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Adrenal Insufficiency Adrenal Insufficiency

Adrenal Insufficiency - PowerPoint Presentation

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Adrenal Insufficiency - PPT Presentation

Haley Minnehan MD Adrenal Insufficiency Definition A disease state that is caused by lack of glucocorticoidsGC andor mineralcorticoids MC by interruption at any level of the hypothalamuspituitaryadrenal axis ID: 545953

cortisol adrenal treatment primary adrenal cortisol primary treatment acth day dosing stress aldosterone pituitary level crisis hydrocortisone life surgery failure hours secondary

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Slide1

Adrenal Insufficiency

Haley

Minnehan

, MDSlide2

Adrenal Insufficiency Definition

A disease state that is caused by

lack of glucocorticoids(GC) and/or

mineralcorticoids

(MC)

by interruption at any level of the hypothalamus-pituitary-adrenal axis.Slide3

Hypothalamus-Pituitary-Adrenal AxisSlide4

Adrenal Insufficiency

Primary?

Secondary?

Tertiary?Slide5

Primary(

Addisons

)

Destruction

Of the Adrenal

CortexSlide6

Primary=Primary Gland Failure = No cortisol or MC

1. Autoimmune

>Most Common

>Lymphocytic infiltration destroys entire adrenal cortex

>Antibodies are detectable

2. Infectious

TB

CMV

Histoplasmosis

3. Drugs

Ketoconazole

Rifampin

EtomidateSlide7

Primary Causes continued…..

4.Deposition Diseases

Sarcoidosis

Amyloidosis

Hemochromatosis

5. Metastatic Disease

6. Congenital Adrenal Hyperplasia

7. Adrenal Surgery

8. Bilateral adrenal gland hemorrhageSlide8

Secondary= Lack of ACTH

=Pituitary ProblemSlide9

Secondary Causes

Autoimmune -

Lymphocytic

hypophysitis

Postpartum hemorrhage-Sheehan’s syndrome

Head Trauma

Tumor

Infiltrative Process

Pituitary surgerySlide10

Tertiary Causes

1

. Exogenous

Glucocorticoids

Prolonged

therapy that is withdrawn

2.

Megestrol

Has

some GC

properties

so will affect the axis

3. Opiates

Affects

the axis at the CRH level.Slide11

What does cortisol do?

MAINTAINS GLYCOGEN (cause of hypoglycemia)

REGULATES IMMUNE FUNCTION (more infections)

PART OF HEPATIC NEOGENESIS (gut absorption, nausea, diarrhea, vomiting)

VASCULAR TONE (cause of hypotension)Slide12

What does MC =Aldosterone do?

Regulates Na and K at the level of the kidney/ renin-angiotensin system

(Very little aldosterone secretion comes from ACTH stim)

Lack of aldosterone as in Primary AI

a) renal wasting of Na

b) retention of K

c)volume loss >>severe intravascular depletion>>>

hyptotension

and shockSlide13

Renin-Angiotensin-Aldosterone LoopSlide14

What does AI feel like?Slide15

Chronic AI Symptoms

GI

Unexplained abdominal pain

Weight loss

Chronic Nausea

Constipation

Vomiting

CNS

Headache

Cognitive clouding

Hypersomnia

Depression

Anxiety

OTHER

Fatigue

Weakness

Chills

Recurrent

infxns

Tan skin (Primary)

HypotensionSlide16

Normal vs SAI Cortisol PatternSlide17

JFK hadAddisons

Hyperpigmented

Chronically ill

How sick was JFK?Slide18

Acute AdrenalCrisis

Always be in your differential of SHOCK

Look

foR

:

1.Hypoglycemia

2. Acidosis

3. Hyponatremia

4. Hyperkalemia

Primary=

low NA, High KSlide19

ACUTE ADRENAL CRISIS CAUSES

#1 IS

gi

FROM VOMITING/DIARRHEA

SURGERY

HEAT

EMOTIONAL DISTRESS

TRAUMA

PREGNANCY

INFECTIONSlide20

ACUTE ADRENAL CRISIS TREATMENT

STEROIDS AND FLUIDS

100 mg IV Hydrocortisone stat then Q8 hours x 24 hours

Normal Saline

Correct hypoglycemia D5 with 100 mg IV HC if neededSlide21

Laboratory Diagnosis 8-9 am cortisol with ACTH

Cortisol Level:

>18 mcg/dl excludes AI

<3 is virtually diagnostic

< or =10 is suggestive and should start therapy before get further testing

11-18 hold therapy and obtain ACTH stim test (

cosyntropin

)

Sepsis workup without AI baseline cortisol < or = to 10 think AI

ACTH Level:

>or = to 22 is virtually diagnostic of primary AISlide22

ACTH STIM TEST (Cosynotropin)

Give 250 mcg IV

Cosynotropin

Measure serial cortisol serum levels at 30 and 60 minutes

Cortisol < 5 = Adrenal Failure

Cortisol >20 = Normal

Cortisol 5-20 = Pituitary FailureSlide23

Goals of Treatment

#1

Physiological

Replacement

Of GC/MC

#2

Improve QOL

#3

Prevent

Adrenal

CrisisSlide24

Glucocorticoid Replacement Options

Hydrocortisone

Prednisone

dexamethasoneSlide25

Glucocorticoids are not all created equal

None are ideal in avoidance of risks of over or under replacement

Most physiological =

hc

Prednisone potency= 4xHC

Dexamethasone=no effect on vascular tone last 24-36

hrsSlide26

Chronic AI Treatment

Primary

Hydrocortisone 20-30 mg/day

Most physiologic

Divide doses up to TID

10/5/5=7am/12 pm/5 pm

Fludocortisone

0.05-0.1 mg/day

Secondary

Hydrocortisone 10-25 mg/day

Divide doses up to TID

7.5/5/2.5 (all combinations)

No need for

fludocortisone

because aldosterone not under ACTH influenceSlide27

Chronic AI Treatment cont…

Tertiary

Removal of the offending drug with a taper if possible (reversible)

Treat with maintenance HC if not able to d/c drug

As little as 20 mg/day prednisone for 7 days can cause AI

Be suspicious in asthmatics, COPD, rheumatology ptsSlide28

Hydrocortisone

GOOD

Restores physiology and relieves some symptoms

Nausea, weakness, headache, abdominal pain, hypotension

Allowed longer life span of AI patients

Prior to GC pts lived max 2 years after diagnosis

BAD

Cannot replace the physiology-circadian rhythm

ie

TIMING

Side effects: depression, irritability, insomnia, bone metabolism

Short acting- peaks at 1-2 hours then rapid decline until next dose at

hr

4-5

Absorption rate variableSlide29

Treatment is Challenging

Physiological demands change throughout the day depending on the day.

No objective measurement of cortisol that is “normal” for that individual.

Ideal world =

fingerstick

rapid test so with symptoms know what to treat

Symptoms checklist:

bp

, fluids, low blood sugar, stress not

accouneted

for, infection, sleep deprivedSlide30

Treatment is often trial and error

Despite treatment morbidity is high and life expectancy is reduced

WHY? Non-physiological nature of replacement therapy

Compliance with twice-three times daily dosing is difficult

Overexposure to GC> 30 mg per day=Cardiovascular Complications, Osteoporosis, Infections, Glucose Intolerance, Insomnia, Obesity

Underexposure= Infections, adrenal crisis, feel “post call” all the time, hypersomnia, significant impairment in physical, emotional and cognitive functioning affecting work, family, social Slide31

Stress Dosing!

Ideal to “anticipate” the upcoming stressful event

Travel, holidays, call, prolonged exercise (football game), emotional distress, illness, surgery, pregnancy

Double or Triple the maintenance dose for 3 days then taper for 3 days until reach maintenance doses

Challenging- again

How much?

How long?Slide32

Stress dose for prolonged exerciseSlide33

Patient and Family Education on Management

Life threatening disease that requires lifelong replacement

Educate and Reiterate the importance of stress dosing and how to avoid v adrenal crisis

ID Emergency bracelet>>”Adrenal Failure- Need steroids”

Emergency kit with 100 mg HC vials, needles, syringes for vomiting/diarrhea or other illness and cannot keep oral down

Give

Prevnar

and Pneumovax to prevent recurrent sinusitis/bronchitisSlide34

Despite treatment….

Patients report poor QOL

Worldwide survey of 1245 pts:

64%

reported compromised health status

40%

missed school or work in last 3 months

38%

hospitalized in last

yr

25%

DisabilitySlide35

Patients need support

Support groups

NADF (National adrenal disease foundation)

Website:

www.nadf.org

provides information on emergency kits, patient information, newsletter and online support group- inspire.com

Quality of Life Discussions:

stress, exercise, routine, dosing compliance, sleep (no dosing after 6 pm), relationships, family life, work

Journaling

dosing times,

bp

, hours of sleep, stress

Slide36

What is in the Pipeline?

Cortisol pumps

Long acting/IR CortisolSlide37

Adrenal Insufficiency after 4 years of TreatmentSlide38

Thank you IAFP.