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Harlequin-type ichthyosis is a genetic disorder that results in thickened skin over nearly the entire body at birth. The skin forms large, diamond/trapezoid/rectangle-shaped plates that are separated by deep cracks. These affect the shape of the eyelids, nose, mouth, and ears and limit movement of the arms and legs. Restricted movement of the chest can lead to breathing difficulties. These plates fall off over several weeks. Other complications can include premature birth, infection, problems with body temperature, and dehydration. The condition is the most severe form of ichthyosis, a group of genetic disorders characterised by scaly skin.
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Joints are sometimes lacking in movement, and may be below the normal size. Hypoplasia is sometimes found in the fingers. Polydactyly has also been found on occasion. In addition, the fish mouth appearance, mouth breathing, and xerostomia place affected individuals at extremely high risk for developing rampant dental decay.
Patients with this condition are extremely sensitive to changes in temperature due to their hard, cracked skin, which prevents normal heat loss. The skin also restricts respiration, which impedes the chest wall from expanding and drawing in enough air. This can lead to hypoventilation and respiratory failure. Patients are often dehydrated, as their plated skin is not well suited to retaining water.
Children who survive the neonatal period usually evolve to a less severe phenotype, resembling a severe congenital ichthyosiform erythroderma. Patients continue to suffer from temperature dysregulation and may have heat and cold intolerance. Patients can also have generalized poor hair growth, scarring alopecia, contractures of digits, arthralgias, failure to thrive, hypothyroidism, and short stature. Some patients develop a rheumatoid factor-positive polyarthritis. Survivors can also develop fish-like scales and retention of a waxy, yellowish material in seborrheic areas, with ear adhered to the scalp.
"On Thursday, April the 5th, 1750, I went to see a most deplorable object of a child, born the night before of one Mary Evans in 'Chas'town. It was surprising to all who beheld it, and I scarcely know how to describe it. The skin was dry and hard and seemed to be cracked in many places, somewhat resembling the scales of a fish. The mouth was large and round and open. It had no external nose, but two holes where the nose should have been. The eyes appeared to be lumps of coagulated blood, turned out, about the bigness of a plum, ghastly to behold. It had no external ears, but holes where the ears should be. The hands and feet appeared to be swollen, were cramped up and felt quite hard. The back part of the head was much open. It made a strange kind of noise, very low, which I cannot describe. It lived about forty-eight hours and was alive when I saw it."
People being swallowed by fish, living inside the fish, then being spit out alive.Talking snakes.Every animal somehow getting on some ridiculous boat for some mythical flood.Burning talking bushes.The earth being only 6k years old.
Just as when he was younger, he was glad for a bench seat in a car. He remembered drive-in movies and the crackle of the speaker attached to the window sill. He kissed her hair and tuned the radio to an AM station. A smooth grooves set.
He was between two large pick-up trucks when he slipped and landed squarely on his hip. He felt something crack. His cheek hit the pavement, and he could see beneath the cars. The rain beat against his face. He coughed and tried to remember the day.
One of hockey's most charming tales involves an epic journey by the Dawson City Nuggets, who travelled nearly 5,000 km by foot, sea and rail from the Yukon to Ottawa for a crack at the Stanley Cup in January, 1905. The Nuggets lost -- badly, as it turned out -- but have come to symbolize a time when the game was young, when amateurs prevailed and played for joy rather than money. There are still places where the sport is new and unspoiled, but it takes an enterprising traveller to find them, as Toronto rock musician-writer Dave Bidini reveals in his sparkling new book, Tropic of Hockey: My Search for the Game in Unlikely Places (McClelland & Stewart, $32.99). Bidini journeyed to Romania, the United Arab Emirates, northern China -- and Hong Kong, where he witnessed Asia's largest annual tournament and the victory lap of the jubilant UAE Nats. "They skated the perimeter of the ice, crouching behind one another in a crazy speeding train," he writes, "15 bearded faces aglow with victory."
Bidini's survey of hockey in exotic locales is full of such spontaneous shows of joy and enthusiasm, which are all too rare at a time when the pro season lasts nearly 10 months and too many teams favour defence over offence. The book is also a refreshing departure from many of the fall season's other hockey offerings -- an uneven mix of life stories and glossy, large-format histories. But there is another title -- Ice Time: A Canadian Hockey Journey (Viking, $32.99) by broadcaster Scott Russell -- that takes the reader to some out-of-the way locales and introduces people, like longtime Edmonton Oiler scout Lorne Davis, with a youthful love of the game. "I'd be a pretty lonely man if I couldn't go to hockey games anymore," says Davis, who is nearing 70. "I get the...