A case of human intramuscular adrenal gland transplantation as a cure for chronic adrenal insufficiency.
Intramuscular endocrine gland transplantation has been well described as it pertains to parathyroid autotransplantation; however, transplantation of the adrenal gland is less well characterized. While adrenal autotransplantation in the setting of Cushing's disease has been described, intramuscular adrenal allotransplantation as a cure for adrenal insufficiency to our knowledge has not been previously carried out. Current treatment for adrenal insufficiency leaves patients without diurnal variation in cortisol release and susceptible to the detrimental effects of chronic hypercortisolism. We describe here the case of a 5-year-old girl with renal failure who had adrenal insufficiency following fulminant meningococcemia that led to requirements for both stress-dose steroid and mineralocorticoid replacement. Ten months after the onset of her disease, she received a simultaneous renal and adrenal gland transplant from her mother. The adrenal gland allograft was morselized into 1 mm(3) segments and implanted into three 2 cm pockets created in her rectus abdominis muscle. Three years after surgery, her allograft remains fully functional, responding well to adrenocorticotropin hormone stimulation and the patient does not require any steroid or mineral-corticoid supplementation. We believe this case represents the first description of successful functional intramuscular adrenal allograft transplantation with long-term follow up as a cure for adrenal insufficiency.
Grodstein
E
E
Division of Abdominal Organ Transplantation, Department of Surgery, Columbia University College of Physicians and Surgeons, New York, NY, USA. eig2103@columbia.edu
Hardy
M A
MA
Goldstein
M J
MJ
D000224:Addison Disease / Q000188:drug therapy; D000311:Adrenal Glands / Q000637:transplantation*; D000309:Adrenal Insufficiency / Q000188:drug therapy; D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use; D002675:Child, Preschool; D002940:Circadian Rhythm; D003480:Cushing Syndrome / Q000188:drug therapy / Q000628:therapy*; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D047748:Pituitary ACTH Hypersecretion / Q000188:drug therapy; D014182:Transplantation, Autologous
D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone