Intradialytic hypertriglyceridemia can be associated with priapism: A case report of congenital adrenal hyperplasia with kidney failure

Abstract

Data on kidney failure in patients with congenital adrenal hyperplasia are rare. To the best of our knowledge, there is no data on how to manage patients with congenital adrenal hyperplasia during hemodialysis sessions. We report a case of a fifteen-year-old boy with a known case of congenital adrenal hyperplasia since 8 months of age who presented with advanced uremia and severe hyperkalemia for which he was initially started on hemodialysis without pre-HD hydrocortisone, during which he developed recurrent intradialytic hypotension. Subsequently, when intradialytic serum cortisol levels were monitored with different hydrocortisone regimens, a serum cortisol level greater than 25 mcg/dl during HD was not associated with significant intradialytic complications. The target was 28 mg of injected hydrocortisone followed by a maintenance dose of 6 mg/hr starting at the end of the first hour of dialysis. He developed intradialytic hypertriglyceridemia-associated priapism, which improved after the correction of the iron deficiency. Intradialytic hypertriglyceridemia was of higher magnitude during heparin-free HD than during HD with heparin. In conclusion, a target serum cortisol concentration > 25 mcg/dl during HD helps reduce intradialytic complications in patients with congenital adrenal hyperplasia and renal failure. Exogenous glucocorticoid administration can be associated with intradialytic hypertriglyceridemia-associated priapism in iron-deficient patients.