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Congenital Muscular Dystrophy Workup
- Author: Glenn Lopate, MD; Chief Editor: Amy Kao, MD more...
Laboratory Studies
Persons with Ullrich congenital muscular dystrophy, rigid spine with muscular dystrophy (deficiency of selenoprotein N), and integrin-α7 deficiency have creatine kinase (CK) levels that are normal to mildly elevated (≤5 times normal).
CK levels are usually more than 1000 in patients with congenital muscular dystrophy with familial junctional epidermolysis bullosa.
CK levels are mildly to markedly elevated (2-150 times normal) in most patients with congenital muscular dystrophy due to abnormal glycosylation or with laminin-α2 mutations.
Imaging Studies
Persons with congenital muscular dystrophies due to mutations in genes for selenoprotein N and in genes for the extracellular matrix proteins integrin-α7 and collagen type VI have normal brain MRI findings.
Patients CMD with familial junctional epidermolysis bullosa often have brain atrophy and enlarged ventricles on MRI.
In those with congenital muscular dystrophies due to mutations in laminin-α2 or with any other congenital muscular dystrophy due to abnormal O-glycosylation, brain MRI findings are abnormal.
The mildest changes are seen in deficiency of laminin-α2, with periventricular white matter changes being the most common abnormality (increased T2 signal).
In the congenital muscular dystrophies due to abnormalities in O-glycosylation, the abnormalities vary, even in patients with mutations in the same gene. Brain MRIs can be normal, or they can show severe changes, such as agyria and severe pontocerebellar hypoplasia.
All patients with muscle-eye-brain disease and Fukuyama congenital muscular dystrophy have abnormal MRIs, which show a range from mild changes of only cerebellar hypoplasia or cysts to severe disease, as described above.
The most severe changes are seen in Walker-Warburg syndrome, with most patients having severe agyria, pontocerebellar hypoplasia, and, in many patients, encephalocele or myelomeningocele.
Muscle MRI can help differentiate muscular dystrophies with rigidity of the spine[50]
SEPN1 – Selective involvement of the sartorius, gastrocnemius spared
COL6A – Bethlem myopathy (BM) patients had concentric atrophy and peripheral involvement, most obvious in vasti and gastrocnemius
COL6A - Ullrich CMD patients had diffuse involvement of thigh muscles with selective sparing of anteromedial thigh muscles; more diffuse than BM, but similar peripheral involvement of gastrocnemius
LMNA – Involvement of vasti at thigh level, medial > lateral gastrocnemius, soleus involved
LGMD2A (CAPN3) – Selective involvement of adductor magnus and posterior thigh muscles, medial > lateral gastrocnemius, soleus involved
Other Tests
Electromyography (EMG) and nerve conduction study (NCS)
EMG and NCS should be performed in all patients with suspected congenital muscular dystrophy to confirm myopathy and to exclude other diseases.
NCS results are normal except in some cases with mutations in laminin-α2, in which mild neuropathic changes may be seen (some with demyelinating features).
EMG usually shows typical small-amplitude, narrow-duration motor-unit potentials with early recruitment.
Prenatal diagnosis
Prenatal diagnosis had been performed most commonly in families with mutations in laminin-α2, in part, because this is the most common congenital muscular dystrophy.
Laminin-α2 is expressed in 9-week trophoblasts, allowing immunohistochemical detection of protein in chorionic villus. However, in families with partial laminin-α2 deficiency, protein detection may not be reliable. Linkage analysis can also be performed but is also at times unreliable, especially in families with partial laminin-α2 deficiency or no brain MRI abnormalities. However, the combination of these 2 techniques along with rigorous controls has been highly accurate and reliable in the prenatal diagnosis of laminin-α2 mutations. The most reliable technique is direct mutation analysis, although this is more time consuming because the entire gene sequence must be analyzed.
Genetic testing
Genetic testing is available for all congenital muscular dystrophies (see http://www.ncbi.nlm.nih.gov/sites/GeneTests/?db=GeneTests)
Muscle biopsy is indicated in all cases of suspected congenital muscular dystrophy to help confirm the diagnosis and exclude other causes of weakness.
Congenital muscular dystrophy with laminin-α2 deficiency
- Complete laminin-α2 deficiency
- Patients may have severe dystrophic pathology with muscle-fiber degeneration and regeneration, fiber necrosis, and endomysial and perimysial fibrosis.
- Mononuclear cell infiltrates may be present in biopsy samples obtained from infants.
- Immunohistochemical studies show complete loss of staining for laminin-α2.
- Antibodies must be used against both the 300- and 80-kd subunits.
- α-dystroglycan staining is also absent.
- Approximately 95% of biopsy samples with absent laminin-α2 staining have a mutation in the LAMA2 gene.
- Partial laminin-α2 deficiency
- Mild myopathic features often occur with little or no necrosis.
- Partial staining for laminin-α2 may be seen in patients with laminin-α2-deficient congenital muscular dystrophy and in those with any congenital muscular dystrophy associated with a glycosyltransferase enzyme deficiency.
- Complete laminin-α2 deficiency
Ullrich congenital muscular dystrophy
Variation ranges from mildly myopathic to dystrophic in terms of muscle fiber size, muscle fiber necrosis, and fibrosis.
Collagen type VI staining around surface of muscle fiber is usually reduced or absent, but staining may occur in connective tissue.
In Bethlem myopathy, routine muscle biopsy and collagen type VI immunohistochemistry usually are normal.
Integrin-α7 deficiency
Mild variations in muscle-fiber size are noted.
Staining for integrin-α7 is decreased. This may also be seen in congenital muscular dystrophy with laminin-α2 deficiency.
Congenital muscular dystrophy with familial junctional epdermolysis bullosa
Variation in muscle fiber size, internal nuclei, increased connective tissue, muscle fiber necrosis and regeneration
Plectin immunostaining is reduced in muscle Z-lines and skin
Rigid spine with muscular dystrophy (deficiency of selenoprotein N)
Myopathic features include small, round muscle fibers, endomysial fibrosis and type 1 fiber predominance or atrophy.
Regenerating and degenerating muscle fibers and fiber necrosis are rare. Severe cases may have significant fibrosis but still little or no necrosis.
Minicores may be present.
Glycotransferases (abnormal O-glycosylation of α-dystroglycan)
All of the α-dystroglycanopathies have similar muscle pathologies that differ in the degree of severity, which is likely correlated with the degree of preserved α-dystroglycan function.
Patients have muscle fiber degeneration and/or necrosis and regeneration, variability in muscle fiber size, and endomysial and/or perimysial fibrosis
Muscle tissue may look fairly normal in persons with muscle-eye-brain disease and with mutations in FKRP shortly after birth.
Immunohistochemical studies show decreased staining for α-dystroglycan, which is localized correctly to the muscle cell surface. Western blot studies show a decreased molecular weight of α-dystroglycan in affected patients. A secondary decrease in staining for laminin-α2 may be noted in some biopsy samples.
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Gary Oldman Net Worth
Gary Oldman
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To celebrate the release of the Rack Extension platform, Pulsar will be gifted to everyone who is using Rack Extension compatible versions of Reason and Reason Essentials for the first three months after release—a $49/€39 value.
"We want all Reason users to get the experience of using Rack Extensions," says Leo Nathorst-Böös, Product Marketing Manager. "Pulsar is an amazing device that opens up a lot of new possibilities for creative sound-sculpting, and with the included ReFill, it's something that all Reason users will enjoy—regardless of whether you're a tweak-head or not. It feels great to be able to provide Pulsar for free to our loyal users."
Thanks to the Reason rack's flexible routing, Pulsar integrates perfectly with all Reason devices, opening up new possibilities for creative modulation within the program. With Pulsar it's easy to create a shuffled tremolo that changes speed as different notes are triggered, or come up with ever-changing wobbles that respond to playing.
Using its audio outputs, Pulsar transforms from a low frequency oscillator to a high-powered synth with plenty of character. The waveforms designed for modulation, combined with the unique lag and shuffle controls, bring tons of unique tones.
For users that are not versed with the modular aspects of Reason, Pulsar also comes with a set of premade patches, containing everything from lush evolving pads to the grittiest of basses.
For more information about Rack Extensions, Pulsar LGM-1 and to listen to audio examples, visit www.propellerheads.se/go/74B1DC9D
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MINEOLA, N.Y. (CBSNewYork/AP) — Nassau County is forgiving $4.2 million in speed camera fines issued in the last month.
County Executive Edward Mangano said there were problems with cameras installed at six school locations because of malfunctions in the equipment and the timing.
Mangano said he is declaring amnesty on the fines. He said enforcement resumes when school opens next month.
"Clearly, there were some errors in the speed zone violations, so we're meeting with the vendor and our traffic safety department to make certain that those errors are corrected," Mangano told 1010 WINS. "So as a matter of fairness, we're eliminating all those tickets."
Gov. Andrew Cuomo signed legislation this year allowing 200 new speed cameras at school zones in New York City and Long Island. The cameras are supposed to operate during and immediately before and after school.
"Many of the residents believed there was not enough notice, so this will serve as a notice of the new state law that there will be speed cameras in school zones come this September with the opening of school," Mangano said.
Mangano said anyone who paid their ticket will be issued a refund from the Traffic and Parking Violations Bureau.
"Those who haven't paid their tickets can have fun ripping them up," Mangano told WCBS 880's Ginny Kosola.
The state allows speed cameras to ticket drivers going more than 10 mph over the school speed limit on school days during school hours, plus an hour before and after school and during school activities.
In total, Nassau issued 30,108 citations at the five schools, totaling more than $2.4 million in fines.
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(TM and © Copyright 2014 CBS Radio Inc. and its relevant subsidiaries. CBS RADIO and EYE Logo TM and Copyright 2014 CBS Broadcasting Inc. Used under license. All Rights Reserved. This material may not be published, broadcast, rewritten, or redistributed. The Associated Press contributed to this report.)


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First he got a kicking on the field, now he's getting one off it.
British media and commentators on social media are putting the boot into the ball-boy who looked to be in such pain after being kicked by Chelsea football star Eden Hazard during a match in Wales yesterday.
It turns out ball-boy 17-year-old Charlie Morgan boasted on Twitter before the game, between Swansea City and Chelsea, about his time-wasting skills.
"The king of all ball-boys is back making his final appearance #needed #for #timewasting," he said in his Twitter post.
After that tweet , he added: "Haven't been doing it this season been ask to come back to run them because the person can't come in cos of the snow."
In the incident yesterday, Hazard rushed to get the ball after it went out of play late in the game.
A dawdling Morgan gets in his way and Hazard pushes him. Morgan falls on top of the ball and appears to be in no hurry to get off it.
Hazard tries to grab the ball and then resorts to kicking it out from under Morgan, who then rolls around grimacing and holding his ribs.
The referee sent Hazard off.
Police are investigating, and have said Morgan was not injured.
Morgan is the son of hotel and property tycoon Martin Morgan, who is said to be the 32nd-richest person in Wales, and is on the Swansea City board of directors. He started his career as a welder but became a company director by the age of 29, and now sits on the board of 11 hotel and property companies.
Ben Watkins, 18, a former school friend of Charlie Morgan's, said: "He must be the richest ball-boy in football," the Daily Mail reported.
"He's mad about Swansea City - what he did was a bit foolish especially after be boasted he was going to time-waste. I guess he's been a bit spoiled because his dad's got pots of money."
In posts on Twitter, Charlie has bragged about drinking Veuve Clicquot champagne and going on holiday to Las Vegas and Dubai.
Hazard told Chelsea TV after the game: "The boy put his whole body on to the ball and I was just trying to kick the ball and I think I kicked the ball and not the boy. I apologise.
"The ball-boy came in the changing room and we had a quick chat and I apologised and the boy apologised as well, and it is over. Sorry."
Hundreds of tweets were posted after the incident, many posting mocked-up pictures of a hospital patient covered in bandages, claiming they were photographs of Morgan recovering from being kicked.
Others suggested that he should be signed up by a football team thanks to his apparent goalkeeping skills. Singer Lily Allen joined in the fun, joking: "Pray for the little Welsh ball-boy."
Swansea City manager Michael Laudrup denied the club had told the ball-boys to hold on to the ball when it went out of play.
But former Chelsea and England manager Glenn Hoddle sympathised with Hazard and claimed managers often instructed ball-boys to waste time.
He told Sky Sports: "As a manager, if you've got ball-boys, you will tell people instructing the ball-boys that if you're winning the game, don't give the ball back quickly."
Some Twitter users called Hazard a "disgrace", "scumbag" and "animal".
Former Liverpool footballer Robbie Fowler added: "Right decision even though ball-boy milked it."
Former Derby County footballer Robbie Savage, now a BBC pundit, said: "Still can't believe he got sent off for that, incredible!! If the ball-boy does his job properly that doesn't happen."
Chelsea's official Twitter feed tried to make light of the incident, saying: "Has football gone mad? Hazard is sent off for kicking the ball under a ball-boy attempting to smother the ball rather than return it. #CFC."
Some outraged Chelsea fans sent Morgan a string of abusive tweets blaming him for the whole incident.
@OfficialDJWalsh said: "Mate, all I wanna say is ... YOU are a disgrace and a bad sportsman for trying to time-waste. I hope he broke your ribs."
Fan David Comlay added: "Five minutes of fame, but you looked an idiot. You should be ashamed."
Morgan went from 600 followers on Twitter to more than 80,000 in the hours after the nationally televised incident.
QPR midfielder Joey Barton, on loan at Marseille, joined the debate, tweeting : "Hazard only crime is he hasn't kicked him hard enough."
Barton later updated on Twitter to clarify his comments: "Just re-read that last tweet and it sounds like I'm advocating kicking ball-boys! Haha I was mearly [sic] trying to point out Hazard, may as well ... of kicked him properly for the way he is going to be punished for this. Public perception is he's volleyed him. In reality he's tried ... all be it in an aggressive manner to dislodge the ball from beneath him. I am strictly against the kicking of balls people#kickitout."
Former Chelsea winger Pat Nevin suggested Morgan had what was coming to him, labelling his behaviour "disgraceful".
"I would have kicked the ball out from underneath the ball-boy if he had been lying like that, 100 per cent," he told BBC Radio Five.
"I was very, very disappointed by the way the ball-boy acted, and I say 'acted'," Nevin said.
"He must have been watching footballers, the way he rolled around and pretended to be more injured."
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Discover a selection of top forums school) city on the internet. Come to share your passion on forums school) city and meet other fans of school) city. Then, why don't you create your own forum of school) city ?
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BABL'S Social Web Sites
Join Our EMail List
Visitor Counter
Signup for Alerts
Get important messages from Brick American Baseball League by Text & Email
Congratulations to the 2016 BABL Division Winners
A Ball - Indians
50/70 Minor - Yankees
50/70 Major - Mariners
Senior League - White Sox
CONGRATULATIONS to Max Cala for winning the Home Run Derby with 14 Homers. Thanks to all the participants and the coaches who helped to put the event together.
Brick Little League and Brick American Baseball League would like to send out a huge THANK YOU to everyone who contributed to the Danicka Fike Benefit event where $14,958.75 was raised for the family. With such an expansive project and the group effort it required, we can all appreciate how the sum of the parts that got us to this end.
Great job Brick!
Congratulations to the BABL Scholarship winners from Brick Memorial High School: Justin Bates and Brian Markoski
We need managers so we can know how many teams we are able to have for our 2016 Fall Ball season. Please see Ray Mannon in the snack stand to sign up.
Player Registration will begin Jun 4th and run until the end of June. Teams will be created and FINALIZED in July.
Senior (pending enough players) $80
50/70 $70
A Ball. $60
Coach Pitch $50
Tee Ball. $50
Players will be placed in the divisons that they will be playing in NEXT YEAR (2017). Exiting 15 year olds will be allowed to play if they want.
****Please register for email and text notifications on the BABL website.****
Thanks to all involved for a successful Opening Day!
Meeting Date Change - The General Membership Meetings will now be held on the third Sunday of each month beginning February 21.
Registration for the 2016 season is complete. Any special requests for registration can be made by calling BABL at 732-458-8127 or by email at firstname.lastname@example.com
Meetings are held at the Brick American Club House, Lanes Mill Rd.
on the 3rd Sunday of the Month in
Jan, Feb, March, May, June, Sept, Oct, Nov. at 7:30 PM
No meetings for the months of April, July, August & December
Managers must attend at least 3 league meetings per year and Coaches 2 meetings per year.
The membership year begins Oct. 1st and ends on Sept. 30th of the following year.
Memberships dues are $20 per year.
***General Membership meetings are open to both our members and players parents.***
Click here General Membership Information
****Minutes for Board Meetings and General Membership Meetings can be found by clicking on "Meeting Minutes" on the menu of the Home Page.*****
Score Booth
We are happy to report that the use of the Score Booth has been very successful and we would like to continue with that success by adding a few rules that will apply at all games.
1. Only players aged 11 and 12 are permitted in the booth.
2. Only Brick American players and Coaches are permitted in the booth.
3. Three players are allowed at one time. One will announce the home team, one will announce the visiting team and one will control the score board. Please no slang or nicknames and be appropriate at all times.
4. At the conclusion of the game the Sound System switch should be turned off, the lights turned off and the booth closed and secured and the remote returned to the snack stand.
Note: Only HR's hit during the regular season are eligible for the HR Derby.
NOTICE:Please let us know if any of our members or family are CPR certified.
We are located across from Brick Memorial High School
BABL is open to all New Jersey Players who are not affiliated with another Babe Ruth League
Current issue's for Youth Baseball Online Magazines
Travel Baseball site featuring the BABL Travel Baseball Home Run Club!!
Travel Home Run Club
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Travel Baseball site featuring the BABL Travel Baseball Home Run Club!!
Travel Home Run Club
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Click here for more information about Our Sponsors
Sponsors if you want to make changes to your information or have specials to post
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Fulfillment ? Irvine, California Job Description: Operations Performance Lead As Mopro?s Operations Performance Lead, you will manage the fulfillment process for our clients, from on-boarding to finished product and ongoing support. You will oversee and support Team Leads who7/11/2016 Irvine, CA Save This Job
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Location: Chicago, Illinois Description Job Description The Lead Software Developer oversees software engineers responsible for the design, development, enhancement, deployment and support of multiple Ruffalo Noel Levitz products. The Lead Software Developer is responsible for5/30/2016 Chicago, IL Save This Job
Location: Chicago, Illinois Description The Lead Software Developer oversees software engineers responsible for the design, development, enhancement, deployment and support of multiple Ruffalo Noel Levitz products. The Lead Software Developer is responsible for design,5/30/2016 Chicago, IL Save This Job
at LogRhythm (View all jobs) Boulder, CO LogRhythm is looking for a Manager, Support Services who will be responsible and accountable for ensuring technical support excellence through the management of a Support Services team. The Manager will work with other teams to support7/2/2016 Boulder, CO Save This Job
Summary This professional is the client-facing representative of MD Revolution?s Technology Team. The SIE Lead works in support of each Implementation Manager with responsibility for all aspects of client technology interface and RevUp implementation from site to site. The SIE6/7/2016 San Diego, CA Save This Job
Delivery Greater New York City, United States Ayasdi is looking for strategic, results-driven Customer Success Director (CSD) to engage, and empower our customers to fully utilize Ayasdi solutions. Reporting to the Chief Customer Officer, the Customer Success Director will be7/2/2016 New York, NY Save This Job
Job Description: Position Reports to CEO ?Lead a team of outstanding service professionals in the healthcare automation space to drive hospital customers to highest level of financial and operational performance. ?Drive customer satisfaction scores and value seen by customer in6/27/2016 Pittsburgh, PA Saved Job
Description Global Head of Product Support Liquidnet is seeking a Leader to manage its global product support function, implement process change while helping to build a team for the future, and support expanding product lines across Equities and Fixed Income. This opportunity5/29/2016 New York, NY Save This Job
Redwood City About QuanticMind QuanticMind, the Platform for Smarter Advertising, is the pioneer of predictive advertising management software for paid search and social channels. By reinventing ad management point solutions through machine learning, distributed cloud computing,5/28/2016 Redwood City, CA Save This Job
Location:Carlsbad, CA Job Code:1060 # of Openings:1 Description POSITION: Supervisor, Technical Support DEPARTMENT: Technical Support REPORTING SUPERVISOR: Technical Support Manager DIRECT REPORTS: Yes FLSA: Exempt TRAVEL REQUIREMENTS: Up to 20% travel in the7/27/2016 Carlsbad, CA Save This Job
Department:Client Service Location:Waltham, MA Click Here to Complete Your Application At ZoomInfo we encourage creativity, value innovation, demand teamwork, expect accountability and cherish results. If you are an individual who takes charge, takes initiative, and gets stuff7/4/2016 Waltham, MA Save This Job
The Vice President of Customer Services & Renewals plays a critical role in the delivery of a world-class service experience to Guardian Analytics customers and channel partners by managing all areas of support and delivery including customer service, technical and product6/20/2016 Mountain View, CA Save This Job
Redwood City, CAFull-time Company Description Satmetrix is the leading global provider of cloud-based software for companies who demand differentiated knowledge about their customers. As co-developer of the Net Promoter methodology, Satmetrix combines unrivaled expertise with its6/1/2016 Redwood City, CA Save This Job
The Company Passenger is an LA-based company with a SaaS platform for sophisticated marketers who want to do more with their community. FUEL, powered by Passenger, is the most advanced community platform that helps brands rapidly build, engage and interact with customers for4/12/2016 Los Angeles, CA Save This Job
Customer Support Palo Alto, California APPLY Description Senior Technical Support Manager, Tier 3: Job Summary The Tier 3 Technical Support Team is the highest and most senior level of support at Bill.com. The Senior Technical Support Manager, Tier 3 is responsible for the7/19/2016 Palo Alto, CA Save This Job
at Prosper (View all jobs) San Francisco, CA We are seeking a Manager, Customer Success to be the primary thought leader on defining the ?playbook? of activities to optimally drive utilization and loan volume. The ideal candidate is also expected to manage the Customer Success7/1/2016 San Francisco, CA Save This Job
PowerReviews works with more than 1,000 global brands and retailers to increase conversion and improve products and the customer experience with ratings and reviews and Q&A software. Ratings and reviews solutions from PowerReviews are essential for consumers as they search7/22/2016 Chicago, IL Save This Job
PowerReviews works with more than 1,000 global brands and retailers to increase conversion and improve products and the customer experience with ratings and reviews and Q&A software. Ratings and reviews solutions from PowerReviews are essential for consumers as they search7/18/2016 San Francisco, CA Save This Job
US-VA-Vienna Apply View All Jobs Share This Job Job Description Telarix is a leading provider of interconnect and settlement software solutions to telecom communication service providers (?CSPs?) worldwide. The Company?s products drive ROI for customers by enabling and7/5/2016 Vienna, VA Save This Job


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Jobs in Medway
Part Time Medway,
Teaching Assistant Medway,
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Hays - Rochester, Kent, South East
As the sole agency supplier to the HM Prison Service for the provision of maintenance trades & support operatives, Hays is seeking to recruit Operational Support Grades (OSGs) for a Prison in Rochester... Medway Work careers ... £8.07 - £11.75 per hourRegister your CV
from: totaljobs.com (+3 sources) - 2 days ago
THE HUNDRED OF HOO SCHOOL - Rochester, Kent, ME3 9HH
We are looking for talented and committed Teaching Assistants to provide 1:1 support for pupils who have a range of particular needs. The Hundred of Hoo Academy is a unique all through academy with pupils... Medway careers... £15,523 - £19,742 pro rata
from: Jobs.guardian.com - 2 days ago
Farrer Barnes Limited - Rochester
This top tier firm are looking to add a Newly or Part Qualified accountant to their financial services department. The ideal candidate for this position will be an ACCA/ ACA studier, or AAT qualified and... medway kent... £28000 - £36000 per annum
from: GAAP web - 2 days ago
Hays - Rochester, Kent, South East
Surrey, £22.00 - £26.00 Per-hour A small Building Services consultancy based in Surrey are looking to recruit an M&E 2D CAD Technician capable of working within a demanding environment. You will be supporting... £22.00 - £26.00 per hourRegister your CV
from: totaljobs.com (+3 sources) - 2 days ago
Michael Page Finance - England, Kent, Rochester
This well known and well regarded professional services company is currently recruiting for an intelligent and highly organised Senior Insolvency Administrator at their Medway based office. As part of...
from: GAAP web (+4 sources) - 4 days ago
Evolution hospitality recruitment - Rochester, Kent, South East
Business Development Executive will need to go out there and find and help to close new opportunities! This is a 100%outward looking "hunter" role. Business Development Executive will have access to an... From £40,000 to £45,000 per annum BASIC 40K PLUSRegister your CV
from: totaljobs.com - Today
Senitor Associates - Rochester, Kent, Kent, South East
I have a new and exciting 1st line support vacancy for a Managed Services Provider based in Rochester with a salary up to £20K with good promotion prospects available in the future and great exposure to... £20000 per annum
from: cwjobs.co.uk (+1 source) - 4 days ago
Akton Recruitment Ltd - Rochester, Kent, South East
A leading UK manufacturer involved with fire safety systems in Kent are looking to take on a Software Test Engineer to work closely with developers and other stakeholders throughout the software development... £23000 - £28000 per annumRegister your CV
from: totaljobs.com - Today
QA Ltd (Apprenticeships) - Rochester
Our long-term clients include many blue-chip and high-street names, and we partner with global ICT solution providers. We operate in the Retail and Commercial industry sectors and we are particularly active... £6864 per year
from: jobsite.co.uk - Yesterday
New Appointments Group Limited - Medway
The successful candidate will while learning how the business runs from start to finish, also be working towards an Engineering qualification and developing skills using Sage software in a busy office...
from: jobsite.co.uk (+1 source) - Yesterday


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validation engineer jobs near Philadelphia, PA
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Validation Engineer/Temperature Mapping
Pindi Solutions - Philadelphia, PA
Analytical Instrumentation Validation Engineer/Temperature Mapping.author and execute protocols on analytical lab equipment.HPLC's, mass spectrometers, etc...
Electrical Engineer
Custom Ultrasonics, Inc. - Ivyland, PA
Plan, identify and execute Design Verification and Validation (V&V) testing of medical devices and their sub-systems confirming that Design Output(s) satisfy...
Validation Engineer
System One - West Point, PA
Authoring validation protocols and final reports, executing validation studies, analysis of validation data....
Process Engineer
Polysciences, Inc - Pennsylvania
Proficient in Commissioning and Validation strategies and execution. Lead commercial polymer facility project from inception through construction and validation...
Video Support Engineer
IntePros Consulting - Philadelphia, PA
Troubleshoot infrastructure and network issues as possible causes of poor service performance.- Takes the lead on defining the requirements for new tools and...
Sr. Engineer; Verification, Validation & Test
General Dynamics Information Technology - Philadelphia, PA
Oversees verification and validation of systems and software. Validation and testing requirements and procedures for Navy systems and software....
Video Operations Engineer
IntePros Consulting - Philadelphia, PA
Experience working with mobile (iOS/Android), web, cloud-based solutions, and IP video technologies in an enterprise environment ideal.• Knowledge with tools...
Automation Engineer- IT
Vertex, Inc. - Berwyn, PA
Employing DevOps concepts and approaches, the Automation Engineer will work with IT, development and testing personnel to maximize agility and minimize delays...
Application Engineer - DevOps
Ascensus - Dresher, PA
Understand, implement, and automate security controls, governance processes, and compliance validation. The Application Engineer - DevOps is responsible for the...
Embedded DEvOps Engineer
Trident Consulting Inc - Philadelphia, PA
Embedded DEvOps Engineer Location:. Setting up Jenkin build automation, scripting after deployment validation. Philadelphia, PA Job Description:....


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NOW With Alex Wagner 06/09/14
Lawrence O'Donnell's return to TV
Lawrence O'Donnell, host of "The Last Word," joins Alex Wagner from Los Angeles to discuss his return to TV after a car accident. ... more Duration: {{video.duration.momentjs}}
Lawrence O'Donnell's return to TV
NOW gives touching final goodbye
The role of music in presidential politics
GOP candidates prep for debate in unique ways
Eyes on Trump ahead of first 2016 GOP debate
Eyes on Trump ahead of first 2016 GOP debate
Trailer for new Benghazi movie released
Ohio campus cop pleads not guilty to murder
Which GOP-ers will be allowed to debate?
Outrage mounts after 'Cecil the Lion' death
Clinton, Boehner, Walker...and a cheesesteak
Politico: Jon Stewart secretly visited Pres.
Pres. Obama claims he could win third term
Anti-abortion group releases third video
Senate bill seeks to defund Planned...
Cosby accusers appear on NY Mag cover
Sanders gains ground on Clinton in key states
Louisiana state rep. calls for stricter...
Parents of Aurora victim react to LA shooting
Louisiana councilman: 'It's unfathomable'
Retrieving data...
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Sanders: Clinton must become president
Sanders thanks supporters
Elizabeth Warren has a message for the GOP
Michelle Obama: Clinton never buckles...
Michelle Obama: 'When they go low, we go...
Sarah Silverman ad lib changes tone of DNC
Silverman calls Bernie or Bust'ers ridiculous
Sen. Al Franken rips on Donald, Trump U.
Russian link probed in DNC e-mail theft
Jealous: What a real convention looks like
Clinton, DNC make room for progressives
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Sanders loses control of angry supporters
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DNC Chair won't gavel in convention
Sanders supporters says she won't vote for...
Talks underway to keep DNC chair off stage
Sanders' call to elect Clinton gets mixed...
Sanders: DNC chair resignation will lead...
Jeff Weaver on Wasserman Schultz, party unity
SNL Weekend Update on MSNBC
Rachel Maddow
Sarah Silverman ad lib changes tone of DNC
Russian link probed in DNC e-mail theft
Jealous: What a real convention looks like
Clinton, DNC make room for progressives
Are Democrats having a Tea Party moment?
Sanders loses control of angry supporters
Washington Post: Trump a threat to democracy
Progressives assess merits of Sen Tim Kaine
Programming notes for the next couple days!
Kaine 'a believer in possibilities'
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Under review: Fact-checking Trump's speech
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Did Trump speak to angry Americans?
Morning Joe
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Morning Joe mentioned in DNC emails
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Emails 'an opportunity for Hillary Clinton'
'Wrong for her': Why Schultz shouldn't speak
Is Russia behind DNC email leak?
Trump gets post-convention bounce: poll
Joe: I think Tim Kaine pick is a home run
Warren set to be Monday DNC keynote
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Used Chevrolet Uplander for Sale in Madison, WI
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"Awesome inexpensive reliable vehicle for large family's"
We love that the seats are oversized and comfortable. It wasn't difficult to fit any car seat in the vehicle and the latch system was easy to use. Our number one thing that we love is that it is reliable. We have never had the vehicle in the shop for any repairs. We have done all scheduled maintenance and it has continued to be completely relia...
2008 Chevrolet Uplander LS Ext Used Cars in Rochelle, IL 61068
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2006 Chevrolet Uplander LS Fleet Used Cars in Milwaukee, WI 53220
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2006 Chevrolet Uplander LS Fleet Ext Used Cars in South Beloit, IL 61080
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Braemar SA, the multi-disiplinary Marine Surveying and Technical Consultancy arm of Braemar Technical Services (BTS) has announced a key appointment to further strengthen its yacht services capability.
The Company's latest recruit, Stefano Scarpa, joins Braemar SA's well established team of marine consultants and surveyors specialising in yacht services and brings with him a wealth of experience to this important sector.
Stefano is a maritime professional with 15 years' experience in working in the superyacht market, including experience in design, naval architecture consulting and project management. In his previous role as Senior Naval Architect and Project Manager with a major international superyacht specialist, he reviewed and monitored the design and construction of new build yachts on behalf of yacht owners, in addition to carrying out pre-purchase surveys, warranty surveying and technical due diligence for financial institutions.
"Alongside our traditional services Braemar SA has a well-established global yacht surveying and consultancy capability," says Nigel Clark, Managing Director of Braemar SA. "With a broad base of experts strategically located around the world, including within the important yacht centres such as London, Miami and Dubai, we are ideally placed to further expand our range of services in this exciting sector of the market and we are delighted to welcome Stefano on board. His move to Braemar SA will add a new dimension to the team and contribute to our continued growth in this sector.
Although technically the challenges facing the surveyor or consultant when dealing with superyachts can be very similar to those when dealing with other vessels, there are still always some differences in approach that make this a niche sector requiring specific skills."
Maritime Today
The Maritime Industry's original and most viewed E-News Service
Hoover Container Solutions' chairman and CEO, Donald Young, has been nominated and elected to the Petroleum Equipment & Services Association (PESA) Advisory Board for a three-year term.
The U.S. Navy conducted a series of cooperative air defense test exercises with the Spanish navy that culminated in live missile firing events using the latest Aegis Weapon System baseline July 20-21.
After 25 years as CEO of Antwerp Port Authority, Eddy Bruyninckx has been raised to the nobility by H.M. the king of Belgium with the rank of baron. In Belgium
As Team Britannia prepares for its bid to break the round-the-world powerboat record later this year, GAC has been named their official shipping and logistics partner.
Blohm+Voss has appointed Jean-Gabriel Tridon as General Manager and Dieter Pladeck as Finance Director for its new maintenance and refit facility in La Ciotat in the south of France.
RINA S.p.A, the holding company of the multinational testing, inspection, certification and consulting engineering group based in Genoa, Italy, has announced that
Plans to ensure there is a full maritime survey and inspection team covering the whole U.K., will see more remote working and a slight change to where some of the marine offices are based.


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Kathy Stevens in the Huff Post
Kathy Stevens pens a story in The Huffington Post about veganism.
On Thursday, December 16, Kathy Stevens, founder and director of Catskill Animal Sanctuary, posted an essay on The Huffington Post titled, "Talking Tofurkey: What Would It Take to Change Your Diet?" In it, Stevens says that the holidays are a time to reflect on the year past, and she encourages readers to consider how compassionately they lived in 2010, especially in regards to eating animal. She addresses the common excuses why people choose to not become vegan, and counters them with information both about agribusiness and the environment. Stevens closes with a message of compassion and resources for readers who are interested in learning more about veganism.
Sales of Veg Products in Denmark Spike 30 Percent
Denmark's growing plant-based population is driving sales of meat and dairy alternatives.
Read More »
Dublin Bans Wild Animal Circuses
Ireland's capital city will no longer allow the use of wild animals such as elephants, tigers, and camels in traveling circuses.
Read More »
Court Protects Whales in Navy Sonar Ruling
Animal and environmental groups successful lawsuit represents a big win for marine mammals.
Read More »
Five Fashion Brands Ban Down Feathers
A group of large clothing brands, including UK-based Topshop, pledges to remove feathers from future collections.
Read More »
Number One Egg Brand in America to Go Cage-Free
Egglands Best has plans to ensure none of its 15 million hens are cruelly confined by inhumane wire cages.
Read More »
More Flashbacks
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- Sculptor Forms Fresh Faces from Fruits and Veggies
- Rhode Island Becomes First State to Ban Bullhooks
- Publix Finally Commits to Going Cage-Free
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- Junk Food is Largest Source of Americans Calories
- Bill Would Protect Plant-Based Foods from Big Ag Attacks
- Vegan Blue Wine To Debut in the United States
- Exhibit Opens in England to Show Importance of Bees
- Portlands Summer of Brunch Starts Sunday
- First Automated Farming Machine Debuts
- Walmart Pilots Ugly Produce Program
- Vegan Diet for Farmed Fish Could Prevent Collapse of Fisheries
- Brand Halts Leather Line in Favor of Vegan Bag Collection
- One in 10 New Products in Germany Is Vegan
- Italian Mayor Plans to Make Turin Italys First Vegan City


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I've got news for you: you're in relationship.
With the person you wake up with, with the barista who hands you your grande dark, with your cubicle mate, with your clients, with whomever you break bread with at the end of the day.
I want you to be in these relationships in a sparkly, lifting-up way. And I'm betting that you're not, because it can be hard to do.
It's okay. We can talk communication, and conflict, and whatever lies in between those things for you. I am like a soccer coach (as in: you get to WIN) for your life and your relationships.
Here's who I work with:
Couples – both as a pair and with the individuals, I am damn good at talking about what makes our relationships tick. I am the referee who makes it all safe to talk about. Even the sex and the stuff you think might just end it all.
New parents – Having a baby is completely magical and a beautiful new chapter. And it will also throw everything you know about being in relationship into a big ol' wood chipper. I give you tools, strategy and support you as you learn the simple things that keep your family together and functioning.
People who want more – Have you landed in the same place again? Frustrated with all of it? Trying to figure out what's next? Scared? Doing great and not sure how to clear the next hurdle? I've got your back. We'll look at what's working and what's not and make a PLAN.
What have you got to lose? Your comfortable, boring life?
Yeah, that would be super sad.


text:
New Music
Enthroned Conjure Black Metal That is "Of Shrines & Sovereigns"
Black metal traditionalists Enthroned hail from Belgium, but heed to calls of the old Norwegian guard for their latest track "Of Shrines and Sovereigns", streaming below for the first time. Culled from the Sovereigns LP, the track is the latest to be released from the band's ninth LP and first in two years. Check it out below and look for your copy via Agonia on April 15th.
The Outfit TX Are Universal Dudes, and 'Green Light: Everythang Goin'' Proves It
Redd Kross and Side Eyes Remind Us How Badass the Go-Go's Actually Were
Fake People Suck: Here's Teen Sensation Mallrat's "Uninvited"
NYC Black Metal Force Anicon's New Video for "The World As Will" Tames The Blurry Wilderness


text:
Learn more
Tea with Jane Austen Hardcover – 1 Dec 2004
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Product Description
The ideal read for any fan of Austen's work, or the enduring British favourite - tea.(Period Living)
Darjeeling with Darcy or earl Grey with Emma?(Field)
Both books are beautifully produced and make an attractive pair for lovers of Jane Austen, filling in all-important background details to the novels.(Good Book Guide) --This text refers to an alternate Hardcover edition.
About the Author
KIM WILSON is a writer, editor, and gardener who lives in Waukesha, Wisconsin, and is a longtime member of the Jane Austen Society of North America. Her other title for Frances Lincoln is In the Garden with Jane Austen (9780711225947).--This text refers to an alternate Hardcover edition.
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Customer Reviews
Top Customer Reviews
Indeed, the passing of the day was marked by many during the late eighteenth and early nineteenth centuries with the drinking of this beverage. Wilson broadens the scope of her work, however, beyond the scope of the novelist and her characters to show how the habit evolved across a broad swathe of society, and how the quality of tea that you drank, as well as the paraphernalia with which you drank it, was an indicator of your social standing.
Although we no longer tend to put such effort into our drinking of tea nowadays, with teabags being the standard order of the day for many, you do still find those who have aspirations to the ilk of Earl Grey and other more select teas, and tea is still one of the most commonly consumed hot liquids. Wilson caters for our needs in this respect, too, as she gives numerous recipes throughout the text of updated versions of, as well as the original recipes for, treats from the teatimes of Austen's day.Read more ›
I am always fascinated by any kind of historical book that sheds light on the everyday lives of people of another era, especially when that is related to one of my favorite authors. I love to see how different people's lives were in the past, but also what similarities might exist. For instance, I was thrilled to find out that although centuries apart, Jane Austen and I, not only share a passion for a good cup of tea, but surprisingly she got her tea from Twinings just like I do today.
The book includes excerpts from novels by Austen and other authors of the time, excerpts from her letters, showing how important tea was to her, and a few period recipes. I think this is a wonderful book, but if you are interested in tea related recipes, as the recipes in this book are not that many, I also strongly recommend The Jane Austen Cookbook, which includes many excellent recipes for tea accompaniments from the Austen household.
She loved it - and apparently the recipies are also great!
Most Recent Customer Reviews
Bought, together with the following two items, as a birthday present for a literary fanatic friend who has enjoyed the vintage experiencePublished on 13 April 2013 by sally coston
I've bought this as a Christmas present for my wife, and I know she's going to love it. Nice presentation, photos, snippets of jane Austen - what's not to like?Published on 15 Dec. 2012 by Dr. D. Brand
I really loved this wonderful book . Very useful for all Jane Austen's fans , and many interesting informations about the Regency area in England . Read morePublished on 25 Aug. 2012 by P. Babin
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From a huge Short Sandringham to a sleek Supermarine S.6, the Solent Sky museum in England is a must-see for any fan of flying boats and seaplanes.
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17 Comments
Realtime comments disabled
Amuseiclovah 1981 days ago
i want to bite someone's neck ugh the hat and the mirror, love'm
anniengel 2561 days ago
hey graham your hat is awesome, in really I love your style greetings
alohalolita 2687 days ago
exhilarating 2709 days ago
it's a pity the tickets for pete's tour are sold out. >< but see you at your own tour mr coxon!
exhilarating 2709 days ago
i'm too shy to leave a comment here but...i LOVE the hat and ...
darrengroucutt 2711 days ago
Hi gra looking good! Did you get my message the other day? I was
Gonna try and catch stu this week again!
Viola_cobain 2711 days ago
nice mirro,nice hat,nice t-shirt,everything in it's right place~
iamyijuan 2711 days ago
will you model more clothes here?
jennyhardy 2711 days ago
was just wondering how you'd look with the pakol... you do have an interesting collection of hats!
Newsie2 2711 days ago
that neck needs to be bitten....
iad2la 2711 days ago
I appear to have left a message from my sister's twitter. Sorry! Tobes x
iad2la 2711 days ago
100% genuine dog hair? Woof! x
delgordon73 2711 days ago
very early 90s.
tofupanny 2711 days ago
mmm... interesting hat... the brown pakol? i see a goatee coming. doesn't look like black to me.
odette9 2711 days ago
Very nice! thumbs up, approved. Now, next, tall hat and red jacket? Yes? Yes! :)
dadis 2711 days ago
:O My dog has a tee like yours


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A look at what's happening across the region
Reports of broken down vehicle on A1 Northbound between A184 (Dunston Interchange) and A694 / A69 (Blaydon / Consett Turn Off).
Seven people have been arrested on suspicion of manslaughter after a man died following an incident at nightclub in Durham.
Emma Jesson brings you Saturday night's forecast
Sandiford, a former legal secretary originally from Redcar, was convicted of drug smuggling offences and sentenced to death in 2013.


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BYU football: Ziggy Ansah will attend NFL draft over graduation
Jeffrey D. Allred, Deseret News
Ziggy Ansah originally thought he would attend his graduation ceremony from BYU instead of the NFL draft in New York City.
Promptly following this decision, he got a lot of grief from his friends.
"I wasn't going to go (to the draft). I was going to be educational — I've got to walk at my graduation," Ansah told USA Today sports. "But then I got the invitation from the NFL, and I have my friends telling me, 'What the heck is wrong with you? You're going to go walk? Don't be stupid.' "
With a little bit of influence from his peers, Ansah changed his mind and will attend the draft.
The two life-changing events are scheduled for the same day, April 25. Ansah's counselor at BYU is arranging for him to walk at a different ceremony so he can have both experiences.
Whitney O'Bannon is currently a new media sports intern for the Deseret News.
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