ADDISON DISEASE

CHRONIC
ADRENOCORTICAL
INSUFFICIENCY INSUFFICIENCY
By Hj. Muaeni, SKp, M.Kep
A. DEFINISI
Is a disorder resulting from
destruction or dysfunction of
the adrenal cortexd. The result
is chronic deficiency of
Hj. Muaeni, SKp, M.Kep
is chronic deficiency of
cortisol, aldesterone and
adrenal andugens ADDD
Primary, cocondary (a lack of
pituitary ACTH).
B. ETIOLOGI
Autoimmune destruction of the adrenals.
This is the most common cause,
accounting for about 80% of
spontaneous cases (tierney et all, 2001).
It may occur alone, or as part of a
Hj. Muaeni, SKp, M.Kep
It may occur alone, or as part of a
polyglandular autoimmune syndrome
(PGA). Type 2 PGA is seen in adults,
often associated with autoimmune
thyroid disease (usually
hypothyroidesm), type 1 diabetes,
primary ovarian or testicular failure, and
pernicious anemia.
Lanjutan...
Clients who are taking
anticoagulants, have major trauma,
or are having open haert surgery.
Such clients may have bilateral
adrenal hemorrhage.
Hj. Muaeni, SKp, M.Kep
adrenal hemorrhage.
Adrenoleukodystrophy, an X-linked
disorder characterized by an
accumulation of very long chain
fatty acids in the adrenal cortex,
testes, brain, and spinal cord.
Lanjutan....
ACTH deficit resulting from pituitary
tumors, pituitary surgery or irradiation,
and the use of exogenous steroids.
Clients who are abrupthy withdrawn
from long-trem high dose steroid
Hj. Muaeni, SKp, M.Kep
from long-trem high dose steroid
therapy. Other client at risk are those
with tuberculosis or acquired immune
deficiency syndrome (AIDS); the
pahogens responsible for either disease
can infiltrate and destroy adrenal tissue.
atau
Sebab utama : Certoimun (80%)
TBC (20%)
Sebab yang jarang :
Infark/perdarahan adrenal
AIDS
Infeksi-infeksi jamur
Hj. Muaeni, SKp, M.Kep
Infeksi-infeksi jamur
Infiltrasi metastasik dari limfomator
Hemokromatosis
Radiasi th/
Adrenalektomi
Inhibitor-inhibitor enzim (ketokonazol)
Sistotoksik (mitotan)
C. Patofisiologi
1. Kurang Sekresi Aldesteron 2. Hilang Glucocoticoid 3. Insufficion Kortisol

Sangat menurunkan
reabsorpsi natrium
tubulus ginjal
Tidak dapat mempertahankan konsentrasi
normal glucosa darah antara waktu
makan (tidak bisa mensintesis glucosa
dalam jumlah cukup melalui
glukoncogenesis)
Meningkatkan sekresi
propiomelanokortin
(POMC) turunan
peptida termasuk
ACTH, MSH LIB
Hilangnya banyak ion
natrium clorida, H2O
dalam urine

sangat berkurangnya
volume cairan ektra
seluler

Kurang kortisol
Mengurangi mobilisasi perotein dan
lemah jaringan

Menekan metabolisme yang lain
Meningkatkan sekresi
propiomelanokortin
(POMC) turunan
peptida termasuk
ACTH, MSH LIB

Akibat hilangnya
umpan balik dari
sumbu hipotraksi dan
Kelambatan Mobilisasi energi sewaktu
Hj. Muaeni, SKp, M.Kep
sumbu hipotraksi dan
hipofisis

Hiperpegmentasi
mikosium membran
dan kulit (bagian kulit
yang tipis)
Hiponatremia, hiper
kalemia dan asidosis
rugen akibat gagalnya
sekresi ion kalium, H guna
menggantikan reabsopsi
natrium di atas
Volume plasma akan
turun
Konsentrasi sel darah
merah meningkat
dengan nyata
Curah jantung
menurun
Klien meninggal
akibat rejatan bila
tidak diobati dalam
waktu 4 hari 2
minggu
tidak ada kortisol efek yang sangat
menganggu.

Bahkan bila tersedia banyak sekali
glukosa dan bahan-bahan makanan lain
otot klien akan lemas

Glukoconticoid yang berikan untuk
mempertahankan fungsi metabolik
jaringan disamping metabolisme energi
Kurang Glucocorticoid

Peka terhadap jenis stress yang berbeda

Diantaranya infeksi pernafasan Rigmen

Kematian
D. MANIFESTASI
Integumentary
System
Delayed wound
healing
Hyperpigmentation
Cardiovascular
Central Nervous System
Lethargy
Tremors
Emotional lability
Confusion
Hj. Muaeni, SKp, M.Kep
Cardiovascular
System
Postural
hypotension
Arrhymias
Tachycardia
Confusion
Musculoskeletal System
Weakness
Muscle wasting
Joint pain
Muscle pain
Lanjutan
Gastrointestinal System
Anorexia
Nausea an vomiting
Diarrhea
Metabolitic Effect
Hyperkalemia
Hyponatremia
Hypoglycemia
Hj. Muaeni, SKp, M.Kep
Reproductive System
Menstrual changes
E. COMPLICATION :
ADDISONIAN CRISIS
Manisfestasi : Panas tinggi,
kelelahan, nyeri abdomen, dll,
hipotensi berat, collap sirculasi,
shock dan coma hipogikemia
Hj. Muaeni, SKp, M.Kep
shock dan coma hipogikemia
tachycardia, hiperkalemia.
Th/ : intravenous replacement of
fluids and glucocorticoid (4-6 hour)
F. DIAGNOSTIC TEST
Serum cortisol levels, which are
decreased in adrenal
insufficiency
Blood glucose levels, which are
Hj. Muaeni, SKp, M.Kep
decreased in adrenal
insufficiency
Serum sodium levels, which are
decreased in adrenal
insufficiency
Lanjutan
Test Normal Values Findings
Serum Cortisol
Blood urea nitrogen
(BUN)
Sodium
8 a.m. to 10 a.m.:
5 to 23 g/dL
4 p.m. to 6 p.m.:
3 to 13 g/dL
5 to 25 mg/dL
135 to 145 mEq/L
Decreased
Increased
Decreased
Hj. Muaeni, SKp, M.Kep
Urine
Sodium
Potassium
Glucose (serum)
17-KS
135 to 145 mEq/L
3.5 to 5.0 mEq/L
70 to 100 mg/dL
Male : 5 to 25
mg/24h
Female: 5 to 15
mg/24h
>65:4 to 8 mg/24h
Decreased
Increased
Decreased
Low/Absent
Lanjutan
Serum potassium levels, which are increased in
adrenal insufficiency
Blood urea nitrogen (BUN) levels, which are
increased in adrenal insufficiency
Plasma ACTH levels, which are increased in
primary adrenal insufficiency but decreased in
Hj. Muaeni, SKp, M.Kep
primary adrenal insufficiency but decreased in
secondary adrenal insufficiency
Possibly ACTH stimulation test, (Cortisol levels
rise whit pituitary deficenecy but do not rise in
primary adrenal insufficiency)
CT scans of the head, which identify any
intracnial lesion impinging on the pituitray
gland
G. NURSING ASSESMENT
Nursing assessment related to the patient
with Addisons disease icludes assessment
of subjective data such as weight loss,
hyperpigmentation, loos of body hair,
anorexia, alst craving, nausea and vomiting,
cramping abdominal pain, and diarrhea,
Hj. Muaeni, SKp, M.Kep
cramping abdominal pain, and diarrhea,
Patients may also complain of exhaustion,
profound weakness, inability to perform
asual activities, muscle aches, light-
headedness, lack of interest in usual
activities and relationship, confusion,
inability to tolerate any stress, decreased
libido, and amenorrhea.
Lanjutan
Objective data noted during the physical
examination include emaciation, pale
skin (but bronzed in sun-exposed areas,
scars. Buccal mucosa, and genitalia),
and sparse body hair (particularly
Hj. Muaeni, SKp, M.Kep
and sparse body hair (particularly
axillary and genital), irritability,
confusion, disorientation, or depresion
may also be noted. Skin tenting (delayed
return of skin to flat position after
pinching) may be observed with poor
skin turgor.
Lanjutan
Hypotension (particularly postural),
decreased cardiac output and heart size,
muscle wasting, and weakness may also
be present. Laboratory values may
indicate hyponatremia, hyperkalemia,
Hj. Muaeni, SKp, M.Kep
indicate hyponatremia, hyperkalemia,
hypoglycemia, low serum cortisol,
increased serum ACTH, decreased 24-
hour urine free cortisol and lack of
response to an ACTH stimulation test.
H. NURSING DIAGNOSA
Nursing diagnoses for the patient with
Addisons disease may include, but are
not limited to, the following
Activity intolerancy related to weakness
and hypotension
Hj. Muaeni, SKp, M.Kep
and hypotension
Self-care deficit related to weakness,
lack of interest and depression
Altered nutrition : less tahn body
requirements related to weakness,
anorexia and nausea and vomiting
Lanjutan
Self-seteem disturbance related to
inability to reform usual activities, loss
of hair hyperpigmentation, and
diagnosis of chronic illness
Altered maintenance related to lack of
knowledge of management of lifelong
hormone replacemen, therapy
Hj. Muaeni, SKp, M.Kep
knowledge of management of lifelong
hormone replacemen, therapy
Decreased cardiac output related to
hypotension an volume depletion
Altered sexuality patterns related to
weakness, malaise, depression and
changing self-consept
Potential complication: hypotension
I. PERENCANAAN
1. Manage self-care activities
2. Gejala-gejala monitor
3. Ajar- pengobatan- dosis dll
4. Cegah acut adrenocortical
Hj. Muaeni, SKp, M.Kep
4. Cegah acut adrenocortical
insufficiency
5. Klien ikut aktif dalam
merencanakan pengobatan jangka
panjang
J. IMPLEMENTASI
Deficient fluid volume hilang cairan
dan sodium vomiting, diare, ektra
celluler fluid volume defisit.
Penurunan cardiac out put, hipotensi,
dll. :
Hj. Muaeni, SKp, M.Kep
dll. :
Monitor intake & out put
Kaji tanda-tanda dehidrasi : mukosa
membraness dry turgor kulit jelek,
sunken eyebolls, urine-gelap,
peningkatan union gravity, BB
menurun, increase hemoconcetration.
Lanjutan
Monitor cardiovascular status :
Vital signs carakter of pulses,
monitor potassin, EGC
BB tiap waktu dehidrasi BB
Hj. Muaeni, SKp, M.Kep
BB tiap waktu dehidrasi BB
menurun
Mendorong out fluid intake 3000
cc/day dan salt intake
Ajarkan duduk dan berdiri pelan-
pelan
Lanjutan
Risk for Inefective Theraupetic
Regimen Management
Ajarkan efek dari sakit dan
pengobatan diskusikan dengan
Hj. Muaeni, SKp, M.Kep
klien dan keluarga plan
Pemberian steroid sendiri
Pemberian cortison parenteral &
sprit emergency ktl
Lanjutan
Meningkatkan cairan per oral
Diet tinggi sodium dan rendah
potasium rutin
Setelah pengobatan (dose)
Hj. Muaeni, SKp, M.Kep
Setelah pengobatan (dose)
emosional & physical shessor
Perlu perwatan kesehatan secara
rutin.
HOME CARE
Perlunya perawatan kesehatan yang
rutin
Referral sosial worker yang tepat
Referral agency komunitas contiuning
education dan support
Bantu sepenuhnya :
Hj. Muaeni, SKp, M.Kep
Bantu sepenuhnya :
Nasional institute of diabetes and
digestive and kidney diseases
(Addisons. D)
Endocrime Sosiety
American Asosociation of Clinical
Endocrinenologists.
Hj. Muaeni, SKp, M.Kep