Endocrine/Metabolic Endocrine/Metabolic AlterationsAlterations

NUR 264NUR 264PediatricsPediatrics

Angela Jackson, RN, MSNAngela Jackson, RN, MSN

Developmental DifferencesDevelopmental Differences The endocrine system is incompletely The endocrine system is incompletely

developed at birthdeveloped at birth Less mature than any other body systemLess mature than any other body system Pituitary gland is formed by the 4Pituitary gland is formed by the 4thth month month

of gestation and measurable amounts of of gestation and measurable amounts of hormone can be detectedhormone can be detected

Newborn’s level of TSH is 10 times higher Newborn’s level of TSH is 10 times higher than levels seen in older children. Initial than levels seen in older children. Initial thyroid function tests cannot be thyroid function tests cannot be interpreted using normal standards of interpreted using normal standards of childhood or adultschildhood or adults

Endocrine GlandsEndocrine Glands Anterior pituitaryAnterior pituitary Posterior pituitaryPosterior pituitary ThyroidThyroid ParathyroidParathyroid Adrenal cortexAdrenal cortex Adrenal medullaAdrenal medulla OvariesOvaries TestesTestes PancreasPancreas

Growth Hormone DeficiencyGrowth Hormone Deficiency Characterized by poor growth and Characterized by poor growth and

short stature short stature Occurs equally in both sexesOccurs equally in both sexes May result from injury, destruction of May result from injury, destruction of

the anterior pituitary gland by a the anterior pituitary gland by a brain tumor, infection, or irradiation, brain tumor, infection, or irradiation, but is usually idiopathicbut is usually idiopathic

GHD: PathophysiologyGHD: Pathophysiology Hypothalamus secretes growth Hypothalamus secretes growth

hormone-releasing hormone (GRH)hormone-releasing hormone (GRH) Production of growth hormone (GH) Production of growth hormone (GH)

by the pituitary is stimulatedby the pituitary is stimulated In GHD, the pituitary is unable to In GHD, the pituitary is unable to

respond to the GRH, and GH is not respond to the GRH, and GH is not producedproduced

GHD: Clinical ManifestationsGHD: Clinical Manifestations Short statureShort stature Deteriorating or absent rate of growth Deteriorating or absent rate of growth Higher weight to height rationHigher weight to height ration Delayed bone age (Determined by x-ray of the Delayed bone age (Determined by x-ray of the

hand and wrist)hand and wrist) Increased fat in trunk areaIncreased fat in trunk area Childlike face with a large, prominent foreheadChildlike face with a large, prominent forehead High-pitched voiceHigh-pitched voice HypoglycemiaHypoglycemia Micropenis and small testes in malesMicropenis and small testes in males Delayed sexual maturationDelayed sexual maturation Delayed dentitionDelayed dentition

GHD: DiagnosisGHD: Diagnosis Family historyFamily history Review of previous growth recordsReview of previous growth records Physical examinationPhysical examination Determination of growth rateDetermination of growth rate Radiographic bone studiesRadiographic bone studies Baseline blood testing Baseline blood testing Pituitary function testing Pituitary function testing

GHD: TreatmentGHD: Treatment Goal of treatment is to promote Goal of treatment is to promote

normal growth rates by administration normal growth rates by administration of growth hormoneof growth hormone

Growth hormone is given IM or SCGrowth hormone is given IM or SC Treatment is discontinued once the Treatment is discontinued once the

epiphyseal growth plates have fusedepiphyseal growth plates have fused Treatment is expensive ($20,000 to Treatment is expensive ($20,000 to

$30,000 / year, depending on dosage)$30,000 / year, depending on dosage)

GHD: Potential Complications of GHD: Potential Complications of TreatmentTreatment

Slipped femoral epiphysisSlipped femoral epiphysis Pseudotumor cerebriPseudotumor cerebri EdemaEdema Sodium retentionSodium retention

GHD: Nursing ManagementGHD: Nursing Management Monitor growthMonitor growth Maintain growth chartMaintain growth chart Provide teaching to family concerning Provide teaching to family concerning

normal growth and developmentnormal growth and development Teach family proper medication Teach family proper medication

administration techniques and side administration techniques and side effectseffects

Monitor medication dosagesMonitor medication dosages Provide emotional supportProvide emotional support

Precocious PubertyPrecocious Puberty Breast development before the age of Breast development before the age of

7 in Caucasian girls and before the 7 in Caucasian girls and before the age of 6 in African-American girlsage of 6 in African-American girls

Development of secondary sex Development of secondary sex characteristics in boys less than 9 characteristics in boys less than 9 years oldyears old

Five times more common in girlsFive times more common in girls Idiopathic in girls, related to central Idiopathic in girls, related to central

nervous system abnormalities in boysnervous system abnormalities in boys

Precocious Puberty: Precocious Puberty: PathophysiologyPathophysiology

Results from premature activation of Results from premature activation of the hypothalamic-pituitary-gonadal axisthe hypothalamic-pituitary-gonadal axis

Hypothalamus secretes gonadatrophin Hypothalamus secretes gonadatrophin releasing hormone, which stimulates releasing hormone, which stimulates the pituitary to produce leutinizing the pituitary to produce leutinizing hormone and follicle stimulating hormone and follicle stimulating hormone. Estrogen or testosterone is hormone. Estrogen or testosterone is also producedalso produced

Precious Puberty: Clinical Precious Puberty: Clinical ManifestationsManifestations

Accelerated growth rateAccelerated growth rate Advanced bone ageAdvanced bone age Secondary sex characteristicsSecondary sex characteristics AcneAcne Body odorBody odor May be emotionally labile, aggressive, May be emotionally labile, aggressive,

and mood swings may occurand mood swings may occur Potentially fertilePotentially fertile

Precocious Puberty: DiagnosisPrecocious Puberty: Diagnosis Physical exam and historyPhysical exam and history Tanner stagingTanner staging Measurement of height and weightMeasurement of height and weight X-rays for bone age, pelvic ultrasound for X-rays for bone age, pelvic ultrasound for

females to identify size of uterus and ovaries, females to identify size of uterus and ovaries, CT, MRI or skull film to detect CNS lesions for CT, MRI or skull film to detect CNS lesions for malesmales

Lab tests for LH, FSH, estradiol or Lab tests for LH, FSH, estradiol or testosteronetestosterone

GnRH stimulation testingGnRH stimulation testing

Precocious Puberty: TreatmentPrecocious Puberty: Treatment Administration of luteinizing hormone – Administration of luteinizing hormone –

releasing hormone (Lupron) SC on a releasing hormone (Lupron) SC on a monthly basismonthly basis

Surgery, radiation or chemotherapy if Surgery, radiation or chemotherapy if caused by CNS tumorcaused by CNS tumor

Treatment results in a decrease in growth Treatment results in a decrease in growth rate, stabilization or regression of rate, stabilization or regression of secondary sex characteristicssecondary sex characteristics

Puberty resumes when therapy is Puberty resumes when therapy is discontinued discontinued

Precocious Puberty: Nursing Precocious Puberty: Nursing ManagementManagement

Monitor growthMonitor growth Provide psychological supportProvide psychological support Teach parents about normal growth and Teach parents about normal growth and

developmentdevelopment Instruct parents that child’s mental age Instruct parents that child’s mental age

is congruent with chronologic age is congruent with chronologic age Teach parents about medication Teach parents about medication

administration and potential side effectsadministration and potential side effects

Diabetes Insipidus (DI)Diabetes Insipidus (DI) Disorder of water regulationDisorder of water regulation Deficiency of ADH results in excretion Deficiency of ADH results in excretion

of large amounts of dilute urineof large amounts of dilute urine Most often seen as a complication Most often seen as a complication

following head injury or cranial surgery following head injury or cranial surgery to remove tumors of the hypothalamic-to remove tumors of the hypothalamic-pituitary regionpituitary region

Other causes include vascular Other causes include vascular anomalies, infection, and genetic defectanomalies, infection, and genetic defect

DI: Clinical ManifestationsDI: Clinical Manifestations PolyuriaPolyuria PolydipsiaPolydipsia Nocturnal enuresisNocturnal enuresis Urine output can range from a few Urine output can range from a few

liters to eighteen liters a dayliters to eighteen liters a day Urine specific gravity is 1.005 or less, Urine specific gravity is 1.005 or less,

urine osmolarity is <200mmol/lurine osmolarity is <200mmol/l Serum sodium concentration and Serum sodium concentration and

plasma osmolarity are elevatedplasma osmolarity are elevated

DI: DiagnosisDI: Diagnosis UA for osmolarity, specific gravity, and UA for osmolarity, specific gravity, and

sodiumsodium Serum osmolarity, sodium and Serum osmolarity, sodium and

creatinine levelscreatinine levels Water deprivation test. Requires Water deprivation test. Requires

several hours to complete with close several hours to complete with close monitoring (I&O, weight, vital signs, monitoring (I&O, weight, vital signs, hydration assessment, and urine and hydration assessment, and urine and blood samples) blood samples)

DI: TreatmentDI: Treatment Goals of treatment include: Goals of treatment include:

antidiuresis, uninterrupted sleep, and antidiuresis, uninterrupted sleep, and increased ability to participate in increased ability to participate in school and other programsschool and other programs

Treated with daily replacement of Treated with daily replacement of ADHADH

Drug of choice is DDAVP, which is Drug of choice is DDAVP, which is given intranasally or orallygiven intranasally or orally

DI: Nursing ManagementDI: Nursing Management Strict I&O and daily weightStrict I&O and daily weight Teach parents about the conditionTeach parents about the condition Teach parents about lifelong need for Teach parents about lifelong need for

medication and medication medication and medication administrationadministration

Teach parents to monitor I&O and Teach parents to monitor I&O and daily weightsdaily weights

Congenital Hypothyroidism (CH)Congenital Hypothyroidism (CH) Present at birthPresent at birth Reduced rate of metabolism caused by a Reduced rate of metabolism caused by a

low concentration of circulation thyroid low concentration of circulation thyroid hormones (T3 and T4)hormones (T3 and T4)

More females than males are affectedMore females than males are affected Caused by a defect in the embryonic Caused by a defect in the embryonic

development of the thyroid gland, inborn development of the thyroid gland, inborn error of thyroid hormone synthesis, and error of thyroid hormone synthesis, and pituitary dysfunctionpituitary dysfunction

Thyroid gland is unable to produce T3 and Thyroid gland is unable to produce T3 and T4 in response to increasing elevated levels T4 in response to increasing elevated levels of TSH secreted by the pituitary glandof TSH secreted by the pituitary gland

CH: Clinical ManifestationsCH: Clinical Manifestations Asymptomatic at Asymptomatic at

birthbirth Large posterior Large posterior

fontanelfontanel Umbilical herniaUmbilical hernia ConstipationConstipation Prolonged jaundiceProlonged jaundice Pallor hypothermiaPallor hypothermia

Enlarged tongueEnlarged tongue Hypotonia, Hypotonia,

hypoactivityhypoactivity Feeding difficultiesFeeding difficulties Delayed mental Delayed mental

responsivenessresponsiveness Cool, dry, scaly Cool, dry, scaly

skinskin Swollen eyelidsSwollen eyelids

CH: DiagnosisCH: Diagnosis Mandatory newborn screeningMandatory newborn screening Low T4 and a high TSH indicate CHLow T4 and a high TSH indicate CH Thyroid scan to evaluate for absence Thyroid scan to evaluate for absence

or ectopic placement of the thyroid or ectopic placement of the thyroid gland gland

CH: TreatmentCH: Treatment Goal of therapy is to quickly normalize Goal of therapy is to quickly normalize

thyroid functionthyroid function Maintain the level of T4 in the upper half of Maintain the level of T4 in the upper half of

the normal range and TSH in the normal the normal range and TSH in the normal rangerange

Thyroid replacement with synthroid is Thyroid replacement with synthroid is initiated as soon as possible, starting dose initiated as soon as possible, starting dose of 10-15 mcg/kg/dayof 10-15 mcg/kg/day

Close monitoring of thyroid functionClose monitoring of thyroid function Lifelong replacement is necessary to Lifelong replacement is necessary to

maintain normal metabolismmaintain normal metabolism

CH: Nursing ManagementCH: Nursing Management Monitor growth and developmentMonitor growth and development Monitor lab values: every 2-4 weeks until Monitor lab values: every 2-4 weeks until

thyroid function is within target range and thyroid function is within target range and medication dose is stabilized, every 3-4 medication dose is stabilized, every 3-4 months for first several years of life, every months for first several years of life, every 6-12 months in adolescence6-12 months in adolescence

Teaching parents proper medication Teaching parents proper medication administration, side effects, importance of administration, side effects, importance of continuing medication for rest of child's continuing medication for rest of child's life and importance of regular blood tests life and importance of regular blood tests to monitor thyroid functionto monitor thyroid function

Questions??Questions??