A 19 year old African American male presented with progressive shortness of breath over one month. Associated symptoms were dry cough and forty pound weight loss. Past medical history was pertinent for meningitis four years ago without any residual deficits. Vital signs: temperature 37°C, heart rate 70, blood pressure 93/35, oxygen saturation 98% on 4L. Examination revealed a tall, thin male with fish-mouth deformity, hypertelorism, marfanoid features, loss of secondary sexual characters, microorchidism and II/VI systolic ejection murmur at the left sternal border. Abnormal laboratory data included ALT: 42, AST: 96, ALP: 125, INR: 1.86. An MRI of the abdomen and pelvis revealed hepatic steatosis with associated perfusion abnormalities and absent testes. Subsequent liver biopsy showed severe steatosis accompanied by bridging fibrosis and nodule formation. As clinically suspected, intrapulmonary shunting was evidenced by a positive bubble stdy on echocardiogram, further confirmed by a 70% intrapulmonary shunt seen on pulmonary perfusion scintigraphy. Other important tests were undetectable testosterone, LH, FSH, FT4, IGF-1 and a failed cosyntropin stimulation test. In light of these findings a brain MRI was ordered which revealed a pituitary mass and consequent biopsy showed fibrous tissue only. Our final diagnosis was hepatopulmonary syndrome (HPS) secondary to non-alcoholic fatty liver disease (NAFLD), the cause of which remains unclear but likely linked to panhypopituitarism. No over-arching genetic diagnosis has yet been identified on genetic testing. The patient was discharged home on hormone replacement therapy and oxygen supplementation, all the while undergoing evaluation for liver transplantation.
The link between hypopituitarism and the development of NAFLD is becoming increasingly recognized, although not well understood. Furthermore, albeit a rare complication of cirrhosis, a few case reports suggest that HPS may actually occur at an increased frequency in patients with hypopituitarism1. This case adds to the sparse literature supporting an interrelated pathogenesis of these conditions. Further studies are required to explore the link between the pituitary and liver and hence improve our understanding of how to manage these patients.
With increasing prevalence of NAFLD alongside obesity in the US, it is important to be aware of the link between hypopituitarism and NAFLD, particularly as these patients may have a higher likelihood of developing cirrhosis complicated by hepatopulmonary syndrome.
- Jankowska I, Pawowska J, Teisseyre M, et al. Recurrence of nonalcoholic steatohepatitis following liver transplantation in 13 years old boy (Abstract). J Pediatr Gastroenterol Nutr 2004;39:S141–S142
To cite this abstract:Alexopoulos A, Patel R, Langah R. Pituitary Origin of Cirrhosis – a Surprising Link. Abstract published at Hospital Medicine 2015, March 29-April 1, National Harbor, Md. Abstract 434. Journal of Hospital Medicine. 2015; 10 (suppl 2). https://www.shmabstracts.com/abstract/pituitary-origin-of-cirrhosis-a-surprising-link/. Accessed January 22, 2020.