Consultation note
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Dr. ___________ has asked for consultation on this patient regarding regarding _________.

Thank you for inviting us to see this patient.

Chief complaint: ___ year old male/female presents today for evaluation and treatment of _______. Patient complains of the following:
menstrual irregularity (oligomenorrhea), bruisability, mental disorders (depression), muscle weakness (wasting), frequent infections, poor wound healing, hemorrhagic diathesis, skin fragility, psychosis, emotional lability, weight gain, hypogonadism, infertility

My key findings of the ROS, Past Hx, Family Hx, and social Hx are:
ROS: Review of 12 systems was negative with exception of those things mentioned above in the HPI and weightloss/weight gain, headache, fever, chills, heat/cold intolerance, chest pain, dyspnea, cough, sinus/allergy complaints, frequent infections, nausea, vomiting, diarrhea, constipation, erectile dysfunction, genitourinary complaints, muscle aches or pains, numbness or tingling, easy bruisability or bleeding, hair changes/alopecia, skin changes/ulcers, tremors, depression or anxiety.

Patient denies weightloss/weight gain, headache, fever, chills, heat/cold intolerance, chest pain, dyspnea, cough, sinus/allergy complaints, frequent infections, nausea, vomiting, diarrhea, constipation, erectile dysfunction, genitourinary complaints, muscle aches or pains, numbness or tingling, easy bruisability or bleeding, hair changes/alopecia, skin changes/ulcers, tremors, depression or anxiety.

Past Medical History:

Past Surgical History:

Family History:

Social History:
Diet:
Exercise:
Smoking:
Recreational Drug use :
Education:
Occupation:
Lives with ____ where ____

Home Meds:

Allergies:

My key findings of this patient's Physical Exam are:
VITALS:
Pulse:
Blood Pressure:
Respirations:
Temperature:
Weight:
Height:
BMI:

GENERAL: sitting up/reclined in bed, awake, alert, and oriented, truncal obesity (centropedal, spares arms), hypertension, osteoporosis
HEAD: normocephalic, atraumatic, no temporal wasting, negative chvostek sign, moon face (with facial plethora)
NOSE, MOUTH: lips and mucous membranes are moist, normal color of buccal mucosa
EYES: EOMI, PERLA, no proptosis
NECK: supple, thyroid normal in size, thyroid small, thyroid low set, no thyromegaly or masses, buffalo hump, supraclavicular fat pads
RESPIRATORY: clear to ascultation bilaterally
CARDIOVASULAR: S1S2, no murmurs/rubs/gallops
GI: soft, non-tender, + bowel sounds, red-purple abdominal cutaneous striae
LYMPHATIC: no cervicoclavicular adenopathy appreciated
MUSCULOSKELETAL: moving all extremities
EXTREMITIES: no edema present, edema, muscle wasting with proximal myopathy
NEUROLOGIC: monofilament sensed in all areas of the foot, vibratory sensation intact, fine/crude touch intact, pulses palpable, DTRs 2+, CN grossly intact
DERMATOLOGY: no xanthomas appreciated, no skin ulcers, no calluses, no onchyomycosis/dermatophyosis, no striae, hirsutism, acne, thinning of skin, ecchymoses
PSYCHIATRY: patient answers questions appropriately, appropriate affectcoherent thoughts without flight of ideas

Labs:

Assessment:

Causes:
Over- or non-functioning adenoma of adrenal cortex.
Pathogenesis:
No or prolonged overproduction of adrenal hormones.

Diagnostic Plans:
The major tests to rule out Cushing's syndrome are 1 mg dexamethasone suppression test (1 mg DST) and 24-hour urinary free cortisol (UFC). Typical clinical use is to rule out Cushing's syndrome in patients with obesity and hypertension or with adrenal incidentalomas. If Cushing's syndrome not ruled out, 48-hour 2 mg DST is used as a confirmatory test. If Cushing's syndrome confirmed, further testing used to distinguish (1) adrenal overproduction of corticosteroids from (2) pituitary (Cushing's disease) or (3) ectopic overproduction of adrenocorticotrophic hormone (ACTH).
Overnight (rapid, 1 mg) dexamethasone suppression test (1 mg DST). It is often used as the initial test due to simplicity and because cortisol suppression effectively rules out Cushing's syndrome (excellent negative predictive value), it is done by administering dexamethasone 1 mg PO at 11 pm, and measuring plasma cortisol at 8 am. Plasma cortisol < 5 mcg/100 mL makes Cushing's syndrome very unlikely, failure to suppress endogenous cortisol (false positive tests) can also be seen in obesity, agitated depression, severe stress, alcoholism, anorexia nervosa, oral contraceptives, and some other medications, rifampicin 600 mg/day caused false positive 1 mg DST in all 16 of 16 healthy volunteers (J Clin Endocrinol Metab 1992 Jul;75(1):315), carbamazepine associated with false positive 1 mg DST reported in 2 case reports (BMJ 2005 Feb 5;330(7486):299).
If failure to suppress cortisol after 1 mg DST, usual next step is 24-hour urinary free cortisol (UFC). It has a 95-100% sensitivity and 94-98% specificity for Cushing's syndrome and 3 or more determinations are often needed.
48-hour low-dose (2 mg) dexamethasone suppression test (2 mg DST) is performed by administering dexamethasone 0.5 mg every 6 hours for 48 hours (8 doses) and then measuring
24-hour urinary free cortisol or plasma cortisol, 24-hour urinary 17-hydroxysteroids (17-OH), and 24-hour urinary creatinine (adequacy of collections indicated by < 10% variability in day-to-day creatinine excretion.
A normal response would be: 24-hour urinary free cortisol < 20-30 mcg, plasma cortisol < 5 mcg/dL, 24-hour urinary 17-OH < 3-3.5 mg. Abnormal response suggests Cushing's syndrome with a 69% sensitivity and 74% specificity. Due to variability among tests, some authors suggest a regimen with four consecutive 24-hour urine collections
two consecutive 24-hour urine specimens before starting 2 mg DST (e.g. 8 am to 8 am), then two consecutive 24-hour urine collections during 2 mg DST (e.g. 8 am to 8 am collections, with dexamethasone 0.5 mg every 6 hours starting at 8 am).
48-hour high-dose (8 mg) dexamethasone suppression test can be performed by administering dexamethasone 2 mg PO every 6 hours for 48 hours, then measuing a 24-hour urinary free cortisol (UFC) or plasma cortisol, 24-hour urinary 17-OH, and a plasma ACTH. Suppression suggests pituitary disease, but can occur with some ectopic tumors. In pituitary excess (Cushing's disease), ACTH - 50% normal, 50% increased. Suppression of UFC to < 10% suggests pituitary-dependent Cushing's disease with 70% sensitivity and 100% specificity, some pituitary ACTH-secreting tumors doe not suppress UFC. Partial suppression of 24-hour urinary 17-OH < 50% and partial suppression of plasma cortisol < 10 mcg/dL. In adrenal Cushing's syndrome, no suppression measuring urinary 17-ketosteroids (17-KS) can help distinguish adenoma from carcinoma (normal in adenoma, > 30 mg/24 hour in carcinoma)
of limited value in ectopic ACTH source, 10% ectopic tumors show suppression
Basal ACTH is best way to discriminate ACTH-dependent (normal or high ACTH levels) and ACTH-independent (suppressed ACTH) Cushing's syndrome. 2-site immunoradiometric assay has high sensitivity and specificity, but fails to recognize some ACTH variants from ectopic sources. Single site assay may detect these ectopic ACTH variants. Samples must be collected in plastic tubes containing EDTA and proteinase inhibitor and kept frozen.
ACTH < 5 pg/mL establishes diagnosis of primary adrenal disease - order abdominal CT and proceed to adrenalectomy if mass present. If elevated ACTH, 90% chance of pituitary origin, 10% ectopic. Pituitary tumors often < 5 mm and may be difficult to identify even with high-resolution CT and MRI.
Other diagnostic approaches:
Corticotropin releasing hormone (CRH) stimulation test: pituitary ACTH sources more likely than ectopic sources to have CRH receptors and exhibit exaggerated ACTH responses to CRH, CRH stimulation and 48-hour DST are the main tests to differentiate pituitary from ectopic ACTH.
An overnight high-dose dexamethasone suppression test measure baseline cortisol (7 am), give dexamethasone 8 mg PO at 11 pm, measure cortisol at 7 am. Suppression of cortisol > 50% baseline is normal with a 92% sensitivity, 100% specificity, 93% accuracy.
Continuous IV dexamethasone (1 mg/hour) for 7 hours is a positive test defined as cortisol decrease > 190 mmol/L. False test results in patients with CRH-secreting tumors.
Metyrapone test: measure plasma 11-deoxycortisol at 8 am, at midnight give metyrapone 30 mg/kg (maximum dose 2 g), measure plasma 11-deoxycortisol at 8 am. There will be an increased 11-deoxycortisol in Cushing's disease. The alternative approach is to measure 24-hour urinary 17-OH or 11-deoxycortisol on the day of and day after metyrapone 750 mg every 4 hours for 6 doses or 500 mg every 2 hours for 12 doses.
Jugular ACTH, especially inferior petrosal sinus sampling reveals increased ACTH in patients with pituitary adenoma inferior petrosal/peripheral ACTH ratio > 2 in Cushing's disease (< 1.5 if ectopic ACTH)
only indicated if biochemical and radiographic data are non-diagnostic.

Rule out:
Must also consider iatrogenic Cushing's syndrome, adrenal carcinoma. Adrenal incidentaloma may be benign in adults, but should be evaluated fully in children (using adrenal tumor resection if necessary) to rule out neuroblastoma, pheochromocytoma and adrenal carcinoma (Pediatric Surgery Update 2005 Feb;24(2):1)
Testing to consider:
CT scan of adrenals with multiple small tomographic cuts
Suggestive blood tests can show high cortisol, low ACTH, hyperglycemia, impaired glucose tolerance, hypernatremia, hypokalemia, hypochloremia, metabolic alkalosis, hypercholesterolemia

Imaging studies:
CT or MRI shows > 90% of lesions especially if > 1 cm (most are large tumors). Size alone is inadequate for predicting malignancy of adrenal masses; study of 76 patients who had resection of adrenal tumor and whose main differential diagnosis of adrenal mass was adrenal cortical carcinoma vs. benign tumor, pheochromocytomas were excluded; of 38 adrenal cortical carcinomas, 5 (13%) were < 5 cm and 4 of these had malignant features on CT scan, the other was 4 cm; of 38 benign tumors, 10 (26%) were > 5 cm; authors suggest that observation is acceptable for masses that are hormonally inactive, have benign features on CT and are < 4 cm in diameter (Surgery 2000 Dec;128(6):973 in J Watch 2001 Feb 1;21(3):26).
Radioisotope scanning

Therapeutic Plans:
Aminoglutethimide?

Surgery:
Surgical resection of affected adrenal, unilateral adrenalectomy, by flank approach. Glucocorticoid replacement treatment for approximately 9-12 months after surgery.
Laparoscopic adrenalectomy promoted as procedure of choice for adrenalectomy except in patients with invasive carcinomas or with tumors > 15 cm, based on observational study of 97 consecutive procedures showing less blood loss and shorter hospital stay than historical controls.

Patient Education:
Return to Clinic:
We have discussed our diagnostic and treatment plans with the patients and family/friends that were present. All questions have been answered to the patient's satisfaction.

Thank you for inviting to see this consult!

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