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harlequin ichthyosis
This is something unbelievable, what do you think?
Length: 37
Rating: 4.40 (64 ratings)
Tags: WTF ufo baby monster
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Harlequin Type Ichthyosis: Proof There Is No God
If there was a loving god, why permit such horrors? If I was an all-powerful god I would intervene! There is NO GODLY REASON to bring about a person born with such an affliction. If there was a god and he/she/it permitted such a thing to happen (something I wouldn't if I was a god), then he is a cruel and sadistic being. Of course, there is no god, which is one this was simply a case of a rare disease striking this poor infant. This poor baby suffers from Harlequin Baby Syndrome.
Harlequin-type ichthyosis (also harlequin ichthyosis, ichthyosis congenita, or keratosis diffusa fetalis), a skin disease, is the most severe form of congenital ichthyosis, characterized by a thickening of the keratin layer in fetal human skin. In sufferers of the disease, the skin contains massive, diamond-shaped scales, and tends to give off a reddish color. In addition, the eyes, ears, mouth, and other appendages may be abnormally contracted. The scaly keratin greatly limits the child's movement. Because the skin is cracked where normal skin would fold, it is easily pregnable by bacteria and other contaminants, resulting in serious risk of fatal infection.
Sufferers are known as harlequin fetuses, harlequin babies, or harlequins.
The harlequin-type designation comes from both the baby's apparent facial expression and the diamond-shape of the scales (resembling the costume of Arlecchino), which are caused by severe hyperkeratosis. The disease can be diagnosed in the uterus by way of fetal skin biopsy or by morphologic analysis of amniotic fluid cells obtained by amniocentesis. In addition, doctors can now usually recognize common features of the disease through ultrasound, and follow up with 3D ultrasound can diagnose the condition.
The features of sufferers are deformed facially and cranially. The ears may be very poorly developed or absent entirely, as may the nose. The eyelids are severely everted (ectropion), which leaves the eyes and the area around them very susceptible to trauma. They often bleed upon birth. The lips, pulled by the dry skin, are fixed into a wide grimace (eclabium). Arms, feet, and fingers are almost always deformed in such a way that they cannot bend properly, and may be below the normal size. They present hypoplasia in the fingers; therefore, they cannot grab things properly, or they can barely touch them. Polydactyly, a condition in which one has more than the usual number of toes or fingers, has also been found in these infants.
They are extremely susceptible to changes in temperature due to their armor-like skin, which prevents normal heat loss. This can result in hyperthermia. Their respiration is also restricted by the skin, which impedes the chest wall from expanding and drawing in enough air. This can lead to hyperventilation and respiratory failure. Harlequins are often dehydrated, as their plated skin is not well suited to keeping water in.
Treatment and prognosis
In the past, the disorder was invariably fatal, whether due to dehydration, infection (sepsis), restricted respiration due to the plating, or other related causes. The most common cause of death was systemic infection and sufferers rarely survived for more than a few days. However, there have been improvements in care, most notably the drug Isotrex. Some patients have survived into adolescence and, in very rare cases, lived to adulthood.
A Real Families documentary broadcast on ITV1 in the United Kingdom in 2005 (and later rebroadcast in other countries) showed the lives of two pairs of sisters afflicted with the condition: Lucy and Hannah Betts (ages 18 and 15), and Dana and Lara Bowen (ages 8 and 1½). The girls all went through a daily routine of getting up early in the morning and bathing for two hours to soften the skin, scrubbing hard to remove as much of the hard extra skin as possible, and then covering their entire bodies in a thick layer of moisturiser. A second and third "creaming" as they called it had to be performed in the afternoon and at bedtime to soften the skin. Even with this treatment a thick layer of hyperkeratotic skin covered them and they were plagued with infections. Scales on the inside of the eyelids had blinded one of Lucy's eyes and left the other eye with 10% vision. Hannah Betts also suffers from cerebral palsy. Their skin was said to grow at fourteen times the normal rate. Vacuuming the carpets was necessary at least a couple of times a day to remove the shed skin.[1]. A documentary on Five in 2008 showed the same children three years later after major advances in medical technology.
The chances of any one person with the disease mating with an unaffected person to produce a child who is also a sufferer would be about 1 in 2000.
Length: 34
Rating: 3.80 (5 ratings)
Tags: World Trade Center Attacks WTC 9/11 Proof There is No God Harlequin Baby Syndrome LethalKillingMachine
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Ichthyosis - Harlequin Type Ichthyosis - 日本オーディオ
This is a serious, hereditary skin disease that causes skin to become abnormally thick and cracked, as well as eyes, ears and noses to be stunted.
Because the skin is the body's way of keeping moisture in and bacteria out, people with this disease can become dehydrated very quickly and the risk of infection is also very high. For this reason, babies born with this disease never used to survive the first few hours or days after birth.
The disease can now be managed to some extent by an intensive regimen of bathing and "creaming" - smearing thick, greasy cream all over one's body three or more times a day. In spite of these treatments, few people with this painful, disfiguring disease live into adulthood.
Subtitles: Portuguese
Audio: Japanese
Length: 168
Rating: 4.40 (111 ratings)
Tags: hereditary Harlequin 日本オーディオ Ichthyosis skin corpse disease Autopsy Forensic pathologist doença bebe
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Bebe con Harlequin Ichthyosis
No es que estemos trágicos últimamente y no pongamos posts alegres, por ahí vendrá uno, pero estas son cosas de las que hay que hablar.
Ichthyosis es una familia de condiciones genéticas dermatológicas no muy común pero que se ve en humanos y animales domésticos. La palabra viene del girego ιχθύωσις que significa "pez formándose", ya que quienes sufren de esto tienen una piel escamosa, como un pez. Existen muchos tipos de Ichtyosis y se ve con mayor frecuencia en perros domésticos, especialmente en razas como Golden Retrievers, Bulldogs, Jack Russell Terriers, etc. Se puede diagnosticar simplemente viendo la piel o con la historia de la familia, y una biopsia para confirmar la enfermedad. No es más o menos común en algún tipo específico de etnia, y la exposición al sol puede afectar, tanto positiva como negativamente, y lo más lamentable es, que de momento, no hay forma de prevenirla.
Pero el Harlequin Ichtyiosis, es la más severa de esta familia, es causado por una mutación en el gen ABCA12. Se caracteriza por un engrosamiento de la capa de queratina en la piel fetal humana -hiperqueratosis- por lo que esta se ve como con escamas en forma de diamante, y toma un color rojizo, además los ojos, orejas y boca pueden tomar formas anormales. El alto contenido de queratina limita el movimiento del niño, y como fácilmente se rompe donde debería doblarse, corre un alto riesgo de infecciones fatales.
Length: 33
Rating: 4.00 (178 ratings)
Tags: Niño Harlequin Ichthyosis bebe baby
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infant with harlequin ichthyosis
Child with rare skin desease.
Length: 184
Rating: 5.00 (4 ratings)
Tags: children skin rare infant
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babies with harlequin ichthyosis are not aliens
http://www.gfmer.ch/genetic_diseases_v2/gendis_detail_list.php?cat3=729 for details and proof... Information about it is presented below... Harlequin-type ichthyosis (also harlequin ichthyosis, ichthyosis congenita, or keratosis diffusa fetalis), a skin disease, is the most severe form of congenital ichthyosis, characterized by a thickening of the keratin layer in fetal human skin. In sufferers of the disease, the skin appears as massive, diamond-shaped scales. In addition, the eyes, ears, mouth, and other appendages may be abnormally contracted. The scaly keratin greatly limits the child's movement. Because the skin is cracked where normal skin would fold, it is easily pregnable by bacteria and other contaminants, resulting in serious risk of fatal infection.
Length: 32
Rating: 4.40 (167 ratings)
Tags: baby babies alien aliens harlequin ichthyosis
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Lamellar Ichthyosis / Exfoliative Dermatitis
Lamellar Ichthyosis, Exfoliative Dermatitis
Length: 359
Rating: 0.00 (0 ratings)
Tags: KNEELUPAK
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