Chapter 27

1. Chapter 27 The Child with an Endocrine or Metabolic Alteration

2. Hormones Secreted by the Pituitary Gland Anterior pituitary • Growth hormone (GH) • Follicle stimulating hormone • Luteinizing hormone • Adrenocorticotropic hormone (ACTH) • Prolactin • Thyroid stimulating hormone (TSH)

3. Hormones Secreted by the Pituitary Gland • Posterior pituitary • Antidiuretic hormone (ADH) • Oxytocin

4. Tissues and Hormones of the Endocrine System

5. Feedback Control in Hormone Production

6. GH Deficiency • Occurs when GH is absent or produced in inadequate amounts • If other pituitary hormones are lacking, the condition is called hypopituitarism

7. GH Deficiency • The child’s growth pattern is an important part of determining normal growth • Growth hormone deficiency can occur at any age

8. Signs Indicative of GH Deficiency • Growth of less than 2 inches per year between the ages of 2 and 11 years in girls or the ages of 2 and 13 years in boys • Leveling off and slowing of growth progress • Growth below the fifth percentile on standardized growth charts Which child is more likely to have growth problems, A or B?

9. Etiology of GH Deficiency • Can be congenital or acquired • Tends to run in families but may occur in an individual with no family history • Congenital GH deficiency may be associated with an abnormal pituitary gland or be a part of another syndrome

10. Etiology of GH Deficiency • Acquired causes • Brain tumors (or other disorders) • Cranial irradiation • Infections • Trauma • Idiopathic

11. Clinical Manifestations of GH Deficiency • Short height for child’s age • Increased amount of fat around waist and in face • Emotional feelings about height or weight • Younger appearance than children of same age • Decreased muscle mass • Delayed skeletal maturation • Delayed onset of puberty • Delayed tooth development • Hypoglycemia

12. Diagnostic Evaluation • Thyroid panel • Serum electrolytes • Blood urea nitrogen • Creatinine • Complete blood count • Insulinlike growth factor 1 • Insulinlike growth factor binding protein 3 • Karotyping • Bone density scan

13. Therapeutic Management • If the cause is a tumor, surgical removal or radiation therapy may be indicated • Replacement of deficient hormones is often required even after successful treatment of a pituitary tumor

14. Treatment for GH Deficiency • Most children receive subcutaneous injections • Injections can be daily or three to four times per week and have increased growth velocity at bedtime • GH must be refrigerated • Close monitoring of growth with endocrinology visits every 3 to 4 months • Replacement therapy is continued until the child achieves an acceptable height or growth velocity drops to less than 1 inch per year

15. Assess compliance with medication regimen Assess and address issues of self-esteem Monitor growth (accurate measurement is important) Teach the child and family about the disorder Teach proper technique for administering medication (shots) Educational resources for parents: Magic Foundation www.magicfoundation.org/www Human Growth Foundation www.hgfound.org Child Growth Foundation www.childgrowthfoundation.org/ghd.htm Nursing Considerations

16. Precocious Puberty • Onset of puberty usually occurring before age 7 or 8 years in girls and before age 9 years in boys • Accompanied by the appearance of secondary sexual characteristics, advanced growth rate, and bone maturation • Causes short stature as an adult from premature closure of epiphyseal ends of the long bones

17. Pathophysiology of Precocious Puberty

18. Etiology of Precocious Puberty • Hormone-secreting tumors • Brain injury caused by head trauma • Infection • Thyroid dysfunction • Ovarian dysfunction • Idiopathic (most cases)

19. Clinical Manifestations of Precocious Puberty Female Breast development Axillary hair Pubic hair Adult body odor Onset of menses Acne Male Testicular enlargement Acne Penile enlargement Axillary and chest hair Pubic hair Adult body odor Facial hair Deepening voice

20. Diagnostic Evaluation • Bone density scan • Gonadotropin-releasing hormone stimulation test • Pelvic and adrenal ultrasound • Computed tomographic scan or magnetic resonance imaging • Blood work: testosterone levels, estrogen fraction test • Treatment involves the suppression of puberty

21. Nursing Considerations • Discuss concerns and issues about body image and sexuality • Offer support • Encourage patients and family to express their feelings about body changes • Assure children that their friends will go through the same body changes • If Lupron is used, plan for monthly or daily injections with the family

22. Sites for Kids, Parents, and Health Care Professionals • KidsHealthkidshealth.org • The Lawson Wilkins Pediatric Endocrine Society www.lwpes.org • The Magic Foundationwww.magicfoundation.org • The US National Library of Medicine National Institutes of Healthwww.nlm.nih.gov/medlineplus/encyclopedia.html

23. Hormones Secreted by the Thyroid • Thyroxin • Triiodothyroinine (T3) • Thyrocalcitonin (T4) • Secretion of thyroid hormones is controlled by TSH • TSH is secreted by the anterior pituitary gland • TSH is regulated by thyrotropin-releasing factor

24. Congenital Hypothyroidism • Present from birth • Thyroid gland does not produce enough thyroid hormone to meet metabolic needs • Caused by an absent, underdeveloped, or ectopic thyroid gland

25. Pathophysiology of Congenital Hypothyroidism

26. Etiology of Congenital Hypothyroidism • Spontaneous gene mutation • Autosomal recessive genetic transmission of an enzyme deficiency • Iodine deficiency • Failure of the central nervous system/thyroid feedback system mechanism to develop

27. Clinical Manifestations of Congenital Hypothyroidism • Large for age despite having poor feeding habits; increased birth weight • Puffy face, swollen tongue • Hoarse cry • Poor muscle tone • Cold extremities • Persistent constipation; bloated or full to the touch • Lack of energy, sleeps most of the time, appears tired even when awake • Little to no growth • Often appears perfectly normal at birth, which is why screening is vital

28. Diagnostic Evaluation for Congenital Hypothyroidism • State-required screening: TSH and T4 • Low T4, elevated TSH, or both indicate hypothyroidism • Positive test results may be followed by scan for bone age • Blood tests before 48 hours after birth may be falsely interpreted because of the rise in TSH immediately after birth

29. Nursing Considerations • Monitor growth and development of the infant (serial measurements of height, weight, and head circumference and screens for developmental milestones) • Assess for retarded physical growth and slow intellectual development; if cognitive impairment has occurred, provide support to family

30. Nursing Considerations • Teach family importance of daily administration of medication; drug therapy is need for life • Medication can be crushed and added to a small amount of formula, food, or water, or can be offered mixed with formula through a syringe or a nipple • Never put medication in a whole bottle of formula in case infant does not finish the bottle • Include instructions on taking pulse in the teaching plan to monitor for signs of drug overdose

31. Juvenile Hypothyroidism(A Case Study) A 10-year-old girl was seen for growth failure. She was a good student who had grown normally until the age of 7 years. The patient and her parents denied any symptoms of hypothyroidism. Physical examination revealed a child at the fifth percentile for height and the ninetieth percentile for weight. Her skin was dry and thick. Reflexes had a slow return phase. The thyroid was three times normal in size. Her bone age was 8 years.

32. Case Study Questions • What symptoms did this child display? • Are these symptoms consistent with juvenile hypothyroidism? • Could other symptoms be manifested in the child with juvenile hypothyroidism? • Diagnostic evaluation? • How will this child be treated? • What is the role of the nurse regarding this child and family?

33. Anterior Pituitary • ACTH stimulates the adrenal cortex to convert cholesterol into adrenal steroids

34. Congenital Adrenal Hyperplasia • Includes a group of disorders, each characterized by a deficiency of one of the enzymes needed to make cortisol • Autosomal recessive condition • More than 90% of the cases in children are caused by a partial or complete 21-hydroxylase enzyme deficiency

35. Cortisol • Maintenance of blood sugar levels • Partial maintenance of body fluids and electrolytes • Protection of the body against stress

36. Normal Adrenal Function • Adrenal gland composed of two regions • The inner region (the medulla) produces adrenaline • The outer region (the cortex) produces adrenal steroid hormones • Three types of adrenal steroids • Glucocorticoids • Mineralocorticoids • Androgens • Androgens are produced in abnormal amounts in children with congenital adrenal hyperplasia (CAH)

37. Cortisol • The pituitary gland located at the base of the brain produces ACTH, which activates the adrenal glands to produce cortisol. • When cortisol levels in the blood are too high, ACTH production stops so that cortisol production returns to normal. • When cortisol in the blood is too low, ACTH production increases until cortisol concentrations return to normal. • Cortisol production doubles or triples at the time of medical, surgical, or psychological stress.

38. Aldosterone • Aldosterone secretion is under the control of angiotensin. • Angiotensin is under the control of renin, which is produced by the kidneys. • When aldosterone is too low, sodium chloride levels in the blood fall, as does total body water, which leads to an increase in renin. This in turn causes an increase in angiotensin production and a subsequent return of aldosterone concentrations to normal. • When aldosterone is too high, serum sodium, total body water, and blood pressure increase, resulting in decreased renin and angiotensin production until aldosterone returns to normal.

39. Adrenal Androgens • Responsible for the development of pubic and axillary hair in girls • Testicular androgens play the major role in male masculinization

40. Adrenal Function and 21-Hydroxylase Deficiency • Three forms of 21-hydroxylase deficiency • Salt losing • Simple virilizing • Late onset or nonclassic

41. Salt-Losing Form of CAH • Near-total deficiency of the 21-hydroxylase enzyme • Results in the complete inability to produce cortisol and aldosterone

42. Salt-Losing Form of CAH • No cortisol = hypoglycemia • No aldosterone = salt and water loss • Increased cortisol precursors (17-hydroxyprogesterone) = salt-losing tendency • Increased androgens = masculinization

43. Simple Virilizing Form of CAH • Partial deficiency of the 21-hydroxylase enzyme • Near-normal or normal amounts of cortisol produced in response to increased ACTH output • Adrenals are able to increase production of aldosterone to compensate for salt loss

44. Simple Virilizing Form of CAH • Normal or near-normal cortisol level • Increased cortisol precursors (17-hydroxyprogesterone) • Increased aldosterone to compensate for salt-losing tendency • Increased androgens = masculinization

45. Adrenal Androgens in Salt-Losing and Simple Virilizing CAH • Excess androgen production during fetal life is associated with salt-losing and simple virilizing CAH and masculinizes the external genitalia of female infants • The simple virilizing form of CAH results in a moderate excess of androgen production with moderate masculinization of the external genitalia in female infants • Salt-losing CAH results in greater masculinization than simple virilizing CAH from greater production of androgens

46. Nonclassic or Late-Onset CAH • Refers to a mild deficiency of the 21-hydroxylase enzyme • Normal amounts of cortisol and aldosterone are produced • Increased amounts of cortisol precursors (17-hydroxyprogesterone) and adrenal androgens are produced • In boys and girls, results in rapid growth and early virilization • Masculinization and abnormal menses seen in girls

47. Nonclassic or Late-Onset CAH • Normal cortisol • Normal aldosterone • Increased 17-hydroxyprogesterone (moderate) • Increased androgens = masculinization

48. Diagnostic Evaluation for CAH • Ambiguous genitalia • Elevated levels of 17-hydroxyperogesterone • Serum electrolytes • Serum sodium will be low • Serum potassium will be high • Karyotype to determine genetic sex

49. Treatment • Cortisol or prednisolone administration (oral preparations available: Cortef tablets and Cortef suspension; Pediapred syrup) • Oral dosage is divided to simulate the normal diurnal pattern of ACTH secretion • Children with salt-losing form require aldosterone replacement • Preparation for salt-retaining hormone treatment is Florinef once or twice daily

50. Nursing Considerations • Recognition of ambiguous genitalia in the newborn and notification of parents of discovery • Assessment for signs of dehydration, electrolyte imbalance, and shock in the salt-wasting form of the disease • Assessment of vital signs and peripheral perfusion