Korean J Pediatr.  2007 Aug;50(8):794-798. 10.3345/kjp.2007.50.8.794.

A case of hepatopulmonary syndrome in a child with fatty liver disease secondary to hypopituitarism after craniopharyngioma resection

• Im SJ
• Lee HD
• Park JH
• Park HJ

• KMID: 2279204
• DOI: http://doi.org/10.3345/kjp.2007.50.8.794

Abstract

Hepatopulmonary syndrome is a triad that includes: hepatic dysfunction, intrapulmonary vascular dilatation and abnormal arterial oxygenation. The incidence of intrapulmonary vascular dilatations, in adults with end-stage liver disease, has been reported to be 13% to 47%, however the incidence in children is unclear and the cases in Korean children have never been reported. The hepatopulmonary syndrome may occur as a result of chronic liver disease following nonalcoholic steatohepatitis in children with hypothalamic or pituitary dysfunction. We report a case of hepatopulmonary syndrome in a 13-year- old child who had rapidly progressive liver dysfunction secondary to panhypopituitarism after craniopharyngioma resection. Careful monitoring and treatment of endocrine abnormalities and metabolic status, as well as liver function, are required in all children undergoing pituitary tumor resection.

Keyword

Hepatopulmonary syndrome; Fatty liver disease; Hypopituitarism; Craniopharyngioma

MeSH Terms

Adult
Child*
Craniopharyngioma*
Dilatation
Fatty Liver*
Hepatopulmonary Syndrome*
Humans
Hypopituitarism*
Incidence
Liver
Liver Diseases
Oxygen
Pituitary Neoplasms
Oxygen