Published online Jun 26, 2021. doi: 10.12998/wjcc.v9.i18.4852
Peer-review started: February 8, 2021
First decision: March 29, 2021
Revised: April 2, 2021
Accepted: April 20, 2021
Article in press: April 20, 2021
Published online: June 26, 2021
Processing time: 122 Days and 20.5 Hours
Metabolic associated fatty liver disease frequently occurs in patients with hypopituitarism and growth hormone (GH) deficiency. Some patients may develop to hepatopulmonary syndrome (HPS). HPS has a poor prognosis and liver transplantation is regarded as the only approach to cure it.
A 29-year-old man presented with progressive dyspnea for 1 mo. At the age of 10 years, he was diagnosed with panhypopituitarism associated with pituitary stalk interruption syndrome. Levothyroxine and hydrocortisone were given since then. To achieve ideal height, he received GH treatment for 5 years. The patient had an oxygen saturation of 78% and a partial pressure of arterial oxygen of 37 mmHg with an alveolar–arterial oxygen gradient of 70.2 mmHg. Abdominal ultrasonography showed liver cirrhosis and an enlarged spleen. Perfusion lung scan demonstrated intrapulmonary arteriovenous right-to-left shunt. HPS (very severe) was our primary consideration. His hormonal evaluation revealed GH deficiency and hypogonadotropic hypogonadism when thyroid hormone, cortisol, and desmopressin were administrated. After adding with long-acting recombinant human GH and testosterone for 14 mo, his liver function and hypoxemia were improved and his progressive liver fibrosis was stabilized. He was off the waiting list of liver transplantation.
Clinicians should screen HPS patients' anterior pituitary function as early as possible and treat them primarily with GH cocktail accordingly.
Core Tip: Liver transplantation is currently known to be the only way to cure hepatopulmonary syndrome (HPS). Even after the successful transplantation surgery, metabolic associated fatty liver disease (MAFLD) always recurs in patients with hypopituitarism who do not receive appropriate hormone replacement therapy. We present herein a case of HPS (very severe) induced by panhypopituitarism that was recovered by complete hormone replacement without surgery, especially growth hormone and testosterone. This case report highlights the importance of screening anterior pituitary function in patients with MAFLD or HPS as early as possible. The growth hormone cocktail therapy, especially growth hormone and testosterone, is expected to avoid liver transplantation.