Saturday, March 21, 2009

BEZOAR


A bezoar is a mass of foreign material trapped somewhere in the gastrointestinal system. Typically, these obstructions are found in the stomach where they are formed from food boli, celulose, medications or hair.


There is even a syndrome specifically described for hairy bezoars in the stomach and the small bowel. Rapunzel Syndrome, named after the heroin of the famous fairytale, typicall occurs in psychiatric patients suffering from trichophagia, which is the compulsive need to ingest hair.


In the middle ages, bezoars were considered magical. Bezoar stones where thought to cure any poison, until the theory was tested by a physician on a convicted fellon condemned to death by poisoning. After the prisoner was poisoned, the physician treated him with a bezoar. The fellon died 7 hours later in extreme agony, proving definitively that bezoars could not cure poisoning.


The consumption of under-ripe persimmons is the leading cause of Bezoars in humans.


Due to modern, medical intervention, bezoars are rarely lethal. However, they can be quite disturbing due to their sheer size and foreign appearance.


This sick gets a

1 on my "lethality scale" (1-10)

and a 3 on my "disturbing scale" (1-10)

Friday, March 20, 2009

RAYNAUD'S DISEASE


Named for French doctor Maurice Raynaud, Raynaud's Disease was first described in 1862.

Raynaud's Disease is characterized by vascular anomalies that restrict blood flow to the extremities when the sufferer is exposed to cold temperatures or stress.

The disease is most often diagnosed in women in their teens and early twenties.

There are two types of Raynaud's. When the vascular anomalies are caused by an apparent secondary cause, the ailment is called Raynaud's Phenomenon. When there is no secondary cause for the symptoms it is called Raynaud's Disease, or Primary Renaud's.

Raynaud's is defined by its extreme visible symptoms. There are 3 cyclic color changes that occur in the extremities of Renaud's disease sufferers.

When a Renaud's sufferer is exposed to cold or stress their fingers, toes, nose or ears will often turn white as their blood vessels constrict diminishing blood flow to the extremities. When the vascular constriction is extreme and extended, the extremities will turn blue from hypoxea. Once the extremities are brought back into a warm environment or the stressor is removed, they will often swell and turn bright red.

Primary Renauds is not typically life threatening, though severe cases of the disease can cause gangrene from reduced blood flow and hypoxeia of the extremities. In these severe cases, medication is sometimes used to increase blood flow, and even vascular surgery might be attempted in an affort to get blood flow normalized.

This sick is typically more of an annoyance than a threat but does have some interesting and apparent physical symptoms. So, I give it a
1 on my "lethality scale" (1-10)
and a 2 on my "disturbing scale" (1-10)

Thursday, March 19, 2009

PROTEUS SYNDROME


Named after the sea-god Proteus, Proteus syndrome is an extremely rare (200 cases since the disease was isolated in 1979) hereditary disorder that causes uncontrolled growth of bones, muscles, tissues, skin and lymphatic vessels leading to severe disfigurement as the disease progresses.
Victims of Proteus Syndrome are born with a completely normal appearance but as they grow older growths of skin and bone begin to alter their appearance and cause health complications.
Affected individuals may die early due to complications from extraneous tissue and bone growth. Sometimes, the very mass of the tumors can end their lives. For instance, the most famous Proteus Syndrome case, Joseph Merrick (The Elephant Man), died when the weight of his head broke his neck while he was sleeping.
Scientists are still trying to figure out what causes Proteus Syndrome, but there is some evidence that it is linked to the PTEN gene on either chromosome 10 or chromosome 16.
There is no prescribed treatment, but there has been some success treating the illness with Rapamycin.

Due to the horrible disfigurement this disease causes and the fact that it often leads to early death, I give this sick a
4 on my lethality scale (1-10)
and a 10 on my disturbing scale (1-10)

Wednesday, March 18, 2009

PROGERIA


Progeria is an extremely rare, genetic condition (1 in 4,000,000) which causes exceptionally rapid aging. Children who are born with this ailment usually die by the time they are 13 years old.

Though the disease is genetic, it is not usually inherited. It is a mutation which causes a change to LMNA, a gene on chromosome 1 that is crucial in producing the membrane which surrounds cell walls. It is this change to the cellular membrane that causes the rapid aging seen in progeria patients.

There is no treatment for Progeria. Instead, doctors focus on solving the problems that the disease causes, such as heart disease, through surgery and profilactic measures.

90% of progeria cases die from cardiovascular causes such as stroke or heart attack.

Progeria was first discovered in 1886 by Jonathan Hutchinson. It was also independently documented in 1897 by Hastingls Gilford. The disease's full name is: Hutchinson-Gilford Progeria syndrome (HGPS).

This is a truly devestating illness with 100% mortality over a relatively long period of time.
Its physical manifestations are disturbing and painful to the victim.

So, this sick gets a:
9 on my lethality scale (1-10)
and a 9 on my disturbing scale (1-10)