Wilson’s Disease

Wilson’s disease is an autosomal recessive disorder caused by mutations in the ATP7B gene. Wilson’s disease causes copper to accumulate in your liver, brain and other vital organs.

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 excess is excreted through a substance produced in your liver (bile).

But in people with Wilson’s disease, copper isn’t eliminated properly and instead accumulates, possibly to a life-threatening level.

Causes

INSECTICIDE,PESTICIDE,RODENTICIDE,WEEDICIDE  IN AGRICULTURAL FARMING

1.ORGANOPHOSPHORUS  POISON(OP POISON)-Malathoin, Tetron, Parathion,Chlorthoin,Diazion(Tik 20)

2.ORGANO CHLORINES-DDT,Aldrin,Endrin,Endosulfan,Benzene Hexa Chloride

3.PARAQUAT(WEEDOL)

4.FLOURIDES(SODIUM FLOURIDES)

5.ZINC PHOSPHIDE

6.ALUMINUM PHOSPHIDE

PATHOGENESIS

ATP7B protein deficiency impairs biliary copper excretion, resulting in positive copper balance, hepatic copper accumulation, and copper toxicity from oxidant damage. Excess hepatic copper is initially bound to metallothionein; liver damage begins as this storage capacity is exceeded, sometimes by 3 years of age.

Serum copper levels are usually lower than normal because of low blood levels of ceruloplasmin, which normally binds >90% of serum copper.

CLINICAL PRESENTATION

 Hepatic Features -Hepatitis, Cirrhosis, Or Hepatic Decompensation

Neurologic Features The neurologic manifestations of Wilson’s disease reveal damage in the basal ganglia and occasionally in the pons,medulla, thalamus, cerebellum, and subcortical areas. The three main movement disorders include dystonia, incoordination, and tremor. Dysarthria and dysphagia are common.

Psychiatric Features– Loss Of Emotional Control (Temper Tantrums, Crying Bouts), Depression, Hyperactivity, Or Loss Of Sexual Inhibition.

Other Manifestations– Abortions, Amenorrheic ,Cholelithiasis ,Nephrolithiasis, Osteoarthritis Knee, Microscopic Hematuria, . Sunflower cataracts and Kayser-Fleischer rings (copper deposits in the outer rim of the cornea) may be seen.

Prevention

• Avoid chemical pesticides, chemical fertilizer for farming
• Avoid herbicides
• Avoid weedicides
• Avoid rodenticide
• Eat organic fruits, vegetable
• Regular exercise
• Avoid smoking
• Eating foods high in fiber
• Drinking an adequate amount of fluids
• Avoid alcohol
• Avoid tobacco

DIAGNOSIS

Blood- CBC,Serum ceruloplasmin levels ,COPPER

KIDNEY FUNCTION TEST

LIVER FUNCTION TEST

EYE-Kayser-Fleischer ring

Ct brain

LIVER BIOPSY

TREATMENT

HOMEOPATHY MEDICINE