Endocrine Abstracts

Introduction: Hereditary hemochromatosis is a common disorder in white population with a prevalence of 4-6.4%. It is a systemic disorder that affects multiple organs. It could affect the pituitary gland leading to hypogonadotropic hypogonadism and could affect other pituitary hormones to a lesser extent. We present a rare case of hereditary hemochromatosis leading to hypotension most likely due to iron infiltration of the pituitary gland.

Case presentation: A middle aged male presented to the emergency department with a gradual onset of progressive dyspnoea, abdominal distension, bilateral leg swelling and jaundice. His past medical history is remarkable for alcohol related liver cirrhosis. He cut off his alcohol 3 months prior to this presentation and last alcohol intake was 2 weeks prior. He was afebrile, tachycardic, on oxygen with normal blood pressure. He had jugular venous distension, bilateral crackles, ascites, and bilateral lower limb oedema. Chest Xray showed right sided pleural effusion. Echocardiography was unremarkable with normal ejection fraction. Abdominal ultrasound and CT were only remarkable for liver cirrhosis with minimal ascites. He had very high ferritin levels (4059 mg/l) and transferrin saturation (89%). He became hypotensive (systolic blood pressure <80) for a few days despite stopping furosemide, giving IV fluids and albumin. With his prolonged persistent hypotension, there was a question whether he has developed hypopituitarism secondary to hemochromatosis. His Serum FSH (1.0 u/l), LH (<1.0 u/l), testosterone (3.2 nmol/l), and AM cortisol were low (104nmol/l). Prolactin (454 mu/l) was high. TSH (2.04 mu/l) and Free T4 (18.9 pmol/l) were normal. He was suspected to have partial hypopituitarism secondary to hemochromatosis. Hydrocortisone was started and his blood pressure improved significantly.

Discussion: Hereditary hemochromatosis causes iron deposition in many organs specially the liver, the heart and the pancreas leading to multi-system affection and consequently liver cirrhosis, heart failure and diabetes. This patient had only liver cirrhosis with no other known organ affection. He was hypotensive despite appropriate treatment. His pituitary hormonal profile showed low gonadotropins, low cortisol despite normal thyroid function. This implies that he had partial hypopituitarism secondary to iron deposition in the pituitary gland secondary to his hemochromatosis. His hypotension resolved once hydrocortisone was started.

Conclusion: Clinicians should be aware that hypopituitarism should be suspected in patients with unexplained hypotension with history of hemochromatosis.