Indian childhood cirrhosis

Indian Childhood Cirrhosis (ICC) is a rare and severe form of liver cirrhosis that affects young children, predominantly in India. This condition leads to the progressive deterioration of the liver, ultimately resulting in liver failure if not treated promptly. The exact cause of ICC is not fully understood, but it is believed to be related to genetic factors and dietary intake of copper.

Causes and Risk Factors

The primary cause of Indian Childhood Cirrhosis is believed to be an overload of copper in the body. High levels of copper can be toxic and may lead to liver damage. In India, the use of brass or copper containers for storing and cooking food has been linked to the high incidence of this condition, as these practices can lead to excessive copper intake. Additionally, genetic predisposition may play a role, with some families showing a higher incidence of the disease, suggesting a hereditary component.

Symptoms

Symptoms of Indian Childhood Cirrhosis typically begin in early childhood. Early signs may include:

• Jaundice (yellowing of the skin and eyes)
• Abdominal swelling due to ascites (accumulation of fluid in the abdomen)
• Poor appetite and weight loss
• Fatigue and weakness
• Itching

As the disease progresses, more severe complications can arise, such as esophageal varices (swollen veins in the esophagus that can bleed), hepatic encephalopathy (brain dysfunction caused by liver failure), and coagulopathy (blood clotting disorder).

Diagnosis

Diagnosis of Indian Childhood Cirrhosis involves a combination of clinical evaluation, laboratory tests, and imaging studies. Blood tests may reveal elevated liver enzymes, indicating liver damage. Imaging tests such as ultrasound, CT scan, or MRI can assess the extent of liver damage and rule out other conditions. A liver biopsy, where a small sample of liver tissue is examined under a microscope, is often necessary to confirm the diagnosis of ICC.

Treatment

Treatment for Indian Childhood Cirrhosis focuses on managing symptoms and preventing further liver damage. This may include:

• Reducing dietary copper intake and avoiding the use of copper or brass utensils for food storage and preparation.
• Medications to remove excess copper from the body, such as chelating agents.
• Nutritional support to address malnutrition and promote liver health.
• In severe cases, a liver transplant may be the only option to save the child's life.

Prevention

Preventive measures for Indian Childhood Cirrhosis include:

• Avoiding the use of copper or brass containers for storing and cooking food, especially acidic foods that can leach copper.
• Ensuring safe drinking water with low copper levels.
• Genetic counseling for families with a history of ICC.

Prognosis

The prognosis for children with Indian Childhood Cirrhosis has improved with early diagnosis and treatment. However, without treatment, the condition is typically fatal due to liver failure. The success of liver transplants and advancements in medical treatment have significantly improved survival rates.