Wilson Disease.
Wilson's disease is a rare inherited disorder that causes too much copper to accumulate in your liver, brain and other vital organs. Symptoms typically begin between the ages of 12 and 23.
Copper plays a key role in the development of healthy nerves, bones, collagen and the skin pigment melanin. Normally, copper is absorbed from your food, and any excess is excreted through bile — a substance produced in your liver.
But in people with Wilson's disease, copper isn't eliminated properly and instead accumulates, possibly to a life-threatening level. When diagnosed early, Wilson's disease is treatable, and many people with the disorder live normal lives.
Wilson disease (WD) is a rare, autosomal recessively inherited disorder of copper metabolism. Patients typically present with progressive neurological dysfunction in association with liver disease due to excess copper storage. Early diagnosis of the disease allows for treatment and prevention of permanent organ damage. Guidelines from two medical societies delineate diagnosis strategies.
What are the signs and symptoms of Wilson disease?
Although abnormal copper accumulation is happening from birth, the symptoms of Wilson disease may not become apparent until late childhood or adolescence. Because copper initially accumulates in the liver, the first sign of the disease is usually liver related and manifests as one of the following:Hepatitis (acute or chronic inflammation of the liver)
Cirrhosis (severe liver disease due to a progressive loss of liver function)
Fulminant hepatic failure (sudden and often fatal liver disease)
The most common initial presentation is with cirrhosis, usually occurring during the first 10 years of life. Possible symptoms may include:
Confusion and drowsiness (hepatic encephalopathy)
Bleeding from the intestines due to high pressure in compressed veins within the liver (portal hypertension and oesophageal varices)
Fluid accumulation in the abdomen (ascites) and legs (oedema)
Susceptibility to infections
Excess fluid in the lungs causing breathlessness
Other symptoms that may occur as copper is deposited into other organs include:
Neurological symptoms – usually becoming apparent around the 2nd or 3rd decade of life, but may even occur as late as 50 years of age
Tremor of the head, arms, or legs
Impaired muscle tone causing unpredictable, jerky, repetitive, or slow movements
Slurred or slow speech, excessive saliva, decreased or slow facial expressions (mask-like facies)
Emotional or behavioural changes
Confusion, delirium or dementia
Eye symptoms Kayser-Fleischer rings (coloured rings) formed by deposits of copper in the corneal membrane. Colour of the rings range from greenish gold to brown.
Muscles and joints Stiffness and difficulty moving extremities as joints become involved
Softening and thinning of the bones
Skin changes Yellowish discolouration of skin and eyes (jaundice)
Development of unusually dark skin patches (hypermelanotic pigmentation)
Other signs of liver disease: spider angiomas (broken capillaries) and palmar erythema (red palms)
Blood problems Haemolytic anaemia
Thrombocytopaenia (low levels of platelets) resulting in easy bruising and bleeding because of defective clotting
Kidney disease Symptoms are variable
Kidney or bladder stones may occur
Blood may be seen in the urine (haematuria)
Symptoms of Wilson's Disease
List of symptoms associated with Wilson's disease, listed in alphabetical order with photos when available. This list can help identify the warning signs of Wilson's disease, but if you're concerned for your health you should visit your physician immediately. Many people in the world have been diagnosed with Wilson's disease, and it's certainly nothing to be ashamed about. If you are wondering "Do I have Wilson's disease?" this list may help you get a ballpark idea of whether or not you might have it.
List is made up of many different items, including Muscle weakness and Jaundice.
1-Abdominal pain.
2-Jaundice (yellowing of the skin and eyes).
3-Anemia.
4-Vomiting of blood.
5-Fatigue, lack of appetite or abdominal pain.
6-Jaundice, a yellowing of the skin and the whites of the eye.
7-A tendency to bruise easily.
8-Problems with speech, swallowing or physical coordination.
9-hypoglycemia.
10-kidney dysfunction.
11-jaundice (caused by liver failure)
12-cirrhosis of the liver.
13-memory impairment
14-gait disturbance
15-vision impairment
16-psychosis
17-tremor
18-Abnormal posture of arms and legs
19-Confusion or delirium.
20-Dementia
21-Difficulty moving arms and legs, stiffness
22-Difficulty walking (ataxia)
23-Emotional or behavioral changes
24-Enlargement of the abdomen (abdominal distention)
25-Personality changes
26-Phobias, distress (neuroses)
27-Slow movements
28-Slow or decreased movement and expressions of the face
29-Speech impairment
30-Tremors of the arms or hands
31-Uncontrollable movement
32-Unpredictable and jerky movement
33-Vomiting blood
34-Weakness
35-Yellow skin (jaundice) or yellow color of the white of the eye (icterus).
Prognosis
The prognosis is good in individuals who are treated with copper-binding medicine, a low-copper diet, and vitamin B6 and zinc supplements before liver or brain damage occurs. Such individuals have a normal life expectancy. If therapy is started soon after symptoms appear, treatment can sometimes improve liver and brain function. If untreated, Wilson's disease is generally fatal, usually by the age 30.Treatment.
Cap Wilsogon (1 cap TDS)
Syp Wilsogon (1 Teaspoonful After Every 6 Hours)
Post a Comment