Delayed puberty in girls having biliary atresia

Area of Science:

• Pediatric Surgery
• Endocrinology
• Hepatology

Background:

• Biliary atresia is a severe neonatal liver disease requiring surgical intervention.
• The Kasai operation (portoenterostomy) is a primary treatment, but long-term sequelae persist.
• Long-term outcomes in female survivors of biliary atresia treated with the Kasai operation are not fully understood.

Purpose of the Study:

• To evaluate the long-term health status of female patients following successful Kasai operation for biliary atresia.
• To investigate the incidence and characteristics of menstrual disorders and pubertal development.
• To explore the relationship between hormonal profiles, portal hypertension, and menstrual abnormalities.

Main Methods:

• Longitudinal follow-up of 14 female patients over 12 years post-Kasai operation.
• Assessment of jaundice status, esophageal varices, hypersplenism, and pubertal development.
• Hormonal evaluation including Luteinizing Hormone-Releasing Hormone (LHRH) testing and estradiol levels.

Main Results:

• All patients were jaundice-free, but 10 had esophageal varices and/or hypersplenism.
• Five patients experienced menstrual disorders, including delayed menarche, primary amenorrhea (18.2%), and secondary amenorrhea (36.4%).
• Amenorrhea correlated with portal hypertension; hormonal evaluation showed abnormal LH response in affected patients.

Conclusions:

• Female survivors of biliary atresia treated with the Kasai operation face significant long-term challenges, including endocrine and pubertal abnormalities.
• Portal hypertension is a key factor associated with menstrual dysfunction in these patients.
• Further investigation and management strategies are needed for the endocrine and pubertal sequelae of biliary atresia.