Indian childhood cirrhosis in the United Kingdom

Area of Science:

• Hepatology
• Pediatric Gastroenterology
• Genetic Liver Diseases

Background:

• Indian childhood cirrhosis (ICC) is a rare, idiopathic liver disease primarily affecting infants and young children.
• Consanguineous marriages are associated with an increased incidence of recessive genetic disorders.
• The genetic basis of ICC remains largely unknown, necessitating further research into its etiology.

Observation:

• Two young brothers, born to Bangladeshi parents with a history of first-cousin marriage, presented with symptoms of cirrhosis.
• The elder brother was born in Bangladesh, while the younger was born in the United Kingdom.
• Clinical presentation and liver histology were consistent with the diagnosis of Indian childhood cirrhosis (ICC).

Findings:

• The diagnosis of Indian childhood cirrhosis (ICC) was confirmed in both brothers based on clinical and histological evidence.
• The younger brother represents the first documented case of ICC in a patient born in the United Kingdom.
• This suggests potential underdiagnosis or a changing geographical distribution of ICC.

Implications:

• The findings underscore the importance of considering ICC in children with unexplained liver disease, especially those with consanguineous parentage.
• This case expands the known geographical occurrence of ICC, highlighting the need for increased awareness among UK pediatricians.
• Further genetic studies are warranted to elucidate the specific factors contributing to ICC in this family and potentially identify novel genetic mutations.