Endocrine/Metabolic Alterations
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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??
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