Endocrine Disorders

1. Endocrine Disorders Jan Bazner-Chandler CPNP, MSN, CNS, RN

2. BMI • In recent years, BMI has received increased attention for pediatric use. In 1994, an expert committee charged with developing guidelines for overweight in adolescent preventive services (ages 11-21 years) recommended that BMI be used routinely to screen for overweight adolescents. In addition, in 1997 an expert committee on the assessment and treatment of childhood obesity concluded that BMI should be used to screen for overweight children, ages 2 years and older, using the BMI curves from the revised growth charts.

3. BMI Calculation • Can be calculated on-line at various sites including www.cdc.gov http://nhlbisupport.com/bmi/

4. Growth Charts • The growth charts consist of a series of percentile curves that illustrate the distribution of selected body measurements in U.S. children. Pediatric growth charts have been used by pediatricians, nurses, and parents to track the growth of infants, children, and adolescents in the United States since 1977. The 1977 growth charts were developed by the National Center for Health Statistics (NCHS) as a clinical tool for health professionals to determine if the growth of a child is adequate. The 1977 charts were also adopted by the World Health Organization for international use.

5. Endocrine Disorders • Growth hormone deficiencies • Hypo and hyper thyroid • Diabetes type I and type II • Diabetes Insipidus • PKU

6. Growth Charts

7. Disorders of the Pituitary Gland • Disorders of the pituitary gland depend on the location of the lesion or physiologic abnormality.

8. Posterior Pituitary • Secretes antidiuretic hormone (ADH or vasopressin) and oxytocin.

9. Anterior Pituitary • The anterior pituitary is made up of endocrine glandular tissue and secretes growth hormone (GH), adrenocorticotropic hormone (ACTH, TSH, FSH, LH, and prolactin).

10. Growth Hormone Deficiency • Hypopituitarism • 80% are idiopathic • Familial patterns • 1 in 10,000 children • Males are referred more often • Short at birth or premie

11. Causes • Growth hormone produced by the pituitary gland • If the pituitary gland doesn’t produce enough hormones for normal growth, growth slows down or stops. • Underdeveloped, damaged or malfunctioning pituitary gland.

12. Growth Hormone • GH stimulates the growth of all organs and tissues in the body, particularly the long bones.

13. Clinical Manifestations • Cherub facial features, frontal bossing, large eyes, and button nose • Males have small testes / micro-penis • Look much younger than chronological age • Delay of onset of puberty as a teenager

14. Emotional Difficulties • Emotional difficulties related to small stature are common. • Short child is often treated as if younger. • Teased by peers. • Child may dress as a younger child. • Body image is altered.

15. Hypopituitarism

16. Diagnostic Tests • Renal and Liver function test • Thyroid function • Sedimentation rate / ESR Done to rule out other causes of delayed growth

17. Definitive Diagnosis • Deficiency in the Growth Hormone • Bone age by x-ray: delayed bone age • Slow growth rate: as documented on standard CDC growth chart

18. Goals of Therapy • The goal of therapy is to augment growth so that at the time of epiphyseal close, a normal or normally expected adult height is attained. • Child will attain a final adult height consistent with their genetic potential

19. Growth Hormone Replacement • GH products are currently labeled for use in “children who have growth failure due to an inadequate secretion of normal endogenous growth hormone”

20. Hormone Replacement Therapy • Children should receive GH injections daily and at a minimum of three times a week • Treatment costs $10,000 to 50,000 dollars annually • Therapy can last for 2-4 years or until the epiphyses close

21. Management • Children should be managed by a pediatric endocrinologist

22. Ethical Issues • Social Justice Considerations • Children must meet specific criteria to be eligible for treatment • Parents must have access to health insurance coverage • Children who receive GH therapy will obtain the economic and social benefits of growing taller

23. Outcomes of Treatment • The child will verbalize positive feelings about his or her body image. • The child will demonstrate an increase in age-appropriate activities with peers. • Child will be able to participate in age related activities of daily living

24. Long Term Effects • Long term follow up needed: • Long term risks unknown • Physiologic trauma of daily injection • Metabolic effects of the therapy: children on GH therapy usually lean muscular • Therapy associated with increase risk of malignancies: leukemia, lymphoma, and tumors

25. Hypersecretion of Growth Hormone • In children called gigantism • Uncommon disease • 15% due to pituitary tumors causing increase release of GH. • Goal of treatment is surgical removal of GH-secreting adenoma.

26. Gigantism Acromegaly Anatomy & Physiology: Mosby

27. Precocious Puberty • Development of sexual characteristics before the usual age of onset of puberty. • Girls • Breast development before 7.5 years • Pubic hair before 8.5 years • Menses before 9.5 years • Boys • Secondary sexual characteristics before age 9

28. Assessment • Chart growth on growth chart. • Chronological timing of pubertal events. • Tanner Scale: true precocious puberty is characterized by 2 signs of puberty • Family history

29. Management / Prognosis • Treatment to halt or reverse sexual development. • Treatment needs to be started prior to closure of epiphysis. • Good outcomes if treatment stared early

30. Delayed Puberty • Failure to develop sexually at an appropriate age. • Girls • No breast development by age 13 or lack on menses within 5 years. • Boys • Secondary sexual characteristics not started by 14 years of age.

31. Etiology and Incidence • 2 to 3% of all adolescents. • Bone age moderately delayed. • History of small stature during infancy and early childhood. • Familial history

32. Rule out any Endocrine Abnormalities • 12% will have a pathologic reason for delayed puberty • Congenital adrenal hyperplasia • Hypothyroidism • Growth hormone deficiency

33. Management • Low dose testosterone for the male. • Oral ethinyl estradiol for the girl.

34. Hypothyroidism • Most common endocrine disorder of childhood • Hypothyroidism can be congenital, acquired, or secondary

35. Congenital Hypothyroidism • Results from absence or abnormal development of the thyroid gland or abnormal synthesis of thyroid hormone. • Most common cause is incomplete development of the thyroid gland

36. Importance of Thyroid Hormones • Thyroid hormones promote normal myelination during brain development in the first two to three years of life and normal skeletal growth • Regulates metabolism

39. Clinical Manifestations • Dull appearance • Feeding difficulties • Inactivity • Constipation • Characteristic faces • Flat nasal bridge • Puffy eyelids • Thick protruding tongue • Low hairline • Large posterior fontanel

40. Diagnosis • Diagnosis • Positive health history • Physical findings • Low levels of T3 and T4 • High levels of TSH • Neonatal screening is mandatory

41. Management • Replacement of sodium-l-thyroxine • Monitor TSH, T3 and T4 • Monitor growth and development • Frequent visits with emphasis on importance of therapy

42. Acquired Hypothyroidism • 15% of Down Syndrome children are hypothyroid • Auto-immune type of thyroiditis is most often the cause • High TSH levels as young as 2 years of age • Difficult to diagnose due to overlap of symptoms

43. Hyperthyroidism • Excessive secretion of thyroid hormone • More common in females 7:1 • Genetic and immunologic components • HLA-B8 • Autoimmune disease of unknown cause

44. Clinical Manifestations • Cry easily • Emotionally labile • Nervous • Short attention span • Can’t sit still / Hyperactive • Fatigue but unable to sleep at night • Accelerated growth / tall for age

45. Physical Exam • Enlarged thyroid gland • Asymmetric or lobular • Patient may present with neck swelling

46. Exophthalmos

47. Diagnosis History and Physical Levels of T3 and T4 are increased Levels of TSH are decreased

48. Treatment • Antithyroid drugs to block T 4 synthesis • Prophylthiouracil • Methimazole (Tapaxole)

49. Permanent Treatment • Radioactive Iodine is given to kill off some of the thyroid cells • Most common negative outcome is giving too much iodine that all thyroid producing cells are killed. • Surgical removal of gland or nodule – not always possible since often it is the entire gland resulting in overproduction of the hormone.

50. Diabetes Mellitus / Type 1 • Lack of insulin production in the pancreas. • Autoimmunity involved in destruction of beta cells. • 15 new cases per 100,000 children under 20 years of age. • Peak incidence between 10 and 14 years.