BACKGROUND 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 prognos...BACKGROUND 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.CASE SUMMARY 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.CONCLUSION Clinicians should screen HPS patients'anterior pituitary function as early as possible and treat them primarily with GH cocktail accordingly.展开更多
基金Supported by the National Natural Science Foundation of China,No.81771576 and No.81971375the Beijing Municipal Natural Science Foundation,No.7202151 and No.7212080.
文摘BACKGROUND 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.CASE SUMMARY 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.CONCLUSION Clinicians should screen HPS patients'anterior pituitary function as early as possible and treat them primarily with GH cocktail accordingly.
