory seizures that are unresponsive to antiepileptic treatments. As with most forms of epilepsy, patients with DS are esquisitely sensitive to external stimuli lowering seizure threshold including fever, vaccines, hot showers/baths, emotional stress, probing lights, and provoking visual stimuli. Treatment of DS typically requires dual anti-seizure therapy. Of note, anti-epileptics that work on sodium channel blockade should be avoided with patients who have DS as they exacerbate symptoms, leading to restricted availability of antiseizure treatments. Although strong evidence of successful management of DS with antiepileptics has been poorly studied, First line treatment is Valproate in addition to Clobazam (ONFI). Second line therapy for patients who have poor seizure control with above include Topiramate, Stiripentol, and Levtiracetam (1). Nonpharmaceutical adjuncts include ketogenic diet and potential surgical therapies including vagus nerve stimulation and deep brain stimulation. In July 2018 the FDA approved the use of Cannabidiol for adjunctive therapy in addition to standard pharmaceutical antiepileptic in children with DS. In the ED setting it is critical to manage different forms of epilepsy, and management of status epilepticus. Although studies are limited on gold standard treatment of status in DS patients, more effort should be attributed to protocols regarding difficult to treat epilepsy syndromes in the ED.https://scholarlycommons.henryford.com/merf2019caserpt/1088/thumbnail.jp

New Opportunities for Economic Benefits for the American Southeast in the International Pearl Industry

Economic participation of the United States in the global pearl industry has been minimal in terms of economic benefits received, although critical in terms of support to the industry. The United States is the primary provider of freshwater mussel shell nucleus and a major consumer of pearls, but economic benefits accrue mainly to foreign companies whereas environmental and economic externalities affect the economy and environment of the southeastern states. The economic role of the U.S. mussel shell industry and aquaculture sector can be enhanced if the mussel shell fishery is stabilized and if options to extract higher returns for the stakeholders are explored. Establishment of a freshwater pearl culture industry can aid in strengthening the U.S. role in the global industry through a variety of means: 1) by providing an economic incentive to conserve freshwater mussel stocks; 2) by establishing a local market for shell nucleus of the smaller size categories; 3) by supplying U.S. demand for pearls; and 4) by providing experimental animals for research and development to create marketable technologies for export to the pearl industry

Immune tolerance induction in 31 children with haemophilia A: is ITI less successful in African Americans?

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/92102/1/j.1365-2516.2010.02429.x.pd

Policing Kentucky\u27s School Children: Issues and Trends

The purpose of this research bulletin is to document the scope and nature of an important dimension of the school safety movement--the degree to which schools in Kentucky are being policed by public police agencies. A shift toward having an active police presence in our public schools, an unprecedented and significiant development, should be examined carefully

Evidence for coal forest refugia in the seasonally dry Pennsylvanian tropical lowlands of the Illinois Basin, USA

The Moscovian plant macroflora at Cottage Grove southeastern Illinois, USA, is a key example of Pennsylvanian (323–299 Million years ago) dryland vegetation. There is currently no palynological data from the same stratigraphic horizons as the plant macrofossils, leaves and other vegetative and reproductive structures, at this locality. Consequently, reconstructions of the standing vegetation at Cottage Grove from these sediments lack the complementary information and a more regional perspective that can be provided by sporomorphs (prepollen, pollen, megaspores and spores). In order to provide this, we have analysed the composition of fossil sporomorph assemblages in two rock samples taken from macrofossil-bearing inter-coal shale at Cottage Grove. Our palynological data differ considerably in composition and in the dominance-diversity profile from the macrofossil vegetation at this locality. Walchian conifers and pteridosperms are common elements in the macroflora, but are absent in the sporomorph assemblages. Reversely, the sporomorph assemblages at Cottage Grove comprise 17 spore taxa (∼16% and ∼63% of the total assemblages) that are known from the lycopsid orders Isoetales, Lepidodendrales and Selaginallales, while Cottage Grove’s macrofloral record fails to capture evidence of a considerable population of coal forest lycopsids. We interpret our results as evidence that the Pennsylvanian dryland glacial landscape at Cottage Grove included fragmented populations of wetland plants living in refugia

Climate and vegetational regime shifts in the late Paleozoic ice age earth

The late Paleozoic earth experienced alternation between glacial and non-glacial climates at multiple temporal scales, accompanied by atmospheric CO 2 fluctuations and global warming intervals, often attended by significant vegetational changes in equatorial latitudes of Pangaea. We assess the nature of climate–vegetation interaction during two time intervals: middle–late Pennsylvanian transition and Pennsylvanian–Permian transition, each marked by tropical warming and drying. In case study 1, there is a catastrophic intra-biomic reorganization of dominance and diversity in wetland, evergreen vegetation growing under humid climates. This represents a threshold-type change, possibly a regime shift to an alternative stable state. Case study 2 is an inter-biome dominance change in western and central Pangaea from humid wetland and seasonally dry to semi-arid vegetation. Shifts between these vegetation types had been occurring in Euramerican portions of the equatorial region throughout the late middle and late Pennsylvanian, the drier vegetation reaching persistent dominance by Early Permian. The oscillatory transition between humid and seasonally dry vegetation appears to demonstrate a threshold-like behavior but probably not repeated transitions between alternative stable states. Rather, changes in dominance in lowland equatorial regions were driven by long-term, repetitive climatic oscillations, occurring with increasing intensity, within overall shift to seasonal dryness through time. In neither case study are there clear biotic or abiotic warning signs of looming changes in vegetational composition or geographic distribution, nor is it clear that there are specific, absolute values or rates of environmental change in temperature, rainfall distribution and amount, or atmospheric composition, approach to which might indicate proximity to a terrestrial biotic-change threshold.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/73033/1/j.1472-4669.2009.00192.x.pd

Response Of Late Carboniferous And Early Permian Plant Communities To Climate Change

Late Carboniferous and Early Permian strata record the transition from a cold interval in Earth history, characterized by the repeated periods of glaciation and deglaciation of the southern pole, to a warm-climate interval. Consequently, this time period is the best available analogue to the Recent in which to study patterns of vegetational response, both to glacial-interglacial oscillation and to the appearance of warm climate. Carboniferous wetland ecosystems were dominated by spore-producing plants and early gymnospermous seed plants. Global climate changes, largely drying,forced vegetational changes, resulting in a change to a seed plant–dominated world, beginning first at high latitudes during the Carboniferous, reaching the tropics near the Permo-Carboniferous boundary. For most of this time plant assemblages were very conservative in their composition. Change in the dominant vegetation was generally a rapid process, which suggests that environmental thresholds were crossed, and involved little mixing of elements from the wet and dry floras