Could factor V Leiden defect cause Addison's disease?

نویسندگان

  • H Soran
  • H Y Osman
  • N Younis
  • S A Sukumar
  • D A Taberner
چکیده

Sir, We describe a case of Addison's disease caused by bilateral suprarenal haemorrhage in a patient heterozygous for factor V Leiden defect. This is the first reported case of isolated bilateral supra-renal haemorrhage in a patient with this disorder. Because of the unique blood supply of the suprarenal glands, we believe that suprarenal haemorrhage was secondary to venous infarction caused by venous thrombosis in suprarenal veins and venous plexus. A 68-year-old man was admitted to hospital with a 3-week history of anorexia, lack of energy, dizziness and weight loss. There was a history of ileo-femoral deep-vein thrombosis in his left leg treated with anticoagulation 4 weeks previously. He was on warfarin and senna tablets. On examination, he was hypotensive and tachy-cardic, and his left leg was swollen. The rest of the examination was unremarkable. Sodium was low (125 mmol/l), potassium was 6.6 mmol/l and glucose 5.6 mmol/l. ECG showed right bundle branch block, and chest X-ray showed clear lung fields. He was treated with low-molecular-weight heparin, rehydration with normal saline, fluid balance and insulin and dextrose to treat his hyperkalaemia. Further investigations showed a low random cortisol level at 117 nmmol/l. Subsequently , a short synacthen test suggested primary adrenal failure: pre-synacthen cortisol was 107 nmol/l and post-synacthen was 117 nmol/l. Supra-renal autoantibodies were negative. Ultra-sound scan of the lower limbs showed bilateral extensive deep-vein thromobosis. Abdominal computerized tomography showed bilateral suprarenal masses suggesting bilateral haemorrhage into suprarenal glands (Figure 1), extensive venous thrombosis affecting the distal splenic vien, inferior mesenteric veins, distal inferior vena cava below the level of renal veins and pelvic veins. Thrombo-philia screen showed increased activated protein C resistance, and the heterozygous factor V Leiden defect was confirmed by genetic studies. Anti-cardiolipin antibodies were weakly positive, but this was thought not to be significant. He was started on lifelong hydrocortisone and fludrocortisone. He made a good recovery and was discharged from hospital. A repeat CT scan 2 years later showed that the changes in suprarenal glands has resolved. Suprarenal glands are particularly vascular. They are supplied by the superior, middle and inferior supra renal arteries, which arise from the inferior phrenic arteries, abdominal aorta and renal arteries, respectively. Most of the branches of the suprarenal arteries ramify over the capsule before entering the gland and dividing to form a narrow sub-capsular plexus. This plexus supplies the zona glomerulosa and then passes through the zona fasciculata to …

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عنوان ژورنال:
  • QJM : monthly journal of the Association of Physicians

دوره 94 12  شماره 

صفحات  -

تاریخ انتشار 2001