Normalization of Serum 11- Deoxycortisol in a Patient with Non-Classic Adrenal Hyperplasia Due to 11-Hydroxylase Deficiency Treated with Vitamin D and a Glucacon-like Peptide(GLP)-1 Agonist

Presentation Number: SAT-0763
Date of Presentation: June 21st, 2014

Milay Luis Lam*1, Alan Scott Sacerdote2 and Gul Bahtiyar3
1woodhull Medical Center, Brooklyn, NY, 2Woodhull Med & Mental Hlth Ctr, Brooklyn, NY, 3Woodhull Medical & Mental Health Center, Brooklyn, NY


Background: We have previously reported that vitamin D replacement can ameliorate both classical and non-classical adrenal hyperplasia (NCAH) due to 11-hydroxylase deficiency much as it does with both polycystic ovarian syndrome, possibly due to a reduction in  insulin resistance. In this case report we show the biochemical benefit that a patient received from vitamin D replacement and GLP-1 agonist treatment in terms of his 11-deoxycortisol levels.

Clinical Case: Our patient is a 60 year old male being followed in Endocrinology clinic since March 2012, after being hospitalized for bowel obstruction and noticing that he was hyperglycemic.  His only past medical history was positive for seizure disorder, for which he was taking oxcarbazepine 300mg and phenobarbital 30mg both three times a day. The latter is known to cause Vitamin D deficiency/insufficiency via increased clearance.

At the time of his diabetes diagnosis he was not taking any insulin sensitizers. His BMI was 36 kg/m2 and has been stable in that range.  His initial HbA1c by immunoturbidimetry on 3/20/2012 was 11.4% (normal range 0-6.99%) so he was started on an insulin regimen with bedtime glargine and lispro before meals.  On 3/20/2012 his 25-OH-vitamin D by liquid chromatography tandem mass spectrometry [LC MS/MS] was 10ng/ml (normal range 30-100 ng/ml). He was started on replacement with ergocalciferol 50 000 IU weekly. The initial measurement of 11- deoxycortisol could not be done since the laboratory lost the corresponding  sample. 

On 5/22/2012 he had a follow-up visit in Endocrinology clinic where he was started on liraglutide,1.2mg SC, since his HbA1c from 5/3/2012 was still above target at 8.8%, 11 deoxycortisol by LC/MS/MS was found to be 79ng/dl (< 42ng/dl) with a 25-OH-Vitamin D level of 41ng/ml.  On 8/6/2012 his HbA1c was 5.8%, 11 Deoxycortisol level was 70ng/dl, 25–OH vitamin D level was 61ng/dl, so on his 8/15/2012 Endocrinology visit, liraglutide was increased to 1.8mg daily.

By 11/8/2012 his 11-deoxycortisol level was 49ng/dl, HbA1c 5.5% and on 2/13/13 11-deoxycortisol level was normal at 34 ng/dl, with a 25-OH-Vitamin D level of 62 ng/ml.

Conclusion: Our findings suggest that vitamin D replacement in combination with a GLP-1 agonist can help in the treatment of NCAH caused by 11- Hydroxylase deficiency by reducing insulin resistance through reductions in inappropriate glucagon secretion and glucose toxicity in addition to weight loss (when it occurs) as well as by a direct effect of Vitamin D, through binding to its adrenocortical receptors and increasing the synthesis of mRNA for 11-hydroxylase.


Nothing to Disclose: ML, ASS, GB