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Etiology of cirrhosis in the young
Maria C Olave1, Ananta Gurung2, Pramod K Mistry3
1Yale University School of Medicine, Department of Pathology, New Haven, CT 06511, United States.
Insights
Cirrhosis causes differ by age: congenital and developmental disorders in children, and viral hepatitis in young adults. Cryptogenic cirrhosis incidence also varies across pediatric age groups.
Area of Science:
- Hepatology
- Pediatric Gastroenterology
- Internal Medicine
Background:
- Cirrhosis etiology is well-documented in adults but understudied in younger populations.
- Understanding pediatric cirrhosis causes is crucial for timely diagnosis and management.
Purpose of the Study:
- To investigate the diverse causes of cirrhosis in patients aged 40 years and younger.
- To analyze etiological differences across pediatric and young adult age strata.
Main Methods:
- Multi-institutional retrospective study of pathology databases (1995-2018).
- Review of clinical charts and pathology reports for 594 patients ≤40 years old.
- Stratification into four age groups: <1, 1-<5, 5-<18, and 18-40 years.
Main Results:
- In patients <18 years, congenital cholestatic/developmental disorders (50.2%) and metabolic/genetic diseases (16.6%) were primary causes.
- In patients 18-40 years, viral hepatitis (39.6%), autoimmune liver disease (22.2%), and fatty liver disease (15.07%) predominated.
- Cryptogenic cirrhosis varied by age, lowest in 1-<5 years (1.4%) and highest in 5-<18 years (10.7%).
Conclusions:
- Cirrhosis etiology in individuals ≤40 years is age-dependent.
- Congenital and metabolic disorders are key in children, while viral hepatitis dominates in young adults.
- Age-specific etiological analysis is vital for understanding and addressing cirrhosis in younger demographics.
Abstract:
The etiology and incidence of cirrhosis in adults has been well studied, however there is scant data in younger patients. The aim of this study was to determine causes of cirrhosis in patients ≤40 years old. In this multi-institutional retrospective study, pathology databases were searched for patients ≤40-year-old with a diagnosis of cirrhosis from 1995 to 2018. Clinical charts and pathology reports were reviewed to identify etiologies of cirrhosis in each case. The patients were divided into 4 age groups (<1, 1- < 5, 5- < 18, and 18-40 years old) for further analysis. We identified 594 patients (264 female, 330 male). Among <18-year-old patients, congenital cholestatic diseases and developmental disorders were the most common causes of cirrhosis (50.2%, 172/342). Metabolic and genetic diseases were also seen more commonly in this age group (16.6%, 57/342). In contrast, viral hepatitides were the most common cause of cirrhosis in 18-40-year-old patients (39.6%, 100/252) followed by autoimmune and fatty liver disease (22.2%, 56/252 and 15.07%, 38/252, respectively). Cryptogenic cirrhosis (overall 7.2%, 42/594) was seen in 3% (4/133), 1.4% (1/69), 10.7% (15/140) and 8.7% (22/252) of patients aged <1, 1- < 5, 5- < 18, and 18-40 years, respectively. Developmental and metabolic disorders are the most common causes of cirrhosis in children (<18), while viral hepatitides are leading causes in adolescents and young adults (18-40) similar to adults. The incidence of cryptogenic cirrhosis also varies depending on the age, being lowest in 1- < 5 year and highest in 5- < 18 year age group children.
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