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...