2. manajemen asuhan keperawatan; hipopituitarisme
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Manajemen Asuhan Keperawatan Pasien Dengan Gangguan Kelenjar Pituitari
Yulius TirandaDepartemen KMB
PSIK STIKes Muhammadiyah Palembang
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.
Anatomy
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 Rathke’s pouch, an evagination of the stomodeal ectoderm. In contrast,the posterior pituitary arises in the infundibulum, a part of the diencephalon.
Hypothalamic-Pituitary
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’).
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
The pituitary gland produces a variety of different hormones:
Target Organ
Hypopituitarism
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’.
An overview
Hypopituitarism, fi rst described clinically by
Simmonds in 1914,2 is the inability of the pituitary gland to provide suffi 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 insuffi cient supply of hypothalamic-releasing hormones.
Incidence and prevalence of hypopituitarism are
estimated to be 4·2 per 100 000 per year and 45·5 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 I’s”: 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 Sheehan’s 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 insufficiency 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, Kallman’s syndrome – X-linked 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
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.
Sign and Symptom
2. TSH deficiency causing thyroid hormone
deficiency: Symptoms include fatigue, weakness, difficulty losing weight, generalized body puffiness, feeling cold, constipation, difficulty 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), difficulty 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 • Sheehan’s 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-Bardet-
Biedl 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 hyperphagia, obesity, and central hypogonadism
Decreased GnRH production in these patients results in 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 insufficiency Diagnose with CT/MRI Differentiate from leaking aneurysm Treatment:
Surgical - Transsphenoid decompression Visual defects and altered consciousness
Medical therapy – if symptoms are mild Corticosteroids
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.
Pathophysiology
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 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 Hipopituitarisme
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).
Manifestasi Klinis
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 Biokimia1. 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
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.
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.
Treatment
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
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)
Nursing Management of The Patient With Hypopituitari
Common nursing dx related to emotional needs include: Fear Aniety Anticipatory grieving Ineffective denial Ect...