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Addison’s Disease. Nasimeh Rakhshani CC3. Adrenal Insufficiency. Addison’s Disease . Addison’s Disease . First described by Thomas Addison in 1855 His description referred to primary adrenal insufficiency At that time, the most common etiology was tuberculosis infiltration

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addison s disease

Addison’s Disease

Nasimeh Rakhshani CC3

adrenal insufficiency
Adrenal Insufficiency

Addison’s Disease

addison s disease3
Addison’s Disease
  • First described by Thomas Addison in 1855
  • His description referred to primary adrenal insufficiency
  • At that time, the most common etiology was tuberculosis infiltration
  • TB infiltration continues to be the leading cause worldwide
adrenal insufficiency ai
Adrenal Insufficiency (AI)
  • Impairment in synthesis and/or release of adrenocortical hormones
  • Classified as:
    • Primary AI  results from disease intrinsic to the adrenal cortex
    • Secondary AI results from impaired release or effect of adrenocorticotropic hormone (ACTH) from the pituitary gland
    • Tertiary AI results from the impaired release or effect of corticotropin releasing hormone (CRH) from the hypothalamus
the hpa axis
The HPA Axis

Glucocorticoids

(-)

Hypothalamus

CRH

(-)

ACTH

Adrenal Gland

clinical manifestations
Clinical Manifestations
  • In general, symptoms of AI include fatigue and GI complaints (nausea and vomiting)
  • Clinical suspicion is important because the presentation of AI may be insidious and subtle
  • Resultantly, clinical diagnosis is frequently delayed or missed early  if unrecognized, may present in a life-threatening crisis with acute cardiovascular collapse (adrenal crisis)
clinical manifestations7
Clinical Manifestations
  • Signs and symptoms of primary adrenal insufficiency vary depending on which hormones are deficient and the severity of the defects
clinical manifestations10
Clinical Manifestations
  • Adrenal Crisis
    • Hypotension or shock
      • Particularly if disproportionate to apparent underlying illness
    • Serum electrolyte and metabolic abnormalities:
      • Hyponatremia
      • Hyperkalemia
      • Metabolic Acidosis
      • Hypoglycemia
    • Vomiting and diarrhea, sometimes with severe abdominal pain
    • Unexplained fever, weight loss and anorexia
clinical manifestations11
Clinical Manifestations
  • Adrenal Crisis: When to be suspicious???
    • In neonates, presents within 1st few days-weeks of life with vomiting, diarrhea, ↓BP, ↓Na, ↑K and ↓BG
      • CAH (21-hydroxylase deficiency) most common cause
      • In females with CAH is suggested by ambiguous genitalia
    • In infants and older children with previously diagnosed AI
      • Weight loss, serum electrolyte abnormalities +/- hyperpigmentation
      • Often a history of an antecedent precipitating stress (eg, surgery or infection)
    • Bilateral adrenal hemorrhage or infarction
      • Children with hypotension and shock that fail to respond to vigorous fluid resuscitation and inotropic medications
      • Especially if have severe hyponatremia and hyperkalemia
etiology
Etiology
  • Steroidogenesis disorders: Defects within the biosynthetic pathways of glucocorticoids +/- mineralocorticoids  lead to impaired synthesis of cortisol and/or aldosterone
    • There are also drugs that inhibit cortisol synthesis (aminoglutethimide, ketoconazole, and etomidate)
  • Adrenal damage: Injury from factors extrinsic to the adrenal gland may impair adrenal function
  • Abnormal adrenal development: A lack of normal adrenocortical cell differentiation may result in adrenal hypoplasia
  • Adrenal unresponsiveness to ACTH: Defects in adrenal responsiveness to ACTH results in cortisol deficiency
  • Peroxisomal disorders: Accumulation of abnormal very long chain fatty acids within peroxisomes which may lead to adrenal impairment
etiology continued
Etiology Continued
  • Perry et al (2005):
    • 20 years of data, 103 patients <18 y/o  Primary AI
    • 73% congenital adrenal hyperplasia (CAH)
    • 13% autoimmune adrenal insufficiency
    • 5% Peroxisomal disorders
    • 3% Wolman disease (lysosomal acid lipase deficiency)
    • 1% Adrenal hypoplasia congenita
    • 1% Triple A syndrome (Unresponsiveness to ACTH)
    • 6% No diagnosis identified
diagnosis
Diagnosis
  • 3 Step Process:
    • Confirm adrenal insufficiency  demonstrating inappropriately low cortisol secretion
    • Determine whether the cortisol deficiency is primary or central AI
    • Determine the cause of the underlying disorder
diagnostic tests
Diagnostic Tests
  • Static Tests
    • Cortisol, ACTH, Adrenal Androgens
    • Mineralocorticoid status
      • Serum electrolytes
      • Plasma renin activity (PRA), direct [renin]
  • Dynamic Tests
    • Short ACTH stimulation test:
      • Serum cortisol levels are measured before and 60 minutes after the rapid IV infusion of synthetic ACTH (cosyntropin)
    • Tests of ACTH secretory ability
      • Insulin-induced hypoglycemia
      • Glucagon
      • Metyrapone test
    • CRH stimulation test: helps determine if 2o or 3o AI
1 confirming ai
1. Confirming AI
  • Initial step: measurement of serum ACTH and cortisol in the morning (8 a.m.) and in the fasting state
    • If serum cortisol is low and serum ACTH is high  likely primary AI and diagnosis can be confirmed with an ACTH stimulation test
      • If low cortisol and normal lytes the rapid ACTH stimulation test often performed simultaneously
    • If serum ACTH is also low, serum cortisol is indeterminate or pituitary disease is suspected  likely central AI  tests of ACTH secretory ability
1 confirming ai cont d
1. Confirming AI cont’d
  • In patients suspected of adrenal crisis:
    • Immediate treatment crucial and must not postpone until diagnosis confirmed
    • Draw required samples and initiate therapy (saline and GC replacement)
      • Cortisol, ACTH, electrolytes, PRA, renin, etc.
    • May perform a short ACTH stimulation test following treatment in these patients if:
      • Treatment has been given for < a few days (no adrenal suppression secondary to treatment)
      • Dexamethasone used to treat (not detected in cortisol assay unlike hydrocortisone and cortisone)
2 establish the level of defect
2. Establish the level of defect
  • Diagnosis of Primary AI:↓ amcortisol and ↑ am ACTH levels + ↓ or absent cortisol in response to ACTH stimulation test
    • Often evidence of mineralocorticoid deficiency  hyponatremia, hyperkalemia, ↑ PRA and/or ↑ [renin]
  • Diagnosis of Central AI: ↓ Basal and ACTH stimulated cortisol secretion and ↓ Basal ACTH  tests of ACTH secretory ability +/- CRF stimulation test
    • Practically, not always necessary to determine if AI is 2o or 3otreatment is often the same
    • Plasma levels of renin and aldosterone are usually unaffected in central AI
3 evaluation of cause
3. Evaluation of Cause
  • Primary AI
    • Evaluate for CAH (most common cause of 1o AI )  adrenal androgens
    • Measure adrenal antibodies  if +ive screen for autoimmune polyglandular syndromes
      • Measuring antibodies to other endocrine glands (thyroid, parathyroid, and islet cell antibodies)
    • If antibodies negative screen for other causes of 1o AI
      • Imaging (CT) identifying adrenal hemorrhage, calcifications, or infiltrative disease
  • Central AI
    • Evaluate for secretion of other pituitary hormones
treatment of adrenal insufficiency
Treatment of Adrenal Insufficiency
  • Principles:
    • Maintenance Therapy (Replacement)
    • Stressed Conditions
treatment cont d
Treatment Cont’d
  • Maintenance Therapy
    • Glucocorticoid Therapy
      • Calculated according to body surface area
      • Often hydrocortisone is preferred because of its short duration of action and low potency  ease in titration to optimal dose (5-16 mg/m2/ddivided into 3 doses)
      • During follow-up must ensure adequate somatic growth (weight, BA, height and weight velocities) and screen for symptoms of insufficiency
      • Must also screen for symptoms of GC excess
    • Mineralocorticoid Therapy
      • Fludrocortisone (Florinef)  0.05-0.2 mg/d do not vary by age or surface area because aldosterone secretion rate is nearly constant throughout the lifespan
      • Monitor for signs of inadequate replacement: dehydration, poor weight gain, salt-craving, and hyponatremia with hyperkalemia
treatment cont d24
Treatment Cont’d
  • Stress Conditions
    • Primary goal is to avoid serious consequences of an adrenal crisis  always wear identification
    • Illness:
      • Minor stress (e.g. sore throat, rhinorrhea, T < 38ºC)  may not require ∆ dose
      • Moderate stress (e.g. severe URTI)  double the GC replacement dose
      • Major stress (e.g. T > 38ºC and/or vomiting), three to four times the GC replacement dose
      • If child unable to keep down oral dose administer IM GC
    • Surgery:
      • During general anesthesia, +/- surgery, the GC requirements increases greatly
      • Protocols vary depending on nature of surgery, length of surgery, age of patient etc
      • Stress dosing is generally continued until the patient can tolerate oral intake, is afebrile, and is hemodynamically stable
thank you
Thank You!!!

Questions???