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Copper metabolic defects and liver disease: environmental aspects
Anand N Pankit1, Sheila A Bhave
1Department of Pediatrics, KEM Hospital Research Center, Pune, India.
Journal of Gastroenterology and Hepatology
|December 11, 2002
Summary
Indian Childhood Cirrhosis (ICC) is linked to environmental copper exposure, particularly from brass vessels. Evidence suggests a combination of copper toxicity and genetic factors may cause this liver disease.
Area of Science:
- Biochemistry
- Environmental Health
- Pediatric Gastroenterology
Background:
- Copper (Cu) is vital for biological processes, with homeostasis maintained by absorption, excretion, and storage.
- Copper deficiency is rare, but toxicity can occur acutely (e.g., copper sulfate poisoning) or chronically.
- Chronic copper toxicity manifests as liver cirrhosis and organ damage, notably in Wilson's Disease and Indian Childhood Cirrhosis (ICC).
Purpose of the Study:
- To describe the clinical, epidemiological, and treatment aspects of Indian Childhood Cirrhosis (ICC).
- To present evidence linking ICC to environmental copper exposure.
- To explore potential genetic factors in ICC causation.
Main Methods:
- Review of clinical and epidemiological data on ICC.
- Analysis of evidence linking environmental copper exposure to ICC.
- Observation of treatment outcomes, including d-penicillamine therapy and remission.
Main Results:
- Significantly elevated hepatic copper levels in ICC patients.
- Association between ICC and boiling/storing milk in brass vessels.
- A decline in ICC incidence correlating with changes in feeding vessels.
- Sustained remission in treated patients after drug withdrawal.
Conclusions:
- Strong evidence supports environmental copper exposure as a key factor in ICC.
- A suspected genetic predisposition may interact with environmental factors.
- Similar conditions in the West (Idiopathic Copper Toxicosis) suggest 'ecogenetic' mechanisms for infantile liver disease.