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Given the low projected demand for new coal-fired generating capacity prior to 2005, the U.S. market for Group 1 systems will likely be small. These systems are essentially based on proven components and do not offer an efficiency advantage over state-of-the-art pulverized coal systems. Projected performance and cost enhancements come from improved systems design and integration. There may be opportunities to market these technologies overseas, where demands for new coal-based power generation capacity are greatest. Despite their limited commercial potential, the first-generation PFBC and IGCC systems constitute important steps toward the development of higher-efficiency Group 2 and Group 3 systems. In contrast, the LEBS does not offer comparable growth potential, since it employs a simple steam (Rankine) cycle, whereas all of the Group 2 and Group 3 systems use combined-cycles with potentially higher efficiencies.
The committee recommends that future investment of DOE resources in first-generation systems be based on realistic market expectations and value as an entry into new technology with high growth potential. At least 50 percent industry cost sharing should be required to demonstrate private sector confidence in these technologies.
Group 2 and Group 3 power generation systems depend on the successful development of several critical components, including high-temperature gas turbines, high-temperature heat exchangers, advanced high-temperature furnaces, fuel cells, hot gas cleanup technology, and high-efficiency gasification. The riskiest components appear to be the high-temperature ceramic heat exchanger required for the externally fired combined-cycle system and the hot gas cleanup systems required for advanced PFBC and needed for maximum-efficiency IGCC and IGFC systems. The 1370 °C to 1430 °C (2500 °F to 2600 °F) gas turbine required for Group 3 systems is within the state of the art for aviation systems but requires further development, demonstration, and testing for power generation applications. Fuel cells hold significant promise for efficiency advantages, but their high cost may be a barrier to widespread use of IGFC systems.
Gas cleanup is necessary to comply with environmental requirements and to protect advanced gas turbines from corrosive impurities, notably chlorine, volatile alkali metals, and particulates. Commercially available cold gas cleanup technology could be used for IGCC and IGFC systems, although this would incur higher costs and an efficiency penalty of approximately 2 percentage points for air-blown second-generation systems. In contrast, advanced PFBC systems require hot gas filtration since cooling the high-temperature, high-pressure combustion products would eliminate the advantages of PFBC. Thus, IGCC is a somewhat less risky technology than PFBC. Environmentally, IGCC has the advantage of producing by-product sulfur or sulfuric acid, whereas the use of limestone or dolomite for in-bed sulfur capture in PFBC systems can as much as


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Santiago Gamboa's Necropolis, which won the La Otra Orilla Literary Award in 2009, is frustratingly good, inventive, and obsessed with story telling. The premise is simple: An author much like Santiago Gamboa himself, is invited to participate in a literary conference about biography—one that will also be attended by a strange array of guests, including a porn star and an ex-con turned evangelical pastor—that takes place in a besieged Jerusalem. During the conference, the ex-con evangelical—who tells one of the most captivating stories in the book—is found dead of an apparent suicide. Maybe.
What's interesting/frustrating about this book is that that plot point takes place on page 165, then is interrupted, textually at least, for almost 200 pages as other participants in the conference tell their stories, each of which is intriguing in its own right, but which, for a reader of traditional, conventional books obsessed with pacing, plot points, and building climaxes, must be crazy-making. (But those sorts of readers don't really read these sorts of books, do they?)
I read this way back in the fall and meant to write up a review back then when all the connections between the various stories in the novel—which, in terms of their themes, ideas, and narrative styles, overlap and play off one another in a beguiling fashion—were still fresh in my mind. Now, I'm just left with the memory that, in contrast to say The Canterbury Tales or The Decameron, this novel is much more circular in its construction, looping back on itself in a way informed by the best of twentieth-century literature.
A lot of people reading this blog probably feel the same way, but god damn is it a good time for Spanish-language literature. Vila-Matas. Gamboa. Neuman. Labbé. Marias. Chejfec. Prieto. Valenzuela. Dozens of writers I can't think of.
Founded in 1960 by such creative pioneers as George Perec, Raymond Queneau and Italo Calvino, the Oulipo, shorthand for Ouvroir de littérature potentielle, came about in when a group of writers and mathematicians sought constraints to find new structures and. . .
There's little to say about a series of prose poems that willfully refuse to identify pronoun antecedents. Or perhaps there are a million things. The poems in Morse, My Deaf Friend— the chapbook by Miloš Djurdjević published by Ugly Duckling. . .
The Crimson Thread of Abandon is the first collection of short fiction available in English by the prolific Japanese writer and all-around avant-garde trickster Terayama Shūji, who died in 1983 at the age of 47. This collection would be important. . .
Last year, NYRB Classics introduced English-language readers to Catalan writer Josep Pla with Peter Bush's translation of The Gray Notebook. In that book, Pla wrote about life in Spain during an influenza outbreak soon after World War I, when. . .
"Your bile is stagnant, you see sorrow in everything, you are drenched in melancholy," my friend the doctor said.
bq. "Isn't melancholy something from previous centuries? Isn't some vaccine against it yet, hasn't medicine taken care of it yet?" I. . .
What to make of Vano and Niko, the English translation of Erlom Akhvlediani's work of the same name, as well as the two other short books that comprise a sort of trilogy? Quick searches will inform the curious reader that. . .
The opening of Jón Gnarr's novel/memoir The Indian is a playful bit of extravagant ego, telling the traditional story of creation, where the "Let there be light!" moment is also the moment of his birth on January 2nd, 1967. Then. . .
Mahasweta Devi is not only one of the most prolific Bengali authors, but she's also an important activist. In fact, for Devi, the two seem to go together. As you can probably tell from the titles, she writes about women. . .
The prolific Spanish author Benito Pérez Galdós wrote his short novel, Tristana, during the closing years of the nineteenth century, a time when very few options were available to women of limited financial means who did not want a husband.. . .
Pedro Zarraluki's The History of Silence (trans. Nick Caistor and Lorenza García) begins with the narrator and his wife, Irene, setting out to write a book about silence, itself called The History of Silence: "This is the story of how. . .

subset: commoncrawl


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Place the butter in a glass pie plate and melt in microwave for 30 €" 45 seconds. Add the coarsely ground Pecans and sugar and mix with the butter. With a fork, press pecans against the pie plate bottom and sides. Bake in oven at 350F for approximately 10 minutes or until toasted. Cool on wire rack.
Combine sweetened condensed milk, lime zest, key lime juice, lightly beaten egg yolks, and salt; blend until mixture thickens. Stir in avocado puree. Pour filling into cooled toasted pecan crust.
Chill pie overnight.
"If you like this video, please remember to visit Real Texas Blog for more videos from Ramona_werst."


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We created Pandora to put the Music Genome Project directly in your hands
It's a new kind of radio –
stations that play only music you like
But It's Better If You Do
Features of This Trackacoustic rock instrumentation
a subtle use of vocal harmony
a busy horn section
minor key tonality
a prominent accordion part
acoustic rhythm guitars
emphasis on instrumental arranging
These are just a few of the hundreds of attributes cataloged for this track by the Music Genome Project.show more
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How can he possibly sing this in like 5 seconds?!?!? ? ?


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Russell Day-Long Group Buy 2013-14
View Single Post
Beastly Adventurer
Joined: Sep 2011
Location: TN/AL
Oddometer: 1,294
Originally Posted by
Matt fe2o3
What part of your post am I not understanding:
1). OP advised he worked off hours and folks needed to be patient in getting a response to a PM and this was not an immediate done deal.
2). You decided not to be patient and not only badgered the OP for a PM, you called the vendor to discuss a GB that you has no hand or effort in setting up?
3). You now post there is no GB without discussion from OP, and you have posted only twice in this entire forum..
That about sums it up. So hokay;
a). Remind me to never do you a favor. You are too high maintenance.
b). The GB attempt has no value to a vendor unless there is substantial interest. You show up with a coordinated effort for 5 or 10 seats and the maker kinda shrugs. You show up with 250+ peeps, then that represents good income that might go elsewhere. That's why it is done, to leverage value for the group while supporting a vendor.
c). Really you can do or call whatever or whoever you like, however nobody is going to drop their world to rub your back and coax you to buy a seat. It was not like the OP was saying to send him money. Instead of being patient you took a nice steaming dump on yourself.
d). Frankly if you called me up out of the blue with your impatience I would tell you to piss of too - just like the lady at RDL did.
Here's a bit of advice: Lighten up, take an anti-anxiety pill or whatever, relax, let the ideas unfold. If you can't handle the concept of a GB don't get involved.
Then again for all I know you are the official spokesman for the OP, but me thinks not.
OP thanks, will wait and see how this develops.
Originally Posted by
I had several people do the same thing as you last year during my group buy and in the same tradition, you have returned to this forum in an attempt to crush the spirits of those on the list.
First of all, thank you Mr. Helper.
RDL will never, and I repeat never, confirm that they are going to accept a group buy months before they actually do so. Reason being... If they did so, people would quit placing full price orders and wait a couple to three months to order at the discount. These are smart business people we are talking about here and this ain't their first rodeo.
I would ask anyone that comes across this thread to first of all, don't call RDL and ask about this forum's group buy. Leave the negotiation with RDL to the administrator of this group buy effort. If you do not have faith that the group buy will take place, simply go to their website and go ahead and order at full price. While there is no guarantee that RDL will do a group buy this year, Kim's denial of it in September certainly doesn't mean it will not happen in December. She said the exact same thing last year. Time will tell...
Secondly... See the last request. No one is forcing anyone to be part of this effort. Don't go out of way though to ruin it for those that do want to be part of it. If RDL goes forward (as they did last year when they said they were not) there will be quite a few people enjoying a new seat come December. If not, we did our very best to make it happen.
The administrator of this group buy works for a living and does not exist to cater to your emails and requests for acceptance. If you do not hear back from him in a timeframe you feel is reasonable, simply attempt to reach out to him again.
Last but not least, go back and read the instructions in the first post. If you did not follow them, you shouldn't expect a response at all.
I ride not to add days to my life, but to add life to my days.
Those who say it cannot be done should not interrupt the people who are doing it."
"Now and then it's good to pause in our pursuit of happiness and
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Knights of Malta
An alternative to the 'Malta Experience' in Valletta, this audiovisual presentation and tour of a former ammunitions store gives a good insight into the history of Malta and how this was shaped by the successes of the Knights of Malta and Knights Templar.
A visit to the Knights of Malta starts with an audiovisual show which I didn't find greatly interesting as the quality of the film wasn't good. Much better is the tour of the building which houses 3D waxworks that depict various scenes relating to the Order. I have to say that I learned quite a bit on the tour and found the building very atmospheric (if kind of creepy!).
Entrance costs 6.50 Euros for adults (3 Euros for children) and you get issued with a pair of headphones that you hand back when you leave.
- Museum Visits
- Historical Travel
- Architecture
The Jewel in the Crown.
Mdina is a great place to visit and walking the tiny streets and the ancient battlements of the "Silent City" are very interesting things to do. The different audio visual "experiences" vary in quality but canbe interesting as well (see seperate tip). For me, and as the title suggests, the jewel in Mdina's crown is undoubtedly the Cathedral of St. Paul right in the centre of the city. In a country well-endowed with impressive places of worship, this one really does stand out.
So where does this wonderful building originate from? Local legend has it that it stands on the site where Publius met St. Paul when he was shipwrecked on Maltain the first century. Nobody is entirely sure when the first Church was built here but it fell into disuse during the Tirkish Islamic occupation of the island. After the Turks left, another Cathedral was built but this was almost totally destroyed in the great earthquake of 1693. A new structure was called for and was built to the design of Lorenzo Gafa primarily between 1697 and 1702 although the final work of adding the cupola was not complete until 1705. This is essentially the Baroque structure you see today.
From the outside it is impressive enough and it is worth noting the two barred cross on the roof as opposed to the single barred cross I am more used to in Western Europe. Also of note are the clocks. Look closely and you will see they are not both standard clocks, one serves as a calender. However, it is only when you go inside the building that it's true glory is revealed. It truly is magnificent.Fortunately, (non-flash) photography is allowed and I did take full advantage of that. I intend to let the images speak for themselves rather than describe in great detail every point of interest. As the VT format only allows for five images here, I have also constructed a travelogue to showcase some more of my photographic handiwork, such as it is.
I would make one suggestion for the visitor. I am always speaking on tips about the value of looking up as you travel around as it is amazing what you see above eye level. This holds true as the work on the cielings is outstanding. In the Cathedral, the visitor is also advised to look down as you are effectively walking on the memorial stones of literally dozens if not hundreds of members of the Knights of St. John of Malta and they do make fascinating reading.
To the logistics now.
The Cathedral, like the Cathedral Museum (see seperate tip) is open Monday to Friday 0930 to 1630 and Sunday 0930 to 1530 with last admission 30 minutes before closing. Admission is €5 for adults (obviously including admission to the Cathedral proper) and €3:50 for students. Under 12's are free. You should buy your ticket in the Museum as there is no ticket office in the Cathedral itself. Also note that the entrance to the Cathedral is not by the front door as you might expect but the door on the right side of the building as you look at the front.
I believe it would be wheelchair accessible as it is all on a level and I did not see any major obstacles.
No visit to Mdina is complete without a visit to the Cathedral and I really do recommend it.
- Religious Travel
- Photography
- Architecture
It's all included in the price.
If you visit Mdina, you simply must see the magnificent Cathedral of St. Paul right in the centre of the old walled city. I have a seperate tip and travelogues about this wonderful building. Included in the ticket price and giving rise to the title of this tip, is admission to the Cathedral Museum which is situated in the old Archbishop's Palace just across the square from the Cathedral itself. In fact, you have to go here to get your ticket for the Cathedral. As you can see from the images, it is an impressive building.
The Palace actually resulted form a natural disaster, namely the great earthquake of 1693 which caused huge damage to the original Cathedral. the building plans for the now structure, which you see today, included encroaching on what had been the previous Palace and so a new building was required. This is the result.
Whilst the building is interesting, I have to say that the actual Museum is less so. There are some good things to see, including some very good paintings but generally it is not that exciting. As I say, though, it is included in the price and you should have a look round. Regrettably, photography is not allowed inside, hence the paucity of images, but the attached website has some good images should you want a look.
To the logistics now.
the Museum is open Monday to Friday 0930 to 1630 and Sunday 0930 to 1530 with last admission 30 minutes before closing. Admission is €5 for adults (obviously including admission to the Cathedral proper) and €3:50 for students. Under 12's are free.
- Historical Travel
- Museum Visits
Inaccessible but worth a look.
I would not normally write tips about a place you cannot enter but I shall make an exception in this case as it is possible to look in from outside and it is a place of significance in the ancient town of Mdina (Imdina in Maltese). It is the chapel of St. Agatha and I am unsure if it is generally open to the public or I was just unlucky when I visited. There was no information at the site and I can find nothing online on the subject.
There has been a place of Christian worship here since 1410, built initially by Francesco Gatt, a local noble. The reason this place so important to the Maltese is because of an event which occurred in 1551 when the Ottoman Turks under Sinam Pasha invaded the island and laid seige to the city which was then called Notabile and was capital of the country. A local nun claimed to have had a vision from St. Agatha who instructed her to have a Mass said and to then process around the bastions in view of the enemy and carrying her image. This was duly done and the beseiging Turks decided to take ship again and went off to invade nearby Gozo instead. How the residents of Gozo feel about the Saint I have no idea. For many years this event was comemorated with a procession around the bastions on the 5th of February every year.
The great earthquake of 1693 more or less laid the old building waste although it was quickly rebuilt in 1694 to the deign of Lorenzo Gafa, who was also responsible for the nearby Mdina Cathedral (see seperate tip). Opened in the presence of the then Grand Master of the Knights of St. John of Malta Adrien de Wignacourt on 26th June 1695, this is the building you presently see.
Despite the popularity of St. Agatha, the church eventually fell into disrepair and it was necessary to launch a public appeal for funds for refurbishment. The building looked OK to me, although there may still be work to do that is not obvious. I do hope they succeed and save what is a very important building. I know the European Union is throwing a vast amount of money at Malta presently and perhas there will be some for this building.
As I mentioned, I was not able to enter the building although I did get a look in through a mtetal gate and took the image you can see of the wonderful inside of the place, which actually looks quite small. It is worth having a look at and perhaps you will be luckier then me and get inside.
- Historical Travel
- Religious Travel
- Budget Travel
Take a walk.
I know it mightt seem like a very obvious thing to say but the very best way to see the glory that is Mdina (Imdina) is to walk it. If you are afraid of getting lost, you can get a free map from the excellent Tourist Information centre (see seperate tip on this page) but it really is not necessary. the walled city is not that large and is easy to navigate. In fact, you cannot roam too far as you are enclosed by the walls. Get yourself off the main streets and go and have a wander rould some of the fascinating little back alleys, it really is very rewarding and there is something interesting to see round every corner you turn.
I have enclosed a few images here of the types of things you may see. Of coursae, I have left the best until last, it is absolutely free!
- Hiking and Walking
- Historical Travel
- Budget Travel
Really not worth bothering with.
This "experience" as they like to style them, was the third one I had visited in a short period of time in an afternoon, having been encouraged to purchase a discounted ticket for all three. The first was the Mdina Experience, the second the "Knights of Malta" and this, the third, the so-called "medieaval Times experience".
Perhaps I was a little jaded with all the "experiences" I had had that afternoon but this really was somewhat of a disppointment. It is effectively a bit of a walk-through selection of tableaux put together in an upstairs room of the "Palazzo Costanzo", one of the many auberges belonging to the various branches of the Knights of St. John of Malta. On entering the building, which I did on an off-season midweek afternoon, I was directed by a sign to the right and into the souvenir shop where I found myself totally alone. Had I been criminally minded, which I am most definitely not, I could have made off with a good proportion of the stock as there was nobody there. There were a few people in the cafe across the corridor.
Eventually, a man turned up and looked slightly surprised when I presented my ticket. He pointed me towards some stairs and off I went. I'll not bore the reader but I walked through a designated route comprising several decently rendered tableaux but it really was nothing special. There was little factual information given and no audio guide. Ralistically, I could have done the whole thing comfortably in fifteen minutes. Frankly, it was a disappointment and really not worth bothering about. If you want to do one "experience" in Mdina, stick to the "Mdina Experience" and then spend the rest of your time actually walking round the streets of this wonderful place soaking up the real history for yourself.
Readers of my other pages will know I do not like writing negative tips but I can find little good to say about this. Give it a miss.
- Historical Travel
Another "experience" but not much of one.
I visited this place in company with a middle aged British couple I had met in the "Mdina Experience" nearby, where we had bought composite tickets for several attractions operated by the same company. The visitor should note that this is a commercial operation and not run by either Heritage Malta or Witt Artna, the two Government / charitable bodies that run many attractions in the country. Having been ushered out of the back door of the first location, we were given fairly comprehensive directions to this place which is fairly nearby and the walk is pleasant enough along some atmospheric little streets. I should add here that much of Mdina, but it's very nature, is perhaps not suitable for mobility impaired visitors as it is very uneven underfoot.
We were greeted by a slightly brusque lady and asked what language we spoke, English for us all. We were given headsets and ushered into a small cinema type place where the presentation had already begun. There were two ladies there, evidently Italian, and the soundtrack was quite understandably in that language. Here was the first problem. The PA system was so loud that it was very difficult to hear the English commentary through the headphones and the sound quality was not great. Aditionally, much of the historical content we had already seen in the previous "Mdina Experience".
After the presentation, the screen raised to show us a brief tableau of some medieaval figures and then the lady came and ushered us through a door into a small corridor. We were told to follow the corridor round, which we did. Movement sensitive sensors lit up the various tableaux and triggering an appropriate commentary on the headsets. The tableaux were well done but again my complaint would be that we had already had most of the information earlier. I know you cannot seperate the history of the Knights of St. John from the history of the island, they are far too closely linked, but it was a little repetitious. If you had done this as the first "experience" in Mdina, no doubt it would have been a lot more interesting.
A small note here. I have included the website for this place which, at time of writing (08/0/2013) does not appear to be working properly but I include it in the hope it will be fixed soon.
If given the choice again, I would pick the Mdina Experience over this. No mannequins but the quality of the presentation is so much better. I really would not recommend this place and a look at various respected websites suggests I am not alone in this view. Regular readers of my pages will know that I do not writing negative tips but I can find little positive to say about this place and I really cannot recommend it to the visitor.
- Family Travel
- Historical Travel
Experience the "experience"!
There are certainly many wonderful things to experience on Malta in the proper sense of the word and then there are a number of "experiences" you may like to try. If this sounds a little cryptic, allow me to explain. For it's relatively small sixe, the island seems to have a disproporionate amount of "experiences", normally audio visual presentations perhaps with a few tableaux and / or special affects and it is easy to become "experienced out" in the same way as visitors to Southeast Asia speak of being "templed out" after visiting so many. Often these "experiences are in direct competition with each other and whilst none of the several I visited were bad, they do vary in quality so it pays to shop around. If you do several, you will be getting the same information over and over again so it pays to do a bit of research first.
The first such place I visited on the island was in Mdina (Imdina) \nd is called the Mdina Experience, situated in a very pleasant old building in Mequitta Square as you can see in the image. I wandered in on a not particularly sunny off-season midweek afternoon and was pleasantly greeted by a lady waeing a vaguely period peasants costume. As seems to be the norm in Malta I was offered a choice of various combination tickets for other "attractions" operated by the same company which I took as it offered a decent discount. I shall deal with the other attractions in other tips. Please note that this is not run by Heritage Malta or Witt Artna, it is a purely commercial operation. The next show was not due for some minutes so I was invited to either purchase something from the well-stocked if expensive shop to having a bite to eat or a drink in the associated cafe. I declined having just enjoyed a coffee shortly before.
There were only myself and another middle aged British couple and we were led into a cineam typr room upstairs which would probably have held a couple of hundred, which felt a bit odd. We were then treated to a forty or so minute, mostly re-enacted, film accompanied by a narrative voiced by an American actor. It was interesting enough and the quality of the film and sound was very good. When it was over, the lady came and ushered us out a back door which was a shortcut to the next "experience" (The Knights of Malta) we were going to visit a little way away. The third attraction on the combined ticket is the "Medieaval Times Experience" in yet another location.
It was OK and the film very well-produced but whether I would recommend it I am not so sure. Reading a decent guidebook or looking here on VT or other internet sites will give you all the same information and you can spend your time actually walking the streets and soaking up the history instead of watching it on a screen. I suppose it just depends on your personal preference.
- Historical Travel
- Family Travel
IL-Foss Tal-IMdina
The name given to the former moat now a wide ditch that surrounds the city's fortress walls,cleared of its natural citrus trees and now replaced by new Laurel trees,very peaceful and illuminating place to take gentle strolls after walking the city streets.There was much debate several years ago when the Government decided to up root thousands of trees that had grown hear naturally but enough money and time has been spent in making the area somewhat more attractive to both locals and visitors.
- Architecture
- Historical Travel
- Castles and Palaces
City Walls
Mdina is completely surrounded by bastions 185 metres above sea level,fortified as long ago as 1000 BC when the Phoenicians built a protective wall here and called their settlement Malet meaning 'place of shelter'.It was given its present name after the Arabs arrived in the 9th century-Medina is Arabic for 'walled city'.they built strong walls and dug deep moat between Mdina and its surrounding suburbs.
- Architecture
- Historical Travel
- Castles and Palaces
Mdina Gate
This is the main entrance to the city of Mdina and is located not far from the bus terminus in Rabat.It was designed by 'de Mondion' ,a prodigious builder for the Order of St.John.This gate was built a few metres to the left of the original gate in 1724.The location of the original gate is clearly visible in the now solid wall.The building of the new gate was to enlarge the Palace,Palazzo Giuratale,damaged in the great earthquake of 1693.The new palace built for 'Grand Master Vilhena' was renamed 'Palazzo Vilhena'.The coat of arms above the gate are of Lions,the lions are repeated as statues at the front of the bridge that leads you to the gate.
- Castles and Palaces
- Historical Travel
- Architecture
Greek Gate
Although the Greek Gate in Mdina is well preserved,it was built in the 17th century.Some are of the opinion that a Greek community once lived here.The site is actually called 'St.Nicholas square',St.Nicholas being the patron saint of Greece,there also used to be a chapel in the area which was also dedicated to St.Nicholas.
- Architecture
- Historical Travel
- Family Travel
Cathedral Museum
This museum traces its origins to a fabulous donation made by 'Marquis Saverio Marchesi' ,who decreed,in his last will,that once his family became exstinct,all the artistic works the family possessed were to be given to the cathedral chapter.This came to be in 1896.The idea of turning some halls adjacent to the Cathedral into a museum matured during the 1960's.It was officially opened and named the Mdina Cathedral Museum in 1969.
Three large halls now feature the earliest painted panels from the Spanish Romanesque period to the 17th century as well as a small collection of 19th and 20th century paintings from both local and foreign artists.There are also a few smaller rooms displaying ornaments and artifacts donated from artists from all over Malta.
Opening times:Mon till Fri:09.00-16.30,Sat:09.00-15.30,closed Sunday,Admission is €5,Children under 12 free,students €3.50.
- Religious Travel
- Museum Visits
- Historical Travel
St.Paul's Cathedral
This is a Roman Catholic Cathedral situated in the heart of Mdina,it is built on the site where governor 'Publius' was reported to have met Saint Paul following his shipwreck off the Maltese coast.According to tradition,the firtst Cathedral of Malta was dedicated to the Blessed Virgin,Mother of God,but having fallen into ruin during the Muslin period,it was re-built following the Norman conquest and re-dedicated to Saint Paul.The old church was modified and enlarged several times.
The building you see today was designed by 'Lorenzo Gafa',it was built between 1687 and 1702 to replace a ruined Norman cathedral destroyed by the 1693 earthquake on Malta.Designed in Baroque style it sits at the end of a rectangular square with two bell towers at both corners and a light octagonal dome with eight stone scrolls leading up to a neat lantern.
Open daily:10.00-17.00,admission is free,no flash on photos.
- Historical Travel
- Religious Travel
- Architecture
A short but useful tip.
I am sure there will be an audible sigh of relief from regualr readers of my pages when I tell you that this is going to be one of the shortest tips I have ever written.
This tip concerns the excellent Tourist information Centre in Mdina (Imdina). I wandered into the beautiful buidling you see in the image on my first visit to the city to be greeted by a charming young Maltese lady. I had only been in Malta a couple of days and was still finding my way around a bit. She answered my many (probably stupid) questions with a smile, suggested which of the many free brochures might suit me and sent me on my way feeling that I had encountered the Maltese hospitality of which I had heard so much.
That's it really, I suggest that if you visit Mdina, and you really should, that you drop in here.
Here are the logistics, with details taken from the very good attached website.
Opening hours are -
Monday to Saturday: 09:00 to 17:30 (Last admission 17:15)
Sunday and Public Holidays: October to March 1000hrs to 1400hrs (Last Admission 13:45); April to September 09:00 to 13:00 (Last Admission 12:45)
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I like the dark red color! And bangs are fun, until you want to grow them out then they're annoying lol
I think working at TDS is going to be SO FUN!!! I can't wait to hear about it!
I like the dark red color! And bangs are fun, until you want to grow them out then they're annoying lol
I think working at TDS is going to be SO FUN!!! I can't wait to hear about it!
Have a great first day Susan!
i'm so stressed! The pins I bought for Kelly and another friend are missing and not where I last put them. Losing things and spending lots and lots of time to find them is probably the story of my life.
Ugh, work. Normal Saturday night gripes. Had both of the new guys tonight, so I'm surprised I got all my signs done. One is doing better than the other; the super new one really needs to be taught how to interact with customers. He's not rude, he's just kind of there. One of my regular customers came in and asked why we were hiring a bunch of hot guys. I told her I didn't know, but it's super unfair since they're practically babies, they're only 18. Hopefully they both get the hang of it all soon, I like new people, but I don't care for babysitting.
Tonight sucks. My order entry system is down AGAIN, it has been down since 2320, and a code blue has been called FIVE TIMES in ICU. FIVE! FIVE FREAKING TIMES! Arrrggghhh!!!!!
So of course they're calling for all kinds of drips, using up everything in their code carts, there are like 10 nurses, the hospitalist, the house supervisor, respiratory, and who knows down there, plus possibly patient family. It's a mad house, and I can't enter any orders. Everything is being hand written. It's scary, slow, and frustrating.
Thanks for letting me vent lol
My system came back up after 3 hours. Hooray!
I also found out that we had been on lockdown for a while due to a shooting in the vicinity, and when I called the house sup to confirm we were still on lockdown she said the man was just caught in our parking lot.
Wayyyyy too much drama for one night/day there Kelly!
I even started questioning if I did buy those pins but I remembered I sent the photo to you. Hopefully they'll turn up soon.
Speaking of pins: We're hitting up the parks this week. Would anyone like anything picked up while we are there?
T-minus 1 hour, 15 minutes until I leave for work.
Peter Pan: "You know, your hair is on fire."
Me: "Yes. It happened after a recent trip to Neverland."
Peter Pan: "Well, you can dunk your head in the pond behind us, if you want."
Lost Child of Neverland since 1996.
~Favorite Hero: Peter Pan. Favorite Villian:Malificent.Favorite Ride: The Haunted Mansion.~
Yes Kelly, the scenery has been much improved with them there even if it's a tad distracting
And that sounds like a very scary night, sheesh! You'd think a lockdown would be more um, not really announced, but widely known? Glad they caught the guy though, that's a good thing. Venting keeps us all sane. I look at this place sometimes as my padded room to come scream in like the mom in Blast From the Past. Ya always feel better!
I sent an e-mail to my director saying lockdowns should be announced overhead. There was also a shooting right across the street from my house, haven't found out yet if they're related.
I need to move.
Brandon - they'll turn up eventually!
Susan - How was your day???
I'm going to raise a terrible human being because I'm going to bring snacks in for my kids in Disneyland.
You've all been warned.
I bring snacks into DL out of necessity. Unfortunately I can't have the usual snacks that everyone enjoys at the parks - pretzels, churro, popcorn and so forth so I'm forced to
You have an excuse.
I'm just going to be a terrible mother.
I've seen families bring KFC buckets into the parks so a few snacks is nothing at all.
Pins found! I can breathe easier now. One problem is that I was searching for those blue Disney Parks bags but I had placed the small Disney parks bags into a larger grocery bag when exiting the car.
It's so aggravating how often I lose things and I try to be organized too but I guess those moments where I'm exhausted when I get home and I don't place things in the spots I usually place them that gets me in trouble. I hate being late to things and my number one reason for being late is not being able to find my keys.
Anyhow I'm off tomorrow!
Story of my life. I often think "I'll put this right here where I won't forget it!" then have no idea where that super special place is when I go looking for the item again.
Hooray for being off tomorrow! I wish I was off!


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The Latest: Offensive line mixing it up to find right combo — 4/9/2015
As the spring winds down, offensive line coach Mike Uremovich is experimenting with his offensive line to make sure the Wolfpack has the right combination up front. [details]


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NOV User Interface for .NET 2015.1
Nevron Open Vision (NOV) implements a complete and feature-rich set of cross-platform User Interface elements (widgets) that aims to completely eliminate the need to use any platform specific controls, that would ultimately bind your Presentation Layer to a specific platform/runtime, look-and-feel and API.
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140--By the end of the tournament Blair's Guzzo, who took third, looked like the best wrestler in the weight class. All 4 Blair freshmen lost early and then ran the table to get to the podium (two lost conso semis, but all won their conso finals).
145--Mason's losses will be discussed ad naseum. There are physically stronger wrestlers who are also skilled, like Burns, who are going to cause him problems.
152--Boyd simply couldn't beat Meade's length. Did any observer feel that Boyd was going to win as it went into overtime? I would term Meade's RO of Boyd in the second OT as "professional" as funny as that sounds.
160--In that early semifinal scramble when Cubberly landed HARD out of bounds I could feel it up in the stands. Oof. It hurt. Condon won but.....
189--Wisonsin's Joe Budi could have gotten some consideration for OW also. VERY impressive.
215--Nice tourney for Blair's Hanzlik (5th place).
285--Ohio's Division 1 hwy ranks were emptied by graduation. Valley Forge's James Meder thrusts himself into the spotlight with a great showing taking Goebel into OT in the final. A pleasure to watch.


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Publications (25)64.97 Total impact
Article: Intragenic mutations in SMN1 may contribute more significantly to clinical severity than SMN2 copy numbers in some spinal muscular atrophy (SMA) patients[Show abstract] [Hide abstract]
ABSTRACT: Background: Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder caused by deletion or intragenic mutation of SMN1. SMA is classified into several subtypes based on clinical severity. It has been reported that the copy number of SMN2, a highly homologous gene to SMN1, is associated with clinical severity among SMA patients with homozygous deletion of SMN1. The purpose of this study was to clarify the genotype-phenotype relationship among the patients without homozygous deletion of SMN1. Methods: We performed molecular genetic analyses of SMN1 and SMN2 in 112 Japanese patients diagnosed as having SMA based on the clinical findings. For the patients retaining SMN1, the PCR or RT-PCR products of SMN1 were sequenced to identify the mutation. Results: Out of the 112 patients, 106 patients were homozygous for deletion of SMN1, and six patients were compound heterozygous for deletion of one SMN1 allele and intragenic mutation in the retained SMN1 allele. Four intragenic mutations were identified in the six patients: p.Ala2Val, p.Trp92Ser, p.Thr274TyrfsX32 and p.Tyr277Cys. To the best of our knowledge, all mutations except p.Trp92Ser were novel mutations which had never been previously reported. According to our observation, clinical severity of the six patients was determined by the type and location of the mutation rather than SMN2 copy number. Conclusion: SMN2 copy number is not always associated with clinical severity of SMA patients, especially SMA patients retaining one SMN1 allele.Brain & development 12/2013; 36(10). DOI:10.1016/j.braindev.2013.11.009 · 1.54 Impact Factor
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ABSTRACT: Spinal muscular atrophy (SMA) is a common neuromuscular disorder with autosomal recessive inheritance, resulting in the degeneration of motor neurons. The incidence of the disease has been estimated at 1 in 6000-10,000 newborns with a carrier frequency of 1 in 40-60. SMA is caused by mutations of the SMN1 gene, located on chromosome 5q13. The gene product, survival motor neuron (SMN) plays critical roles in a variety of cellular activities. SMN2, a homologue of SMN1, is retained in all SMA patients and generates low levels of SMN, but does not compensate for the mutated SMN1. Genetic analysis demonstrates the presence of homozygous deletion of SMN1 in most patients, and allows screening of heterozygous carriers in affected families. Considering high incidence of carrier frequency in SMA, population-wide newborn and carrier screening has been proposed. Although no effective treatment is currently available, some treatment strategies have already been developed based on the molecular pathophysiology of this disease. Current treatment strategies can be classified into three major groups: SMN2-targeting, SMN1-introduction, and non-SMN targeting. Here, we provide a comprehensive and up-to-date review integrating advances in molecular pathophysiology and diagnostic testing with therapeutic developments for this disease including promising candidates from recent clinical trials.Annals of Human Genetics 07/2013; DOI:10.1111/ahg.12031 · 1.93 Impact Factor
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ABSTRACT: We report a newborn with intestinal malrotation who developed a severely high serum unbound bilirubin level and a low serum albumin level without a marked increase in serum total bilirubin level after abdominal surgery, which required exchange transfusion and albumin supplementation. The serum unbound bilirubin level may be highly relative to the serum total bilirubin level in newborns who have undergone abdominal surgery soon after birth and are hypoalbuminemic after surgery.Pediatrics International 06/2013; 55(3):e59-62. DOI:10.1111/ped.12018 · 0.73 Impact Factor
Article: Minimal residual disease monitoring in neuroblastoma patients based on the expression of a set of real-time RT-PCR markers in tumor-initiating cells[Show abstract] [Hide abstract]
ABSTRACT: Minimal residual disease (MRD) is derived from tumor-initiating cells (TICs) and is responsible for tumor relapse. Neuroblastoma is characterized by extreme tumor heterogeneity, and more than half of high-risk patients experience tumor relapse. To overcome tumor heterogeneity and achieve more sensitive detection of MRD, several sets of real-time RT-PCR markers have been reported for MRD monitoring in neuroblastoma patients from different centers. However, these markers vary across centers and are still being validated. In the present study, we validated the ability of 14 commonly used real-time RT-PCR markers to detect MRD based on their expression in neuroblastoma TICs, and we developed a novel MRD detection protocol, which scored the samples as MRD-positive when the expression of one of the 11 real-time RT-PCR markers (CHRNA3, CRMP1, DBH, DCX, DDC, GABRB3, GAP43, ISL1, KIF1A, PHOX2B and TH) exceeded the normal range. By using this protocol, we prospectively monitored MRD in 73 bone marrow (BM), 12 peripheral blood stem cell and 8 peripheral blood samples from 14 neuroblastoma patients treated at a single center. We scored 100, 56, 56 and 57% BM cytology-positive, elevated vanillylmandelic acid (VMA), elevated homovanillic acid (HVA) and elevated neuron-specific enolase (NSE) samples as MRD-positive, respectively. MRD was also positive in 48, 45, 46 and 43% of the BM cytology-negative and normal VMA, normal HVA and normal NSE samples, respectively. These results suggest that the present MRD detection protocol based on the expression of a set of 11 real-time RT-PCR markers in neuroblastoma TICs achieves sensitive MRD monitoring in neuroblastoma patients.Oncology Reports 04/2013; 29(4):1629-36. DOI:10.3892/or.2013.2286 · 2.19 Impact Factor
Article: Quantitative evaluation of ventricular dilatation using computed tomography in infants with congenital cytomegalovirus infection[Show abstract] [Hide abstract]
ABSTRACT: BACKGROUND: Infants with congenital cytomegalovirus infection (CCMVI) may develop brain abnormalities such as ventricular dilatation, which may potentially associate with sensorineural hearing loss. There is currently no recognized method for quantitative evaluation of ventricle size in infants with CCMVI. Our objectives were to establish a method for quantitative evaluation of ventricle size using computed tomography (CT) in infants with CCMVI, and determine a cut-off value associated with abnormal auditory brainstem response (ABR) early in life. DESIGN/SUBJECTS: This study enrolled 19 infants with CCMVI and 21 non-infected newborn infants as a control group. Infants with CCMVI were divided into two subgroups according to ABR at the time of initial examination: normal ABR (11 infants) or abnormal ABR (8 infants). Ventricle size was assessed by calculating Evans' index (EI) and lateral ventricle width/hemispheric width (LVW/HW) ratio on brain CT images, and was compared among groups. A cut-off ventricle size associated with abnormal ABR was determined. RESULTS: EI and LVW/HW ratio were significantly higher in the CCMVI with abnormal ABR group than the control and CCMVI with normal ABR groups. Cut-off values of 0.26 for EI and 0.28 for LVW/HW ratio had a sensitivity of 100% and 100%, respectively, and a specificity of 73% and 91%, respectively, for association with abnormal ABR. CONCLUSIONS: We established a method for quantitative evaluation of ventricle size using EI and LVW/HW ratio on brain CT images in infants with CCMVI. LVW/HW ratio had a more association with abnormal ABR in the early postnatal period than EI.Brain & development 01/2013; 36(1). DOI:10.1016/j.braindev.2012.12.009 · 1.54 Impact Factor
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ABSTRACT: The patient was an 8-year-old Japanese girl with Gilbert's syndrome (GS). Based on the DNA analysis, she was homozygous for a T-to-G transversion at nucleotide position 1456 in the UGT1A1 gene, leading to the substitution of aspartate for tyrosine at position 486 of the UGT1A1 enzyme. Because this mutation is located in an exon common to UGT1A genes, all the UGT1A enzymes may be affected. It is well-known that UGT1A1, UGT1A6 and UGT1A9 enzymes glucuronidate acetaminophen. To evaluate acetaminophen tolerance in the patient, serum acetaminophen levels were determined after oral administration of acetaminophen (15 mg/kg). The maximum serum acetaminophen level reached (12.8 µg/mL) was far below the toxic level. The finding suggested that the usual therapeutic dose of acetaminophen is safe for the GS patient. The combination of mutation analysis in UGT1A1 and acetaminophen loading test may be useful to avoid adverse effect in GS patients.Pediatrics International 12/2012; 54(6):934-6. DOI:10.1111/j.1442-200X.2012.03602.x · 0.73 Impact Factor
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ABSTRACT: BACKGROUND: Serum unbound bilirubin (UB) level measures bilirubin not bound to albumin, and has been reported to be better than total bilirubin level at identifying infants at risk of developing bilirubin-induced neurotoxicity, including auditory abnormalities. A detailed treatment strategy for newborns with high serum UB levels has not been established. Our objective was to assess auditory outcomes in newborns with serum UB levels of ≥1.00 μg/dL who were treated according to our novel treatment protocol. METHODS: A prospective clinical study was conducted in newborns weighing >1,500 g with serum UB levels of ≥1.00 μg/dL who were admitted to Kobe University Hospital and Kakogawa Municipal Hospital, Japan from 2006 to 2011. Enrolled newborns were treated as follows: (1) if the serum UB level was 1.00-1.50 μg/dL, phototherapy and infusion were administered with or without albumin or immunoglobulin therapy; and (2) if the serum UB level was >1.50 μg/dL, exchange transfusion was performed immediately. Auditory brainstem responses were evaluated at the time of discharge. RESULTS: A total of 89 Japanese newborns with UB levels of ≥1.00 μg/dL were enrolled at a median age of 4 days. Of these, 85 had UB levels of 1.00-1.50 μg/dL and four had UB levels of >1.50 μg/dL. After being treated according to our protocol, no newborns were diagnosed with auditory brainstem response abnormalities. CONCLUSIONS: Our treatment protocol for Japanese newborns with serum UB levels of ≥1.00 μg/dL may be useful for the prevention of bilirubin-induced auditory abnormalities.Pediatrics International 09/2012; 55(1). DOI:10.1111/j.1442-200X.2012.03726.x · 0.73 Impact Factor
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ABSTRACT: Recent Japanese epidemiology of neonatal sepsis and its predominant pathogens has not been reported. It is also unknown whether there are center differences in the incidence of neonatal sepsis, including early-onset sepsis (EOS) and late-onset sepsis (LOS) in Japan. To investigate the morbidity and characteristics of neonatal sepsis in recent years and the differences in the incidence of sepsis among Japanese neonatal care units. We retrospectively collected the data of newborn infants with culture-proven sepsis that occurred in five Japanese centers of perinatal care from 2006 to 2008. The incidence of sepsis was calculated, including EOS and LOS, and compared among centers. Morbidity from sepsis occurred in 51/6,894 (0.74%) infants. The incidence of EOS and LOS was 0.13 and 0.61%, respectively. The incidence of total sepsis and LOS in infants <1,000 g of birth weight was significantly higher than that in infants who weighed >1,000 g at birth, whereas there were no significant differences in the incidence of EOS between the different birth weights. Methicillin-resistant Staphylococcus aureus was the most common pathogen involved in morbidity and mortality of neonatal sepsis. Significant center differences were observed in the incidence of LOS, but not EOS. The majority of culture-proven neonatal sepsis is LOS, which differs among centers, especially in infants who weigh <1,000 g at birth in Japan. We consider that it is important to control nosocomial infection in newborn care units to further reduce the morbidity of neonatal sepsis in Japan.Neonatology 05/2012; 102(1):75-80. DOI:10.1159/000337833 · 2.37 Impact Factor
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ABSTRACT: Background: Paramyotonia congenita (PMC) is an autosomal dominant disorder characterized by cold- or exercise-induced myotonia. PMC is caused by a mutation in SCN4A which encodes the α-subunit of the skeletal muscle sodium channel. Methods: The patient was an 11-year-old Japanese girl who was diagnosed as having PMC. To confirm the diagnosis, an orbital ice-pack test and blinking tests were performed. Next, to identify the mutation, genetic analysis of SCN4A was performed. Finally, to evaluate the mutation effect on the protein structure, in silico protein modeling analysis was performed. Results: Cold- and exercise-induced myotonia was reproduced in the patient with non-invasive bedside tests: ice-pack and blinking tests. In the genetic analysis, a missense mutation, c.4343G>A in SCN4A, was identified, which may result in an arginine to histidine substitution at 1448 in the protein sequence (p.Arg1448His). According to the protein modeling analysis, the mutation neutralized the positive electrostatic charge at 1448 in the DIV/S4 segment and disrupted the beginning of the helical structure in the DIV/S3-S4 linker of the SCN4A protein. Conclusions: Diagnostic physical interventions in the patient confirmed the phenotype presentation consistent with PMC, and the in silico protein modeling analysis of p.Arg1448His predicted structural changes which can affect function of the protein. All the data confirmed the diagnosis of PMC in the patient and added to existing literature emphasizing the important role of arginine residue at 1448.Pediatrics International 04/2012; 54(5):602-612. DOI:10.1111/j.1442-200X.2012.03646.x · 0.73 Impact Factor
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ABSTRACT: Neuroblastoma is an aggressive pediatric tumor that accounts for 15% of cancer-related deaths in children. More than half of high-risk neuroblastoma patients develop tumor relapse that is lethal in most cases. A small population of tumor-initiating cells (TICs), recently identified from high-risk neuroblastoma patients as spheres, is believed to be responsible for tumor relapse. Rab family small G proteins are essential in controlling membrane traffic and their misregulation results in several cancers. Rab15 was originally isolated as a brain-specific Rab protein regulating the endocytic recycling pathway and was recently identified as a downstream target of the neural transcription factor Atoh1. Previously, we identified two alternatively spliced Rab15 isoforms in neuroblastoma cells and showed a significant correlation between Rab15 expression and neuronal differentiation. As aberrant alternative splicing is intimately associated with an increasing number of cancers, its use as a new diagnostic and/or prognostic biomarker has attracted considerable attention. In the present study, we explored cancer-associated changes of Rab15 alternative splicing in neuroblastoma TICs. We found that Rab15 alternative splicing generated two novel isoforms designated as Rab15(AN2) and Rab15(AN3) in addition to two known isoforms designated as Rab15(CN) and Rab15(AN1). Although both Rab15(AN2) and Rab15(AN3) contained premature termination codons, they were detected in not only neuroblastoma cells but also in normal human tissues. One isoform was predominantly expressed in the brain and testis, while the other isoform was more specifically expressed in the brain. In neuroblastoma, Rab15 isoform balance measured by the Rab15(CN)/Rab15(AN1+AN2+AN3) ratio was significantly decreased in spheres compared to parental cells. These results suggest that Rab15 alternative splicing may serve as a biomarker to discriminate TICs from non-TICs in neuroblastoma.Oncology Reports 03/2012; 27(6):2045-9. DOI:10.3892/or.2012.1731 · 2.19 Impact Factor
Article: Correlation and precision of serum free bilirubin concentrations determined by single and two peroxidase concentration methods in term or late-preterm newborn infants using a FDA-approved analyzer.[Show abstract] [Hide abstract]
ABSTRACT: Free bilirubin concentration (B(f)) is an index for identifying newborns at risk for developing bilirubin-induced neurotoxicity. It has been suggested that B(f) measured by a single peroxidase concentration (B(f-single)) does not equal the equilibrium concentration of B(f), which is confirmed by B(f) at two different peroxidase concentrations (B(f-two)). However, the differences between B(f-single) and B(f-two) are unknown in the serum of term or late-preterm newborn infants. Furthermore, to apply B(f-single) with savings on time and cost to the clinical setting, it is very important for us to clarify the differences between B(f-single) and B(f-two). Forty serum samples were obtained from 21 term or late-preterm newborns who were admitted at Kobe University Hospital. Using a peroxidase method, B(f-single) was measured at one peroxidase concentration, and B(f-two) was determined at two different peroxidase concentrations (the manufacturer's recommended peroxidase concentration and half the manufacturer's recommended peroxidase concentration). To clarify the relationship between B(f-single) and peroxidase concentrations, B(f-single) was measured at five different concentrations of peroxidase reagent. Intra-day and inter-day analyses were performed to assess the precision of B(f-single) and B(f-two). 1/B(f-single) increased as peroxidase concentration increased. B(f-single) was significantly lower than B(f-two) (B(f-single): 0.50 microg/dL [0.13 - 1.22 microg/dL] versus B(f-two): 0.59 microg/dL [0.15 - 1.76 microg/dL], p < 0.001), but B(f-single) was significantly correlated with B(f-two) (r = 0.953, p < 0.0001). Intra-day analysis showed that the CV was 9.7% for B(f-two) and 3.3% for B(f-single), and the inter-day CV was 12.4% for B(f-two) and 3.2% for B(f-single). Although B(f-single) and B(f-two) are not identical, B(f-single) is significantly correlated with B(f-two) and it is more precise than B(f-two) in term or late-preterm newborns.Clinical laboratory 01/2012; 58(5-6):507-14. · 1.08 Impact Factor
Article: Spinal Muscular Atrophy Patient Detection and Carrier Screening Using Dried Blood Spots on Filter Paper[Show abstract] [Hide abstract]
ABSTRACT: Spinal muscular atrophy (SMA) is a common autosomal recessive neuromuscular disorder. It is caused by mutations in the SMN1, and its clinical severity is modified by copy number variations of the SMN2. According to previous studies, deletion of SMN1 exon 7 is the most frequently observed in patients with SMA. Therefore, molecular analyses exploiting this genetic lesion could be beneficial in the diagnosis of SMA. Unfortunately, in many geographical regions, physicians do not have the latest molecular screening technologies at their immediate disposal. Thus, to overcome this issue, we developed an SMA-diagnosing system using dried blood spots (DBS) placed on filter paper to facilitate remote diagnosis. In this study, we validate the applicability of DBS on Flinders Technology Associates (FTA) filter paper for detecting SMN1 exon 7 deletions and copy number variations of SMN1 and SMN2. To detect exon 7 deletions in SMN1, polymerase chain reaction (PCR)-restriction fragment length polymorphism analysis was conducted by using DNA extracted from the DBS on FTA filter paper that had been stored at room temperature for a period of up to 4 years. To determine the copy numbers of SMN1 and SMN2, we carried out SYBR green-based real-time PCR by using the same blood specimens. The results obtained from the DBS on FTA filter paper were in complete concordance with those analyses using fresh blood specimens. This indicates that DBS on filter papers is a reliable method for SMA patient detection and carrier screenings. The SMA-diagnosing system, combined with the mailing of DBS on filter paper, will be beneficial for patients suffering from neuromuscular disorders in areas with limited or no access to diagnostic facilities with molecular capabilities.Genetic Testing and Molecular Biomarkers 09/2011; 16(2):123-9. DOI:10.1089/gtmb.2011.0109 · 1.15 Impact Factor
Article: Renin is activated in monochorionic diamniotic twins with birthweight discordance who do not have twin-to-twin transfusion syndrome[Show abstract] [Hide abstract]
ABSTRACT: To assess renin, aldosterone, human atrial natriuretic peptide (hANP) and brain natriuretic peptide (BNP) levels in cord blood from monochorionic diamniotic (MD) twins with a birthweight (BW) discordance that do not satisfy the criteria of twin-to-twin transfusion syndrome (TTTS). Cord blood samples were obtained from 28 MD twins without TTTS. They were divided into two groups on the basis of BW discordance as follows: large (>7.5%) and small (7.5%). Cord blood renin, aldosterone, hANP and BNP levels were measured. Renin levels in MD twins with a large BW discordance were significantly higher than those in MD twins with a small BW discordance, with no significant differences in aldosterone, hANP and BNP levels. A significant correlation was found between renin levels and BW discordance. Renin is activated in MD twins with a BW discordance of >7.5%, even in non-TTTS.Journal of perinatology: official journal of the California Perinatal Association 09/2011; 32(7):514-9. DOI:10.1038/jp.2011.136 · 2.35 Impact Factor
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ABSTRACT: A growing number of epidemiological studies have demonstrated that the consumption of green tea inhibits the growth of a variety of cancers. Epigallocatechin gallate (EGCG), the most abundant catechin in green tea, has been shown to have an anti-cancer effect against many cancers. Most cancers are believed to be initiated from and maintained by a small population of tumor-initiating cells (TICs) that are responsible for chemotherapeutic resistance and tumor relapse. In neuroblastoma, an aggressive pediatric tumor that often relapses and has a poor prognosis, TICs were recently identified as spheres grown in a serum-free non-adherent culture used for neural crest stem cell growth. Although EGCG has been reported to induce growth arrest and apoptosis in neuroblastoma cells, its effect on neuroblastoma TICs remains to be defined. Gene expression was analyzed by real-time reverse transcription polymerase chain reaction (RT-PCR). The effects of EGCG on cell proliferation, apoptosis, and sphere formation were determined by cell counting, propidium iodide staining, and sphere (>100 μm in diameter) counting, respectively. Neuroblastoma BE(2)-C cells showed increased expression of stem cell markers (nanog homeobox [NANOG] and octamer-binding transcription factor 4 [OCT4]), as well as decreased expression of neuronal differentiation markers (Cu(2+)-transporting ATPase alpha polypeptide [ATP7A] and dickkopf homolog 2 [DKK2]) in spheres grown in serum-free non-adherent culture, compared to parental cells grown in conventional culture. Although EGCG induced growth arrest and apoptosis in the parental cells in a dose-dependent manner, it was not effective against spheres. However, EGCG potently inhibited sphere formation in the BE(2)-C cells. The present results suggest that EGCG may inhibit the development of TICs in BE(2)-C cells.Environmental Health and Preventive Medicine 09/2011; 17(3):246-51. DOI:10.1007/s12199-011-0239-5
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ABSTRACT: The American Academy of Pediatrics guidelines recommend that the total bilirubin (TB)/albumin (Alb) ratio (B/A ratio), instead of serum concentration of unbound bilirubin (UB), can be used with TB for determining treatment modality for jaundiced newborns ≥ 35 weeks of gestation. It is unknown, however, whether the B/A ratio is actually correlated with serum UB. Four hundred and ninety-seven serum samples were obtained from 209 newborns ≥ 35 weeks of gestation, who were admitted to Kobe University Hospital. Serum UB concentration was measured using the glucose oxidase-peroxidase method. Serum TB and Alb concentrations were measured on spectrophotometry. B/A ratios were calculated and were linearly compared with serum UB. Furthermore, the accuracy of the B/A ratio was evaluated. The B/A ratio was significantly correlated with serum UB concentration. A serum UB concentration of 0.6 µg/dL was in agreement with a B/A ratio of 0.5. For comparison of the number of newborns who had serum UB concentrations ≥ or <0.6 µg/dL and B/A ratios ≥ or <0.5, we found the following characteristics: the concordance rate between serum UB concentrations and the B/A ratio was 94%, sensitivity was 51%, and specificity was 99%. The B/A ratio is significantly correlated with serum UB concentration in newborns ≥ 35 weeks of gestation. The B/A ratio, however, is underestimated when serum UB concentrations are >0.6 µg/dL.Pediatrics International 08/2011; 54(1):81-5. DOI:10.1111/j.1442-200X.2011.03457.x · 0.73 Impact Factor
Article: Rab15 expression correlates with retinoic acid-induced differentiation of neuroblastoma cells[Show abstract] [Hide abstract]
ABSTRACT: Neuroblastoma is the most common extracranial solid tumor in children and accounts for 15% of pediatric cancer deaths. Although retinoic acid (RA) is currently used to treat high-risk neuroblastoma patients in the clinic, RA-responsiveness is variable and unpredictable. Since no alterations in the RA-signaling pathway have been found in neuroblastoma cells, molecules correlated with RA-induced differentiation will provide predictive markers of RA-responsiveness for clinical use. The Rab family of small G proteins are key regulators of membrane traffic and play a critical role in cell differentiation and cancer progression. Although an increasing number of cancer-associated alternative splicing events have been identified, alternative splicing of Rab proteins remains to be characterized in neuroblastoma. In the present study, we focused on Rab15 that was originally identified as a brain-specific Rab protein and regulates the endocytic recycling pathway. We identified alternatively spliced Rab15 isoforms designated as Rab15CN and Rab15AN in neuroblastoma cells. Rab15CN was composed of 7 exons encoding 212 amino acids and showed brain-specific expression. Alternative splicing of exon 4 generated Rab15AN that was predicted to encode 208 amino acids and was predominantly expressed in testis. RA induced neuronal differentiation of neuroblastoma BE(2)-C cells and specifically up-regulated Rab15CN expression. Reciprocally, RA-induced differentiation was observed in Rab15CN-expressing BE(2)-C cells in preference to Rab15AN-expressing BE(2)-C cells. Furthermore, Rab15CN expression was also specifically up-regulated during RA-induced differentiation of newly established neuroblastoma cells from high-risk patients. These results suggest that Rab15 expression correlates with RA-induced differentiation of neuroblastoma cells.Oncology Reports 07/2011; 26(1):145-51. DOI:10.3892/or.2011.1255 · 2.19 Impact Factor
Article: Diagnosis of Spinal Muscular Atrophy Via High-Resolution Melting Analysis Symmetric Polymerase Chain Reaction Without Probe: A Screening Evaluation for SMN1 Deletions and Intragenic Mutations[Show abstract] [Hide abstract]
ABSTRACT: Spinal muscular atrophy (SMA) is a well-defined autosomal recessive neuromuscular disorder caused by mutations in the survival motor neuron 1 (SMN1) gene. The most frequently observed mutation is a deletion of exon 7, which has been documented in >95% of SMA patients. A novel technique for detecting mutations known as high-resolution melting analysis (HRMA) has rapidly become the tool of choice for screening pathogenic genetic variants. In the present study, we attempt to validate the applicability of HRMA to the detection of exon 7 deletions and other intragenic mutations in SMN1. Three primer sets were adopted in our HRMA screening for deletion of SMN1 exon 7. In screening attempts utilizing two primer sets, the results of HRMA were not compatible with those obtained by polymerase chain reaction-restriction fragment length polymorphism. Therefore, we applied a modified protocol using revised primer sets, which resulted in an absolute compatibility of results between HRMA and polymerase chain reaction-restriction fragment length polymorphism. With regard to screenings for intragenic mutations in SMN1 exon 3, two primer sets were adopted for use in HRMA. In the initial HRMA screening using the first primer set, we failed to identify any intragenic mutations; however, when using a revised primer set, HRMA successfully detected the presence of a c.275G>C mutation. HRMA is a simple but versatile tool to add to the existing arsenal of diagnostic techniques that could aid clinicians/researchers in diagnosing SMA. However, as we demonstrate in the present study, the design and selection of primers is of monumental importance in ensuring the successful application of HRMA to screening for pathogenic variants.Genetic Testing and Molecular Biomarkers 06/2011; 15(10):677-84. DOI:10.1089/gtmb.2010.0237 · 1.15 Impact Factor
Article: Valproic acid increases SMN2 expression and modulates SF2/ASF and hnRNPA1 expression in SMA fibroblast cell lines.[Show abstract] [Hide abstract]
ABSTRACT: Spinal muscular atrophy (SMA) is a common autosomal recessive neuromuscular disorder that is caused by loss of the survival motor neuron gene, SMN1. SMA treatment strategies have focused on production of the SMN protein from the almost identical gene, SMN2. Valproic acid (VPA) is a histone deacetylase inhibitor that can increase SMN levels in some SMA cells or SMA patients through activation of SMN2 transcription or splicing correction of SMN2 exon 7. It remains to be clarified what concentration of VPA is required and by what mechanisms the SMN production from SMN2 is elicited. We observed that in two fibroblast cell lines from Japanese SMA patients, more than 1mM of VPA increased SMN2 expression at both the transcript and protein levels. VPA increased not only full-length (FL) transcript level but also exon 7-excluding (Δ7) transcript level in the cell lines and did not change the ratio of FL/Δ7, suggesting that SMN2 transcription was mainly activated. We also found that VPA modulated splicing factor expression: VPA increased the expression of splicing factor 2/alternative splicing factor (SF2/ASF) and decreased the expression of heterogeneous nuclear ribonucleoprotein A1 (hnRNPA1). In conclusion, more than 1mM of VPA activated SMN2 transcription and modulated the expression of splicing factors in our SMA fibroblast cell lines.Brain & development 05/2011; 34(3):213-22. DOI:10.1016/j.braindev.2011.04.010 · 1.54 Impact Factor
Article: A homozygous mutation in UGT1A1 exon 5 may be responsible for persistent hyperbilirubinemia in a Japanese girl with Gilbert's syndrome.[Show abstract] [Hide abstract]
ABSTRACT: The UGT1A1 gene encodes a responsible enzyme, UDP-glucuronosyltransferase1A1, for bilirubin metabolism. Many mutations have already been identified in patients with inherited disorders with hyperbilirubinemia, Crigler-Najjar syndrome and Gilbert's syndrome. In this study, we identified a UGT1A1 mutation in an 8-year-old Japanese girl with persistent hyperbilirubinemia who was clinically diagnosed as having Gilbert's syndrome. For the mutational analysis of UGT1A1, we performed a full sequence analysis of the gene using the patient's DNA. She was homozygous for a T to G transversion at nucleotide position 1456 in UGT1A1 exon 5 (c.1456T>G), leading to the substitution of aspartate for tyrosine at position 486 of the UGT1A1 protein (p.Y486D). In conclusion, the homozygous mutation of UGT1A1 may be responsible for persistent hyperbilirubinemia in this patient.The Kobe journal of medical sciences 01/2011; 57(1):E26-31.
- Nature medicine 01/2011; 17(1):29-30; author reply 30-1. DOI:10.1038/nm0111-29 · 28.05 Impact Factor
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Kōbe, Hyōgo, Japan
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I had a miscarriage 2 months ago. How soon can I start trying to become pregnant again? Do I have to take any special precautions to make sure I don't miscarry again?
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Some doctors recommend that women wait 2 to 3 months or more before trying to get pregnant again to give their bodies time to heal, and so that they are emotionally ready to be pregnant again. Continue to take preconception precautions, like taking folic acid; abstaining from drinking, smoking, or using drugs; and avoiding other substances that might harm a pregnancy.
If you miscarry more than once, you may want to talk with your doctor about medical conditions or other factors that could be putting you at risk for miscarriage.
If you become pregnant again, be sure to maintain a healthy, nutrient-rich diet and limit or avoid caffeine. Also avoid foods that can cause illnesses, such as soft unpasteurized cheeses or undercooked meats.
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Ocean Export Coordinators aren't exactly in the most profitable line of business; these folks earn a belt-tightening average of $41K per year. Residence and career duration each impact pay for this group, with the former having the largest influence. A fair number report receiving medical coverage from their employers and a strong majority collect dental insurance. Most workers in this position report high levels of job satisfaction. Female Ocean Export Coordinators make up most of the survey respondents at 73 percent. The data for this snapshot was collected from individuals who took PayScale's salary survey.
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An ocean export coordinator helps control the flow of products and/or information to clients and customers across international waters; this means shipments headed overseas are ultimately the ocean export coordinator's responsibility. The coordinator is expected to respond to client need, and providing an accurate timeline for delivery. They keep up with changes to import and export laws for every country from which their company ships or which receive items. Ocean export coordinators are also expected to perform regular in-depth audits of all workflow operations.Read More...
Most ocean export coordinators work for shipping companies, and they typically work in a shipping warehouse environment. Since shipping is a 24/7 field, an ocean export coordinator may work any shift based on the needs of the company. This is generally a full-time job.
While most companies have a minimum educational requirement of a high school diploma or equivalent, most prefer a bachelor's degree or equivalent experience in the field. Having a flexible work schedule is also a plus. Ocean export coordinators must be detail-oriented, a team player, and customer-focused. On top of that, being able to communicate effectively in both verbal and written communication is essential.
Survey takers who work as Ocean Export Coordinators report a focused set of job skills. Most notably, skills in Logistics, Oral / Verbal Communication, Customer Service, and Documentation are correlated to pay that is above average.
Median of all compensation (including tips, bonus, and overtime) by years of experience.
Workers in their first five years can expect to earn $38K, but people who have been around for five to 10 years earn a noticeably bigger sum of $42K. Ocean Export Coordinators bring in $44K after working for 10 to 20 years. Survey participants with more than two decades under their belts actually report earning less than people with less experience; the former take home only $41K on average.
|Extremely satisfied
Rated 5 out of 5
based on 48 votes.
73 %
27 %
|Medical: 80%
|Dental: 70%
|Vision: 55%
|None: 16%


text: http://askubuntu.com – I upgraded from 10.04 to 12.04 LTS. Upgrade went fine, even restarted couple of times. Then the next day while booting into Ubuntu, after the grub, it gave the error 'Kernel panic : not syncing vfs unable to mount root fs on unknown block (0,0). I then booted into live CD and tried the following commands, based on other posts on this forum: sudo fdisk -l As the 8 was on /dev/sda1, sudo mount /dev/sda1 /mnt sudo mount --bind /dev /mnt/dev Now I got the message: mount: mount point /mnt/dev does not exist Then tried sudo mount --bind /proc /mnt/proc Again got the message: mount point point /m (HowTos)


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The SETI project scientists are known for tracking possible extraterrestrial signals, but now they are also considering sending messages from Earth telling of our position. A researcher from the University of Cádiz (Spain) questions this idea in view of the results from a survey taken by students, revealing the general level of ignorance about the cosmos and the influence of religion when tackling these matters.
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1995 Oldsmobile Cutlass Ciera Q&A
1995 Oldsmobile Cutlass Ciera Question: How do I get my power windows to work?
My power windows aren't working at all. I checked the circuit breakers: OK. Replaced master power window switch: check. Still no response from the switch. Is there a power window relay? Or could there be some wires disconnected? - oldslady50
Answer 1
on the older models, there is a big rubber boot where the wires from the car go to the door. inside, the wires bend and break over time. cut the boot open and check for broken wires. Roy - mastertech6371
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I´m planning a trip from Argentina to Peru. Will fly Bs As-Lima, and then Lima-Cuzco, to go to Machu Picchu. After that, taking the PeruRail, will go to Puno and titicaca lake. The question is: to return from Puno to Lima, what route should I take? again the same train, or I should take a Bus (I´ve been said that train between Puno Arequipa are not available) to Arequipa traveling a new route, and there fly to Lima? thanks