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Pediatric Endocrinology

Emergency
Rogatianus Bagus P
Presented by Jose RL Batubara

PKB V Palembang 18 Feb 2012

ADRENAL INSUFFICIENCY

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Adrenal Anatomy &


Physiology
The adrenals are endocrine organs
that sit on top of each kidney

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Each adrenal gland has two parts


Adrenal Medulla (inner area)

Secretes catecholamines which


mediate stress response (help
prepare a person for
emergencies).
Norepinephrine
Epinephrine
Dopamine
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Adrenal Cortex (outer area, encloses


Adrenal Medulla)
Secretes steroid hormones

Glucocorticoids: exert a
widespread effect on
metabolism of carbohydrates
and proteins
Mineralocorticoids: are essential
to maintain sodium and fluid
balance
sex hormones (secondary
source) PKB V Palembang 18 Feb 2012

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A person can survive without a


functioning adrenal medulla.
A functioning adrenal cortex (or the
steady availability of replacement
hormone) is essential for survival.

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The Essential Steroids


Primary glucocorticoid:
Cortisol (a.k.a. hydrocortisone)

Primary mineralocorticoid:
Aldosterone

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Cortisol
A glucocorticoid
Frequently referred to as the stress
hormone
Released in response to physiological or
psychological stress

Examples: exercise, illness,


injury, starvation, extreme
dehydration, electrolyte
imbalance, emotional stress,
surgery, etc.
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Cortisol
Critical actions on many physiologic
systems, including:
Maintains cardiovascular function
Provides blood pressure regulation
Enables carbohydrate metabolism

acts on the liver to maintain


normal glucose levels
Immune function actions

Reduces inflammation
Suppresses immune system
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Cortisol
When cortisol is not produced or
released by the adrenal glands,
humans are unable to respond
appropriately to physiologic
stressors.
Rapid deterioration resulting in
organ damage and
shock/coma/death can occur,
especially in children
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Aldosterone
a mineralocorticoid
Regulates body fluid by influencing
sodium balance
The human body requires certain
amounts of sodium and water in
order to maintain normal
metabolism of fats, carbohydrates
and proteins.
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Water/sodium balance is maintained


by aldosterone.
Without aldosterone, significant
water and sodium imbalances can
result in organ failure/death.

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Why we need cortisol


Cortisol has a necessary effect on
the vascular system (blood vessels,
heart) and liver during episodes of
physiologic stress

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Vascular Reactivity
In adrenally-insufficient individuals
experiencing a physiologic stressor,
the vascular smooth muscle will
become non-responsive to the
effects of norepinephrine and
epinephrine, resulting in vasodilation
and capillary leaking.
The patient may be unable to
maintain an adequate blood
pressure
The blood vessels cannot respond to
the stress andPKB
will
eventually
V Palembang 18 Feb 2012

Energy Metabolism
In adrenally-insufficient individuals
under increased physiologic stress,
the liver is unable to metabolize
carbohydrates properly, which may
result in profoundly low blood sugar
that is difficult to reverse without
administration of replacement
cortisol
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The speed at which patient


deterioration occurs is difficult to
predict and is related to the
underlying stressor, patient age,
general health, etc.
Young children can be at high risk for
rapid deterioration, even when
experiencing a simple
gastrointestinal disorder.
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Endocrinologist
Testimony
In adrenal insufficiency, because
of the inability to produce
glucocorticoids and often
mineralocorticoids from the adrenal
glands, there is a risk of lifethreatening hyponatremia,
hyperkalemia, hypoglycemia,
seizures and cardiovascular collapse,
in particular at times of physiologic
stress to the body, such as in injury
or illness
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Who has adrenal


insufficiency?
Anyone whose adrenal glands have stopped
producing steroids as a result of:
Long-term administration of steroids
Pituitary gland problems, including growth
hormone deficiency, tumor, etc.
Trauma, including head trauma that affects
pituitary
Loss of circulation to adrenals/removal of tissue
Auto-immune disease
Cancer and other diseases (TB and HIV may
cause)
There is also an inherited form of adrenal
insufficiency
(CAH)
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Adrenal Insufficiency
Can occur from long-term
administration of steroids (over-rides
bodys own steroid production)
Examples:

Organ transplant patients


Long-term COPD
Long-term Asthma
Severe arthritis
Certain cancer treatments
PKB V Palembang 18 Feb 2012

Primary Adrenal
Insufficiency= Addisons
Disease
The adrenal glands are damaged
and cannot produce sufficient
steroid
80% of the time, damage is caused
by an auto-immune response that
destroys the adrenal cortex
Addisons can affect both sexes and
all age
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Addisons symptoms
This disease has a gradual onset and
can be difficult to diagnose:

Chronic, worsening fatigue


Weight loss
Muscle weakness
Loss of appetite
Nausea/vomiting
Low blood pressure
Low blood sugar
Skin hyperpigmentation
Salt-craving

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Acute manifestation of
Addisons is called Addison
Crisis
Severe vomiting/diarrhea
Dehydration
Hypotension
Sudden, severe pain in back, belly or
legs
Loss of consciousness
Can be fatal

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Presentation of Adrenal
Crisis
The patient may present with any
illness or injury as the precipitating
event.
A patient history of adrenal insufficiency warrants a
careful assessment under specific protocols
Children may deteriorate into adrenal crisis from a
simple fever, a gastrointestinal illness, a fall from a
bicycle or some other injury.
A mild illness or injury can easily precipitate
an adrenal crisis in any age group
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Critical Clinical
Presentation
The early indicators of an adrenalcrisis onset can be vague and nonspecific. Some or all signs/symptoms
may be present.
Infants:
Poor appetite
Vomiting/diarrhea
Lethargy/unresponsive
Unexplained hypoglycemia

Seizure/cardiovascular collapse/death
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Critical Clinical
Presentation
(not all S&S may be
present)
Older Children/Adults
Vomiting
Hypotensive, often unresponsive to
fluids/pressors
Pallor, gray, diaphoretic
Hypoglycemia, often refractory to D50

May have neurologic deficits


Headache/confusion/seizure
lethargy/unresponsive

Cardiovascular collapse
Death
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Clearly, the signs/symptoms of


adrenal crisis are similar to other
serious shock-type presentations.
For these patients, standard shock
management requires
supplementation with corticosteroid
medication (Solu-Cortef or SoluMedrol)
It is important to ANTICIPATE the
evolution of an adrenal crisis and
medicate appropriately under the
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2012
specific protocols.
Do not
until a

Patient Management
Follow standard ABC and shock
management treatment.
BLS/ILS: notify ALS intercept as soon
as possible; transport without
delay
ALS: administer steroid IM/IV/IO as
soon as possible after initial lifethreat and shock management have
been initiated.
Transport without delay to
appropriate hospital
with
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2012

It is important to note that you are


caring for a patient with multiple
issues:
1. The precipitating event (a trauma/illness that
may be a critical issue on its own)
and
2. The evolution towards adrenal crisis, which
will result in organ failure/death if not
reversed.
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This phrase has been added to


Paramedic Standing Orders in
certain ADULT treatment protocols:
For patients with confirmed adrenal
insufficiency, give hydrocortisone
100 mg IV, IM or IO OR
methylprednisolone 125 mg IV, IM or
IO
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PEDIATRIC Protocol
Updates
This phrase has been added to
Paramedic Standing Orders in
certain PEDIATRIC protocols:
For patients with confirmed adrenal
insufficiency, give hydrocortisone
2mg/kg to maximum 100 mg IV, IM
or IO OR methylprednisolone
2mg/kg to maximum 125 mg IV, IM
or IO
PKB V Palembang 18 Feb 2012

Please define Confirmed


Adrenal Insufficiency
Confirmation of a pediatric patients
condition is determined by the
presence of a medic-alert
bracelet/necklace, OR by the child,
parent or care provider verbally
confirming a history of adrenal
insufficiency
In a school or daycare setting, it is
acceptable for the school nurse or
daycare provider to relay this
information toPKB
you
V Palembang 18 Feb 2012

Solu-Cortef
Indications: replacement of absent
corticosteroid in identified adrenallyinsufficient patients being managed
under specific treatment protocol;
many other uses as well
Contra-Indications: Do not use in the
newly-born or any individual with a
known hypersensitivity to SoluCortef
PKB V Palembang 18 Feb 2012

Solu-Cortef
Side Effects: in emergency use,
transient hypertension and/or
headache, sodium/water retention
may occur. Not usual in a 1-time
dose
Dosage: Adult:
100 mg IV, IM,
IO
Pediatric:
2 mg/kg to a
max of
100 mg, IV, IM, IO
Protect from heat
PKB V Palembang 18 Feb 2012

Solu-Cortef
Administration route: IM or slow IV
bolus. Give IV Bolus over 30
seconds. IV infusion is not
acceptable for emergency
administration
For young children, the preferred IM
site is the vastus lateralis muscle

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Onset of action: for the indicated use


(emergency steroid replacement in
patient experiencing stressor) the
onset of action is minutes. Do not
delay transport.

PKB V Palembang 18 Feb 2012

Solu-Medrol
Indications:Protocol: replacement of
absent corticosteroid in identified
adrenally-insufficient patients being
managed under specific treatment
protocol; Other: many uses,
including acute bronchial asthma
(not first-line); anaphylaxis (not
first-line); acute exacerbation of
multiple sclerosis
Contraindications: any patient with
systemic fungal infection, any
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person with known
hypersensitivity

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Solu-Medrol
Dose: Adult: 125 mg IM/IV/IO
Pediatric: 2mg/kg to a max
of 125 mg
IM/IV/IO
Administration route: IM or slow IV
bolus. Give IV Bolus over 30
seconds. IV infusion is not
acceptable for emergency
administration
For young children, the preferred IM
PKB V Palembang
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2012
site is the vastus
lateralis

Solu-Medrol
Onset of action: for the indicated use
(emergency steroid replacement in
patient experiencing stressor) the
onset of action is minutes. Do not
delay transport.

PKB V Palembang 18 Feb 2012

Thankyou

PKB V Palembang 18 Feb 2012

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