Disorders Endocrine/Lymphatic/Immune Systems

Compiled by Sen.T/Sr. Navuta

Disorders of the Endocrine System

May

be related to either an excess or a deficiency of a specific hormone May be related to a defect at its receptor site Onset of an Endocrine Disorder can be either slow and insidious or abrupt and life-threatening

Laboratory Testing for Endocrine Functioning

Blood Work Simulation Testing: measured amounts of hormones are given to stimulate target gland to maximal production (for suspicion of under activity). Failure of hormone level to rise denotes hypofunction Suppression Testing: (for suspicion of over acitivity). Failure of hormone level to lower denotes hyperfunctioning

Laboratory Testing for Endocrine Functioning

Radioimmunoassay: competitive binding assay test in which radioactively labeled amounts of hormones compete with unlabeled hormones from plasma or serum for antibody binding sites.

 Various

techniques measure amount of unbound and bound hormone. Radioisotope is added to clients specimen after it is obtained, so client is not exposed to radiation.

Urine Testing: measures hormone levels and metabolites of specific hormones in urine Usually done in a 24 hour urine collection to better reflect the functioning of a gland such as adrenal gland Certain hormones may require addition of additives to the collection container No unnecessary medications should be used by client during collection process

24 Hour Urine Collection

Test starts when client first arises and urinates The first specimen is discarded but time is noted - (first specimen is not collected because it is hard to tell how long it has been in bladder) Client adds all urine voided after that first discarded specimen during next 24 hours Some hospital labs may have it put on ice

Glucose Testing
Can be done via finger stick or venous puncture Glycosylated hemoglobin (HbA1c) value reveals the average blood glucose level over a period of 2-3 months

Radiographic Examinations
Anterior, posterior and lateral skull x-ray studies may be used to visualize sella turcica (an indentation of sphenoid bone which houses the pituitary gland) Erosion of this structure indicates invasion of the wall from an abnormal growth CT and MRI scans can show extent of growth or location of a tumor buried within pituitary

Radiographic Examinations
Size and shape of other glands and nearby structures may be visualized by use of a contrast media Angiography and Venography may reveal structural abnormalities in blood vessels and ultrasonography of thyroid gland can indicate whether nodules or masses are solid or cystic

Needle Biopsy
Quick outpatient procedure can be used to indicate the composition of thyroid nodules Primarily used to determine whether surgical intervention is necessary

Pituitary Hypofunction
Caused by a deficiency of one or more anterior pituitary hormones Results in metabolic abnormalities and sexual dysfunction

Pituitary Hypofunction

Interventions focus on replacement of deficient hormones to treat delayed puberty

Testosterone therapy is initiated with high-dose testosterone derivatives and continued until virilization is achieved (deepened voice, increased chest, facial, pubic and axillary hair growth) Dose is then decreased, but therapy continues throughout lifetime

Androgen therapy is avoided in men with prostate cancer Male fertility may be decreased and/or depleted due to testosterone therapy Side effects include: gynecomastia (male breasts) baldness prostatic hypertrophy

Women may be treated with a combination of estrogen & progesterone administered at their menstrual cycle The risk for hypertension or thrombosis is increased with estrogen therapy especially if the woman smokes Female fertility may be decreased and medications are needed to induce ovulation

Growth Hormone Deficiency changes tissue growth patterns indirectly In children, GH deficiency leads to short stature & other manifestations of growth retardation GH deficiency in adults produces no obvious anatomic changes - does increase rate of bone destructive activity leading to thinner, more fragile bones Adult clients may be treated with injections of GH, although this treatment is rare

Pituitary Hyperfunction

A condition of hormone oversecretion that occurs when client has pituitary tumors or hyperplasias Tumors usually arise from:

somatotropic cells (growth hormone) lactotropic cells (prolactin) corticotropic cells (adrenocorticotrophic hormone)

Can also be caused by hypothalmic dysfunction

Adenomas can develop in clients without a family history or as part of a syndrome known as multiple endocrine neoplasia Multiple endocrine neoplasia is a familial disorder inherited as an autosomal dominant trait may include parathyroid and pancreatic tumors

Benign epithelial tumor, which is classified by

size, degree of invasiveness and hormone secreted As it grows, it compresses brain tissue causing neurologic symptoms, as well as endrocrine symptoms (i.e. visual changes, headache and increased ICP)

Prolactin secreting tumors are most common of the Pituitary Adenomas Produces excessive prolactin inhibiting secretion of gonadal steroids and gonadotropins in men Men (gynecomastia due to low levels of testosterone & muscle weakness) Women (no production of breast milk, no menses)

Overproduction of Growth Hormone

Results in gigantism or acromegaly Onset may be gradual, slow progression, and changes may remain unnoticed for years before diagnosis Early detection and treatment is essential to prevent irreverible changes in the soft tissues, such as those of the face, hands, feet and skin

Enlarged hands and feet in an adult are an indicator of growth hormone excess Although some changes are reversible after treatment for overproduction of growth hormone, skeletal changes are permanent

Gigantism

Onset of Growth Hormone Hypersecretion occurs before puberty causing rapid proportional growth in the length of all bones Back pain and arthralgias (joint pain) may be symptoms in response to bone changes

Gigantism

Gigantism

Acromegaly

Occurs after puberty and produces increased skeletal thickness, hypertrophy of the skin and enlargement of many visceral organs such as liver and heart.

Acromegaly

Acromegaly

Cushings Disease

Caused by hypersecretion of Adrenocorticotrophic hormone (ACTH) Results in overstimulation of Adrenal Cortex producing excessive amounts of glucocorticoids, mineralcorticoids and androgens Clients have alterations of nitrogen, carbohydrate and mineral metabolism

Cushings Disease

Hyperpituitarism

Is a rare disorder. Of all pituitary tumors for which surgery is performed, approximately 70% secrete one or more hormones. Surgical procedure is called Transsphenoidal hypophysectomy

Posterior Pituitary

Disorders of posterior pituitary are directly related to a deficiency or excess of hormone Vasopressin (antidiuretic hormone - ADH) Two disorders associated with ADH deficiency or ADH excess are:

Diabetes Insipidus Syndrome of Inappropriate Antidiuretic Hormone (SIADH)

Diabetes Insipidus

Two clinical manifestations/signs that alert you to possible endocrine disorder would be:

increased thirst increased urination

Diabetes Insipidus is a disorder of water metabolism caused by a deficiency of ADH

Diabetes Insipidus

Source of ADH insufficiency is caused either by:

decrease in ADH synthesis inability of kidneys to respond appropriately to ADH

ADH deficiency results in excretion of large volumes of dilute urine called polyuria Hypernatremia is a potential problem

Diabetes Insipidus

Dehydration caused by massive diuresis caused by polyuria results in an increase in plasma osmolality causing thirst However, if thirst mechanism is inadequate or absent, or if person is unable to obtain water, dehydration becomes more severe

Types of Diabetes Insipidus

Nephrogenic diabetes insipidus is an inherited disorder where the renal tubules do not respond to the action of ADH. This results in inadequate water reabsorption by kidney Urine output is then increased and specific gravity is decreased. With effective treatment, urine output is effectively decreased and specific gravity is increased

Primary Diabetes Insipidus: caused by a defect in Hypothalmus or Pituitary Gland resulting in a lack of ADH production or release Secondary Diabetes Insipidus: results from tumors within/adjacent to hypothalmus or pituitary gland, head trauma, infectious processes, surgical procedures or metastatic tumors.

Drug-related Diabetes Insipidus : caused by administration of lithium carbonate or demeclocycline as these drugs can interfere with kidneys response to ADH.

Clinical Manifestations of Diabetes Insipidus

Loss of free water produces expected changes in blood and urine tests Initial step in diagnosis is to measure a 24 hour fluid I&O. Urine output during this period must be more than 4 liters for diagnosis. However, it may vary from 4-30 liters in a day

Insipidus vs. Mellitus

Diabetes Mellitus is endocrine problem caused by a decreased sensitivity of insulin receptors to presence of insulin Diabetes Insipidus is endocrine problem caused by an insufficiency of Antidiuretic Hormone (ADH)

Drug Therapy for Diabetes Insipidus

Client with permanent Diabetes Insipidus requires life-long vasopressin therapy Client is instructed to recognize poluria and polydipsia signals for another dose of medication All clients taking vasopressin need to record daily weights to identify weight gains

Syndrome of Inappropriate Antidiuretic Hormone (SIADH)

Occurs when vasopressin (ADH) is secreted when plasma osmolarity is low or normal Decrease in plasma osmolarity normally inhibits ADH production and secretion In SIADH, the feedback mechanisms that regulate ADH do not function properly ADH continues to be released even when plasma is hyposmolar

As a result of ADH secretion, water is retained, resulting in:

dilutional hyponatremia expansion of the extracellular fluid volume

Pulmonary disorders (including Emphysema & other chronic lung diseases) can cause SIADH either by causing increased secretion of ADH or ectopic synthesis of ADH.

Adrenal Gland Hypofunction

Addisons Disease is caused by an insufficiency of adrenocortical steroids causing problems through loss of mineralocorticoid (aldosterone) and glucocorticoid (cortisol) action Reduced aldosterone secretion causes potassium, sodium and water imbalances:

Hyperkalemia Hyponatremia Hypovolemia

Lower adrenal andogen levels result in decreased/loss of body/axillary/pubic hair (esp. in women) because adrenals produce most of androgrens in females Excessive production of melanocytestimulating hormone increases pigmentation of skin and causes darkening of skin It can cause vitiligo patchy areas of depigmentation of skin

Acute Adrenal Insufficiency

Called Addisonian crisis a life-threatening event in which physiologic need for glucocorticoid and mineralocorticoid hormones is greater than the available supply Usually occurs in response to a stressful event (surgery, trauma, severe infection)

Hormone Replacement

Glucocorticoid and mineralocorticoid deficiencies are completely corrected by replacement therapy Drug regimen is divided into thirds 2/3 given in morning and 1/3 given in late afternoon to mimic normal adrenal hormone secretion rhythm

Additional mineralocorticoid hormone may be needed (such as Florinef) to maintain correct electrolyte balance (esp. Na and Kcl) Adjustments may be needed in hot weather to compensate for sodium loss due to excessive perspiration Salt restriction or diuretic therapy should not be started without considering whether it might precipitate an adrenal crisis

Endocrine Disorders: Thyroid, Parathyroid and Pancreas

Graves Disease (Hyperthyroidism)

Also known as Toxic Diffuse Goiter Autoimmune disorder antibodies or immunoglobulins are made and attach to thyroid stimulating hormone (TSH) receptor sites on thyroid tissue Thyroid gland increases in size and overproduces thyroid hormones

Presentation:

Goiter (enlargement of the thyroid gland) Exophthalmos (abnormal protrusion of the eyes giving a wide-eyed startled look) Pretibial myxedema (dry, waxy swelling of the front surfaces of the lower legs)

Graves Disease

Clients may have excessive tearing, bloodshot eye appearance or have sensitivity to light. Fine, soft, silky hair and smooth, moist skin are common with hyperthyroidism. Emotional lability (mood instability) is often experienced. Client may also appear extremely restless, irritable and fatigued.

Extremity muscle weakness, hyperactive deep tendon reflexes or tremors Not all clients with goiter have hyperthyroidism A hallmark assessment finding: heat intolerance

Hypothyroidism

Decreased metabolism from low levels of thyroid hormones Can occur at any time throughout life span; however most often occurs in women between ages 30-60. Women are affected 710 times more than men Link between diabetes mellitus and development of hypothyroidism has been established

Most tissues and organs are affected by low metabolic rate Cellular energy production is decreased and many metabolites build up (compounds of protein and sugars)

Cellular energy is decreased and metabolites build up inside of cells, which increases mucous and water, thus forming cellular edema and changes organ texture. Cellular edema is called myxedema because it is NOT formed from water alone. Nonpitting edema forms everywhere Myxedema coma is rare serious complication

Myxedema

Hypothyroidism

Client reports more time sleeping (14 -16 hrs per day) Generalized weakness, anorexia, muscle aches and paresthesias Constipation is common, as is cold intolerance Women have difficulty getting pregnant or changes in menses; Men have problems with impotence and fertility

Hyperparathyroidism

Primary hyperparathyroidism results when 1 or more parathyroid glands does not respond to normal feedback of serum calcium In 80-85% of cases, cause is benign tumor in 1 parathyroid gland

Hyperparathyroidism

Client may develop renal calculi (kidney stones) and deposits of calcium in soft tissue of kidney Pathologic fractures, bone cysts and osteoporosis is caused due to increased rate of bone destruction (osteoclastia) Anorexia, nausea, vomiting, epigastric pain, constipation and weight loss are common when serum calcium levels are high

Peptic ulcer disease can result from elevated serum gastrin levels (hypergastrinemia) caused by hypercalcemia. Fatigue and lethargy may be present and will become more severe as calcium levels rise

Disorders of the Lymphatic System

blockage of lymph drainage

Lymphedema - swelling in tissues

- due to tumor pressure, parasites, or surgery

Elephantiasis

Immune System Disorders

Hypersensitivity Autoimmune Disease Immunodeficiency Diseases

Hypersensitivity
Production of antibodies to substances most tolerate, ie allergies.

Type I (acute) - Most common, starts within seconds

and

most often ends within 30 minutes.

Anaphylaxis causes edema, mucus, and congestion Asthma reaction to inhaled allergen.

Causes massive release of histamine and spasmatic contraction of the bronchioles.

Anaphylactic shock systemic response to an injected allergen.

Can cause bronchiolar constriction, circulatory shock, and possible death.

Type II (antibody-dependant cytotoxic)- as in

transfusion reaction.

Type III (immune complex)- large antibodyantigen complexes that get trapped under the tunic interna of blood vessels and cause inflammation.

Type IV (delayed)- occur 12 to 72 hours after

exposure. Delay commonly associated with travel time to lymph nodes. Cosmetics and poison ivy hapten commonly do this.

Autoimmune Diseases
Failure of the immune system to distinguish self from foreign antigens. Immune systems produces antibodies against bodies own tissues. Causes: - Cross reactivity fight against a foreign antigen leds to antibodies that attack self. - Abnormal exposure to self-antigens in the blood - Changes in the structure of self-antigens

Immunodeficiency Diseases

SCID Severe combined immunodeficiency disease - congenital deficiency of both T and B cells. - susceptible to opportunistic infections. - Bubble babies

AIDS Acquired Immunodeficiency diseases Acquired after birth, like HIV. HIV targets helper T cells Without these cells, all 3 immune responses are hampered. Most patients with AIDS die of opportunisitic infections.

HIV virus

Tutorial Questions

Discuss the following conditions Diabetes Mellitus Goiter Cushings Syndrome Pancreatitis HIV/AIDS Systemic Lupus Erythmatosus

The End