Endocrine Dysfunction PDF
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Sara Headley
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This chapter covers endocrine dysfunction, specifically focusing on disorders of pituitary function, such as hypopituitarism, and hyperfunction disorders, including acromegaly, and precocious puberty. It also discusses diabetes insipidus and Syndrome of Inappropriate Antidiuretic Hormone (SIADH).
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A l l E l s e Chapter 47...
A l l E l s e Chapter 47 v i e r i ENDOCRINE DYSFUNCTION t e m s a Sara Headley, DNP, CPNP-PC, CNE n d d e r i v e d i t e m s The Endocrine System 2 The Endocrine System ► Three components ► Cells send chemical messages using hormones ► Target cells or end organs that receive the message ► Environment through which chemicals are transported (e.g. Blood, lymph, extracellular fluids) ► Hormones ► Complex chemical substances that exert controlling effect on other cells ► Endocrine glands (see Table 47.1) ► Master gland: anterior pituitary 4 The Endocrine System 5 Pituitary Gland ► Anterior lobe ► Growth hormone ► Prolactin ► Follicle stimulating hormone ► Posterior lobe ► Oxytocin ► Antidiuretic hormone All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 6 Disorders of Pituitary Function ► Disorders may be due to organic defects or have an idiopathic cause ► Problem may involve a single hormone or a combination of several hormonal deficiencies ► Clinical manifestations depend upon the hormone involved ► Disorder may result in an overproduction or hormone deficiency All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 7 Hypopituitarism: Growth Hormone Deficiency ► Deficient secretion of pituitary hormones ► Inhibits somatic growth and development of secondary sex characteristics ► Consequences: depend upon the degree of dysfunction ► Gonadotropin deficiency ► Growth hormone (GH) deficiency ► Thyroid-stimulating hormone (TSH) deficiency ► Corticotropin deficiency All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 8 Diagnostic Evaluation of Growth Hormone Deficiency ► Family history ► Growth patterns and previous health status ► Normal growth the first ► Slowed growth after infancy ► Delayed sexual development ► Physical examination ► Psychosocial evaluations ► Radiographic survey ► Endocrine studies All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 9 Therapeutic Management of Growth Hormone Deficiency ► Treatment is directed toward correction of the underlying disease process ► Replacement with GH is successful in 80% of affected children ► Therapy is ended when growth rates are less than 1 inch per year ► Girls: at 14 years of age ► Boys: at 16 years of age ► Care Management: ► Child and family support ► Preparation for testing and medication administration All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 10 Pituitary Hyperfunction ► Excess GH before closure of epiphyseal shafts results in overgrowth of long bones ► Patients can reach heights of 8 feet or more ► Vertical growth is accompanied by increased muscle ► Weight is generally in proportion to height All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 11 Pituitary Hyperfunction: Acromegaly ► Excess GH after epiphyseal closure is called “acromegaly” ► Typically, several facial features undergo overgrowth ► Head ► Lips, tongue, jaw, nose ► Paranasal, mastoid sinuses ► Separation and malocclusion of the teeth All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 12 Diagnostic Evaluation of Pituitary Hyperfunction ► History of excessive growth during childhood ► Evidence of increased levels of GH ► Radiographic studies ► May reveal a tumor ► Endocrine studies All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 13 Therapeutic Management of Pituitary Hyperfunction ► Surgical treatment to remove tumor ► Radiation and radioactive implants ► Hormone replacement therapy after surgery in some cases ► Thyroid extract ► Cortisone ► Sex hormones All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 14 Care Management of Pituitary Hyperfunction ► Early identification of children with excessive growth rates ► Early treatment for improved outcomes ► Observation for signs of tumor ► Emotional support ► Addressing body image concerns All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 15 Precocious Puberty ► Defined as sexual development before age 9 years in boys or before age 8 years in girls ► Evaluation for pathologic cause: required in white girls younger than 7 years and African American girls younger than 6 years ► Potential causes: ► Disorder of gonads, adrenal glands, or hypothalamic-pituitary gonadal axis ► 95% are idiopathic All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 16 Types of Precocious Puberty ► Central precocious puberty (CPP) ► 80% of cases of precocious puberty ► Early maturation and development of gonads and secondary sex characteristics ► Peripheral precocious puberty ► Premature development of breasts, pubic and axillary hair, and menses All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 17 Therapeutic Management of Precocious Puberty ► Treatment of specific cause if known ► May be treated with leuprolide (Lupron) ► Slows prepubertal growth to normal rates ► Treatment is discontinued at age when normal pubertal changes are expected to resume ► Psychologic support for child and family All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 18 Diabetes Insipidus All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 19 Diabetes Insipidus ► The principal disorder of the posterior pituitary gland ► Results from hyposecretion of antidiuretic hormone (ADH) ► Produces uncontrolled diuresis ► Primary causes: familial or idiopathic ► Secondary causes: trauma, tumors, central nervous system infection, aneurysm All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 20 Clinical Manifestations of Diabetes Insipidus ► Cardinal signs: polyuria and polydipsia ► First sign is often enuresis (involuntary urination: wetting self) ► Infants: irritability relieved with feedings of water but not milk; dehydration often occurs ► Diagnosed by reducing fluid intake with little or no effect on urine output All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 21 Therapeutic Management of Diabetes Insipidus ► Instruct parents in difference between diabetes insipidus and diabetes mellitus ► Daily hormone replacement ► Drug of choice: vasopressin (DDAVP) ► Nasal spray or intravenous administration ► Subcutaneous or intramuscular injection ► Treatment required for life All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 22 Syndrome of Inappropriate Antidiuretic Hormone (SIADH) All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 23 Syndrome of Inappropriate Antidiuretic Hormone (SIADH) All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 24 Syndrome of Inappropriate Antidiuretic Hormone (SIADH) ► Results from oversecretion by the posterior pituitary gland (increased ADH) ► Fluid retention and hypotonicity ► Kidneys unable to reabsorb water ► Manifestations ► Anorexia, nausea/vomiting, irritability, personality changes ► Fluid retention and hypotonicity ► Symptoms alleviated when ADH is decreased All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 25 Care Management of SIADH ► Accurate measurements of intake and output ► Observation for signs of fluid overload ► Seizure precautions ► ADH-antagonizing medications ► Child and family education All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 26 Disorders of Thyroid Function ► Thyroid hormone regulates basal metabolic rate ► Thyroid secretes two types of hormones ► Thyroid hormone, which is made up of ► Thyroxin (T4) ► Triiodothyronine (T3) ► Calcitonin ► Patients may have hypothyroidism or hyperthyroidism ► Patients may have disturbance in secretion of TSH (anterior pituitary) All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 27 Goiter ► Hypertrophy of the thyroid gland ► Congenital ► Usually results from maternal ingestion of antithyroid drugs during pregnancy ► Acquired ► Result of neoplasm, inflammatory disease, dietary deficiency (but rarely in children), or increased secretion of pituitary thyrotropic hormone All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 28 Care Management of Goiter ► Goiters may become noticeable during periods of rapid growth ► Large goiters may be obvious; smaller nodules may be evident only on palpation ► Thyroid hormone replacement is necessary for treatment of hypothyroidism ► Immediate surgery may be life-saving in infants born with goiter All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 29 Lymphocytic Thyroiditis ► Also known as “Hashimoto disease” or “autoimmune thyroiditis” ► Most common cause of thyroid disease in children and teenagers ► Accounts for largest percentage of juvenile hypothyroidism ► Occurs most frequently after age 6 years, peaks during adolescence All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 30 Clinical Manifestations of Lymphocytic Thyroiditis ► Enlarged thyroid gland ► Usually symmetric ► Firm, nontender ► Freely moveable ► Tracheal compression ► Sense of fullness ► Hoarseness, dysphagia ► Hyperthyroidism possible All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 31 Diagnostics & Management of Lymphocytic Thyroiditis ► Diagnostics ► Thyroid function tests usually normal ► TSH may be mildly elevated ► Antithyroid antibodies are present ► Goiter may be transient, asymptomatic ► Goiter may resolve spontaneously within 1-2 years ► Oral thyroid hormone administration often decreases the goiter significantly ► Surgery is contraindicated in this disorder All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 32 Hyperthyroidism (Graves Disease) ► Graves disease constitutes largest percentage of cases of childhood hyperthyroidism ► It is believed to be caused by autoimmune response to TSH receptors ► Signs include enlarged thyroid gland and exophthalmos ► Incidence peaks between ages of 12 to 14 years, but condition may be present at birth ► Familial association exists All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 33 Management of Graves’ Disease ► Therapy: controversial ► Goal of therapy: to retard rate of hormone secretion ► Treatments ► Antithyroid drugs (propylthiouracil and methimazole) ► Subtotal thyroidectomy ► Ablation with radioiodine All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. Care Management of Hyperthyroidism ► Identify children with hyperthyroidism ► Be alert for signs and symptoms ► Child needs quiet environment, rest periods ► Help family cope with emotional lability ► Dietary requirements are higher to meet child’s increased metabolic rate ► Medications have side effects 35 Disorders of Parathyroid Function ► Parathyroid glands secrete parathormone (PTH) ► Function of PTH: to maintain serum calcium level by ► Increasing release of calcium and phosphate from bone demineralization ► Increasing absorption of calcium and excretion of phosphate by the kidneys ► Promote calcium absorption in GI tract All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 36 Clinical Manifestations of Hypoparathyroidism ► Dry, scaly skin with eruptions ► Brittle hair, thin nails with transverse grooves ► Tetany, paresthesias, tingling, laryngeal stridor, spasms, or a combination of these ► Headache, seizures, emotional lability, depression, confusion, memory loss ► Diagnosis based on clinical symptoms ► Low plasma PTH All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 37 Clinical Manifestations of Hypoparathyroidism All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. Clinical Manifestations 38 Chvostek’s Sign Trousseau’s Sign Both due to hypocalcemia Therapeutic Management Hypoparathyroidism ► Goal is to maintain calcium and phosphate levels ► Tetany immediately corrected (with Ca, Vit D, and Mg) ► Monitor renal function, blood pressure, serum vitamin D levels ► Maintain seizure and safety precautions ► Monitor for laryngospasm 40 Disorders of Adrenal Function ► Adrenal cortex secretes three groups of “steroids” ► Glucocorticoids (cortisol, corticosterone) ► Mineralocorticoids (aldosterone) ► Sex steroids (androgens, estrogens, and progestins) ► Altered levels of these produce significant dysfunction ► Adrenal medulla secretes catecholamines: epinephrine and norepinephrine ► Catecholamine-secreting tumors are the primary cause of adrenal medullary hyperfunction All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 41 Acute Adrenocortical Insufficiency ► Adrenal crisis: LIFE THREATENING! ► Without Tx, 20% will DIE! (Cleveland Clinic, 2022) -Compare this to risk of death from anaphylaxis = 0.7-2% (Up To Date, 2021) ► Etiologic factors: hemorrhage into the gland from trauma, fulminating infections, abrupt withdrawal of exogenous cortisone, failure to increase cortisone during times of stress ► Diagnosis generally based on clinical symptoms ► Child in Adrenal Crisis Video, before and after injection: https://www.youtube.com/watch?v=ecJdXDG-6XY All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 42 Acute Adrenocortical Insufficiency (Cont.) All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 43 Acute Adrenocortical Insufficiency (Cont.) ► Clinical symptoms ► Increased irritability, headache ► Abdominal pain, nausea, vomiting ► Hyperpyrexia, cyanosis, seizures ► Diagnosis based on clinical presentation ► Therapeutic management ► Replace cortisol ► IVFs ► Nursing- monitor electrolytes, s/s of dec K+ All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 44 Chronic Adrenocortical Insufficiency (Addison Disease) ► Rare in children ► Causes: ► Infections, neoplasms, autoimmune processes, or idiopathic ► Symptoms: appear gradually after 90% of adrenal tissue is nonfunctional ► Diagnosis ► Measurement of functional cortisol reserve ► Therapeutic management ► Replacement of cortisol and aldosterone ► Should be taken qd-qod (AM) with food ► Care management ► Parental guidance and education All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 45 Cushing Syndrome ► TOO MUCH CORTISOL: ► A characteristic group of manifestations caused by excessive circulating free cortisol ► May be caused by excessive or prolonged steroid therapy ► Condition: reversible once steroids are discontinued ► Abrupt withdrawal of steroids: may precipitate acute adrenal insufficiency All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 46 Cushingoid Appearance (Cont.) All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 47 Diagnostic Evaluation of Cushing Disease ► Measurement of serum cortisol levels ► Imaging of pituitary and treatment glands ► Skull films to examine the sella turcica ► Measurement of bone density (brittle) ► Laboratory tests ► Fasting blood glucose level ► Serum electrolyte levels ► 24-hour urine collection All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 48 Therapeutic Management of Cushing Disease ► Dependent upon the cause ► Surgical intervention ► Replacement of GH, ADH, thyroid hormone, gonadotropins, and steroids ► Care management ► Steroid administration early morning ► Alternate-day schedule ► Postoperative complications All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 49 Congenital Adrenal Hyperplasia ► Occurs in 1 per 12,000-15,000 births ► Overproduction of adrenal androgens ► Results in virilization of the female fetus ► Varying degrees of ambiguous genitalia ► Salt-wasting crisis frequently occurs All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 50 51 Therapeutic Management of Congenital Adrenal Hyperplasia ► Many different types: Vary in severity ► Administer glucocorticoids (prednisone or hydrocortisone) ► Diagnose and assign sex to the child according to genotype (Karyotyping) ► Reconstructive surgery may be required; but may be hindered by state legislation….. ► **Not all cases are diagnosed at birth ► Family Planning: Genetics referral ► Family experience: https://www.youtube.com/watch?v=Dq33NSLcWUg&t=1s ► Late onset: https://www.youtube.com/watch?v=gv6lVfJ_lPQ All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 52 Disorders of Pancreatic Hormone Secretion ► Diabetes mellitus ► Characterized by a total or partial deficiency of the hormone insulin ► The most common endocrine disorder of childhood ► Peak incidence between 10 and 15 years of age ► Increased risk for African American and Hispanic children ► Types: Table 47.2 page 1435 All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 53 Type 1 Diabetes Mellitus ► Characterized by destruction of beta cells, usually leading to absolute insulin deficiency ► Onset typically from autoimmune process (such as a virus) in childhood and adolescence but can occur at any age ► Most childhood cases of diabetes mellitus are type 1 ► More prominent in caucasian people All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 54 55 Type 2 Diabetes Mellitus ► Arises because of insulin resistance ► Onset usually after age 45 ► Native American, Hispanic, and African American children: at increased risk for type 2 diabetes mellitus ► Affected people may require insulin injections All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 56 57 Pathophysiology of Diabetes Mellitus ► With a deficiency of insulin, glucose is unable to enter the cell and remains in blood, causing hyperglycemia ► When serum glucose exceeds renal threshold, glucose spills into urine (glycosuria) ► Cells break down protein for conversion to glucose by the liver (glucogenesis) All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 58 59 Ketoacidosis ► When glucose is unavailable for cellular metabolism, the body breaks down alternative sources of energy; ketones are released, and excess ketones are eliminated in urine (ketonuria) or by the lungs (acetone breath) ► Ketones in the blood are strong acids that lower serum pH and produce ketoacidosis All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 60 Diabetic Ketoacidosis ► Pediatric emergency ► Results from progressive deterioration with dehydration, electrolyte imbalance, acidosis, coma; may cause death ► Therapy: should be instituted in an intensive care unit setting All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 61 Manifestations and Diagnosis ► Manifestations ► Polydipsia ► Polyphagia ► Polyuria ► Labs ► Random BG >200 mg/dL with s/s ► Fasting BG > 120 mg/dL All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 62 Long-Term Complications of Diabetes Mellitus ► Microvascular complications, especially nephropathy and retinopathy ► Macrovascular disease, neuropathy ► With poor control, vascular changes as early as 2.5-3 years after diagnosis ► With excellent control, can be delayed 20 years All Elsevier items and derived items © 2014, 2010, 2006, 2002, Mosby, Inc., an imprint of Elsevier Inc. 63 Therapeutic Management of Type 1 Diabetes Mellitus ► Multidisciplinary approach ► Insulin therapy ► Glucose monitoring; goal, near normal levels of