+This lecture was conducted during the Nephrology Unit Grand Ground by Medical Student rotated under Nephrology Division under the supervision and administration of Prof. Jamal Al Wakeel, Head of Nephrology Unit, Department of Medicine and Dr. Abdulkareem Al Suwaida. Nephrology Division is not responsible for the content of the presentation for it is intended for learning and /or education purpose only.

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Ali Ibrahim Alsagheir

Addison Disease

+Index :

Introduction

ADDISON disease Definition Pathophysiology Clinical manifestation Diagnosis RX

Addison crisis

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Causes of adrenal insufficiency : primary adrenal insufficiency

((ADDISON’sDISEASE)):The problem due to a disorder of the adrenal glands themselves.

secondary adrenal insufficiency: Inadequate secretion of ACTH by the pituitary gland .

+Diff. between primary & secondary:

Primary adrenal ins. Secondary

(↑ACTH) (↓ACTH)

Glucocorticoid insufficiency Glucocorticoid insufficiency

Mineralocorticoid insufficiency

normal

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is a rare endocrine disorder, first described by British physician Thomas Addison.

1 in 100,000 people.

It occurs in all age groups and affects men and women equally.

> 90% of adrenal tissue is destroyed .

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+Etiology of Primary adrenal insufficiency :

Autoimmune

TB

HIV/AIDS

Metastatic cancer

Bilateral Adrenalectomy

Rare: amyloidosis, inta-adrenal heamorrhage, lymphoma

+Clinical manifestations of chronic adrenal insufficiency

symptoms Frequency

Weakness, tiredness, fatigue 100

Anorexia 100

Gastrointestinal symptoms 92

Postural dizziness 6 -13

Muscle or joint pains 12

+Clinical manifestations of chronic adrenal insufficiencySign Frequency, percent

Weight loss 100

Hyperpigmentation 94

Hypotension (systolic BP <110 mmHg)

88-94

Vitiligo 20

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+Clinical manifestations of chronic adrenal insufficiencyLaboratory abnormality Frequency

Hyponatremia 88

Hyperkalemia 64

Hypercalcemia 6

Azotemia 55

Anemia 40

Eosinophilia 17

+Diagnosis :

Random Plasma Cortisol: usually low

Acth Stimulation Test (short Synacthen test): 250 μg ACTH1-24 (Synacthen) by i.m. injection at any time

of day Blood samples: 0 and 30 minutes for plasma cortisol Normal subjects plasma cortisol> 460 nmol/l Inadrenal insufficiencycortisol level fail to increase.

Then see ACTH: high ((primary)) , low ((secondary))

Plasma renin and aldosterone

+Treatment:

Glucocorticoid replacement : Cortisol (hydrocortisone) is the drug of choice . 15 -25 mg/day in 2-3 divided does 2/3 in morning , 1/3 afternoon

Mineralocorticoid replacement : Fludrocortisone 0.05 – 0.2 mg/daily

Adjust both on clinical ground

+ADVICES:

Intercurrent stress: eg. Febrile illness - *2 does of hydrocortisone

Surgery: 150 -300mg parenteal hydrocortisone daily (in 3 divided

doses)

Gastroenteritis: Parenteral hydrocortisone

Instructed in the use of IM emergency hydrocortisone.

All ptn should wear a medical information bracelet.

+ADDISION CRISIS

45y/o, female, c/o anorexia, not feeling well, hyperpigmentation , lethargy, wt. loss for 1 year

Now present to the E/R with severe diarrhea and loss of consciousness

On examination: Decrease BP , dehydration, hyperpigmentation, no axillary

hair

Labs : Na = 124 , K= 5.9 , cl = 82 , HCO3= 17 , ph = 7.2

+ADDISION CRISIS

It is a medical emergency.

Untreated, an Addisonian crisis can be fatal.

therapy should be instituted immediately upon suspicion.

Precipitating factor : Infection, trauma, surgery . Or sudden withdrawal of steriods.

+Clinical manifestations :

SHOCK ((low blood pressure, tachycardia, oliguria))

sudden penetrating pain in the legs, lower back or abdomen

severe vomiting and diarrhea, resulting in dehydration

loss of consciousness

hypoglycemia

+ADDISION CRISIS

Diagnosis : Serum Cortisol, confirmation by an ACTH stimulation test should be

postponed until the patient has recovered.

RX : IV HYDROCORTISONE SUCCINATE 100 MG/6H for 48

hour ,then start oral . IV FLUID ((NORMAL SALINE AND 10% DEXTROSE )) Precipitating cause should be treated.

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