DIETARY MODIFICATIONS AND DIETARY

GUIDELINES FOR WILSON DISEASE

(Hepatolenticular degeneration)

APPLIED CLINICAL NUTRITION

PRESENTED BY – DILSHAD TASNIM
 CONTENTS
1. INTRODUCTION OF WILSON DISEASE
2. PATHOPHYSIOLOGY
3. GENETICS
4. SYMPTOMS
5. DIAGNOSIS
6. TREATMENT
7. DIETARY MODIFICATIONS
8. DIETARY GUIDELINES
 RDA
 FOOD SOURCES
9. REFERENCES
 INTRODUCTION
 Wilson's disease is an inherited condition in which copper is not excreted
properly from the body.
 Excess copper can build up in the liver and/or brain causing liver
damage and/or neurological problems.
 It can also collect in other parts of the body including the eyes and the
kidneys.
 Wilson's disease is caused by a mutation in the Wilson disease
protein (ATP7B) gene.
 This protein transports excess copper into bile, where it is excreted in
waste products.
 The condition is autosomal recessive; for people to be affected, they
must inherit a mutated copy of the gene from both parents.
PATHOPHYSIOLOG
Y
Healthy Person
Diagram II: Wilson's disease patients before treatment:
reduced excretion and retention of copper
GENETICS
 SYMPTOMS
Symptoms usually begin between ages 5 and 35 but can begin anytime from
age 2 to 72.

In almost half of affected people, the first symptoms result from brain
damage. They include
 Tremors
 difficulty speaking and swallowing
 drooling
 incoordination
 involuntary jerky movements (chorea)
 personality changes, and even psychosis (such as schizophrenia or
manic-depressive illness).
 the first symptoms result from liver damages due to inflammation
(hepatitis) and eventually scarring (cirrhosis).

 Gold or greenish gold rings (Kayser-Fleischer rings) may appear around Kayser-Fleischer Ring
the irises (the colored part of the eye). These rings develop when copper Kayser-Fleischer rings (indicated by the
accumulates. In a few people, these rings are the first sign of Wilson black arrow) are gold or greenish gold
disease. rings around the cornea. These rings
develop when copper accumulates in
the eye. In a few people, these rings are
People may have fatigue and weakness due to decreased numbers of red
the first sign of Wilson disease.
blood cells (anemia) because red blood cells rupture (causing hemolytic
anemia). The urine may contain blood. Women may have no menstrual
periods or have repeated miscarriages.
Home
 DIAGNOSIS
Listed below are the standard laboratory tests used to diagnose Wilson's
disease:
• Urine copper is high; this should be measured in a 24 hour urine
collection.
• "Caeruloplasmin", a copper-containing protein in blood plasma is usually
low.
• The copper concentration measured in a liver biopsy specimen will be
high.
• The cerebral imaging (MRI) may be abnormal.
• In cases which are difficult to diagnose, copper isotope studies (more
complex copper tests) may be performed.
 TREATMENT
• Dietary changes
• Drugs and zinc supplements
• Possibly liver transplantation

People with Wilson disease must follow a diet that is low

in copper. Foods to avoid include beef liver, cashews,
black-eyed peas, vegetable juice, shellfish, mushrooms,
and cocoa. People with this disease should not take any
vitamin or mineral supplement that contains copper.
 What Is the Significance of Zinc in treatment of Wilson
Disease ?
1. The primary mechanism through which zinc helps in Wilson's disease is by inducing metallothionein, a protein that
binds to copper in the intestinal cells.
This binding prevents the absorption of copper from the food in the digestive tract, reducing the overall copper
levels in the body. Essentially, zinc competes with copper for absorption, and by increasing zinc levels, the
absorption of copper is diminished.

2. Patients with Wilson's disease often have a defective transporter protein (ATP7B) that is responsible for eliminating
excess copper from the body. Zinc, when taken orally as a zinc salt, such as zinc acetate or zinc sulfate, helps
stimulate the production of metallothionein, which then binds to copper and prevents its absorption. This results in a
gradual decrease in copper levels in the body and prevents further copper accumulation in organs, especially the
liver and brain.

3. Zinc therapy is typically used as maintenance treatment in Wilson's disease after the initial phase of chelation
therapy, which aims to rapidly reduce copper levels. Chelation therapy involves the use of medications like
penicillamine or trientine to remove excess copper from the body. Once initial copper levels are under control, long-
term maintenance with zinc is often recommended to prevent copper re-accumulation.
 DIETARY MODIFICATIONS
1. Low Copper Diet:
 The most crucial dietary modification for Wilson's disease is reducing dietary copper intake.
Since copper is primarily absorbed through the gastrointestinal tract, a low copper diet can
help control copper levels in the body.
 Avoid or limit high-copper foods such as organ meats (liver, kidneys), shellfish, mushrooms,
nuts, chocolate, and dried fruits.
 Choose lean meats and poultry as they generally contain lower levels of copper.
 Be cautious with food cooked in copper cookware, as this can leach copper into your food.
2. Limit Dairy Products:
 Dairy products like milk, cheese, and yogurt can contain varying levels of copper. It's
essential to monitor your intake of these foods and choose low-copper alternatives when
available.
3. Fruits and Vegetables:
 Most fruits and vegetables are generally low in copper. However, spinach, sweet potatoes,
and avocados have slightly higher copper content, so it's advisable to consume them in
moderation.
4. Grains and Cereals:
 Grains, cereals, and bread are typically low in copper and can be included in your diet
without concern. Just be cautious of any fortification with copper.
5. Beverages:
 Limit the consumption of tap water if it has high copper content. It's advisable to use
filtered water or water from sources with low copper levels.
6. Supplements:
 Avoid multivitamin or mineral supplements that contain copper. Consult with a healthcare
professional before taking any supplements.
7. Alcohol:
 Alcohol can worsen liver damage and impair copper metabolism. It's best to avoid or limit
alcohol consumption as per your doctor's recommendations.
8. Maintain a Balanced Diet:
 While reducing dietary copper is essential, it's also crucial to maintain a balanced and
nutritionally adequate diet. Focus on obtaining necessary nutrients, such as proteins,
carbohydrates, fats, vitamins, and minerals, from foods with low copper content.
 DIETARY GUIDELINES
• Copper Restriction: The most critical
aspect of the diet for Wilson's disease is
restricting dietary copper intake. The RDA
for copper in adults is typically around 900
micrograms (0.9 milligrams) per day.
However, for individuals with Wilson's
disease, it is crucial to significantly reduce
copper intake. The exact amount may vary
depending on the severity of the condition
and the individual's specific needs. Some
guidelines suggest aiming for less than 1 mg
of dietary copper per day. This is achieved
by avoiding high-copper foods, such as
organ meats, shellfish, nuts, chocolate, and
mushrooms.
• Zinc Supplementation: To help lower copper
absorption in the digestive system, many
individuals with Wilson's disease are advised
to take zinc supplements. Zinc competes with
copper for absorption in the intestines. The
RDA for zinc in adults is typically around 11
milligrams for men and 8 milligrams for
women. However, those with Wilson's
disease may require higher zinc doses, often
in the range of 150 to 600 milligrams per day,
as prescribed by a healthcare provider.
Monitoring zinc levels and liver function is
essential when taking zinc supplements.
• High-Quality Protein: It's important to consume high-quality proteins to support overall
health and liver function. Lean meats, poultry, fish, and dairy products are good sources
of protein. However, you should be cautious about the copper content in some of these
foods and choose low-copper options.
• Calcium and Iron: Calcium and iron are essential nutrients. Some people with Wilson's
disease may be at risk of developing osteoporosis, so it's important to ensure adequate
calcium intake. The RDA for calcium varies by age and gender but is generally around
1000 milligrams per day for adults. For iron, the RDA is typically around 8 milligrams for
men and 18 milligrams for women. Iron supplements may be necessary for individuals
with anemia due to copper accumulation.
• Vitamins and Nutrients: A well-balanced diet should provide adequate vitamins and
nutrients to support overall health. This includes vitamin D, vitamin B12, and folate,
among others. RDAs for these vitamins can be used as guidelines for ensuring proper
nutrition.
RDA (RECOMMENDED DIETARY ALLOWANCE)
(2010)
Infants · 0 to 6 months: 200 mcg/day
· 7 to 12 months: 220 mcg/day

· 1 to 3 years: 340 mcg/day

Children
· 4 to 8 years: 440 mcg/day
· 9 to 13 years: 700 mcg/day

· Males and females age 14 to 18 years: 890 mcg/day

(0.89mg/day)
Adolescents and
· Males and females age 19 and older: 900 mcg/day (0.90 mg
adults
/day )
· Pregnant females: 1,000 mcg/day
· Lactating females: 1,300 mcg/day
FOOD SOURCES
Very high copper foods High copper foods
Animal liver (lambs, pigs,
Cereal grains (barley, millet)
cows)
Shell fish (oysters, crab, Pulses (Bengal gram, horse gram, red gram,
clams) lentils, kidney beans, soya bean)
Sesame seeds Legumes (peas)

Baker’s yeast Nuts (chestnuts, pistachios, cashewnuts, walnuts)

Chocolates and cocoa powder Oil seeds (gingelly, sunflower, pumpkin)

Condiments and spices (green chillies, basil, black pepper)

Lobster and fish

Candy and fruit gums
 Medium copper foods Low copper foods

Cereals (bajra, jowar, maize, ragi,

Rice
sanwa millet, wheat)

Pulses (black gram, green gram, Leafy vegetables (amaranthus, Brussels sprouts, cabbage, sorrel, lettuce, fenugreek, drumstick
khesari dal, mothe beans) leaves, curry leaves, coriander leaves, mint, radish leaves, spinach, tamarind leaves)

Vegetables (celery, beet root, white

Eggs, butter, milk, milk products, cheese and yogurt
radish, snake gourd, mushroom)

Nuts and oil seeds (almonds, arecanut,

Roots and tubers (carrot, colocasia, onion, potato, pink radish, sweet potato, tapioca, yam)
coconut, groundnut, mustard, piyal)

Condiments and spices (asafetida,

cardamom, cloves, coriander seeds,
Vegetables (bitter gourd, bottle gourd, brinjal, tomato, broad beans, cauliflower, cluster beans,
cumin seeds, fenugreek seeds, garlic,
cucumber, drumstick, field beans, French beans, okra, raw mango, ridge gourd)
ginger, nutmeg, cumin, poppy seeds,
turmeric)

Fruits (kiwi, lime, orange, pears, Fruits (pumpkin, apple, apricot, banana, cherries, gooseberry, dates, guvava, jack fruit, lemon,
pomegranate, custard apple) litchi, ripe mango, muskmelon, watermelon, peach, pineapple, plum, sapota)

Meat (duck, goose, rabbit) Freshwater and marine fish

Sweets (jams, fruit cakes, pudding,

peanut butter)
Questions ???????
Q.1 What is Wilson disease ?
Q.2 Any two symptoms of Wilson Disease ?
Q.3 Why zinc is used in Wilson disease treatment ?
Q.4 What are the low copper food sources ?
Q.5 What are the high copper food sources ?
Q.6 What are the parts affected in Wilson Disease ?
Q.7 What is the RDA for Infants ?
Adults ?
Q.8 What is role of ceruloplasmin ?
REFERENCES
1. https://www.mdpi.com/2227-9067/9/8/1132
2. https://www.childrenliverindia.org/node/1437
3. http://
jmsronline.com/archive-article/Low-copper-containing-diet-for-Wilson-d
isease-patients
4. https://
www.msdmanuals.com/en-in/home/disorders-of-nutrition/minerals/wilso
n-disease
5. http://www.eurowilson.org/en/living/guide/pathway/index.phtml
6. https://en.wikipedia.org/wiki/Wilson%27s_disease#Ceruloplasmin