APRIL 2017 VOLUME 23 SUPPLEMENT 3

Clinical and Investigative Endocrinology and Diabetes

2017 AACE ANNUAL SCIENTIFIC & CLINICAL CONGRESS ABSTRACTS

ABSTRACT CATEGORIES
1 Adrenal Disorders 121 Obesity
29 Diabetes Mellitus/Prediabetes 131 Other
83 Hypoglycemia 161 Pituitary Disorders/Neuroendocrinology
95 Lipid/Cardiovascular Disorders/Hypertension 227 Reproductive Endocrinology
99 Metabolic Bone Disease 247 Thyroid Disease

OFFICIAL JOURNAL OF
THE AMERICAN ASSOCIATION OF CLINICAL ENDOCRINOLOGISTS AND
THE AMERICAN COLLEGE OF ENDOCRINOLOGY • WWW.AACE.COM
 ABSTRACTS – Adrenal Disorders
months she agreed to surgery and underwent laparoscopic
adrenalectomy after withholding mifepristone treatment
for 2 weeks. She was discharged on dexamethasone
replacement. Post surgery dexamethasone was tapered
in 2 weeks with no symptoms. After steroid withdrawal
AM cortisol was 11-13mcg/dL, and ACTH was 70-98pg/
ml. She has continued to lose weight intentionally with no
recurrence of hyperglycemia, hypertension or hypokalemia.
Discussion: Prolonged steroid therapy with slow tapering
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takes months before recovery of normal function in the
remaining adrenal gland. This case demonstrated very
rapid recovery of normal adrenal function after pre-
treatment with Mifepristone. Treatment was both effective
and well tolerated
Conclusion: Mifepristone therapy for non ACTH
dependent Cushing syndrome is effective therapy for
hypercortisolism and appears to offer the advantage of
rapid recovery of the pituitary adrenal axis after surgical
treatment of the disease. Larger scale investigation this
approach may be warranted.
Abstract #134
PEDIATRIC CUSHING DISEASE: DESMOPRESSIN
VERSUS OVINE CRH STIMULATED INFERIOR
PETROSAL SINUS SAMPLING
Henry Rohrs, MD
University of Florida
Case Presentation: An 8 year, 7 month old male was
referred for evaluation of precocious puberty. He
developed pubic hair, body odor and acne at 7 years of
age. During the previous year he had gained 10 kg and his
height deviated from the 90th to 75th percentile. Physical
examination revealed hypertension, height +0.64 SD,
weight +2.95 SD, BMI +2.68 SD, plethoric moon face,
Tanner 3 pubic hair, 5 mL testicles bilaterally, and mild
facial acne. Cortisol level drawn at 1430 was 16.4 mcg/dL
and testosterone was 14 ng/dL.
Presentation was concerning for Cushing syndrome and
1 mg overnight dexamethasone suppression test was
performed with AM cortisol 2.0 mcg/dL and ACTH 15
pg/mL. Despite the indeterminate results, over a 3 month
period he gained an additional 4.1 kg without height
gain and appeared more Cushingoid. A 48-hour low dose
dexamethasone suppression test was performed; baseline 8
am cortisol was 22.1 mcg/dL, 48-hour cortisol was 5.8 mcg/
dL, and ACTH was 29 pg/mL. With biochemical evidence
of Cushing disease, MRI of the brain was performed
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right lateral aspects of the pituitary. A microadenoma was
- 20 -
not visualized, but could not be excluded.
Inferior petrosal sinus sampling (IPSS) was performed to
further evaluate the patient. Notably, our institution is now
using desmopressin in place of ovine corticotropin-releasing
hormone (oCRH) as the stimulant for IPSS. The results
were negative for microadenoma. Clinical and biochemical
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at the NIH using oCRH revealed increase of ACTH in the
right IPS from 162 pg/mL to 1250 pg/mL at 3 minutes with
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diagnosis of Cushing disease (stimulated ACTH IPSS/
peripheral ratio greater than 3 suggestive of pituitary source
of ACTH). He then underwent transsphenoidal surgery
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of his Cushing disease.
Conclusion: Following nationwide shortage of oCRH,
desmopressin was used as an alternate stimulant to
perform IPSS. While desmopressin stimulated IPSS has
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adult studies and case reports in pediatrics have suggested
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Desmopressin stimulated IPSS failed to detect Cushing
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,366 FRQ¿UPHG WKH GLDJQRVLV $GGLWLRQDO VWXGLHV RI
desmopressin as a stimulant for pediatric IPSS are
warranted and clinicians should be aware of the potential
lack of sensitivity of desmopressin stimulated IPSS.
Abstract #135
AN UNUSUAL CASE OF ALDOSTERONE-
PRODUCING ADENOMA PRESENTED WITH
SEVERE HYPOKALEMIA, MYOPATHY, RHAB-
DOMYOLYSIS, AND DEVELOPED RENAL
FAILURE RESULTING FROM MYOGLOBINURIA.
Mohammed Ahmed, MD, FACP, MACP, Ashwag Alqahtani,
MD, Abdulaziz Alroshodi, MD, Balsam Bohlega, MD,
Muhammad Butt, MD
King Faisal Specialist Hospital & Research Centre
Objective: We present a case of Aldosterone-producing
Adenoma (APA) with profound hypokalemia, severe
myopathy, who developed acute renal failure due to
myoglobinuria resulting from rhabdomyolysis. She was
treated initially medically with resolution of renal failure,
followed by resection of adenoma, resulting in resolution
of HTN, hypokalemia and myopathy.
Results: A 35 yrs. old lady presented with BP of 180/120,
and myopathy. Labs: serum K+ 1.7 mmol/l, Na+ 146
mmol/l, metabolic acidosis pH 7.53, HCO3 40, CK
5061 IU/l , plasma renin activity < 1 ng/ml/hr, plasma
aldosterone 693 pmol/l (Ref. value up to 582), abnormal
 ABSTRACTS – Adrenal Disorders
aldosterone renin ratio 36.2. CT showed right adrenal
adenoma measuring 28x12mm with Hounsfield units of 30,
70 and 46 at baseline, 1, and 10 minutes post IV contrast
injection respectively consistent with a benign adenoma
using percentage loss of enhancement relative to the amount
of initial enhancement. Initial renal functions were within
normal limits (creatinine 47 umol/l, GFR >60), followed
rapidly by acute renal failure (creatinine 142, GFR 37) due
to severe muscle necrosis, abnormal urinary myoglobin
2600 resulting in renal papillary necrosis. Patient was treated
using spironolactone, aggressive IV hydration, IV and oral
K+. She recovered from renal failure with normalization
of serum K+ to 3.9. Following stabilization, resection of
adenoma was done for a large 3x2x1.3 cm adenoma. she
has maintained normal BP and normokalemia.
Discussion: Distinctly unusual features of our case
included a large but benign adenoma, severe hypertension,
profound hypokalemia causing muscle necrosis as
evidenced by abnormal CK. Muscle necrosis resulted in
myoglobinuria causing renal papillary necrosis and acute
renal failure (ARF). Protracted severe hypokalemia results
in rhabdomyolyisis. There have been at least 16 published
cases linking rhabdomyolysis to hypokalemia. This
associated is reported in cases of Barter’s and Gitelman’s
syndrome and congenital tubular acidosis as well. With
aggressive medical management ARF got reversed,
providing the opportunity for definitive surgical resection
of the adrenal adenoma resulting in cure. The case
underscores obviating the need to undertake confirmatory
test for APA given undetectable plasma renin, very high
aldosterone, an abnormal aldo/renin ratio and clearly
defined adenoma on imaging.
Conclusion: When rhabdomyolysis occurs in conjunction
with hypokalemia, hypertension and metabolic acidosis,
aldosterone-producing adenoma is a strong suspect.
It should warrant aggressive management to prevent/
reverse acute renal failure and correction of metabolic
abnormalities, followed by resection of the adenoma
whenever feasible.
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Abstract #136
IATROGENIC CUSHING`S SYNDROME PRE-
SENTING SEVERE ATIPICAL SKIN LESIONS
EXTENDED IN THE BODY
Maria Ramos Guifarro1, Jhenny Chavez1, Daniel Guifarro2,
Erick Martinez2, Ariel Figueroa2, Incia Orellana2
1. Hospital Escuela Universitario, 2. Universidad Nacional
Autónoma de Honduras
Results: Cushing’s syndrome comprises a wide variety of
signs and symptoms, that are result of a high exposure of
body tissue to glucocorticoids. The most common cause
is iatrogenic from prescribed corticosteroids. Skin tends
to become thin, fragile, and more susceptible to bruises
and infections, wounds heal poorly, and striae appear
in areas of weight gain. Although some skin lesions are
not rare, it is not common to see widespread skin lesions
in these patients. We describe the case of a 61-year-old
female patient with history of high blood pressure and
rheumatoid arthritis, and generalized tremor associated
with a cerebellar vascular event; she had been receiving
intramuscular betamethasone every fifteen days for the
last 3 years. The patient had extremely thin and fragile
skin, presented with widespread lesions with ecchymosis,
ulcers and necrosis located in arms, forearms, chest and
legs. Her family referred that the patient had progressive
muscular weakness that prevented her from walking,
and had been bedridden for 2 months; associated with an
altered mental status for the last two weeks. She arrived
into our emergency service with a 2-day history of a very
intense headache, nausea, vomiting and fever. Blood
pressure (BP) was not audible, cardiac frequency was
120 bpm, neurological examination was normal except
for a 3/5 muscle strength according to the oxford scale,
plantar response was indifferent. The abdomen presented
striae, telangiectasia and central adiposity. White blood
cells: 22,300, 83% neutrophils, 11% lymphocytes, with
increased CSF proteins 474 mg/dl, glucose 84 mg/dl, 57
cells (70% monocytes), creatinine: 2.18mg/dl, albumin:
1.5 g/dl, rheumatoid factor was positive, serum cortisol
was suppressed. She was diagnosed with Iatrogenic
Cushing`s syndrome, in septic shock, with possible source
of infection attributed to the skin lesions and meningitis.
Wide spectrum antibiotics were initiated (meropenem and
vancomycine), as well as norepinephrine, hydrocortisone,
and IV albumin. The skin lesions were attributed as a
severe manifestation of the Cushing`s syndrome by the
Dermatology and Endocrinology services. The symptoms
resolved within 72 hours of treatment; as she improved
a steroid tapering program was prescribed with oral
deflazacort and she was sent home.