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Whether your kid is homeschooled or they are in community college, they may require extra practice when it arrives to math. So what are some ways that you can reinforce math abilities without luxury car rentals investing a great deal of cash?
Establish at-house guidelines, making research and lsat prep your #1 sports car rental priority, regular family members dinners, limits on cell telephone, pc, and video sport use, and early-to-bed routines.
Make sure that you have all the required paperwork prepared with you such as school information, LSAT scores, letter of recommendations, your resume and so on. you we buy homes have to submit them online at the College Administration Council's web site (wwwdotlsacdotorg/).
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Btw, about the man who took the SAT 20-five occasions? Relax: it wasn't a pupil, but the head of The Princeton Evaluation. And after 25 exams and a lot of bubble sheets, he has some good guidance for SAT students here.
Higher Sats Or Functions - Much More School Cash
Whether your kid is homeschooled or they are in community college, they may require extra practice when it arrives to math. So what are some ways that you can reinforce math abilities without luxury car rentals investing a great deal of cash?
Rawhide Lamp Shades And Antler Chandeliers
Whitetail deer usually begin to drop their racks in December and January depending circumstances. Of program exceptions happen and I have heard of bucks carrying racks till late February.
These hand crafted products are a great way to deliver history and tradition into your southwestern or Indigenous fashion house. Your home decor will be unique with a one-of-a-kind aspiration catcher. Some are usually made the exact same way, but with different bead work and various feathers for decoration. You can even find that some of the Native American style aspiration catchers are made with a natural shed deer antler velvet for a distinctive wall decor design. They are occasionally produced with not only the beads, feather and leather-based, but with a medicine wheel and/or medication bag.
An essential part of knowing how to reduce estrogen is how and what to consume. Steer clear of consuming drinking water in plastic bottles, beer and higher levels of alcohol in general. Drink water using a reverse-osmosis filter rather, which is the proven very best kind of filter.
Since liver is impotant to remodel the yin power to yang energy in the 3rd phrase of menstrual cycle, if liver is damaged or qi deficiency, it will cause the transformation dysfunction, top to yang deficiency and lower BBT in the phrase.
All rooms include a mini fridge, microwave, coffee maker, a personal outdoors balcony, ironing board and iron, hair dryer, and high speed internet access at an extra charge. For Television you have all of the basic local channels and HBO family members. The environment of the rooms tends to make the whole encounter of staying in the Northwoods total with all handcrafted furnishings and log walls. A couple of of the other rooms also consist of fireplaces and whirlpool tubs.
The epidermal growth aspect current in the antler velvet helps in the healthy tissues development. The gastric acid deer antler velvet development decreases with the help of the growth aspect current in antler velvet. They can help in the repairing the damaged vessels restoration.
Antler chandeliers arrive in various styles and they are provided at diverse expenses. They are created from whitetail deer, moose, elk, mule deer, reindeer and caribou. They are regarded as renewable property and there is no damage to the ecosystem. These days, many interior designers like to use these chandeliers to beautify residences since they are in a place to produce "magnificent atmosphere".
Sticks developed for the country walker and for other for outside pursuits like capturing or bird viewing are another much more interesting idea. Thumb sticks and waders are tall employees like sticks than provide an aid to lean on. Many are historically made from Hazel, Ash or Chestnut and can useless to say turn into personalised to suit. A deer antler can certainly include a nice contact and a fantastic talking about stage.
Herbs: Consider Antler And Lower Your Blood Pressure, Remain Rigid And Reside Wholesome
There are fairly a couple of perceptions about how hair on the scalp can grow. People generally hold the mistaken belief that following a routine which is typical among the other people will make their hair look better. However, there are facts, which must be comprehended if you truly want to have luscious hair. Let us look at some of the typical perceptions which individuals hold in their minds.
Today I would like to speak specifically about deer antler velvet chandeliers. The antlers utilized to make these ornamental lighting arrangements can vary within the species of the deer family. Moose horns along with elk and mule deer are common, however the much more exotic the more likely the cost will improve. The white tailed deer is most commonly the subject when it comes to utilizing horns to make a chandelier. The are the most prized game animal in North America and are 1 of the most majestic.
A close buddy of mine grew up on a farm in Iowa and throughout his childhood he labored! In reality, their entire yard was a garden growing create for the twelve member family's meals for the relaxation of the yr. And as I image a dinner table with twelve hungry mouths, my mouth drops to consider what issues would be like only fifty years ago. But lately, I asked my buddy a question.
For events or weddings you experienced two large convention space s that you could lease. Or if you wanted a unique outside party, you could rent the big white tent complete with a stunning wooden floor dance floor. We looked inside the tent and it was absolutely incredible. There had been strings of lights draped from the ceiling, spherical tables coated with best deer antler velvet white desk garments and a long banquet desk along the aspect of the tent. I could just imagine what it would appear like after there were decorations all over the space. It was extremely intimate and romantic in this region, and I think that it would be the ideal location for a wedding ceremony reception.
Also as the snow recedes and you can get near to quite great, even with no snowshoes. If you research ahead of the snow is fully absent you can uncover the deer tracks and the sheds fairly quickly.
Green unicellular algae will sometimes reproduce so rapidly that the drinking water will turn eco-friendly. This is generally called an "algae bloom" and is usually caused by too a lot mild like direct daylight. An algae bloom can be eliminated by filtering with micron cartridges or diatom filters. UV sterilizers can stop the bloom in the initial place. Green water is very helpful in the elevating of daphnia and brine shrimp.
If your not the kind that likes to lookup for antlers, you can usually place an advert in your close by paper and provide you to purchase antler sheds from individuals that enjoy looking for them. If you dwell in a cold climate and get snow, the extremely very best time to lookup for sheds is coming rite up soon. Mice and squirrels are most likely to chew up the sheds if they aren't retrieved beforehand.
Like my buddy, if you are unsure of where to begin in your special rustic area, you can begin with whitetail deer antler lamps. They are some of the most stunning whilst also being some of the least expensive. Antler chandeliers are anti also produced for whitetail antlers. In larger spaces antler chandeliers may have several tiers of massive elk antlers or moose antlers. Remember, it doesn't make a difference if you area is large or little. You can use antler lighting with rawhide lamp shades or include them to your personal rustic lamps to deliver in the really feel of the previous west, and mountain cabin or a southwest adobe ranch.
Learn Forex Forex Buying And Selling On-Line - A Feasible Proposition?
Lets encounter it, if your working a 9 to 5 occupation and find yourself punching numbers or staring at a computer display all day it can get fairly dull. Want to spice up your desk image? Looking for stuff that will be certain to make individuals want to quit and consider discover for a couple of minutes? Try a couple of of the subsequent products that I know will be a strike if you enjoy toys in your workplace area as much as I do.
You can develop on what you know by performing a bit of study on the internet. Make a key phrase lookup for "digital cameras" or what ever your area is, and you will find some internet pages giving info. Frequently the info is just outlined in a point-sensible fashion in bulleted lists. Consider this information, and blend it with your own experience to produce new articles. You can estimate or cite the other articles (with hyperlinks) that you wireless internet service providers discover on the internet if they are especially helpful. In reality this is the authentic concept powering Google's method of PageRank: web pages that are especially good will earn hyperlinks or "votes" from other websites.
Cost: It differs with each store you buy from, but generally $15.95-$19.ninety five. Or you can purchase on-line from any of the numerous retailers presently promoting them.
This indicates that every solitary piece of info your computer gets has its corresponding key in the register. Whilst you delete some of this info, the keys do not always get deleted with them. Also, every thing you do in the computer leaves traces in the register as nicely. Programs, even uninstalled ones, leave a great deal of info in the sign-up as well. These are the issues that cause the build-up of obsolete information in the registry.
There are many people who are caught, who don't see to be able to go ahead in their life. They do not comprehend that the influence of their atmosphere is at work 24 hours a working day every 7 days of their lifetime. The flow of power that surrounds you influences you significantly in either achieving your goals or blocking your dreams. Keep in mind, you are a residing, respiration, energy area and your environment is component of that field. You can alter your life and level of joy by changing the energy around you.
"Smartphones and tablets are growing at a tempo that PCs [laptop or desktop] can't realistically keep up with simply because of device costs and to some extent disposability," said Ryan Reith, program supervisor of Worldwide Mobile Device Trackers study at IDC. Not only are smartphones and tablets more portable than laptops and desktop computers, Reith says the previous are coming down in price as nicely. And by "disposability," he's referring to the reality that consumers are changing their smartphones, in particularl, frequently, thus producing much more device revenue for these goods.
Take candid pictures. Instead of taking posed pictures, attempt taking photos of your subject with family at work or performing something they adore. When your topic is comfy they will be more at simplicity and you will finish up obtaining some distinctive shots. This functions particularly well when trying to photograph children.
Provide a link in between the item and the individuals. You don't have to do the difficult sell, but you do have to enable the people to discover a item that is of interest to them. Once you can do this successfully, then you'll be earning commission and that's why any of us do affiliate advertising. Certain it's great to community, but at the finish of the day, we all want to get paid for our difficult work. The merchant is the individual who will do that promoting for you by supplying a well place with each other revenue or landing web page. Don't place any pressure on your connections, just introduce them to the item and they can determine if it's for them.
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Ive been playing guitar for more than 20 five many years. Many people inquire me to teach them and I explain there is a much simpler way to learn. Use the power of the computer to learn to play guitar. Discover what you want to discover and at your personal tempo. If you have some extra time, you can teach more, and discover to play fairly nicely in a quantity of weeks instead of many years.
CLUTTER, Clutter, GO Away! Don't come back again another working day. Clearing clutter is the initial step to opening your bedroom to the flow of new energy. Keep the clutter in the closets, if you must. Keep it out of sight! Clutter stops concentrate and can cause melancholy. Another little tip is to maintain the garbage cans hidden.
Filters can also have an affect on the water system in your fridge. purifyed water is a lot much less most likely to clog the lines in the drinking water method. This is a good reason to maintain the filters thoroughly clean in your refrigerator, whether or not you treatment about consuming filtered water or not.
This means that each internet service solitary piece of info your pc gets has its corresponding key in the sign-up. While you delete some of this information, the keys do not necessarily get deleted with them. Also, everything you do in the computer leaves traces in the register as nicely. Applications, even uninstalled types, leave a lot of information in the register too. These are the issues that trigger the develop-up of out of date info in the registry.
Now, following getting their personal IP, your laptops can be linked to every other. You have to go to the taskbar and find "Wireless Network Connection" symbol. Correct-click on and select "Change the purchase of preferred networks" in the appeared window.
Compared to smartphones and tablets, notebook and desktop computers are so "been there, done that" that shoppers select the new gadgets. Personally, though, while I no longer have a desktop computer, there are some tasks that my tablet can't do that my laptop can. Just to give you one instance, if I want to edit a publish I just produced on my Fb web page, I can't do it on my Apple iPad; I require to switch over to my MacBook Air.
Easter eggs: Each kid and even some adults adore portray Easter eggs. You can buy the real eggs in some of the oriental supermarkets or you can purchase the plastic eggs in the greenback stores. You can use yellow crayons and draw pictures on the eggs and even print messages on each egg with the yellow crayon. Once you do that then dye the egg in any color. The name and image that you drew on the egg will be crystal distinct and will display up on the egg completely.
It's a trade off but that's the character of something to do with computer systems. Obtaining like some flash drive safety in place is the very best first stage you can take - you can good tune it later.
Nfl Football: 2006 Nfc East Preview
Inpart two of our NBA week two critique, we'll look at four NFL games. These activities: Texans at Colts, Saints 'll be reviewed by me at Eagles and Raiders at Ravens at Packers, Leaders. The Giants Horned at Eagles could be the week from this group's matchup. Let us take a peek.
it had divided some 8 weeks ago although I badly wanted to observe the nfl games last year. After looking to get the client assistance of the TV to address the situation for just two weeks, I dumped it altogether and quit. The soccer games started in a months period and that I was getting quite desperate to get yourself a solution to view them.
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Because my friend demonstrated not so unsuccessful at nfl football, I'd him take a look at College basketball plus it worked as well. Why control it to only 5 weeks annually, next we tried NBA Basketball, with this particular form of accomplishment? Well, do you know what? An NBA method that introduced us as much money while the NBA program was found by us! Like hitting on the lottery it was. TWICE!
One idea for betting on basketball games is, do not guess all on your own group. The reason being you might have a solid bias and never be thinking logically. You can easily discover games that you just haven't any emotional interest in, because you'll find a large number of games over a weekend.
To get a very long time the Horned Frogs trainer that is best was Dutch Meyer. Meyer acquired 109 activities for TCU as well as their only agreement National Championship. But Gary Patterson is creating a solid disagreement. Gary Patterson may be the existing TCU coach and it has an 80 - 27 record. In a couple of more times H Patterson might have a winning percent that is greater and more wins in a smaller timeframe. Meyer comes with the merely opinion of TCU National Title nonetheless it is difficult to fault Patterson for college football today not knowing small conferences as competitors.
The favorite fantasy football league structure is known as a Survivor Group. Betting within this form of structure can also be a gambling cashcow that is good. The Survivor League is not unpopular as the idea of this structure is simple. You simply have to choose one NBA staff to acquire the motion for every week. Nevertheless, you cannot employ the exact same team twice in a single year. It's entertaining to play, than it seems nonetheless itis tougher.
There's a fairly remarkable quantity of luncheon specials in Colorado, with restaurant , grill or just about any club offering some offer on other weekend favorites and also toast. Regardless of options' selection, I find myself spending manya Saturday at the Nightclub on Pearl for one basic cause: $2 breakfast burritos.
How fast could Bill Parcells switch around things in Miami? - 2 yrs. That is usually each time a Bill Parcells crew will recovery. Bill might not have acquired a playoff game in 9 years, but he definitely has made around his share of basketball clubs throughout his profession. He'll gut this Miami Dolphins team this off season and commence finding a couple of fresh players about the subject in 2008, while blending in experts that could lead tutor the young folks. Thatis what he did in Houston together with the Cowboys, and thatis what he did in Nyc together with the Jets. It'll be no unique in Arkansas, merely this time he'll be about the sidelines in place of in the dog owner's box.
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The NBA Core Ice may be the great deal for any hockey fan. Presenting you use of as much as 40 various nfl games every week, you'll not be unable to follow your favorite groups from everywhere in the united kingdom. In addition, you can choose to listen from either group to discourse. Instead of just playing commentary in the house team, you can pick which teamis criticism always feel like your team may be the house team and you intend to pay attention to.
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Because it has definite playoff implications for that Browns, this can be a big game for me personally. I'm not planning to get fed up with declaring "playoffs" and "Browns" inside the same sentence. I selected at Colorado last week but I had been with how which great they looked quite surprised. Denver will not be exactly the same crew ontheroad with nevertheless. I'm not deeply in love with Houston's fashion but I do at home like them.
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My boy and North Park L.T. Are finally turning on it and it is about time. This week my push to the imagination title begins! I should be the only person in the united kingdom who's still surprised at the total collapse of the Tigers. These are two squads that are on two very different roads now of the growing season.
Cal State Northridge placed a finish to the Cougars' season having a 3-1 success (30-26, 30-24, 27-30, 30-27) within the beginning round of the Pile Pacific Sports Federation Event in Northridge, Calif.
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Over the many years, my spouse has discovered the sight of me dragging out the suitcase is his cue to split. Throughout the ordeal that takes a couple of hrs, I attempt on should a selection of clothes to see which I like much better and what goes with what. All sorts of tops, shorts, slacks, attire and shoes spew from my closet into and on to each corner of the bed room. It's not a pretty sight!
Get to your dentist glasses pronto. It's the basic response to most other dental emergencies, from a tooth that's partially broken off to 1 that's pushed in or pulled out.


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Good Morning Everyone,
It has been a while since my last blog, so I guess I better get with the program and catch you all up with my life.
I have ALL of my Christmas presents bought, wrapped and mailed off.
That is a very good thing, because I really fall prey to all the pretty trappings in the stores at this time of year.
It seems I can't even go grocery shopping without looking for seasonal wonders that I don't need but am tempted to buy anyway.
Yesterday I went to my small Applique group's Christmas party. We had a wonderful time.
Irene showed us all how to make these little pincushions and then hot glueing them to golf tees so they fit on top of a spool of thread. Really cute little things.
I, being the klutz of the club, had a heck of a time with them. I kept looping my threads and making my petals in a line rather than a circle, but my darling friend Irene saved me from myself and got them all where they were supposed to be. She got the leaf petals in a circle, and then I did the flower petals.
Again, I didn't do it "right", but Irene, used to bailing me out, saved the day again and got them all in a circle. ( I had managed to get them into TWO lines this time! Don't ask.)
We had a potluck for our lunch. Everyone brought yummy stuff, most of which they made.
I HAD planned on making a Trifle, using a store-bought angel food cake, but can you believe it? None of the grocers had any Angel Food Cakes, so that idea went by the wayside. I have a wonderful Trifle recipe from Weight Watchers, so that would have worked out well.
At least I had the good intention!!
I, being the lazy one, decided I didn't have time to cook anything, called Subway and ordered a variety sandwich platter. That went over very well. Expensive, but was worth not having to take the time to cook anything.
I even had a small piece of apple pie Loretta made, but didn't eat the crust.
There were even two sandwiches left so I could feed the bear......otherwise known as my DH.
I am a slow eater, and Irene finished before me. So, being the kind and generous friend she is, she finished off my pincushion.
Our applique group is very talented. Actually all of THEM are very talented when it comes to applique, but I am the Klutz of the group. They don't seem to mind, and at least I bring smiles and challenges to the group. I guess that is a good thing.
Did I tell you that I have all my Christmas gifts completed, wrapped and sent on their way?
That isn't because I am so organized. It is a life preservation measure. I really fall victim to all the seasonal trappings the stores have on display.
I am better off staying out of the stores during this time of year. I will buy "for next year", and come home with all next year's gifts, hide them somewhere and then don't find them for years.
Can you relate?
I have found doll houses and doll house furniture 7 years after I purchased it for my granddaughter for Christmas. She was too old for doll houses by the time I found it again.
I have given away small children's socks and clothing because I couldn't find them until the kid was already grown and driving.
I found a set of Lincoln Logs and a Beginner's microscope purchased for my Grandson when he was younger. But I found them when he was in high school.
I have found crystal vases meant for weddings years after the couple divorced. I have found completed hand made afghans made for people I haven't seen in years.
I have boxes and boxes of "Oh, won't he or she just LOVE this!", found in very weird places and years after purchase.
I have uncovered purchases and then wondered "Now why in hell did I ever buy THIS thing!?".
When I moved from CA to WA, I loaded up my trusty F250 pick-up truck with seven loads of new stuff, all purchased between November 20-January 5 (must take advantage of the after Christmas sales, you know!) and taken the loads to the Cancer Discovery Shop.
I have three artificial Christmas trees. Two of which were bought during the after-Christmas sale days. Good prices that should not be ignored, right?
Good Will shudders when they see me this time of year. They know I will be off-loading on them a whole bunch of Christmas stuff that they are already inundated with.
The Post Office sees me coming and know they will get their Christmas bonuses due to all the mailing of packages I do.
Ah, Christmas. The most wonderful time of year. Right?
Only if I stay the heck out of the stores!!!!!
Merry Christmas to all, and to all, wishes you can manage to stay within your budget, or at least find all the gifts you have hidden from past years!
This is certianly a busy time of year, isn't it!
That's why I call it the "holidaze". We all have so much decorating, baking, cooking, cleaning, schedules to keep, gifts to get all in order and wrapped....you know the drill.
I will have you all know that I am FINISHED with my Christmas shopping, and have sent all the packages out. I'm glad to strike THAT off my list!
But I still have a long list of Ally Cat Quilts by Krackers requests to fill, which I would very much like to complete by Christmas. I know the kids would love to have a nice cuddly quilt for Christmas, and between me and God, we are trying our best to do it.
Honestly, at my age, I can't for the life of me figure out why I do so much decorating!! I mean, once we become Senior Citizens, don't we get a decorating pass?
Mine has not arrived.
Therefore, I drag out all the boxes of too many decorations, get the step stool and ladder, wash the windows, put up a tree, get the Christmas towels in the bathrooms, hang wreaths, Cinnamon spray pinecones for the basket, fill stockings and all that other STUFF!
Once it all gets up, I love it. I turn on Christmas music, light candles and really enjoy life to the fullest with the beauty of the Season.
It's funny, though. I spend weeks getting everything done, and then on the day after New Year's, (typically January 2) can't wait to get them down and return my home to "normal".
"Normal" to me means cobwebs become Dream Catchers, dust becomes slates for guests to leave their messages, dirty floors are proof that I get outside. Piles of ironing prove I have an abundance of clothes for which I should be grateful.
But for now, I am much much too busy to sit and eat cookies!!
I am thankful we don't have to wait in long lines for shoes in our country.
I am grateful for our military.
I am grateful for our border patrols.
I am grateful for weather reports.
I am grateful for charity organizations, and the ability to volunteer to help.
I am grateful for an over abundant pantry that I can clean out and take good healthy food to the food bank.
I am grateful for the spontaneous energy of our Teen-agers.
I am grateful for artists that beautify our world.
I am grateful for the beauty of the changing seasons.
Isn't is wonderful that we live in a country that sets aside a full day for gratitude and Thanks giving!
Despite all the problems we are experiencing with our government, the economy, the riots that have emmanated from the sit ins, unemployment, we can still find reasons for giving thanks.
We can give thanks for our beautiful country, where we can travel anywhere in it without being checked at state borders by armed guards.
We have the right to vote and speak freely of our own opinions without the fear of reprisal.
We have the freedom to worship, and the right to read whatever we choose.
We (still) have the right to medical treatment with a doctor of our choice.
We have the right to attend schools and further our education.
No one can demand we work in a particular field, like many other countries do.
We have the right to bear arms, and the right to be heard in our courts.
We can have wonderful friendships with those of different faiths than our own without the worry of reprisal.
Oh, indeed, beautiful for spacious skies, for amber waves of grain.
For purple mountains, majesty, among the fruited plains!
America, AMERICA! God shed His grace on Thee!
And bound Thy GOOD with BROTHERHOOD,
from sea to shining sea!!
Christmas/Holidays 2011 -- Birth of a New Tradition
A dear friend sent this to me, and I am sure most of you will love it like I do.
Great Idea!
Hi folks. This seems like a very good idea to me.
Christmas/Holidays 2011 -- Birth of a New Tradition
As the holidays approach, the giant Asian factories are kicking into high gear
to provide North Americans with monstrous piles of cheaply produced goods --
merchandise that has been produced at the expense of North American labor. This
year will be different. This year we can give the gift of genuine concern for
our countrymen. There is no longer an excuse that, at gift giving time, nothing
can be found that is produced by North American hands. Yes there is!
It's time to think outside the box, people. Who says a gift needs to fit in a
shirt box, wrapped in Chinese produced wrapping paper?
Everyone -- yes EVERYONE gets their hair cut. How about gift certificates from
your local hair salon or barber?
Gym membership? It's appropriate for all ages who are thinking about some health
Who wouldn't appreciate getting their car detailed? Small, privately owned
detail shops and car washes would love to sell you a gift certificate or a book
of gift certificates.
Are you one of those extravagant givers who think nothing of plunking down your
dollars on a Chinese made flat-screen? Perhaps that grateful gift receiver would
like his driveway sealed, or lawn mowed for the summer, or driveway plowed all
winter, or games at the local golf course.
There are a bazillion owner-run restaurants -- all offering gift certificates.
And, if your intended isn't the fancy eatery sort, what
about a half dozen breakfasts at the local breakfast joint. Remember, folks this
isn't about big National chains -- this is about supporting your home town
friends and neighbors with their financial lives on the line to keep their
doors open.
How many people couldn't use an oil change for their car, truck or motorcycle,
done at a shop run by the neighborhood working guy?
Thinking about a heartfelt gift for mom? Mom would LOVE the services of a local
cleaning lady for a day.
My computer could use a tune-up, and I KNOW I can find some young guy who is
struggling to get his repair business up and running.
OK, you were looking for something more personal. Local crafts people spin their
own wool and knit them into scarves. They make jewelry, and pottery and
beautiful wooden boxes.
Plan your holiday outings at local, owner operated restaurants and leave your
server a nice tip. And, how about going out to see a play or ballet at your
hometown theater.
Musicians need love too, so find a venue showcasing local bands.
Honestly, people, do you REALLY need to buy another ten thousand Chinese lights
for the house? When you buy a five dollar string of light, about fifty cents
stays in the community. If you have those kinds of bucks to burn, leave the
mailman, hair stylist or babysitter a nice BIG tip.
You see, Christmas is no longer about draining North American pockets so that
China can build another glittering city. Christmas is now about caring about US,
encouraging local small businesses to keep plugging away to follow their dreams.
And, when we care about other our neighbors, we care about our communities, and
the benefits come back to us in ways we couldn't imagine.
THIS is the new North American Christmas tradition.
Forward this to everyone on your mailing list -- post it to discussion groups --
throw up a post on Craigslist in the Rants and Raves section in your city --
send it to the editor of your local paper and radio stations, and TV news
departments. This is a revolution of caring about each other, and isn't that
what Christmas is about?

subset: commoncrawl


text:
Dock your iPod, iPhone or iPad in the black iPod, iPhone & iPad Multimedia Entertainment Docking Station from Pyle Audio and incorporate your 30-pin iDevices into your home entertainment setup. This docking station has a bevy of connections that allow you to connect your device to speakers and a TV or other display to stream your media. When it's docked, your device will even be charging to avoid a dead battery. You can watch videos, listen to music and sync up your device with your computer. The included wireless remote control lets you command your entertainment from a comfortable distance.
- Compatibility
- Works with iPod, iPhone and iPad with a 30-pin connector
- A/V Connections
- Stereo audio out
- Component video out
- USB host/device control
- HDMI out
- Mini USB port
- Standard USB port
- Charge As You Play
- Charges your device whenever it's docked to avoid a dead battery
- Convenience
- Wireless remote control included


text:
Live Music Fridays @ Indulgence
Bring Back The Rock
Please Support South African Music.
If you are looking for artists for an event, to attend an event, perform at an event or even just be a part of our South African Rock Revolution, you can find it all at Hit Music. Contact us now


text:
Eid al-Adha preparation in Iran
Similar imagesView all
sacrificial animal is being carried in a trunk of a car on October... News Photo 184685111Animal,Car,Eid al-Adha,Horizontal,Iran,Religion,Tehran,TrunkPhotographer Anadolu AgencyCollection: Anadolu Agency 2013 Anadolu AgencyTEHRAN, IRAN - OCTOBER 15 : A sacrificial animal is being carried in a trunk of a car on October 15,2013 in Tehran, Iran. Eid al-Adha, also called Feast of the Sacrifice will be celebrated by Iranian Muslims on October 15,2013 in Iran. (Photo Fatemah Bahrami/Anadolu Agency/Getty Images)


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text:
Defcon 2012 - an inside look
Summary: From Defcon 2012, probably the biggest hacker conferences of the year, we'll take you on a tour of some of the more interesting sights at this year's show. You'll see hacker ware, fake iPads, mysterious machines, schwag and much more.
25 of 29Image
The back room behind the expo had interesting table centerpieces.
Defcon's Lockpick Village was a hands-on learning environment and always busy. It also was where the male-female gender ratio neared 50-50.
Lockpick Village staff showing how to escape handcuffs in seconds with a simple shim.
Kick off your day with ZDNet's daily email newsletter. It's the freshest tech news and opinion, served hot. Get it.


text:
DC's Dirty Dozen (Ragnar - Washington D.C.) by NKSIII - about 2 years ago
"This is the second time we have ordered shirts from CustomInk. The staff have always been responsive and helpful. In particular, the graphic artists have been exceptional in ensuring that the our d..." view full details
Let's Go Crazy! by Prince Concert (Guitar15 designed by Debbie) - about 2 years ago
"I absolutely love this T-shirt. Debbie helped me design it. Since I could not use any copyrighted material, we had to be very creative. The shirt has an image of a hot pink guitar with a crown on ..." view full details
Yep - Even the Dog! by TEAM CSI - Light The Night for LLS - about 2 years ago
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Team Raechel 2012 by Jake Verterano - about 2 years ago
"This is from the Mayor's Annual 5k to "Break the Silence on Ovarian Cancer" in West Orange, NJ. We walked in memory of my mother, Raechel Verterano, who passed away in 2010 from ovarian cancer. The..." view full details
Amaretto Slushie Scrapbookers by MamaErin - about 2 years ago
"This was our annual retreat 2011. Our group of friends meet every year to scrapbook and have a few days of pure bliss! This year was in Brevard NC - also known as White Squirrel. We took an afterno..." view full details
Big or Small, Save Them All (Collage) by Gina - about 2 years ago
"Breast cancer pub crawl - a 8 hour crawl all in the name of breast cancer - it was organized by the breast cancer coalition of Rochester, and I decided my group of friends needs to stand out by mak..." view full details
Mudley Crew is ready for NorCal Tough Mudder! by mcdev - about 2 years ago
"Mudley Crew at Northern California Tough Mudder in Lake Tahoe. We completed the 12-mile course together for our own personal achievement as well as for the Wounded Warrior Project. Yes, everyone go..." view full details
Kickin' it for Nick by Angie - about 2 years ago
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Team Bride-Bachelorette at the Finger Lakes! by reebach - about 2 years ago
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Team Thunder Buddies- Ragnar Relay, Napa Valley by Kasia - about 2 years ago
"My friends and I have been running this overnight relay race for 2.5 years now. Starting from the Golden Gate Bridge, we run through the night (and through wine country) until we arrive at Calistog..." view full details


text:
Hill Makes Pitch to Get GOP Endorsement for Congressional Run
Members of the Ocean County Republican organization will be deciding from a strong field of potential 3rd District Congressional Candidates during their convention next week.
Members of the Ocean County Republican organization will be deciding from a strong field of potential 3rd District Congressional Candidates during their convention next week.
Gov. Chris Christie's 112th town hall meeting started with a focus on Superstorm Sandy and the second round of federal aid headed to New Jersey later this year, but much of the question-and-answer segment addressed issues unrelated to the October 2012 disaster.
Trenton Mayor Tony Mack has been convicted of bribery, fraud and extortion.
U.S. Rep. Rob Andrews will leave Congress in two weeks after 23 years in office.
Gov. Chris Christie said Monday night that his office is cooperating with a federal subpoena from the U.S. Attorney investigating Bridgegate while it conducts its own internal probe.
New Jersey Sen. Robert Menendez's political campaign paid a law firm $250,000 in December for legal costs related to Justice Department and Senate Ethics Committee investigations into his ties with a major donor. The Democrat also has set up a legal trust to raise money as the investigations continue.
Donald Trump made a trip to New Hampshire on Tuesday fueling speculation that he is interested in a presidential run in 2016 and says he "feels badly" for Chris Christie (R) about Bridgegate.
Gov. Chris Christie is still being dogged by the Hoboken and George Washington Bridge scandals, but most people wouldn't have known that by listening to his inaugural address on Tuesday.
Gov. Chris Christie today took the oath of office for a second term and delivered his inaugural address at the War Memorial in Trenton.
The first big test of how Bridgegate is playing on the national political stage comes this weekend in Florida for Gov. Chris Christie.
Speaker John Boehner (R-Ohio) says the House won't take up legislation aimed at neutering a 2012 overhaul of the federal flood insurance program.


text:
Great rocky landscape and shot, beautiful dynamic colors and view, Like 42, best wishes from Germany, Rainer
Singular shapes in a rocky mountains, great frame. F Best regards.
Impressive! I like it a lot! have a nice weekend Leo. Hanka
Incredible view, it is excellent!
With best wishes from Latvia,
Photo taken in Bryce Canyon National Park, Hwy 63, Bryce Canyon, UT 84764, USA


text:
News Details
Alonso attributes Q3 deficit to power loss
Scuderia Ferrari Fernando Alonso says he was unable to set a competitive time during the final segment of qualifying due to a loss of power in his Ferrari F14T. Alonso was the slowest of the Q3 runners but will start from ninth on the grid due to Daniel Ricciardo's 10 place grid penalty. "We tried our best as usual today and now we need to wait for the car to come back from parc ferme to work out why there was a drop in power in Q3," he said. "In winter testing here we had already seen that this circuit is particularly suited to the Mercedes powered cars, because the long straights allow them to use their top end performance. "But given how free practice and the first...
Full Story »
Full Story »
7mo ago from F1Zone.net 0
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Abu Dhabi GP: Post Race press conference
36m ago from Pitpass.com


text:
lightgrid, St-Germain, gridwork, 7-Ray, Violet Ray, net-of-light
TELOS-Underground City of Mt. Shasta.doc
Views: 432
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Posted by Paul Lange on November 26, 2014 at 12:02am
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My morning meditation started very nicely. It was foggy and misty outside, with a constant Northwest drizzle falling. It was actually quite nice and offered a magical backdrop as the trees disappeared into the mist.
As I was relaxing and…
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I've been in recovery mode following an ancestral healing workshop Raven Many Voices…
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More clearing work for me it seems. Another disturbing dream last night.
It started with pretty normal dreamtime stuff. I had moved into a new…
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A failure, that's how I felt waking up from a nightmare last night. The 'F' could stand for fear as well.
In the dream I was having a phone…
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July 3rd-4th 2014 and March 20th-21st 2015:
ANCHORING the GOLDEN LIGHT FREQUENCY of CHRIST LOVE and CONSCIOUSNESS!
SUN and Central Sun Connection
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How to Write an Item Listener
by, 04-26-2012 at 09:32 PM (909 Views)
Components fire the item events which are involved in implementation of the interface ItemSelectable. ItemSelectable components usually maintain off and on state for the items. Swing components involved in firing items include certain buttons, for example, check menu items, combo boxes, check boxes and toggle buttons etc.
Code for the item event handling that has been taken by ComponentEventDemo.java.
Java Code://where initialization occurs checkbox.addItemListener(this); ... public void itemStateChanged(ItemEvent e) { if (e.getStateChange() == ItemEvent.SELECTED) { label.setVisible(true); ... } else { label.setVisible(false); } }


text:
Thought I'd take a little "upper level" approach to the theme for 'Sunday Stills' today with "fire in the sky."
Just one more week to the Christmas writing challenge!
Originally posted on Merry Christmas, World!:
The fun is about to start. Next Saturday, we begin the Christmas story writing challenge here on "Merry Christmas, World!" I'll be hosting a three-week challenge, in which everyone who wants to participate can write a Christmas story — or poem for each of the first three weeks in December.
Stories can be any length — up to 800 words. And once published on your own site, you can post the link to your stories here on "Merry Christmas,World!" I'll put a story up each Saturday, and then other participants can post their stories any time throughout the week up to the next Saturday — when I will post another story, and we'll keep going until December 20.
Don't post anything yet. I just want you to have a heads up. Get your thinking caps on. Sharpen up your imagination. You're free to take your story in any direction, as…
View original 43 more words
This week's WordPress Photo Challenge is "Angular."
This little Christmas bird is here to let you all know that my Christmas Blog, "Merry Christmas, World!" will be hosting a writing challenge the first three weeks in December.The title of the challenge will be "A Stocking Full of Stories."
I'll re-blog the challenge on this site, but all the links to stories will have to be posted onto the Christmas site. I just wanted to give everyone a little advanced notice, and next Saturday, I will post the actual challenge. Stories can be any length — up to 800 words, but they do not have to be long at all. If you can write one in 25 words, go for it.
The first week's story can be written anywhere between November 29 and December 6. The second week's story can be written and posted any time up through December 13, and the last week's story can be posted up through December 20. That will bring us to Christmas week, and I figured most people would be too busy to write a new story that week.
Visit the Christmas blog anytime, but please do not post links to stories before I put the challenge up on the 29th of November. I wouldn't want any of them to get lost in the preliminaries and not get read. Just put on your thinking caps and get ready.
Watch for this picture, and you'll know the challenge has begun:
Share Your World this week has us traveling. Visit Cee's photography blog to learn how to participate in the fun.
Question # 1: On vacation, what would you require in any place that you sleep?
Scrupulously clean bed linens and scrupulously clean bathroom. (And they are extremely hard to find anywhere.)
Question # 2: Music or silence while working?
When I'm cleaning house, washing dishes, or fixing things, I like to listen to music. But when I'm writing or preparing a lesson, I have to have ABSOLUTE quiet.
Question # 3: If you were to move, and your home came fully furnished with everything you ever wanted, list at least three things from your old house that you wish to retain.
Well, I'm going to assume that we would all take things like our personal photos, books, cooking utensils, computers, etc. So I'll refer mainly to furniture items. I wouldn't move without taking the maple secretariat that was my mothers, the book case my father made me, the fold-down oak table that was my great-aunts, a chair that has been in my family since I was six years old, and the child's padded rocking chair that my parents bought for me when I was 2 years old. None of those things are worth any significant amount monetarily, but they are priceless to me.
Question # 4: What's your least favorite mode of transportation?
Flying. I don't have any particular fear concerning plane crashes, but I do hate to go through all the lengthy, aggravating processes of security, and, most of all, I hate the idea that I am imprisoned with a crowd of strangers in a rather cramped, artificial environment, and I have absolutely no say in when I can get off.
I realize that on a train or a bus, I have no real say either, but there's some subconscious sense that, as long as I'm on the ground, I could manage to get someone to stop if I really had to. Ships don't bother me as much, because on a ship, I can get outside and walk around on deck and feel I have more control.
Whenever I tell people how I feel about plane travel, they assume I must have claustrophobia, but I don't have any particular problem with being in small enclosed places in general. Elevators don't bother me. Neither do telephone booths. When all is said and done, I think it's primarily a control thing. I don't really like traveling in any vehicle where I cannot control when we start, stop, get in, or get out.
Bonus Question: What are you grateful for from last week, and what are you looking forward to in the week coming up?
I'm very grateful that I finally sat down and studied the tutorials for YouTube and Vimeo and learned how to make the videos and post them to those two networks. I'm even more grateful that I was able to learn how in a relatively short time.
This week I am looking forward to all the delightful anticipation of celebrating Thanksgiving and preparing for Christmas — including getting my lights up on my house and putting up my little tree.
The Daily Post issued another 10-minute free-write challenge this morning. So I decided, since I didn't have anything else to do — except edit a novel for a budding author, grade 7 stories for Saturday's writing class, and wash a cabinet full of dishes that had been sitting and waiting for my kitchen drain to get unplugged — I'd sit here and write. (I'm also a procrastinator.) I glanced over at a ceramic nose on a pedestal — a friend's gift that is to be used as a repository for my glasses. All at once I heard the first line of this story running through my head. I let my imagination take flight, and 10 minutes later here's what I ended up with:
"Holy Cow! I've never seen a nose that big on anybody!"
"Exactly. Nobody wants a nose this big. And nobody wants their nose to feel this big either. But that's what happens when sinus pressure builds up, congests the head and all the breathing passages, and virtually closes off the nose. Then it starts to feel big and bulbous and like some kind of foreign appendage that's been stuck on where it doesn't belong."
"I don't remember feeling like my nose was some 'foreign appendage' when I had a cold."
"Well, you wouldn't. You live on a plane above everyone else."
"Now, what's that supposed to mean?"
"Just what I said. You think you live on a higher level than everyone else, so, naturally, you wouldn't relate to the description. But most people will relate, and this bulbous nose, with its accompanying voice is going to sell 'D-GEST' decongestant to millions of people."
"What kind of voice?"
"It sounds exactly like a stuffed up little kid. Just enough to get attention and garner sympathy from every adult who's watching the commercial."
" I still don't think it's a winner, and Mr. Sanders is not going to like it either when you present it at the meeting today."
"Wanna bet?"
"How much?"
"Let's say if Sanders likes it, you have to buy me dinner Saturday night. And if he doesn't like it, I'll buy you dinner."
"Okay, it's a deal."
(Three days later.)
"Hi, I just called to say I'll be by to pick you up for dinner about 6:00 if that's okay. Since Sanders loved my idea and I'm choosing the restaurant, I thought we'd do Longshore Seafood."
(Cough, cough) "I'm sorry, Ben." (Cough) "I can't go tonight." (Cough, cough).
"Oh, wow! You sound all stuffed up. In fact you sound terrible. What's going on?"
"I have this horrible cold, and my nose feels like it weighs a ton! I wish I could cut it off."
"Hmmmm. See … I told you that's how it feels. Tell you what. I'll be right over with a box of D-GEST. It's just become the hottest thing on the market, you know."
Well, this week, I finally took the plunge to learn how to set up a YouTube account and channel — and then start posting videos. I began with the audio of healing scriptures read by my father. Of course, I had to add still pictures and convert them into what YouTube calls a "movie." It took a while, but I finally jumped through all the hoops successfully.
Unfortunately, on my old operating system and browser, some of the YouTube videos sound scratchy, and this one does as well. However, on my laptop, with a newer operating system and browser, it sounds great. So if any of you do hop over and listen to it, let me know what you found as far as sound quality.
Thanks a lot. I'm believing the Lord to use it to minister to many people.
Choose the one you like best.
Check out the WP Daily Post site for instructions on how to participate.
Can't resist this little tidbit of Christmas cheer from my Christmas blog. Thanksgiving, will all its delights, is just around the corner in the U. S., and then it's full speed ahead into the joy of Christmas. Hope you enjoy this little appetizer with video from 1950.
Originally posted on Merry Christmas, World!:
"White Christmas," by Irving Berlin has been one of America's favorite Christmas songs since the day in 1950 when Bing Crosby introduced it in a movie entitled "Holiday Inn." The song became such an instant success that a short time afterward, Hollywood made an entire movie with the title "White Christmas," and the song became the finale of the show.
Personally, this song holds special value for me, because it was one of my mother's favorite Christmas songs, and she sang it frequently. In fact, not very long after it became a sensation, she sat with me on our living room couch and sang it over and over for me, to teach me the words and the tune. I've loved it ever since that year when I was six. I adored my mother, and everything that was important to her was important to me. This song will always be a…
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I just want to say at the outset that my writing this particular story is Doug Macilroy's fault. If you read his contribution to this week's Friday Fictioneers, you will find a charming, romantic piece that melts your heart. How he got romance out of this picture I could not begin to fathom, but by the time I had tried to figure it out, I was caught in the web of romance and could not get lose. However, not being possessed of the imaginative insight that Doug has, I could not seem to get free of the subject of cars, so my love story is of a slightly different caliber than his. Nevertheless, love — as they say — is where you find it — and an old, abandoned car lot is as good a place as any.
Our picture this week is courtesy of Jean L. Hayes. (Sorry, no link available). My story is below the photo.
Revvin' Up The Love
"Hey, Babe, wanna take a spin?"
"You talkin' to me?" Ethel Edsel looked across the lot at the ugly yellow Edsel with the bulldozer nose. What was that contraption anyway?
"Yeah. You wanna go for a run?"
"You wouldn't want to run with someone nicknamed Rusty Ethel."
"They call me Bull, and it's what's inside that counts. Take me: sort of ugly outside, but inside I got a heart a' gold. And I bet your little engine still purrs like a kitten."
Ethel giggled. Bull grinned.
"Come on. Rev 'er up and let's make it a date."


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June 1, 2008
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Potential conflict of interest: Nothing to report.
Inherited disorders of cholestasis in adulthood
Article first published online: 30 OCT 2013
DOI: 10.1002/cld.245
© 2013 by the American Association for the Study of Liver Diseases
Clinical Liver Disease
Special Issue: Liver Disease in Children
Volume 2, Issue 5, pages 200–203, October 2013
Additional Information
How to Cite
Bass, L. M. and Kamath, B. M. (2013), Inherited disorders of cholestasis in adulthood. Clinical Liver Disease, 2: 200–203. doi: 10.1002/cld.245
Publication History
- Issue published online: 30 OCT 2013
- Article first published online: 30 OCT 2013
- Abstract
- Article
- References
- Cited By
Watch a video presentation of this article
Watch the interview with the author
adenosine triphosphate–binding cassette
Alagille syndrome
bile salt export pump
familial intrahepatic cholestasis 1
hepatocellular carcinoma
multidrug resistance 3
progressive familial intrahepatic cholestasis.
With improvements in medical and surgical care, more children with inherited cholestatic disorders are expected to survive into adulthood and require care by adult hepatologists. In addition to their ongoing hepatic needs, new management issues, including pregnancy, disease transmission to offspring, and extrahepatic manifestations of their disease, arise in adulthood. For the purposes of this review, the discussion is limited to Alagille syndrome (ALGS) and progressive familial intrahepatic cholestasis (PFIC) as the hallmark and most frequently inherited disorders of cholestasis.
ALGS is an autosomal dominant condition involving the liver, heart, face, eyes, skeleton, kidneys, and vasculature[1] (Fig. 1). The disease gene is JAGGED1 in 94% of patients and NOTCH2 in <1%, and clinical molecular testing is widely available for the former. JAGGED1 and NOTCH2 function as a ligand and a receptor, respectively, in the evolutionarily conserved Notch signaling pathway. Highly variable expressivity is a hallmark feature of ALGS.
Hepatic Disease: Implications for Adulthood
ALGS liver disease ranges from asymptomatic biochemical abnormalities to profound, unremitting cholestasis with severe pruritus and xanthomas and end-stage liver disease. The hyperlipidemia of ALGS is not thought to be associated with an increased risk of cardiovascular disease because the lipoprotein profile is characterized by an increase in lipoprotein X, which is not considered atherogenic.[2] ALGS children with liver disease transitioning into adulthood will likely already have a declared hepatic phenotype. Those with mild liver involvement (biochemical or minimal cholestasis) virtually never have disease progression outside childhood (in the absence of another insult such as alcohol use). ALGS children with significant cholestasis who do not experience the spontaneous improvement of early childhood seen in many children may continue to progress and require liver transplantation. A small subset of individuals with ALGS have progressive fibrosis and develop cirrhosis in late childhood or adulthood with minimal cholestasis. There is no new onset of liver disease in patients with ALGS in adulthood.
Hepatocellular carcinoma (HCC) has been reported in patients with ALGS, although predominantly in isolated case reports. Approximately half the reported cases have involved adults, and it is interesting to note that they have occurred in the absence of cirrhosis.[3] This, of course, complicates the selection of appropriate candidates for surveillance, and there is no consensus regarding this, although an annual ultrasound examination may be considered appropriate.
Liver transplantation is required for 15% to 20% of individuals with ALGS, with most cases occurring in childhood because of complications of cholestasis. The outcomes of liver transplantation for ALGS are largely excellent, although they are slightly impaired in comparison with the outcomes for biliary atresia.[4] This difference in patient and graft outcomes seems to be attributable to the multisystem disease burden of ALGS, and renal complications are particularly frequent and require modifications to immunosuppressive regimens and increased vigilance. Similar findings have been described for ALGS adults who have undergone liver transplantation.[5]
Extrahepatic Disease in Adulthood
The liver and cardiac disease in ALGS typically has a stable or predictable phenotype by the time of adulthood (Table 1). There is no new onset of cardiac or hepatic disease after childhood. However, this is not the case for renal or vascular involvement. Although many renal anomalies in ALGS are structural and present from birth, other anomalies manifest with age, and there are substantial reports of hypertension and renal dysfunction with onset in adulthood.[6] Similarly, though of more concern, there are data supporting the notion of a widespread vasculopathy in ALGS, with stroke and cerebrovascular anomalies occurring at any age, even in individuals who previously had normal intracranial imaging.[7, 8] These data, although not validated in large studies, support surveillance imaging with magnetic resonance angiography in adults and certainly aggressive investigations in the event of a neurological symptom or sign.
|Known liver disease from childhood
|Annual ultrasound and laboratory work as indicated by severity of hepatic involvement
|No known liver disease in childhood
|No surveillance required
|Known cardiac anomaly
|Management as indicated by severity of congenital anomaly
|No known or significant cardiac involvement
|No surveillance required
|Annual urinalysis, blood pressure, laboratory work
|Head magnetic resonance imaging/ magnetic resonance angiography every 5 years and before any major medical intervention
Pregnancy and Disease Transmission
Pregnancy in ALGS is a frequent event because 40% of cases are inherited and mothers are, therefore, commonly affected. Complications arise as a result of the severity of cardiac or hepatic involvement in the mother. As an autosomal dominant condition, the risk of mutation transmission is 50% with each pregnancy. Prenatal and even pre-implantation diagnosis is possible; however, because there are no clear genotype-phenotype correlations in ALGS, the prenatal detection of a mutation provides little clinical prediction.[9]
The 50% risk of disease transmission for ALGS is also relevant for other family members. It is important to consider genetic screening for first-degree relatives, even if they have only subtle features of ALGS, because they retain the same risk of disease transmission.
Outcomes and Mortality
Approximately 70% of ALGS children with liver involvement reach adulthood, although these data are derived from patient series from hepatology centers, and there are likely more JAGGED1-positive adults than appreciated.[10, 11] Mortality from ALGS in childhood can largely be predicted by the severity of cardiac involvement. Causes of death in large series of ALGS patients that include young adults are complex congenital heart disease, intracranial bleeding, and factors related to hepatic disease or transplantation.
PFIC is a heterogeneous group of disorders encompassing familial intrahepatic cholestasis 1 (FIC1; ATP8B1) and bile salt export pump (BSEP) deficiency [adenosine triphosphate–binding cassette B11 (ABCB11)], low–gamma-glutamyltransferase (GGT) inherited cholestatic conditions, and multidrug resistance 3 (MDR3) deficiency (ABCB4), which results in high-GGT cholestasis and other clinical scenarios. ATP8B1 and ABCB11 are autosomal recessive conditions caused by mutations in ATP8B1 and ABCB11, respectively. Mutations are detected in approximately 60% of clinically defined patients.
Children with ATP8B1 and ABCB11 deficiencies manifest with profound cholestasis and pruritus. Patients with BSEP deficiency have aggressive hepatobiliary disease and develop HCC at a high frequency. ATP8B1 disease is characterized by extrahepatic manifestations in addition to cholestasis such as failure to thrive, short stature, diarrhea, pancreatic disease, and sensorineural hearing loss.[12] ABCB4 deficiency is associated with a wide phenotypic spectrum ranging from neonatal cholestasis to cirrhosis in young adults (Table 2).
|Familial intrahepatic cholestasis 1
|Bile salt export protein deficiency
|Multi-drug resistance 3 deficiency
|Gene defect
|Extrahepatic features
|Diarrhea, Hearing loss, Pancreatitis, Pruritus
|Gallstones, Pruritus
|Gallstones, Pruritus
|Risk of HCC
|Recurrence of antibody-mediated disease after transplant
Hepatic Disease: Implications for Adulthood
Low-GGT PFIC patients typically progress to end-stage liver disease before adulthood, and before reaching adulthood, almost all patients will undergo either liver transplantation or a surgical intervention via a biliary diversion procedure.
Biliary diversion can relieve pruritus and slow disease progression in patients without cirrhosis.[13] Biliary stoma losses can contribute to severe dehydration in gastroenteritis. Transplantation should be considered for patients who have progressive liver dysfunction or continue to have symptoms of pruritus, cholestasis, liver cirrhosis, and growth retardation despite external biliary diversion. Persistent diarrhea, graft steatosis, growth failure, and pancreatitis have all been observed after liver transplantation in children with ATP8B1 disease.[14, 15] Patients with ABCB11 disease have demonstrated good graft survival with a rapid increase in growth acceleration after liver transplantation. Notably, the recurrence of low-GGT cholestasis associated with the presence of anti-BSEP antibodies in patients with ABCB11 deficiency who have undergone liver transplantation has been described.[16] This appears to be an immune-mediated recognition of the new hepatic antigen (ABCB11 protein) in the donor organ by the transplant recipient. The remission of these episodes may be achieved by the intensification of the immunosuppressive regimen.[17]
HCC has been identified in many children with severe ABCB11 disease, with the largest study demonstrating malignancy in 15% of patients.[18, 19] HCC has been identified even in patients with biochemical and symptomatic improvements after biliary diversion, and patients should be screened with ultrasonography at regular intervals according to American Association for the Study of Liver Diseases guidelines.[20]
MDR3 defects have been reported in children and adults with intrahepatic lithiasis, in adults with chronic hepatitis, biliary cirrhosis, and fibrosing cholestasis, and in patients with drug-induced liver injuries. There appears to be an association between heterozygosity for ABCB4 defects and a propensity for the development of cholestasis of pregnancy.[21] Gallstone patients with ABCB4 mutations may have low-phospholipid–associated cholelithiasis syndrome, which is characterized by cholesterol gallstones and intrahepatic microlithiasis, along with recurrent biliary symptoms despite cholecystectomy.
Extrahepatic Disease in Adulthood
The extrahepatic features of ATP8B1 disease have been mentioned previously. All PFIC patients, regardless of whether they have undergone biliary diversion or transplantation, also have significant metabolic bone disease when they reach adulthood. All patients should have close follow-up and monitoring for osteopenia and osteoporosis.
In addition to HCC, other malignancies in patients with ABCB11 disease, including cholangiocarcinoma and pancreatic adenocarcinoma, have been described.[22, 23] As such, clinicians should consider the increased risk of cancer when they consider surveillance for patients with ABCB11 disease.
Pregnancy and Disease Transmission
PFIC patients have had successful pregnancies after biliary diversion, although all have had significant intrahepatic cholestasis of pregnancy (Peter F. Whitington, M.D., personal communication). A prenatal diagnosis is possible for PFIC as long as the mutations have been identified in the proband and the heterozygosity of the parents has been confirmed.
- 1Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia): review of 80 cases. J Pediatr 1987;110:195–200., , , , , .
- 2Evaluation of risk for atherosclerosis in Alagille syndrome and progressive familial intrahepatic cholestasis: two congenital cholestatic diseases with different lipoprotein metabolisms. J Pediatr 2005;146:329–335., , , , , , et al.
- 3Management of large hepatocellular carcinoma in adult patients with Alagille syndrome: a case report and review of literature. Dig Dis Sci 2010;55:3052–3058., , , , , .
- 4for Studies of Pediatric Liver Transplantation. Outcomes of liver transplantation for patients with Alagille syndrome: the Studies of Pediatric Liver Transplantation experience. Liver Transpl 2012;18:940–948., , , , , , et al.;
- 5Orthotopic liver transplantation for adults with Alagille syndrome. Clin Transplant 2012;26:E94–E100., , , , , , et al.
- 6Renal anomalies in Alagille syndrome: a disease-defining feature. Am J Med Genet A 2012;158A:85–89., , , , , , et al.
- 7Intracranial vascular abnormalities in patients with Alagille syndrome. J Pediatr Gastroenterol Nutr 2005;41:99–107., , , , , , et al.
- 8Vascular anomalies in Alagille syndrome: a significant cause of morbidity and mortality. Circulation 2004;109:1354–1358., , , , , , et al.
- 9Prenatal molecular diagnosis of inherited cholestatic diseases. J Pediatr Gastroenterol Nutr 2007;44:453–458., , , , , , et al.
- 10Features of Alagille syndrome in 92 patients: frequency and relation to prognosis. Hepatology 1999;29:822–829., , , , , .
- 11Outcome of liver disease in children with Alagille syndrome: a study of 163 patients. Gut 2001;49:431–435., , , .
- 12Differences in presentation and progression between severe FIC1 and BSEP deficiencies. J Hepatol 2010;53:170–178., , , , , , et al.
- 13Follow-up in children with progressive familial intrahepatic cholestasis after partial external biliary diversion. J Pediatr Gastroenterol Nutr 2010;51:494–499., , , , , .
- 14Progressive familial intrahepatic cholestasis type 1 and extrahepatic features: no catch-up of stature growth, exacerbation of diarrhea, and appearance of liver steatosis after liver transplantation. J Hepatol 2003;39:447–452., , , , , , et al.
- 15Progressive familial intrahepatic cholestasis: a single-center experience of living-donor liver transplantation during two decades in Japan. Clin Transplant 2011;25:776–785., , , , , , et al.
- 16De novo bile salt transporter antibodies as a possible cause of recurrent graft failure after liver transplantation: a novel mechanism of cholestasis. Hepatology 2009;50:510–517., , , , , .
- 17Recurrence of bile salt export pump deficiency after liver transplantation. N Engl J Med 2009;361:1359–1367., , , , , , et al.
- 18Severe bile salt export pump deficiency: 82 different ABCB11 mutations in 109 families. Gastroenterology 2008;134:1203–1214., , , , , , et al.
- 19Hepatocellular carcinoma in ten children under five years of age with bile salt export pump deficiency. Hepatology 2006;44:478–486., , , , , , et al.
- 20for AASLD Practice Guidelines Committee. Screening for hepatocellular carcinoma: the rationale for the American Association for the Study of Liver Diseases recommendations. Hepatology 2012;56:793–796., , , ;
- 21Intrahepatic cholestasis of pregnancy: the severe form is associated with common variants of the hepatobiliary phospholipid transporter ABCB4 gene. Gut 2007;56:265–270., , , , , , et al.
- 22Mutations in bile salt export pump (ABCB11) in two children with progressive familial intrahepatic cholestasis and cholangiocarcinoma. J Pediatr 2007;150:556–559., , , , , .
- 23Pancreatic adenocarcinoma in type 2 progressive familial intrahepatic cholestasis. BMC Gastroenterol 2010;10:30., , , , .


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Enhanced Dimensions presents a 48 Hour Film Project Film - The Collection (3D).
Hanging around with the collection obsessed Professor Kit Young isn't quite what research assistant Julian Lamb expected.
The Collection (3D) is a short horror film from Enhanced Dimensions produced as part of the 2012 leg of The 48 Hour Film Project. Written, filmed and edited over the weekend of 18th -20th of May 2012 this is the first 3D entry to the 48 Hour Film Project outside of the US.
The film premiered at the Cameo Picturehouse in Edinburgh on 22nd May in glorious anaglyph 3D and was included in the 48 Hour Film Project 5th year "Best of" DVD!
The cast include the brilliant Nick Cheales as the obsessive collector Professor Kit Young and an equally strong performance from Frasier Fulton as his young research assistant Julian Lamb. Nick and Fraser are scheduled to work together again later in 2012. The crew were again dragged in front of the cameras as extra's including Dale Peet as the rather creepy "Pigboy", Rhiannon Grist's muddy feet and Andrew Murchie's pierced bleeding brow!
The score is provided by Neil Warden who again managed to deliver a brilliantly suitable soundtrack enhancing the film's menacing mood.
For more details visit enhanced-dimensions.com/wordpress/?page_id=4157
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