Wilson’s Disease – All you need to know

Wilson’s Disease – All you need to know

Wilson’s disease is a rare genetic syndrome in which the liver, brain, and other vital organs accumulate copper. Wilson’s syndrome is most common in children between the ages of five and thirty-five. Continue reading to learn more.

Wilson’s disease is a rare genetic condition in which copper builds up in the liver, brain, and other essential organs. Wilson’s disease most often occurs between the ages of 5 and 35. However, it can also affect younger and older adults. 

There are people in Mumbai who suffer from the disease mainly due to their genetic issues. A patient with Wilson’s disease can lead a quality life by availing of proper treatment from a liver transplant surgeon in Mumbai.

Wilson's Disease

 

Copper is essential for developing healthy nerves, muscles, collagen, and the skin’s pigment melanin. Generally, our body absorbs copper from the food we eat and exerts the excess through bile secretion.

On the other hand, the excess copper accumulates in the body of people with Wilson’s disease. This high level of copper accumulation is very harmful to your health. Wilson’s disease is treatable if identified early, and even individuals with this condition lead normal lives.

What are the symptoms of Wilson’s disease?

Wilson’s disease occurs by birth, but symptoms do not occur until copper levels in the brain, liver, or other organs have increased. The signs and symptoms of the disease differ based on the affected body parts. They may contain the following:

  • Fatigue, a loss of appetite, or stomach pains
  • Jaundice
  • Discoloration of the eyes (Kayser-Fleischer rings)
  • In the legs or lungs, there is a build-up of body fluid.
  • Issues while talking, chewing, or physical coordinating
  • Muscle tightness or uncontrollable motions

If a person experiences any of the mentioned symptoms, he or she should avail liver transplant after having a consultation.

How does Wilson’s disease happen?

Wilson’s disease is hereditary. It is an autosomal recessive disorder, which means the patient must inherit one problematic gene from each parent. The person won’t get sick if you get one rare gene, but you will be a vector and pass the gene on to your offspring.

The cases of Wilson’s disease in India are increasing and many liver surgeons observe patients complaining about this condition. More and more awareness needs to be made so that early diagnosis is possible for Wilson’s patients.

How does a liver transplant doctor diagnose Wilson’s disease?

Wilson’s disease is challenging to diagnose, and its signs and symptoms are also difficult to distinguish from those of other liver disorders, like hepatitis. The diagnoses are based on the symptoms and tests. These include:

  • Tests of the blood and urine: Blood tests are essential to evaluate your liver function. It helps to check the level of ceruloplasmin, a protein that binds copper in the blood and copper in the blood. The doctor will also want to monitor the amount of copper excreted in your urine over a day.
  • Eye examination: An ophthalmologist tests Kayser-Fleischer circles’ eyes. Excess copper causes complications in the eyes. Wilson’s disease is also related to a cataract form known as a sunflower cataract, which can come out during an eye test.
  • Biopsy: A thin needle is inserted through your skin into your liver, where we take a small tissue sample. Then the tissue goes for examination in a laboratory to check the excess copper.
  • Genetic testing: If Wilson’s disease occurs due to genetic abnormalities, genetic testing will detect that from the patient’s blood sample. Knowing the family’s mutations helps doctors to test siblings and start treating them before signs occur.

Now let us see the treatment for Wilson’s disease

The doctor may prescribe proper medications to bind copper and then release them into the bloodstream. Finally, they will be released through your urine. After that, the treatment stops the copper formation. The doctor may also suggest a liver transplant if there is serious damage to the liver.

Prescription drugs

Wilson's Disease - Prescription disease

If a patient has Wilson’s disease, they will have to take drugs for the rest of your life. The medications include the following:

Note: Below drugs can be taken only on the prescription of a qualified doctor. Self-diagnosis can land a patient in trouble.

  • Penicillamine: It is a chelating agent that may have severe side effects, including skin and renal disorders, bone marrow suppression, and deteriorating neurological symptoms. If a person is allergic to penicillin, he or she will require proper supporting remedies for allergy. You will need to take vitamin B6 supplements in limited doses.
  • Triennial: Trientine is similar to penicillamine, but it has fewer side effects. However, it can escalate few neurological problems also.
  • Galzin: Galzin drug prevents the body from consuming copper from food. It may bring stomach disturbance.

Surgical techniques

A patient may require a liver transplant if the liver damage is severe. The liver transplant removes the diseased liver and replaces it with a healthy liver from a donor during a liver transplant.

Are there any home treatments/remedies available for Wilson’s disease?

If a person has Wilson’s disease, the doctor would typically encourage limiting the copper intake from a regular diet. Even the person should avoid copper-containing multivitamins.

Avoid food items like mushrooms, chocolate, shellfish, liver, or any other copper-rich food. Further diet control can be prescribed only after proper diagnosis of the patient

Frequently Asked Questions :

1. How long can you live with Wilson disease?

Life expectancy without treatment is expected to be 40 years, although patients who receive quick and effective treatment may live a normal lifespan.

2. Is Wilson’s disease reversible?

Wilson disease has no known treatment. Copper-chelating drugs, which help your body’s organs and tissues get rid of excess copper, are one type of treatment that may be required for the rest of your life.

3. Who is most likely to get Wilson’s disease?

Wilson disease is more common in people under the age of 40. By the age of four, children begin to display symptoms. The organs that are impacted, stop functioning normally. Eastern Europeans, Sicilians and southern Italians are the most affected, but it can affect anyone.

Alcoholic Liver Disease Treatment in Mumbai

Alcoholic liver disease results from overconsumption of alcohol. It leads to liver damage, causing fat build-up, inflammation, and scarring of the liver. It can affect the whole body and can be fatal. The first and foremost step after diagnosis of alcoholic liver disease is consulting a liver specialist.

Dr. Gaurav Gupta, an experienced liver specialist in Mumbai, provides effective alcoholic liver disease treatment in Mumbai. Presently, he is a Consultant and HOD of the Liver Transplant and HPB Surgery at Fortis Hospitals, Mulund, Mumbai.

Dr. Gupta is well-known for his expertise in the field of Liver Transplant. He has conducted 500+ liver transplants. Furthermore, Dr. Gupta has 15+ years of surgical experience, out of which more than ten years in Liver Transplantation.

 

Overview of alcoholic liver disease, its stages, symptoms, diagnosis, and alcoholic liver disease treatment.

What is Alcoholic Liver Disease?

Excessive alcohol consumption causes alcohol-related liver disease. It’s a widespread liver disease that can be prevented at an initial stage. Alcohol abuse causes injury to the liver as the alcohol metabolizes in the liver.

Liver specialists advise patients with alcoholic liver disease to take a preventive vaccination for Hepatitis A and Hepatitis B.

Stages of Alcohol-Related Liver Disease and Associated Symptoms

 

Stage I: Fatty liver 

Fatty liver is an excessive accumulation of fats inside the liver cells. It is a common alcohol-related liver disorder. The liver enlarges, causing upper abdominal discomfort on the right side.

Stage II: Alcoholic hepatitis 

Alcoholic hepatitis is liver inflammation that causes the destruction of liver cells as well as scarring. Fever, jaundice, an elevated white blood cell count, a rise in liver enzymes ( SGOT and SGPT) a swollen, tender liver, are all possible symptoms.

Stage III: Alcoholic cirrhosis 

Alcoholic cirrhosis causes the loss of normal liver tissue and the formation of non-functioning scar tissue. Alcoholic hepatitis if left unchecked leads to permanent scarring and irreversible damage to the liver which is called cirrhosis. In this stage liver changes to a hard, nodular irregular surface and small in size. Symptoms may include portal hypertension (blood vomiting), enlarged spleen ascites, heavy bleeding (due to poor clotting), kidney failure, confusion, and liver cancer.

How is Alcoholic Liver Disease diagnosed?

  • Alcoholism history: 

Drinking over 20 units of alcohol a week (one unit equals one bottle of beer or a peg of whisky or a glass of wine) for more than ten years.

  • Liver function tests:

A series of blood tests is used to see whether the liver is working correctly.

  • Imaging tests:

CT scans or ultrasounds to view the structural anomalies of the liver.

  • Fibroscan:

Fibroscan tells us about liver damage. It let us know if fibrosis has started in the liver which will suggest advanced alcoholic liver disease.

A process in which liver tissue samples are examined under a microscope to diagnose any underlying conditions. These days use of liver biopsy is very limited in diagnosing Liver disease.

Alcoholic Liver Disease Treatment in Mumbai

A liver specialist initiates specific treatment for the alcohol-related liver disease based on the disease’s stage and the patient’s acceptance to quit alcohol. Treatment aims to recover some or all of the liver’s normal functions.

In Stage I: The first step of treatment for fatty liver and early stages of alcoholic hepatitis is avoiding alcohol.

  • Alcohol Cessation

If a person has alcoholic hepatitis, the only way to recover is to stop drinking alcohol. It will help reverse the liver’s damage and prevent it from worsening. If the patient is heavily dependent on alcohol and wants to quit, there are many options available. Medication, counseling, community groups, and a residential treatment facility are some of the alternatives.

  • Treatment for Malnutrition

If the patient has appetite loss, the doctor can suggest a special diet to balance nutrients and vitamins. In extreme situations, tube feeding is also advised. A high protein and high-calorie diet help in recovering from alcoholic liver disease.

  • Medication to Reduce Liver Inflammation

If the patient has severe alcoholic hepatitis, the liver specialist can prescribe corticosteroids and pentoxifylline to decrease liver inflammation.

In Stage II, simply abstaining from alcohol should be enough to regulate and even cure the problem. The liver is a potent healer. It can also restore some of the damage caused by alcohol. Cirrhosis scarring, though, is the only condition it can’t repair.

In Stage III, after the progression of cirrhosis, the disease will eventually worsen. With alcohol abstinence, however, the pace of improvement is substantially slowed. When advanced cirrhosis sets in, the only treatment is total abstinence from alcohol and liver transplantation. Once there are Liver cirrhosis complications, unfortunately, medication will not be able to cure the problem and the only long-term solution remains a liver transplant.

 

Book an appointment with Dr. Gaurav Gupta to avail of the most effective alcoholic liver disease treatment in Mumbai.

 

Frequently Asked Questions:

Q. Is Alcoholic Hepatitis More Prevalent In Men Or Women?

Women tend to be more susceptible to alcohol-related liver injury. Even if a man and a woman have relative weight and consume the same amount, the woman’s blood-alcohol level is higher than the man’s due to her more body fat and lower water content.

Q. Is It True That All Alcoholics Develop Alcoholic Hepatitis And Cirrhosis?

No, it’s not true. Some alcoholics can experience extreme physical and psychological symptoms of alcoholism while preventing severe liver damage. Alcoholic cirrhosis affects between 10% to 25% of alcoholics.

Q. Does Alcoholic Hepatitis Always Develop Into Cirrhosis?

No, it’s not true. Cirrhosis typically takes several years to develop after alcoholic hepatitis has caused enough liver damage. Cirrhosis can be prevented if alcoholic hepatitis is detected and treated earlier.

Q. How Long Can One Live With Alcoholic Liver Disease?

Depends on the stage of liver disease. In the initial stages, abstinence from alcohol and proper treatment can cure the disease. Once Cirrhosis sets in then depending upon the stage of cirrhosis different complications can develop.

Q. Can Patients start alcohol after Liver Transplant?

Strictly No. Once patients undergo Liver transplants they are advised to abstain from alcohol for life long.

Portal Hypertension – Causes, Symptoms and Treatment

Portal hypertension is a complication where pressure increases within the portal venous system. The portal vein forms by merging veins from the stomach, intestine, spleen, and pancreas. It then divides into smaller vessels and flows through the liver. 

Our body cannot circulate blood properly through the liver if there is any blockage due to liver damage. As a consequence, patients will experience high pressure in the portal system.

It may lead to swollen veins (varices) in the esophagus, stomach, rectum, or umbilical region. Varices can rupture and bleed, posing a life-threatening danger.

Many people in Mumbai and the surrounding area search for a proper treatment for Portal Hypertension. To know about the possible treatments, visit Liver Care, one of the best liver transplant center in Mumbai.

Portal Hypertension in Cirrhosis

Cirrhosis of the liver is the most significant reason for portal hypertension. Cirrhosis is scarring that occurs after a liver injury, whether from hepatitis, alcohol, or other less frequent cause of liver damage. Cirrhosis causes increased resistance to portal blood in the liver.

This increased resistance leads to backpressure in the Portal venous system leading to Portal hypertension. In patients with cirrhosis, portal hypertension is one of the most common causes of hospitalization, liver transplantation, even death. The best treatment for cirrhosis-related advanced portal hypertension is liver transplantation surgery.

Causes of Portal Hypertension, and to prevent it?

Other than the cirrhosis of the liver,

  • Blood clots in the portal vein.
  • Blockages of the veins that carry blood from your liver to the heart.
  • A bacterial infection like schistosomiasis.
  • NCPF. Non cirrhotic Portal hypertension

Symptoms of Portal Hypertension?

The symptoms of Portal hypertension are as follows:

  • Due to a sudden rupture and bleeding from varices, patients may experience dark, tarry stools or blood in the stool.
  • Patients may experience blood in vomiting too.
  • There can be an accumulation of fluid in  abdomen known as ascitis
  • Patients may suffer from confusion and forgetfulness due to impaired liver function.
  • The condition can lower platelet levels in your blood.
  • Weight loss as a result of malnutrition.
  • There can be Kidney malfunctions.
  • Patients may experience fluid accumulation in your lungs.

 

What is the treatment for Portal Hypertension?

It is important to know the cause of portal Hypertension. In patients of cirrhosis, the only therapeutic and permanent treatment is Liver Transplant. Till the patient is waiting for a liver transplant, the treatment revolves around preventing or controlling complications, especially variceal bleeding. Proper diet, medicines, endoscopic therapy, surgery, and radiology procedures are there to protect patients from any difficulties.

Endoscopic therapy: It consists of either banding or sclerotherapy. It is typically the first line of treatment for variceal bleeding. Banding is a process in which a gastroenterologist closes a blood vessel with rubber bands to avoid bleeding. Sclerotherapy involves the insertion of a blood-clotting solution into the bleeding varices to prevent bleeding.

Medications: Your doctor may suggest Nonselective beta-blockers (nadolol or propranolol) alone or with endoscopic therapy. It will reduce the risk of bleeding by lowering the varices pressure. Nonselective beta-blockers are also used to avoid first variceal bleeding in patients with varices and suspected of bleeding.

Changes in your lifestyle and diet: Stop drinking alcohol and avoid over-the-counter or prescription medications without consulting your doctor. These drugs can aggravate your condition.

Patients must adopt a low-sodium diet, which means the patient will have to cut down on their salt intake. Patients should limit their sodium intake to no more than 2 grams a day.

All of these are the primary treatments. If these treatments do not work, doctor may suggest:

Transjugular intrahepatic portosystemic shunt (TIPS):

It is a radiological method where doctors insert a stent into the liver. A radiologist inserts a needle into the liver to make a tunnel that connects the portal vein and hepatic veins. A metal stent is inserted into this tunnel to hold it open.

This operation redirects blood flow through the liver and decreases pressure in portal veins, including those in the stomach, oesophagus, bladder, and liver.

Splenorenal shunt (SRS):

In this surgical procedure, Surgeon connect the splenic vein to  left kidney vein. It helps in controlling the pressure in varices and prevents bleeding. This procedure is advocated in Non Cirrhotic Portal hypertension( NCPH)

Fatty Liver Disease – Types, Symptoms and Causes

atty liver, also known as hepatic steatosis, occurs when the liver builds up with fat. Small amount of fat is always present in the liver. However, if the amount of fat in the liver increases it can be detrimental to health.

Too much fat in the liver can lead to inflammation, which can harm and scar the liver. In serious cases, this scarring can lead to liver failure.

There are 2 types of fatty liver diseases Alcoholic fatty liver diseases(AFLD) and Non-alcoholic fatty liver diseases(NAFLD).

If fatty liver builds up in someone who drinks a lot of alcohol, it is known as alcoholic fatty liver disease. When fatty liver develops in someone who does not drink alcohol, it is known as non-alcoholic fatty liver disease.

In this article, the focus is on non-alcoholic fatty liver disease. If you have a serious issue, you can book an appointment with Dr Gaurav Gupata, one of the renowned liver specialist in Mumbai.

What is Non-alcoholic Fatty Liver (NAFL)?

Non-alcoholic fatty liver (NAFL) is the first and reversible stage of liver disease. Unfortunately, it usually goes unnoticed. After some time, NAFL progresses to non-alcoholic steatohepatitis, or NASH, a more severe liver disease.

NASH is characterised by increased fat accumulation and inflammation, both of which harm liver cells. When liver cells are excessively damaged, it results in fibrosis or scar tissue.

Some NAFL further develops into NASH raising the risk of cirrhosis and liver cancer. NAFL also increases the risk of other diseases such as heart disease, diabetes, and kidney disease.

 

Stages of Liver Damage

What are the symptoms of Fatty Liver?

There are many fatty liver symptoms, but not all of them are present at the same time. In fact, the patient might not even be aware that they have fatty liver. Some of the symptoms of fatty liver include:

  • Weakness and exhaustion
  • Ache or feeling of fullness in the right or central abdomen
  • Elevated liver enzyme levels, such as AST and ALT
  • Elevated insulin levels
  • Elevated triglyceride levels

The following symptoms can occur if fatty liver progresses to NASH:

  • Appetite loss
  • Vomiting and nausea
  • Abdominal pain that ranges from mild to extreme
  • Yellowing of the skin and eyes

It is essential to see a doctor regularly for routine examinations and blood tests. It can detect fatty liver disease at an early, reversible stage.

What causes Fatty Liver?

Causes of Fatty Liver

Several factors may cause or lead to the development of fatty liver:

  • Obesity  – It causes low-grade inflammation in the liver, leading to fat accumulation NAFL affects 30–90% of obese people, and it is becoming more common in children.
  • Excess belly fat – Normal-weight people who are “viscerally obese,” meaning they carry too much fat around the waist, can develop fatty liver.
  • Insulin tolerance – Insulin resistance and elevated insulin levels have been shown to increase liver fat storage in patients with type 2 diabetes and metabolic syndrome,
  • High consumption of carbs – A high carbohydrate diet promotes the storage of liver fat, particularly in overweight or insulin-resistant people.
  • Sugary beverage consumption – Sugar-sweetened beverages, such as soda and energy drinks, are rich in fructose. Fructose is linked to the accumulation of liver fat in both children and adults.
  • Impaired gut health – According to recent studies, an imbalance in gut bacteria, problems with gut barrier function (“leaky gut”), and other gut health issues can all lead to NAFL production.

How to get rid of Fatty Liver?

Some of the ways a patient can get rid of non-alcoholic fatty liver are mentioned below:

Dietary changes

DIetary changes

There are several things a patient can do to get rid of fatty liver, including losing weight and reducing the intake of carbs. What is more, certain foods can help lose liver fat as well.

Lose weight and avoid overeating

Patients who are overweight or obese should lose weight and avoid overeating. One of the simplest ways to reverse fatty liver is to lose weight.

In fact, in people with NAFL, weight loss has been proven to promote liver fat removal. It makes no difference whether a patient reduces weight by dietary adjustments, exercise or weight loss surgery.

Exercises

Physical exercise can help lose liver fat. Studies have shown that engaging in endurance or resistance training many days a week will substantially reduce the amount of fat contained in liver cells.

Since regular exercise is important for reducing liver fat, patients can look for an exercise routine that suits them best.

Fatty liver can cause several health issues. Fortunately, if caught early enough, it can be reversed. The patient can reduce excess liver fat by consuming a healthier diet, increasing physical activity, and taking prescribed supplements.

All you need to know about: Nonalcoholic Steatohepatitis (NASH)

Do you know that a person can get a liver disease even if he or she doesn’t drink alcohol?

Non-alcoholic fatty liver disease-NAFLD is a condition in which fat builds up in the liver. Non-alcoholic steatohepatitis (NASH) is a type of NAFLD that can impair the liver.

NASH

 

NASH occurs when the fat build-up in the liver leads to inflammation (hepatitis) and scarring. In a study, liver experts estimated that 3% to 5% of the global population is affected by NASH, though it is considered underdiagnosed.

This blog gives an overview of Non-alcoholic steatohepatitis, its symptoms, causes, diagnosis, and treatment.

 

What is Nonalcoholic Steatohepatitis (NASH), and how does it affect an individual?

The inflammation and destruction caused by fat accumulation in the liver are known as nonalcoholic steatohepatitis (NASH). It is a type of nonalcoholic fatty liver disease.

An excess of fat in the liver affects many individuals, but it usually causes no signs of complications. Fat, on the other hand, can cause inflammation and damage to liver cells in certain people. The liver does not function as well as it does as a result of the damage.

Stages of Liver Damage - NASH

NASH can worsen and lead to liver scarring, which can lead to cirrhosis. However, the condition does not necessarily deteriorate.

NASH affects the liver similarly as in liver conditions caused due to excessive alcohol consumption. But NASH develops in individuals who don’t drink alcohol.

 What are the signs and symptoms of nonalcoholic steatohepatitis (NASH)?

In the initial stages of NASH, the patient will have no symptoms. The majority of people with NASH are entirely unaware that they have it.

If NASH progresses, the liver damage worsens so that the patient can experience symptoms such as:

  • Fatigue
  • Sudden weight loss
  • Weakness
  • Pain in the upper right part of the abdomen

NASH will take several years to progress to the point that it causes symptoms.

 

What causes Nonalcoholic Steatohepatitis (NASH)?

Experts are unsure why some patients accumulating fat in the liver develop NASH, and others do not. Some environmental factors may be causing inflammation in specific individuals. Or maybe it is something that runs through their family.

A variety of factors can cause NASH and liver damage, including:

  • Obesity
  • Insulin resistance and type 2 diabetes
  • Elevated cholesterol and triglycerides levels
  • Metabolic syndrome
  • Hypertension

The majority of NASH patients are between the ages of 40 and 50 and have one or more of the mentioned conditions. However, NASH can also develop in individuals who do not have any of these risk factors. 

How is Nonalcoholic Steatohepatitis (NASH) diagnosed?

A single examination cannot diagnose NASH. A liver specialist can inquire about the patient’s medical history.

To see whether fat is accumulating in the liver and to rule out other diseases, the liver specialist can order specific tests such as:

Diagnosis of NASH
  • Blood tests
  • Ultrasound
  • CT scan
  • MRI scan

The liver specialist may perform a liver biopsy to diagnose NASH. In a liver biopsy, the specialist takes a sample of tissue from the liver and observes it for signs of NASH.

How is Nonalcoholic Steatohepatitis (NASH) treated?

Managing symptoms that raise the chance of NASH or make it worse is part of NASH treatment.

The liver specialist instructs and guides the patient for:

  • Regulating the total cholesterol level.
  • Maintaining a healthy weight. It can make a difference if the patient can correct his/her BMI to bring it down to less than 25
  • Controlling diabetes.
  • Quitting or reducing alcohol consumption.
  • Exercising daily

In some cases, the liver specialist may prescribe medicines to control or reverse liver damage caused by NASH.

In some NASH patients, severe liver damage may cause its failure. For such patients, a liver transplant is the only option for survival. The patients can lead a healthy life after transplant if they eat a balanced diet and take prescribed medications.

 

Make an appointment with an experienced liver specialist to receive the most effective treatment for liver-related conditions.