1. Endocrine/Metabolic Alterations NUR 264 Pediatrics Angela Jackson, RN, MSN

2. Developmental Differences The endocrine system is incompletely developed at birth The endocrine system is incompletely developed at birth Less mature than any other body system Less mature than any other body system Pituitary gland is formed by the 4 th month of gestation and measurable amounts of hormone can be detected Pituitary gland is formed by the 4 th month of gestation and measurable amounts of hormone can be detected Newborn’s level of TSH is 10 times higher than levels seen in older children. Initial thyroid function tests cannot be interpreted using normal standards of childhood or adults Newborn’s level of TSH is 10 times higher than levels seen in older children. Initial thyroid function tests cannot be interpreted using normal standards of childhood or adults

3. Endocrine Glands Anterior pituitary Anterior pituitary Posterior pituitary Posterior pituitary Thyroid Thyroid Parathyroid Parathyroid Adrenal cortex Adrenal cortex Adrenal medulla Adrenal medulla Ovaries Ovaries Testes Testes Pancreas Pancreas

4. Growth Hormone Deficiency Characterized by poor growth and short stature Characterized by poor growth and short stature Occurs equally in both sexes Occurs equally in both sexes May result from injury, destruction of the anterior pituitary gland by a brain tumor, infection, or irradiation, but is usually idiopathic May result from injury, destruction of the anterior pituitary gland by a brain tumor, infection, or irradiation, but is usually idiopathic

5. GHD: Pathophysiology Hypothalamus secretes growth hormone- releasing hormone (GRH) Hypothalamus secretes growth hormone- releasing hormone (GRH) Production of growth hormone (GH) by the pituitary is stimulated Production of growth hormone (GH) by the pituitary is stimulated In GHD, the pituitary is unable to respond to the GRH, and GH is not produced In GHD, the pituitary is unable to respond to the GRH, and GH is not produced

6. GHD: Clinical Manifestations Short stature Short stature Deteriorating or absent rate of growth Deteriorating or absent rate of growth Higher weight to height ration Higher weight to height ration Delayed bone age (Determined by x-ray of the hand and wrist) Delayed bone age (Determined by x-ray of the hand and wrist) Increased fat in trunk area Increased fat in trunk area Childlike face with a large, prominent forehead Childlike face with a large, prominent forehead High-pitched voice High-pitched voice Hypoglycemia Hypoglycemia Micropenis and small testes in males Micropenis and small testes in males Delayed sexual maturation Delayed sexual maturation Delayed dentition Delayed dentition

7. GHD: Diagnosis Family history Family history Review of previous growth records Review of previous growth records Physical examination Physical examination Determination of growth rate Determination of growth rate Radiographic bone studies Radiographic bone studies Baseline blood testing Baseline blood testing Pituitary function testing Pituitary function testing

8. GHD: Treatment Goal of treatment is to promote normal growth rates by administration of growth hormone Goal of treatment is to promote normal growth rates by administration of growth hormone Growth hormone is given IM or SC Growth hormone is given IM or SC Treatment is discontinued once the epiphyseal growth plates have fused Treatment is discontinued once the epiphyseal growth plates have fused Treatment is expensive ($20,000 to $30,000 / year, depending on dosage) Treatment is expensive ($20,000 to $30,000 / year, depending on dosage)

9. GHD: Potential Complications of Treatment Slipped femoral epiphysis Slipped femoral epiphysis Pseudotumor cerebri Pseudotumor cerebri Edema Edema Sodium retention Sodium retention

10. GHD: Nursing Management Monitor growth Monitor growth Maintain growth chart Maintain growth chart Provide teaching to family concerning normal growth and development Provide teaching to family concerning normal growth and development Teach family proper medication administration techniques and side effects Teach family proper medication administration techniques and side effects Monitor medication dosages Monitor medication dosages Provide emotional support Provide emotional support

11. Precocious Puberty Breast development before the age of 7 in Caucasian girls and before the age of 6 in African-American girls Breast development before the age of 7 in Caucasian girls and before the age of 6 in African-American girls Development of secondary sex characteristics in boys less than 9 years old Development of secondary sex characteristics in boys less than 9 years old Five times more common in girls Five times more common in girls Idiopathic in girls, related to central nervous system abnormalities in boys Idiopathic in girls, related to central nervous system abnormalities in boys

12. Precocious Puberty: Pathophysiology Results from premature activation of the hypothalamic-pituitary-gonadal axis Results from premature activation of the hypothalamic-pituitary-gonadal axis Hypothalamus secretes gonadatrophin releasing hormone, which stimulates the pituitary to produce leutinizing hormone and follicle stimulating hormone. Estrogen or testosterone is also produced Hypothalamus secretes gonadatrophin releasing hormone, which stimulates the pituitary to produce leutinizing hormone and follicle stimulating hormone. Estrogen or testosterone is also produced

13. Precious Puberty: Clinical Manifestations Accelerated growth rate Accelerated growth rate Advanced bone age Advanced bone age Secondary sex characteristics Secondary sex characteristics Acne Acne Body odor Body odor May be emotionally labile, aggressive, and mood swings may occur May be emotionally labile, aggressive, and mood swings may occur Potentially fertile Potentially fertile

14. Precocious Puberty: Diagnosis Physical exam and history Physical exam and history Tanner staging Tanner staging Measurement of height and weight Measurement of height and weight X-rays for bone age, pelvic ultrasound for females to identify size of uterus and ovaries, CT, MRI or skull film to detect CNS lesions for males X-rays for bone age, pelvic ultrasound for females to identify size of uterus and ovaries, CT, MRI or skull film to detect CNS lesions for males Lab tests for LH, FSH, estradiol or testosterone Lab tests for LH, FSH, estradiol or testosterone GnRH stimulation testing GnRH stimulation testing

15. Precocious Puberty: Treatment Administration of luteinizing hormone – releasing hormone (Lupron) SC on a monthly basis Administration of luteinizing hormone – releasing hormone (Lupron) SC on a monthly basis Surgery, radiation or chemotherapy if caused by CNS tumor Surgery, radiation or chemotherapy if caused by CNS tumor Treatment results in a decrease in growth rate, stabilization or regression of secondary sex characteristics Treatment results in a decrease in growth rate, stabilization or regression of secondary sex characteristics Puberty resumes when therapy is discontinued Puberty resumes when therapy is discontinued

16. Precocious Puberty: Nursing Management Monitor growth Monitor growth Provide psychological support Provide psychological support Teach parents about normal growth and development Teach parents about normal growth and development Instruct parents that child’s mental age is congruent with chronologic age Instruct parents that child’s mental age is congruent with chronologic age Teach parents about medication administration and potential side effects Teach parents about medication administration and potential side effects

17. Diabetes Insipidus (DI) Disorder of water regulation Disorder of water regulation Deficiency of ADH results in excretion of large amounts of dilute urine Deficiency of ADH results in excretion of large amounts of dilute urine Most often seen as a complication following head injury or cranial surgery to remove tumors of the hypothalamic- pituitary region Most often seen as a complication following head injury or cranial surgery to remove tumors of the hypothalamic- pituitary region Other causes include vascular anomalies, infection, and genetic defect Other causes include vascular anomalies, infection, and genetic defect

18. DI: Clinical Manifestations Polyuria Polyuria Polydipsia Polydipsia Nocturnal enuresis Nocturnal enuresis Urine output can range from a few liters to eighteen liters a day Urine output can range from a few liters to eighteen liters a day Urine specific gravity is 1.005 or less, urine osmolarity is <200mmol/l Urine specific gravity is 1.005 or less, urine osmolarity is <200mmol/l Serum sodium concentration and plasma osmolarity are elevated Serum sodium concentration and plasma osmolarity are elevated

19. DI: Diagnosis UA for osmolarity, specific gravity, and sodium UA for osmolarity, specific gravity, and sodium Serum osmolarity, sodium and creatinine levels Serum osmolarity, sodium and creatinine levels Water deprivation test. Requires several hours to complete with close monitoring (I&O, weight, vital signs, hydration assessment, and urine and blood samples) Water deprivation test. Requires several hours to complete with close monitoring (I&O, weight, vital signs, hydration assessment, and urine and blood samples)

20. DI: Treatment Goals of treatment include: antidiuresis, uninterrupted sleep, and increased ability to participate in school and other programs Goals of treatment include: antidiuresis, uninterrupted sleep, and increased ability to participate in school and other programs Treated with daily replacement of ADH Treated with daily replacement of ADH Drug of choice is DDAVP, which is given intranasally or orally Drug of choice is DDAVP, which is given intranasally or orally

21. DI: Nursing Management Strict I&O and daily weight Strict I&O and daily weight Teach parents about the condition Teach parents about the condition Teach parents about lifelong need for medication and medication administration Teach parents about lifelong need for medication and medication administration Teach parents to monitor I&O and daily weights Teach parents to monitor I&O and daily weights

22. Congenital Hypothyroidism (CH) Present at birth Present at birth Reduced rate of metabolism caused by a low concentration of circulation thyroid hormones (T3 and T4) Reduced rate of metabolism caused by a low concentration of circulation thyroid hormones (T3 and T4) More females than males are affected More females than males are affected Caused by a defect in the embryonic development of the thyroid gland, inborn error of thyroid hormone synthesis, and pituitary dysfunction Caused by a defect in the embryonic development of the thyroid gland, inborn error of thyroid hormone synthesis, and pituitary dysfunction Thyroid gland is unable to produce T3 and T4 in response to increasing elevated levels of TSH secreted by the pituitary gland Thyroid gland is unable to produce T3 and T4 in response to increasing elevated levels of TSH secreted by the pituitary gland

23. CH: Clinical Manifestations Asymptomatic at birth Asymptomatic at birth Large posterior fontanel Large posterior fontanel Umbilical hernia Umbilical hernia Constipation Constipation Prolonged jaundice Prolonged jaundice Pallor hypothermia Pallor hypothermia Enlarged tongue Hypotonia, hypoactivity Feeding difficulties Delayed mental responsiveness Cool, dry, scaly skin Swollen eyelids

24. CH: Diagnosis Mandatory newborn screening Mandatory newborn screening Low T4 and a high TSH indicate CH Low T4 and a high TSH indicate CH Thyroid scan to evaluate for absence or ectopic placement of the thyroid gland Thyroid scan to evaluate for absence or ectopic placement of the thyroid gland

25. CH: Treatment Goal of therapy is to quickly normalize thyroid function Goal of therapy is to quickly normalize thyroid function Maintain the level of T4 in the upper half of the normal range and TSH in the normal range Maintain the level of T4 in the upper half of the normal range and TSH in the normal range Thyroid replacement with synthroid is initiated as soon as possible, starting dose of 10-15 mcg/kg/day Thyroid replacement with synthroid is initiated as soon as possible, starting dose of 10-15 mcg/kg/day Close monitoring of thyroid function Close monitoring of thyroid function Lifelong replacement is necessary to maintain normal metabolism Lifelong replacement is necessary to maintain normal metabolism

26. CH: Nursing Management Monitor growth and development Monitor growth and development Monitor lab values: every 2-4 weeks until thyroid function is within target range and medication dose is stabilized, every 3-4 months for first several years of life, every 6-12 months in adolescence Monitor lab values: every 2-4 weeks until thyroid function is within target range and medication dose is stabilized, every 3-4 months for first several years of life, every 6-12 months in adolescence Teaching parents proper medication administration, side effects, importance of continuing medication for rest of child's life and importance of regular blood tests to monitor thyroid function Teaching parents proper medication administration, side effects, importance of continuing medication for rest of child's life and importance of regular blood tests to monitor thyroid function

27. Questions??