Sei sulla pagina 1di 71

Manajemen Asuhan

Keperawatan Pasien Dengan


Gangguan Kelenjar Pituitari

Yulius Tiranda
Departemen KMB
PSIK STIKes Muhammadiyah Palembang

Anatomy

The adult pituitary gland is a pea-sized midline


structure located below the optic chiasm. It hangs
down on a pedicle (the hypothalamic-pituitary
stalk) from the inferior portion of the
hypothalamus.
The pituitary gland contains two anatomical
components. The anterior portion, also known as
the adenohypoph- ysis, receives its signals
through the portal system of blood vessels that
arise in the hypothalamus and tra- verse the stalk.


The posterior section, also known as the
neurohypophysis,receives its signals through
axons that arise in neural bodies within the
hypothalamus and also traverse down the
stalk. Embryologically,the ante- rior pituitary
gland is derived from Rathkes pouch, an
evagination of the stomodeal ectoderm. In
contrast,the posterior pituitary arises in the
infundibulum, a part of the diencephalon.

HypothalamicPituitary

Pituitary Gland

Located within the sella tursica


Contiguous to vascular and neurologic structures
Cavernous sinuses
Cranial nerves
Optic chiasm

Hypothalamic neural cells synthesize specific


releasing and inhibiting hormones
Secreted directly into the portal vessels of the
pituitary stalk

Blood supply derived from the superior and


inferior hypophyseal arteries

Pituitary Gland

Anterior pituitary gland


Secrete various trophic hormones
Disease in this region may result in syndromes of hormone
excess or deficiency

Posterior pituitary gland


More of a terminus of axons of neurons in the supraoptic and
paraventricular nuclei of the hypothalamus
Storehouse for the hormones
The main consequence of disease in this area is disordered
water homeostasis

Anterior Pituitary Gland


Anterior Pituitary

Master gland

Major blood source: hypothalamic-pituitary portal plexus

Allows transmission of hypothalamic peptide pulses without


significant systemic dilution

Consequently, pituitary cells are exposed to sharp spikes of


releasing factors and in turn release their hormones as discrete
pulses

Production of six major hormones:

Prolactin (PRL)

Growth hormone (GH)

Adrenocorticotropin hormone (ACTH)

Luteinizing hormone (LH)

Follicle-stimulating hormone (FSH)

Thyroid-stimulating hormone (TSH)


Secreted in a pulsatile manner
Elicits specific responses in peripheral target
tissues
Feedback control at the level of the
hypothalamus and pituitary to modulate
pituitary function exerted by the hormonal
products of the peripheral target glands
Tumors cause characteristic hormone
excess syndromes
Hormone deficiency

may be inherited or acquired


The six main pituitary hormones are Growth
Hormone (GH), Luteinising Hormone (LH), Follicle
Stimulating Hormone (FSH), thyrotropin (TSH),
Adrenocorticotropic Hormone (ACTH) and Prolactin
(PRL).
When one or more of these hormones is being
under-produced, the condition is called
hypopituitarism (also sometimes called multiple
pituitary hormone deficiency, or MPHD). When all
of these hormones are deficient, the condition is
called panhypopituitarism (pan means all).

The pituitary gland produces a variety of different


hormones:

1. Adrenocorticotropic hormone (ACTH): controls production of


the adrenal gland hormones cortisol and dehydroepiandrosterone
(DHEA).
2. Thyroid-stimulating hormone (TSH): controls thyroid
hormone production from the thyroid gland.
3. Luteinizing hormone (LH) and Follicle-stimulating
Hormone (FSH): LH and FSH together control fertility in both sexes
and the secretion of sex hormones (estrogen and progesterone from
the ovaries in women and testosterone from the testes in men).
4. Growth hormone (GH): required for growth in childhood and
has effects on the entire body throughout life.
5. Prolactin (PRL): required for breast feeding.
6. Oxytocin: required during labor and delivery and for lactation
and breast feeding.
7. Antidiuretic hormone (also known as vasopressin): helps
maintain normal water balance

Target
Organ

Hypopituitarism

An overview

Hypopituitarism is a condition whereby your


pituitary gland secretes lower levels of
hormones than normal. these hormones play
an important role in maintaining your health
and well-being. over time, having low
hormone levels will leave you feeling very
tired and off-colour.


Hypopituitarism, fi rst described clinically by
Simmonds in 1914,2 is the inability of the
pituitary gland to provide su cient hormones
adapted to the needs of the organism.
It might be caused by either an inability of the
gland itself to produce hormones or an insu
cient supply of hypothalamic-releasing
hormones.


Incidence and prevalence of hypopituitarism are
estimated to be 42 per 100 000 per year and 455 per
100 000, respectively. Although the clinical symptoms
of this disorder are usually unspecifi c, it can cause
life-threatening events and lead to increased mortality.
Current research has refi ned the diagnosis of
hypopituitarism.
Identification of growth hormone and corticotropin defi
ciency generally requires a stimulation test, whereas
other defi ciencies can be detected by basal hormones
in combination with clinical judgment.

Hypopituitarism

Etiology
Anterior pituitary diseases
Deficiency one or more or all anterior pituitary
hormones

Common causes:

Primary pituitary disease


Hypothalamic disease
Interruption of the pituitary stalk
Extrasellar disorders

Causes

More then 75% of the gland must be destroyed


before clinical manifestations are evident
Nine Is: invasive, infarction, infiltrative, injury,
immunologic, iatrogenic, infectious, idiopathic,
isolated.
Invasive: adenomas, craniopharyngioma, other
primary CNS tumors, metastatic lesions
Infarction:
Simmonds /1914/: pituitary necrosis in a woman
with severe puerperal sepsis Sheehans syndrome /
1937/: pituitary ischaemic infarction due to
postpartum hemorrhage and vascular collapse
failure to lactate, to resume normal menstrual periods

Causes (2)

Pituitary apoplexy = spontaneous hemorrhagic infarction


of a pituitary tumor: a fulminant clinical syndrome: severe
headache, visual impairment, ophtalmoplegias,
meningismus, altered level of consciousness. Sometimes
related to DM, radiotherapy, open heart surgery.
Corticosteroids, transsphenoidal decompression
of the intrasellar contents may be lifesaving.
Silent pituitary apoplexy: may cure the hipersecretory
pituitary adenoma without impairing the secretion of other
anterior pituitary hormones.
Infiltrative: sarcoidosis, hemochromatosis, histiocytosis X
Injury: severe head trauma anterior pituitary
insuciency and/or DI

Causes (3)

Immunologic: lymphocytic hypophysitis anterior


hypopituitarism; most often in women during pregnancy or in
the postpartum period; may result in isolated hormone
deficiences (ACTH, PRL); NMR; glucocorticoids, self-limiting
disease
Iatrogenic: surgery, radiotherapy
Infectious: TBC, syphilis, mycotic infections
Idiopathic: either isolated or multiple deficiences
Isolated (monotropic):
- congenital monotropic GH deficiency (sporadic, familial),
- monotropic ACTH deficiency (rare;
most acquired cases due to lympocytic hypophysitis), - isolated
gonadotropin deficiency (not uncommon, Kallmans syndrome Xlinked dominant, defect in LHRH secretion associated with
hyposmia/anosmia, sometimes color blindness and nerve deafness

Causes (4)

Other causes of isolated hypogonadotropic


hypogonadism:
weight loss, emotional or physical stress,
intensive athletic training, anorexia nervosa,
marked obesity, sickle cell anemia, other
chronic ilnesses, eg. poorly controlled DM and
malnutrition, autoimmune hypophysitis

Causes of
hypopituitarism

Brain damage*
Traumatic brain injury
Subarachnoid
haemorrhage
Neurosurgery
Irradiation
Stroke
Pituitary tumours*
Adenomas
Others

Non-pituitary tumours
Craniopharyngiomas
Meningiomas
Gliomas
Chordomas
Ependymomas
Metastases
Infections
Abscess
Hypophysitis
Meningitis
Encephalitis

Symptoms and
Signs of Pituitary
Hormone
Deficience

Hyperpituitarism

Acromegaly

Hypopituitarism

Dwarfism

Sign and Symptom

Symptoms depend on which hormone or


hormones are missing.
ACTH deficiency causing cortisol deficiency:
Symptoms include weakness, fatigue, weight
loss, abdominal pain, low blood pressure and low
serum sodium levels.
During a period of severe stress such as infection
or surgery, cortisol deficiency may potentially
result in coma and death. ACTH also stimulates
DHEA secretion from the adrenal cortex.


2. TSH deficiency causing thyroid hormone
deficiency:
Symptoms include fatigue, weakness, diculty
losing weight, generalized body puness, feeling
cold, constipation, diculty with memory and an
inability to concentrate. Skin may become dry and
the complexion pale.
In addition, anemia, high cholesterol levels and liver
problems may also occur. Patients with severe or
long-term deficiency can appear lethargic. Rarely,
severe thyroid hormone deficiency can cause coma,
low body temperature, and even death


3. LH and FSH deficiency in Women: LH and FSH
deficiency may cause loss of menstrual
cycles, infertility, decrease in sex drive and
vaginal dryness and osteoporosis, which can result
in a tendency to develop bone fractures.
LH and FSH deficiency in Men: LH and FSH
deficiency may cause loss of libido (interest in
sexual activity), diculty in achieving and
sustaining an erection and infertility due to a low
sperm count, and osteoporosis, which can result in
a tendency to develop bone fractures.


4. GH deficiency:
In children, GH deficiency causes slowing or
lack of growth and an increase in body fat.
In adults, GH deficiency may cause a
decrease in energy and physical activity,
change in body composition (increased fat,
decreased muscle mass), a tendency toward
increased cardiovascular risk factors/diseases
and decreased quality of life (including an
increased sense of social isolation).


5. PRL deficiency: In the case of PRL deficiency, the
mother might not be able to breast feed following
delivery.
6. Antidiuretic hormone deficiency: This hormone
deficiency results in diabetes insipidus (DI). DI is not the
same as diabetes mellitus, which is also known as type 1
or type 2 diabetes or sugar diabetes.
Symptoms of DI include increased thirst and frequent
urination, particularly at night. Pituitary adenomas
themselves rarely cause DI unless it occurs after surgery.
If DI occurs spontaneously, it usually indicates that some
other sort of tumor or inflammation is present in the area.

Gonadotropin Deficiency

Women
Oligomenorrhea or
amenorrhea
Loss of libido
Vaginal dryness or
dyspareunia
Loss of secondary
sex characteristics
(estrogen
deficiency)

Men
Loss of libido
Erectile dysfunction
Infertility
Loss of secondary sex
characteristics
(testosterone deficiency)
Atrophy of the testes
Gynecomastia
(testosterone deficiency)

ACTH Deficiency

Results in hypocortisolism

Malaise
Anorexia
Weight-loss
Gastrointestinal disturbances
Hyponatremia

Pale complexion
Unable to tan or maintain a tan

No features of mineralocorticoid deficiency


Aldosterone secretion unaffected

TSH Deficiency

Hypothyroidism
Atrophic thyroid gland

Prolactin Deficiency

Inability to lactate postpartum


Often 1st manifestation of Sheehan syndrome

Growth Hormone Deficiency

Adults

Often asymptomatic
May complain of
Fatigue
Degrees exercise tolerance
Abdominal obesity
Loss of muscle mass

Children
GH Deficiency
Constitutional growth delay

Common
test


Infarction
Apoplexia
Sheehans syndrome
Autoimmune disorders
Lymphocytic hypophysitis

Haemochromatosis, granulomatous diseases, histiocytosis


Empty sella
Perinatal insults
Pituitary hypoplasia or aplasia
Genetic causes
Idiopathic causes

Hypopituitarism

Primary pituitary disease

Tumors
Pituitary surgery
Radiation treatment

Hypothalamic disease

Functional suppression of axis

Exogenous steroid use


Extreme weight loss
Exercise
Systemic Illness

Interruption of the
pituitary stalk
Extrasellar disorders

Craniopharyngioma
Rathke pouch

Hypopituitarism

Hypopituitarism

Developmental and
genetic causes
Dysplasia

Septo-Optic dysplasia

Developmental
hypothalamic
dysfunction

Kallman Syndrome
Laurence-Moon-BardetBiedl Syndrome
Frohlich Syndrome
(Adipose Genital
Dystrophy)

Acquired causes:
Infiltrative disorders
Cranial irradiation
Lymphocytic
hypophysitis
Pituitary Apoplexy
Empty Sella syndrome

Hypopituitarism: Developmental and


Genetic causes

Septo-Optic dysplasia
Kallman Syndrome
Laurence-Moon-Bardet-Biedl Syndrome
Frohlich Syndrome (Adipose Genital Dystrophy)

Hypopituitarism: Genetic

Septo-Optic dysplasia

Hypothalamic dysfunction and hypopituitarism


may result from dysgenesis of the septum pellucidum or corpus
callosum
Affected children have mutations in the HESX1 gene
involved in early development of the ventral prosencephalon
These children exhibit variable combinations of:
cleft palate
syndactyly
ear deformities
hypertelorism
optic atrophy
micropenis
anosmia
Pituitary dysfunction
Diabetes insipidus
GH deficiency and short stature
Occasionally TSH deficiency

Hypopituitarism: Developmental

Kallman Syndrome

Defective hypothalamic gonadotropin-releasing hormone (GnRH)


synthesis

Associated with anosmia or hyposmia due to olfactory bulb


agenesis or hypoplasia

May also be associated with: color blindness, optic atrophy,


nerve deafness, cleft palate, renal abnormalities, cryptorchidism,
and neurologic abnormalities such as mirror movements

GnRH deficiency prevents progression through puberty

characterized by

low LH and FSH levels


low concentrations of sex steroids

Hypopituitarism: Developmental

Kallman Syndrome

Males patients

Delayed puberty and hypogonadism, including micropenis


result of low testosterone levels during infancy
Long-term treatment:
human chorionic gonadotropin (hCG) or testosterone

Female patients

Primary amenorrhea and failure of secondary sexual development


Long-term treatment:
cyclic estrogen and progestin

Diagnosis of exclusion
Repetitive GnRH administration restores normal pituitary
Fertility may also be restored by the administration of
gonadotropins or by using a portable infusion pump to deliver
subcutaneous, pulsatile GnRH

Hypopituitarism: Developmental

Laurence-Moon-Bardet-Biedl Syndrome

Rare autosomal recessive disorder


Characterized by mental retardation; obesity; and
hexadactyly, brachydactyly, or syndactyly
Central diabetes insipidus may or may not be
associated
GnRH deficiency occurs in 75% of males and half
of affected females
Retinal degeneration begins in early childhood

most patients are blind by age 30

Hypopituitarism: Developmental

Frohlich Syndrome (Adipose Genital


Dystrophy)

A broad spectrum of hypothalamic lesions

Decreased GnRH production in these patients


results in

hyperphagia, obesity, and central hypogonadism

attenuated pituitary FSH and LH synthesis and


release

Deficiencies of leptin, or its receptor, cause these


clinical features

Hypopituitarism

Acquired causes:

Infiltrative disorders
Cranial irradiation
Lymphocytic hypophysitis
Pituitary Apoplexy
Empty Sella syndrome

Hypopituitarism: Acquired

Lymphocytic Hypophysitis
Etiology

Presumed to be autoimmune

Clinical Presentation

Women, during postpartum period


Mass effect (sellar mass)
Deficiency of one or more anterior pituitary hormones

ACTH deficiency is the most common

Diagnosis

MRI - may be indistinguishable from pituitary adenoma

Treatment

Corticosteroids often not effective


Hormone replacement

Hypopituitarism: Acquired

Pituitary Apoplexy
Hemorrhagic infarction of a pituitary adenoma/tumor
Considered a neurosurgical emergency
Presentation:

Variable onset of severe headache


Nausea and vomiting
Meningismus
Vertigo
+/ - Visual defects
+/ - Altered consciousness

Symptoms may occur immediately or may develop


over 1-2 days

Hypopituitarism: Acquired
Pituitary Apoplexy

Risk factors:

Diabetes
Radiation treatment
Warfarin use

Usually resolve completely


Transient or permanent hypopituitarism is possible

undiagnosed acute adrenal insuciency

Diagnose with CT/MRI


Differentiate from leaking aneurysm
Treatment:

Surgical - Transsphenoid decompression

Visual defects and altered consciousness

Medical therapy if symptoms are mild

Corticosteroids

Pathophysiology

The pituitary gland is supplied with blood by branches of the


internal carotid artery. These vessels form a capillary plexus
in the region of the median eminence of the hypothalamus.
Blood from this area reaches the anterior pituitary by means
of long and short portal veins via the pituitary stalk. The
middle and inferior hypophyseal arteries supply the pituitary
stalk and neurohypophysis with arterial blood.
However, the anterior lobe is not included in this arterial
blood supply; it is provided with oxygenated blood only
through the internal and external plexus of the median
eminence. The pathophysiology of hypopituitarism is
dependent on the cause of the disorder and is not
understood completely in some cases.


For pituitary adenomas, mechanical compression of portal
vessels and the pituitary stalk, and ischaemic necrosis of
portions of the anterior lobe, have been postulated to be the
pre dominant mechanism causing hypopituitarism.
Moreover, increases in intrasellar pressure have been
recorded in patients with pituitary macroadenomas, which
could be the cause of reduced blood fl ow through the portal
vessels and the pituitary stalk, resulting in diminished
delivery of hypothalamic hormones to the anterior pituitary.
Empty sella is caused by herniation of the subarachnoid
space and associated with fl attening of the pituitary gland.
This process is sometimes, but not necessarily, accompanied
by hypopituitarism.

Manifestasi Hipopituitarisme

Manifestasi yang muncul pada hipopituitarisme ini


bermacam-macam berdasarkan usia penderita.
Padaanak-anak, terjadi gangguan pertumbuhan
somatik akibat defisiensi pelepasan GH.
Dwarfisme hipofisismerupakan konsekuensi dari
hal tersebut. Ketika anak-anak tersebut mencapai
pubertas, maka tanda-tanda seksual sekunder dan
genitalia eksterna gagal berkembang. Selain itu
sering itemukaninsufisiensi adrenal dan
hipotiroidisme, hipoglikemia, kulit terlihat pucat
karena tidak adanya MSH.

Manifestasi Klinis

Tubuh kerdil (dwarfisme hipofisis) akibat defisiensi


GH (bila terjadi pada anak)
Tanda seksual sekunder dan genitalia eksterna gagal
berkembang, akibat dari defisiensigonadotropik
hormon sehingga produksi FSH dan LH berkurang.
Akibat dari FSH defisiensi,pematangan folikel tidak
terjadi, tidak dihasilkannya estrogen pada
perempuan. Hal inimenyebabkan payudara tidak
tumbuh, dan terjadi amenore primer. Jika terjadi
pada pria akibatLH berkurang pembentukan
testosteron berkurang, akibatnya tidak timbul ciri-ciri
kelaminsekunder pada pria (bila terjadi pada anak).


Insufisiensi adrenal. hal ini terjadi akibat defisiensi ACTH
sehingga pembentukan hormon-hormon korteks adrenal
terganggu, defisiensi kortisol, aldosteron, dan
adrenalandrogenberkurang.
Hipoglikemia, hal ini disebabkan karena defisiensi hormone
kortisol yang pengeluarannya dirangsang oleh ACTH yang
disekresi oleh hipofisis, salah satu fungsi kortisol
adalahmeningkatkan glukoneogenesis, dan menurunkan
glikolisis. Akibat dari defisiensi hormone inimaka terjadilah
penurunan glukoneogenesis, peningkatan glikolisis
akibatnya gula darahmenurun yang mengakibatkan
hipoglikemia. Hal ini diperparah dengan adanya defisiensi
GH,GH merupakan hormone yang bersifat hiperglikemia,
akibat defisiensi GH efek darihiperglikemiknya tidak ada.


Takikardia, hipoglikemia menyebabkan
epineprin bekerja yang selanjutnya akan
memengaruhidenyut nadi mengakibatkan
takikardi.
Kelemahan otot dan penurunan berat badan,
hal ini terjadi akibat dari hipoglikemia
yangmenyebabkan pengaktifan saraf simpatis
dan menghambat pelepasan insulin sehingga
jugamemengaruhi lipolisis dan pemecahan
protein.


Anemia, nerutropenia, eosinofilia, trombopenia,
llimfositosis. Salah satu fungsi glukokortikoidyang
sekresinya diatur oleh ACTH adalah meningkatkan
pembentukan eritrosit, trombosit, dangranulosit
basofil, limfosit dan monosit. Akibat dari kekurangan
efek dari glukokortikoid padasel pembentuk darah
menyebabkan anemia, neutropenia, eosinofilia,
limfositosis.
Rambut pubis yang jarang akibat dari defisiensi
androgen karena kadar ACTH menurun.
Tekanan darah menurun. Penurunan sensitivitas
katekolamin di jantung dan pembuluh
darahmenyebabkan hipotensi.


Amenore sekunder pada wanita dewasa. Akibat
defisiensi gonadotropin hormon,
menyebabkankorpus luteum tidak terbentuk dan
dinding endometrium tidak hiperplasi, yang
menyebabkantidak adanya peluruhan.
Atrofi payudara dan genitalia eksterna, pertumbuhan
payudara salah satunya diatur olehestrogen
hormone yang dihasilkan oleh oleh adanya peran
FSH dan LH, akibat dari kekuranganhormon ini
menyebabkan defisiensi pada estrogen akibatnya
terjadi atrofi payudara dangenitalia eksterna.

Pemeriksaan

Pemeriksaan Biokimia
1.Pasien dengan hipopituirarisme tidak akan merespon jika diberikan
pengujian hormon perangsangsekresi. Uji fungsi hipofisis kombinasi dapat
dilakukan pada pasien ini dengan menyuntikkan :1.
2.Insulin. Insulin akan menyebabkan hipoglikemia, pada saat hipoglikemia
dengan kadar serumglukosa yang kurang dari 40 mg/dl, normalnya
menyebabkan pelepasan GH, ACTH, kortisol.Namun pada penderita
hipopituitarisme mungkin tidak terjadi pelepasan tersebut atau hanyasedikit
hormon yang dilepaskan.2.
3.CRH. Pada orang normal CRH akan merangsang hipofisis untuk
mensekresikan ACTH, MSH.3.
4.TRH. Pada normalnya penyuntikan TRH akan merangsang hipofisis
mensekresikan TSH.4.
5.GnRH. Pada normalnya GnRH akan merangsang pelepasan hormon FSH dan
LH.
Pada penderita hipopituitarisme gagal untuk merespon empat rangsangan tersebut


Pemeriksaan Radiografi
Pada pasien hipopituitarisme yang diduga
akibat tumor hipofisis, perlu dilakukan
pemeriksaan radiografi untuk mencari
kebenaranya. Tumor-tumor pada hipofisis ini
sering menyebabkan hipopituitarisme.

Treatment

Penatalaksanaan hipopituitarisme diobati


dengan penggantian hormon-hormon yang
mengalami defisiensi. Defisiensi GH
menyebabkan pemberian GH secara injeksi
setiap hari. Pemberian GH pada anak-anak
dapat menyebabkan penigkatan tinggi badan
yang berlebih. Hormon hipofisis hanya dapat
diberikan secara disuntikan. Sehingga,
pengobatan ini dilakukan hanya sebagai
alternatif.


Sebagai contoh insufisiensi kelenjar adrenal
akibat dari defek ACTH diobati dengan
pemberian hidrokortisonoral. Pemberian
tiroksin oral pada defisiensi TSH. Pemberian
androgen dan estrogen oral juga
diberikansebagai pengganti dari FSH, LH yang
mengalami defisiensi akibat hipopituitarisme

Nursing Management of The


Patient With Hypopituitari

The specifics of nursing management for


patients with hypopituitari depends on the
nursing history and neurological, endocrine
metabolic assessment, with physical care
focusing on the functional deficits incurred.
The appropriate nursing diagnosis for
addressing informational needs of the patient
and the family is Knowledge deficit --- NIC:
individualized teaching plan for the patient
and family (medical plan of care, mgt of
current deficits and disabilities ect)


Common nursing dx related to emotional needs
include:
Fear
Aniety
Anticipatory grieving
Ineffective denial
Ect...