Pediatric cholestatic liver disease: Successful transition of care

Praveen Kumar Conjeevaram Selvakumar; Vera Hupertz; Naveen Mittal; Kris V. Kowdley; Naim Alkhouri

doi:10.3949/ccjm.86a.18140

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Figure 1
Cholestasis in children can be caused by biliary tract obstruction such as in biliary atresia (top) or defects in forming and excreting bile acids and other components of bile (bottom).
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Figure 2
Transitional model in childhood-onset cholestatic diseases.

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TABLE 1

Implications of some childhood-onset liver diseases in adulthood

Liver disease	Implications in adulthood	Screening strategies in adulthood
Biliary atresia	Progressive liver fibrosis, cirrhosis Portal hypertension with variceal hemorrhage Cholangitis Hepatopulmonary syndrome and portopulmonary hypertension Hepatocellular carcinoma Possible need for liver transplant	Periodic monitoring of liver function depending on severity of liver disease Screening for esophageal varices with upper endoscopy at least annually; primary prophylaxis can be considered with beta-blockers and endoscopic variceal ligation to prevent variceal bleeding Adequate treatment of cholangitis with oral or intra-venous antibiotics depending on the severity of presentation; role of prophylaxis is unclear Screening for hepatopulmonary syndrome and portopulmonary hypertension with agitated saline echocardiography in appropriate clinical setting Ultrasonography of the abdomen and alpha-fetoprotein every 6 months or annually to screen for hepatocellular carcinoma
Alagille syndrome	No new onset of cardiac or liver disease with liver transplant being rare in adulthood Hepatocellular carcinoma Onset of renal manifestations such as hypertension or renal artery stenosis Neurovascular accidents such as stroke or intracranial hemorrhage Pregnancy complicated by severity of cardiac or liver disease	Annual ultrasonography and alpha-fetoprotein to screen for hepatocellular carcinoma Renal ultrasonography with Doppler to screen for renal vascular anomalies Magnetic resonance angiography every 5 years and before any major intervention to screen for intracranial vascular abnormalities Screening for any cardiac disease before conception Genetic counseling should be considered before conception
Progressive familial intrahepatic cholestasis (PFIC)	Adult-onset cholestasis Gallstones Hepatocellular carcinoma Intractable diarrhea Intrahepatic cholestasis of pregnancy Benign recurrent intrahepatic cholestasis	Management of extrahepatic manifestations, especially in PFIC 1 Periodic assessment of liver function for low gamma-glutamyl transferase cholestasis after liver transplant in patients with PFIC 2 Annual ultrasonography and alpha-fetoprotein to screen for hepatocellular carcinoma, cholangiocarcinoma and pancreatic adenocarcinoma in patients with PFIC 2