Changes: Wilson's disease

Revision as of 15:58, 23 January 2020

Wilson's Disease is a rare genetic disorder that causes copper poisoning in the body caused by the inability of the body to properly dispose copper from the bloodstream.

In a healthy body, the liver filters out excess copper and disposes of it in bile into the digestive tract releasing it through urine. With Wilson’s disease, the liver cannot remove the extra copper properly. The extra copper then builds up in organs such as the liver, brain, and eyes eventually poisoning them.

One of the only definitive signs of the disease is a Kaiser-Fleischer ring a copper colored ring around the cornea in the eye. As such, many cases of Wilson's are discovered during routine eye exams.

If Kaiser-Fleischer rings are not present samples of the patients hair and nails must be tested with acetone and will glow blue if any traces of copper are found in the keratin at the base of the cuticle. This test must be done if eye exams come back negative for Kaiser-Fleischer rings.

If Kaiser-Fleischer Rings are found or if the patient has cataracts surgery is the only option for treatment to remove it and they will have to get a liver transplant but only if they have an extreme case.

Chelation therapy will remove excess copper from the body, but patients cannot be cured and must thereafter avoid foods that are rich in copper, such as liver, shellfish and nuts.

See also Haemochromatosis, a similar disease where the body cannot dispose of iron.

Wilson's disease at Wikipedia

Wilson's disease at Mayo Clinic

@@ Line 6: / Line 6: @@
 |symptom=[[Jaundice]], [[tremors]], [[spasm|spasms]], difficulty speaking, drooling, [[personality]] changes
 |mortalityrate=Inevitably fatal if untreated
-|treatment=Low [[copper]] diet, [[chelation therapy]]
+|treatment=Low [[copper]] diet, [[chelation therapy]], [[surgery]] if [[Kayser-Fleischer ring|Kayser-Fleischer rings]] or [[cataracts]] found, [[liver]] [[transplant]] if it is an extreme case
 |appearances=*[[The Socratic Method]]
 *[[Remorse]]
 }}
-'''Wilson's Disease''' is a rare genetic disorder caused by the inability of the body to properly dispose of copper from the bloodstream. In normal people, the [[liver]] filters excess copper from the body and disposes of it in [[bile]] into the [[digestive tract]]. However, in patients with Wilson's, excess copper accumulates in the body's organs, particularly the liver, eventually poisoning them.
+'''Wilson's Disease '''is a rare genetic disorder that causes '''copper poisoning''' in the body caused by the inability of the body to properly dispose copper from the bloodstream.
+In a healthy body, the [[liver]] filters out excess copper and disposes of it in [[bile]] into the [[digestive tract]]  releasing it through [[urine]]. With Wilson’s disease, the liver cannot remove the extra copper properly. The extra copper then builds up in organs such as the liver, brain, and eyes eventually poisoning them.
 One of the only definitive signs of the disease is a [[Kaiser-Fleischer rings|Kaiser-Fleischer ring]] a copper colored ring around the [[cornea]] in the [[eye]]. As such, many cases of Wilson's are discovered during routine eye exams.
+If Kaiser-Fleischer rings are not present samples of the patients hair and nails must be tested with [[acetone]] and will glow blue if any traces of copper are found in the [[keratin]] at the base of the [[cuticle]]. This test must be done if eye exams come back negative for Kaiser-Fleischer rings.
-Chelation therapy will remove excess copper from the body, but patients cannot be cured and must thereafter avoid foods that are rich in copper, such as liver, shellfish and nuts.
+If Kaiser-Fleischer Rings are found or if the patient has [[cataracts]] [[surgery]] is the only option for treatment to remove it and they will have to get a [[liver]] [[transplant]] but only if they have an extreme case.
+[[Chelation therapy]] will remove excess copper from the body, but patients cannot be cured and must thereafter avoid foods that are rich in copper, such as liver, shellfish and nuts.
 See also [[Haemochromatosis]], a similar disease where the body cannot dispose of [[iron]].