Aagenaes syndrome

A syndrome characterised by congenital hypoplasia of lymph vessels, which causes lymphedema of the legs and recurrent cholestasis in infancy, and slow progress to hepatic cirrhosis and giant cell hepatitis with fibrosis of the portal tracts.^[1][2]

The genetic cause is unknown, but it is autosomal recessively inherited and the gene is located to chromosome 15q^1,2. A common feature of the condition is a generalised lymphatic anomaly, which may be indicative of the defect being lymphangiogenetic in origin¹. The condition is particularly frequent in southern Norway, where more than half the cases are reported from, but is found in patients in other parts of Europe and the U.S..^[3] It is named after Oystein Aagenaes, a Norwegian paediatrician.

Also called cholestasis-lymphedema syndrome (CLS)

References

1. ^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
2. ^ Bull LN, Roche E, Song EJ, Pedersen J, Knisely AS, van Der Hagen CB, Eiklid K, Aagenaes O, Freimer NB (2000) Mapping of the locus for cholestasis-lymphedema syndrome (Aagenaes syndrome) to a 6.6-cM interval on chromosome 15q. Am J Hum Genet. 2000 Oct;67(4):994-9. Epub 2000 Aug 30.
3. ^ Heiberg A (2001) Aagenaes syndrome: lymphedema and intrahepatic cholestasis. Tidsskr Nor Laegeforen. 2001 May 30;121(14):1718-9.

Best 9 Amazing “Above-Water” Shipwrecks

The world’s oceans abound with shipwrecks and a multitude of other nautical artifacts. Lurking deep beneath the waves of the planet’s most inhospitable waters are some of history’s most grand and feared ships, presenting divers with a fascinating incarnation of a bygone age. But what about those among us who don’t scuba dive? Here are 9 corroding old vessels that can be seen clearly from the relative safety of terra firma.

1.SS Maheno, Fraser Island, Queensland, Australia

Image by NeilsPhotography

Fraser Island off the coast of Queensland in Australia is the largest sand island in the world and a breathtaking place to visit. But its most notable man-made landmark is easily the wreck of the SS Maheno. The Maheno had a varied history: built in Scotland in 1905 as a luxury passenger liner for the trans-Tasman crossings, it served as a hospital ship in the English Channel during before being declared outdated in 1935. That same year, the ship was being towed to Melbourne when a cyclone struck, setting it adrift for several days before it finally became beached on Fraser Island. It has since been used for target practise by the Royal Australian Air Force and is now a popular tourist attraction. More than three storeys of the vessel are reportedly buried beneath the sand.

2.SS American Star, Canary Islands

Image by Wollex

The incredible image above shows the wreck of the SS American Star, built in 1940 for United States Lines and wrecked in January 1994 off Fuerteventura in the Canary Islands. At the time of the accident, the vessel was being towed to Thailand to be used as a floating hotel… More to come on this one later!

3.Chatham Islands, New Zealand

Image by wili hybrid

This rusty old vessel (above) was captured on the archipelago of the Chatham Islands, New Zealand, in September 2007. The islands are named after the ship HMS Chatham of the Vancouver Expedition, captained by William Robert Broughton, who claimed possession of the archipelago for Great Britain in 1791. But it’s fair to say that this old boat doesn’t quite date back to that period in history. Today, the islands have a grand population of 609 people of European and Maori descent, with surroundings that conjure an atmosphere of exploration and adventure harking back to the early settlers.

4.Republic of Vanuatu, South Pacific

Image by Graham Crumb

This ill fated ship ran aground here in the early 1980s when Cyclone Uma lashed the islands of Vanuatu. The volcanic archipelago in the Melanesia region has doubtless seen countless shipwrecks in its time. This one has rested on the reef since the day it foundered, and has since become a popular tourist attraction.

5.The Peter Iredale, Clatsop Spit, Oregon, United States

Image by joannapoe

Jutting out of the sand along the northwest coast of Oregon can be found the remains of the Peter Iredale, which ran aground here in 1906. Built in June 1890 in Maryport, northern England, by R. Ritson & Co., the vessel was attempting to enter the Columbia River when high winds literally drove her into the shore. Thankfully all 27 souls on board, including two stowaways, were saved. The weather scuppered any plans to tow the ship off the beach and it soon became embedded in the sand, where it remains to this day. Rumour has it that the ship’s skipper, Captain Harry Lawrence, waded ashore from the wreck armed with the log book and a bottle of whisky. He passed the whisky around his crew and offered up a final salute to his ship, saying:

6.Point Reyes, California

Image by Orin Zebest

It might not be the most impressive looking wreck of all time, but the photographer has done a nice job of capturing the eponymous Point Reyes fishing boat.

7.The Kakapo, Cape Town

Image by HelgaRainbow

The two remaining propeller blades framing a rusty old boiler is all that remains of the Kakapo, which ran aground on May 26, 1900. On that fateful night, the Kakapo left Cape Town bound for Sydney, reportedly shrouded in “mist and fog”. The captain turned sharp east when he saw what he thought was Cape Point, which would have allowed him to cruise into False Bay. But unbeknown to him, what he had actually seen was Chapman’s Peak, and instead of turning into False Bay he rammed the ship on Noordhoek Beach, where it has been slowly rotting away for the last century. The Cape of Good Hope is known for its treacherous conditions, and was originally called The Cape of Storms. Since 1918, the Slangkop Lighthouse has ensured subsequent captains have not made the same mistake.

8.Lake Ontario, Canada

Image by shane2u

Can anyone fill in the blanks in on this one? Name of vessel? How it came to be washed-up here? What is known is that this amazingly intact shipwreck can be found on the shores of Lake Ontario. And if you think this is cool, the lake has a plethora of wrecks – some of them commanding important places in history.

9.River Princess, Candolim Beach, Goa

Image by Jason Turner

Perhaps more of an eyesore than historic wreck, the vast River Princess lies abandoned on Candolim Beach in Goa after it was washed-up in a storm. The local government, apparently, cannot afford to get rid of the ship, and by all accounts the water is so shallow that people can almost wade out to it – not recommended! One side of the hull has had a gaping hole cut out so that the sand and water that has gushed in will ensure the ship doesn’t move, preventing it drifting into the busy shipping lanes. Eyesore or not, this is one leviathan that could be with us for generations to come…

source:

Belize’s Great Blue Hole

Off the coast of Belize, lies one of the most famous diving spots in the world – the Great Blue Hole, the world’s largest known underwater sinkhole. Surrounded by the Lighthouse Reef atoll, the Great Blue Hole is famous for its rich, blue water, stretches over 300m (984ft) across with a depth of 125m (410ft).

The Great Blue Hole was made famous by Jacques Cousteau in 1971, when the ocean explorer declared it one of the best diving spots in the world. Today, the underwater sinkhole continues to attract divers around the world who flock to see the amazing geological formations of stalactites and stalagmites in the vertical cave. Divers also encounter many species of fish in the crystal clear water including giant groupers, nurse sharks, Caribbean reef sharks and Blacktip sharks.

The Great Blue Hole is a World Heritage Site of the United Nations Educational, Scientific and Cultural Organization.

Underwater Jesus Statues

Worship knows no boundaries – and nowhere is this more evident than with the various underwater Jesus statues – Christ of the Abyss – located around the world.

The underwater Jesus statues, located in John Pennekamp Coral Reef State Park in Florida, USA, the Tyrrhenian Sea near Giglio Island in Italy, and off the coast of St. George in the Caribbean, are popular diving attractions, but also draw people from around the world for underwater worship, marriage ceremonies, burials and annual religious processions.

All three statues depict Christ with his arms raised in blessing.

Best Place Grenada’s Underwater Museum Update

Internationally renowned sculpture and installation artist Jason deCaires Taylor recently sent us some updated images from his Moiliniere Bay underwater museum in Grenada.“After three years beneath the Grenadian waters the latest growth of sponges, tunicates, hydroids, soft and hard corals has been beyond all expectations,” explains deCaires Taylor.Moilinere Bay is now home to sixty-five sculptures, covering an area of 800sq.m (8600sq.ft). It is located two miles (3.2kms) north of the capital St Georges on the west coast of the island, within an area designated as a National Marine Park.Jason deCaires Taylor’s underwater museums are designed to promote love of the ocean and to preserve marine life and increase bio-diversity.

Best 10 Coolest Google Earth Finds

This heart-shaped island in the Adriatic became a hit on Google Earth for Valentine's Day. The uninhabited island is only 130,000 square yards and is called Galesnjak. The owner didn't even know how perfectly this island off the Croatian coast was until he was swamped with requests from couples to stay there.

Dubbed the "Badlands Guardian" by locals, this geological marvel (Google Earth coordinates 50.010083,-110.113006) in Alberta, Canada, bears an uncanny resemblance to a human head wearing a full Native American headdress--and earphones, to boot. Of course, The Guardian was produced naturally.

A synthetic wonder that can be truly appreciated only from above, this giant man-shaped lake (-21.805149,-49.089977) is located near Bauru, Brazil.

This fingerprint can be found in Hove Park, near Brighton and Hove in the UK. It measures 38 metres around. Imagine the size of the hand.

Lion, at ZSL Whipsnade Zoo located at Whipsnade, near Dunstable in Bedfordshire, England.

Some of the sights you find in Google Earth are just plain mysterious. For example, why is a fighter jet parked (Google Earth coordinates 48.825183,2.1985795) in what looks to be a residential neighborhood lot near Paris?

This is a US Navy building in Coronado, Ca. that was unintentionally built in the shape of a swastika. Now, because of a public uproar, the Navy will be spending $600,000 to alter the shape of the building.

In the early days of Firefox, a few devoted fans wanted to generate more PR for their beloved site and so gathered a team of people in Amity, Oregon, to recreate the Firefox logo in a field; their very own fox crop.

Last year, one of the Google Earth Community members called 'ear1grey' posted an amazing discovery. He found a huge picture (36 miles tall) of Santa!

So here's a giant Ipod Shuffle! It looks like a farmer's field with a horse pen or something on it? Either that or a hard core apple enthusiast. Unfortunately, it looks like he moved the pen on the map since it was first spotted.

Google Earth doesn't have advertising, unless you consider the corporate logos and trademarks big enough to be seen from space. Check out this massive Coca-Cola logo (-18.529225,-70.25002) etched into a hillside of Chile with 70,000 Coke bottles. Haven't they heard of recycling?

Best World’s Tallest Statue of JESUS from POLAND

Statue of Christ the King high as 33 meters, has been established in the city Swiebodzin, Poland. With that height, the statue has become the world’s tallest statue of Jesus.

“I was never as happy as today. It is the culmination of my work as pendega,” said Sylwester Zawadzki, 78-year-old priest who created this statue, told local media.

Local City officials say high statue of Jesus was actually reached 51 m, if the foundation and crown of golden statues counted. Thus, they claim the statue in Swiebodzin higher than the statue of Jesus who was in Bolivia and Brazil.

Overall height statue of Cristo de la Concordia in Cochabamba, Bolivia, is 40.4 m is the statue of Christ the Redeemer in Rio de Janeiro, Brazil as high as 38 m.
The cost of building a statue in Swiebodzin comes from private donations worldwide and pastor Zawadzki not willing to mention the cost.

The city government hopes the statue can promote their city and attract tourists from around the world. However some residents are not too enthusiastic to welcome the establishment of the statue was to argue the money would be better used for social projects.