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. Tests and Procedures • BUN, Creatinine, electrolytes, glucose • Hormone levels • Stimulation studies • Urinalysis • Fluid deprivation studies • Radiographs: bone age studies • Thyroid scan • Ultrasound to thyroid

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

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

8. 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).

9. Growth Hormone Deficiency • Failure of the pituitary to produce sufficient growth hormone to sustain normal growth in children • 80% are idiopathic • Familial patterns • Males are referred more often • Review growth charts • Short at birth or preemie

10. Assessment • 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

11. 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

12. Hypopituitarism

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

14. 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

15. 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

16. 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”

17. Hormone Replacement Therapy • Parents and child need to be educated on proper way to reconstitute and administer the GH. • Subcutaneous injection 3 to 7 days per week

18. Interdisciplinary Interventions • Children should be managed by a pediatric endocrinologist • Height and weight is obtained every 3 months and plotted on the growth chart • Bone age study yearly

19. 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

20. 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

21. 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 are usually lean and muscular • Therapy associated with increase risk of malignancies: leukemia, lymphoma, and tumors

22. 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

23. 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

24. 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

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

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

27. Pharmacologic Interventions • Low dose testosterone for the male. • Oral ethinyl estradiol for the girl.

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

29. 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

30. 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

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

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

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

36. CaREminder • Early diagnosis and prompt treatment of congenital hypothyroidism is essential for normal growth and development. The greater the delay treating congenital hypothyroidism, the greater the degree of cognitive challenge. With early diagnosis and treatment, children with congenital hypothyroidism can develop normally.

37. 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

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

39. Assessment • 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

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

41. Exophthalmos

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

43. Pharmacologic Interventions • Antithyroid drugs to block T 4 synthesis • Prophylthiouracil • Methimazole (Tapaxole)

44. 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.

45. Disorders of the Pancrease • Diabetes Mellitus – type 1 diabetes • Type 2 Diabetes

46. 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.

47. Diabetes Type I • Result of a genetic-environmental interaction • Seasonal variation – midwinter to spring • Family history • Illness or infection preceding the onset • Virus triggers the autoimmune response

48. Genetic Marker • Genetic Markers: • HLA –DR4 and HLA – DR3 • 20 to 40 % more susceptible

49. Natural History • Exposure of genetically predisposed individuals to environmental triggers • Leads to inflammation of beta cells of the pancreatic islets (islitis) and subsequent beta-cell injury.

50. Beta Cell Function • Hyperglycemia • 80 to 90% if beta cell function must be lost before hyperglycemia develops