The Child With an Endocrine or Metabolic Alteration - Chapter 51

Sick-Day Management for the Child With Type 1 Diabetes Mellitus

1. Always give insulin injections, even if the child does not want to eat. If concerned that the child will become hypoglycemic with the usual dose, contact a diabetes healthcare team member for instructions. If ordered, use sliding scale, rapid- or short-acting insulin for hyperglycemia every 3 to 4 hours. 2. Test blood glucose level at least every 4 hours or more often if hypoglycemic or hyperglycemic. 3. Test for urine ketones with each voiding. Notify diabetes team member if moderate or large amounts of urine ketones are present. Additional regular insulin may be ordered. 4. Encourage intake of calorie-free liquids. Liquids aid in clearing ketones from the blood. 5. Follow the child's usual meal plan. If the child has a poor appetite, a sick-day diet replacing the usual grams of carbohydrate with simple carbohydrate foods is used. 6. Encourage rest. Exercising while ketones are present results in increased ketone formation. 7. Notify the diabetes healthcare team member of the following: • Nausea and vomiting • Fruity odor to the breath • Deep, rapid respirations • Decreasing level of consciousness • Moderate or high urine ketones • Persistent hyperglycemia

Growth Hormone Deficiency Therapeutic Management

A child with GH deficiency requires replacement therapy. Synthetic GH comes in a powdered form that must be diluted for administration or a premixed liquid form. It is given as a subcutaneous injection daily, usually at bedtime; however, alternative forms are also available, requiring administration a few times or once weekly. Dosage ranges from 0.18 to 0.3 mg/kg/week, depending on the child's age, pubertal stage, and response to therapy Once diluted, GH must be stored at 36° F to 46° F (2.2° C to 7.7° C). With treatment, the growth response is most evident in the first year, then gradually decreases; many children experience linear growth 200% of the pre-treatment velocity during the first year. After several years of treatment, linear growth averages 150% from the baseline (Ergun-Longmire, & Wajnrajch, 2013). When treatment is started at a younger age, the child's height potential is increased. GH therapy continues until the child's growth plates close or the child reaches an acceptable or predicted final height.

Galactosemia

A deficiency of galactose-1-phosphate uridyltransferase prevents the conversion of galactose to glucose in lactose digestion. Infants cannot properly digest milk or sugar. Although rare (1 in 40,000-60,000 live births), infants exhibit intrauterine growth retardation, hypotonia, liver damage, cataracts, and infections. The urine contains reducing substances. Vomiting and diarrhea occur after feedings. The child is on a lifelong lactose-restricted diet and close monitoring for and treatment of infections. If untreated, the infant usually dies; infants who have been treated may have developmental or learning deficits. The condition is genetically transmitted through an autosomal recessive inheritance pattern; referral to a genetic counseling center is warranted.

Tay-Sachs Disease

A genetic condition that affects primarily infants in the Ashkenazi Jewish population. It is caused by an abnormal buildup of gangliosides (normal constituents in nerve synapse membrane) in the neurons. After a 6-mo period of relatively normal development, the infant begins to demonstrate developmental delay and progressive neurologic deterioration. The infant usually exhibits macrocephaly, seizures, blindness, and deafness; death occurs during early childhood. Management is symptomatic and supportive to the child and family. Referral to a genetic counseling center is essential.

Congenital Adrenal Hyperplasia Nursing Considerations

All newborn girls should be assessed for ambiguous genitalia, fused labia, enlarged clitoris, or migration of the urethral opening. Infant boys and girls with unexplained dehydration and low serum sodium levels should be considered to have adrenal insufficiency and undergo careful assessment of fluid and electrolyte status.

Congenital Adrenal Hyperplasia Therapeutic Management

An accurate diagnosis and prompt treatment of fluid and electrolyte abnormalities may avert a salt-wasting crisis. The child with CAH requires lifelong glucocorticoid therapy. Oral glucocorticoid (hydrocortisone acetate, cortisone acetate) dosage is prescribed on the basis of body size and is given two or three times per day in either a liquid suspension or tablet form. For children with salt-wasting CAH, mineralocorticoid replacement therapy is required using fludrocortisone acetate (Florinef), which is taken once or twice daily. Therapy effectiveness is evaluated with serum electrolyte, 17-hydroxyprogesterone level, and renin levels. Special sick-day instructions should be provided to the family. The glucocorticoid dosage is usually doubled or tripled when the child is ill, has a broken bone, or is undergoing a surgical procedure. Bone-age radiographs are performed yearly to assess skeletal maturity; poor adherence to the medication regimen or under-treatment can result in advanced bone aging and decreased final adult height.

Actions of Insulin

Anabolic Actions of Insulin Promotes glucose as a fuel source Promotes storage of glucose as glycogen Prevents breakdown of fat stores Increases protein synthesis Catabolic Consequences of Insulin Deficit Promotes fats and proteins as fuel sources Allows glycogen stores to be broken down Allows fat stores to be depleted Allows protein breakdown into amino acids

Diabetes Insipidus Pathophysiology

Antidiuretic hormone (ADH) is produced in the hypothalamus, transported through the pituitary stalk, and stored in the posterior pituitary. It is carried through the blood to the kidneys, where it acts on the distal tubules and collecting ducts to increase reabsorption of free water, thereby concentrating urine and decreasing urinary output. ADH is under the control of osmoreceptors in the anterior hypothalamus. These osmoreceptors operate on a negative feedback system based on serum osmolality, particularly sodium concentration. When the serum osmolality is low, production of ADH decreases, causing increased urine output and normalizing osmolality; conversely, when serum osmolality is increased, ADH production increases, causing water retention and decreasing urine output. In diabetes insipidus, a deficiency of ADH makes the body unable to conserve water, which results in the excretion of large volumes of dilute urine. Loss of free water leads to an elevated serum sodium concentration. If the child has an intact thirst center, increasing oral intake might compensate for the large fluid loss. If the thirst drive is not intact or the child is unable to drink enough, the child can become dehydrated and develop a high serum sodium level. A child with an intact thirst center is able to self-regulate fluid needs and intake. An infant who is too young or a child who had head trauma or surgery may not able to recognize thirst; the healthcare provider should prescribe a 24-hour fluid intake requirement to ensure adequate hydration.

Insulin Therapy Administration.

Because insulin is a protein and would be digested if taken orally, it is given parenterally. Insulin is administered by subcutaneous injection using a specialized insulin syringe into the adipose tissue over large muscle masses. Preferred sites include the back of the upper arms, the top and outer portion of the thighs, the abdomen, and the hip To avoid injecting into the muscle or vascular space, a 45-degree angle of injection is used with a -inch needle or a 90-degree angle with a -inch needle. Rotation of injection sites helps prevent areas of adipose hypertrophy (fatty lumps), which interfere with insulin absorption. Various injection sites absorb insulin at slightly different rates. Absorption is also affected by body temperature and the level of muscle activity (exercise) under a given injection site. To help decrease variations in absorption, the child should use different locations within a major injection site for one day. For example, the child would inject insulin in one location on the back of the upper right arm for the morning injection, then rotate to another location on the back of the upper right arm for the afternoon injection, and a third location again on the upper right arm for the evening injection. The next day, another major site such as the top, outer portion of the right thigh may be used, depending on the site rotation schedule. Insulin can be administered by an insulin syringe, air injector, or insulin pump. Disposable syringes are to be used only one time and then safely discarded (with the syringe/needle placed in a puncture-resistant, opaque container before placing in the trash). The air injector uses compressed air to deposit the insulin within the fatty tissue without the use of a needle. The child or family must learn to use the air injector device by correctly loading insulin, adjusting pressure settings to avoid intramuscular delivery, and cleaning. The insulin pump is a battery-operated device that provides a continuous infusion of rapid-acting insulin Use of the pump in the pediatric population continues to increase. Evidence suggests that the insulin pump provides tighter control of blood sugar levels and more flexibility in lifestyle. The pump is a mechanical device, approximately the size of a pager, which is often worn on a belt or in a pocket. It delivers insulin to the body through an infusion set consisting of thin plastic tubing attached to a cannula or needle inserted into the subcutaneous tissue of the thigh, abdomen, or buttocks. A continuous basal rate of insulin infusion is maintained, and bolus dosages are infused as determined by blood glucose testing. The pump most closely mimics physiologic delivery of insulin A candidate for insulin pump therapy must be confident in diabetes management, willing to measure blood glucose meticulously (at least four glucose checks per day), be able to count carbohydrates and calculate appropriate insulin coverage, and have a supportive home environment. Parents and/or other caregivers are responsible for the insulin pump when used by infants and toddlers. Dietary recommendations are different for children or adolescents using an insulin pump and are based on carbohydrate counting.

Diabetes Insipidus Etiology

Both forms of DI can occur from inherited defects or acquired conditions Central DI frequently results from head trauma, tumors, or infection in the area of the hypothalamus. The most common type of tumor involving the hypothalamus that causes DI is craniopharyngioma. Cranial radiation for treatment of tumors can lead to ADH deficiency. Other causes include infections of the CNS such as meningitis or encephalitis, and congenital malformations such as septo-optic dysplasia or isolated pituitary malformation or ectopy. Several genetic mutations in the vasopressin gene causing DI have been identified. Central DI can also be caused by an idiopathic autoimmune process. Nephrogenic DI can be caused by genetic mutations or hypercalcemia, low protein diet, hypokalemia, the release of a ureteral obstruction or certain medications

Diabetic Ketoacidosis

DKA is the metabolic consequence of a severe insulin deficit leading to hyperglycemia and the presence of ketone bodies in the blood, followed by metabolic acidosis. For individuals with type 1 diabetes, DKA is seen more frequently in young children and adolescents than in adults DKA affects up to 40% of children diagnosed with new-onset type 1 diabetes mellitus DKA results from an absolute or relative insulin deficit. In the younger diabetic child, the most common cause is insulin resistance, as in a stress response initiated by an infection. In the adolescent, the most common cause is one or more missed insulin injections.

Indicators of Diabetes Insipidus or Syndrome of Inappropriate Antidiuretic Hormone (SIADH)

Diabetes Insipidus Increased urination (polyuria) Nocturia Increased thirst (polydipsia) Dehydration Hypernatremia Urine specific gravity <1.005 Elevated serum osmolality (>300 mOsm/kg) Decreased urine osmolality Syndrome of Inappropriate Antidiuretic Hormone (SIADH) Decreased urination Hypertension Weight gain Fluid retention Hyponatremia Urine specific gravity >1.030 Decreased serum osmolality (<280 mOsm/kg) Increased urine osmolality

Congenital Adrenal Hyperplasia Etiology/Incidence

CAH is caused by a defect in the enzymatic pathway of adrenal steroid production. Diminished glucocorticoid production prompts increased ACTH production by the pituitary gland, further increasing adrenal androgen excess. Several enzymatic defects have been identified, the most common being 21-hydroxylase deficiency (21 OHD), accounting for more than 90% of cases. Infants with diminished mineralocorticoid production (specifically aldosterone) will waste salt through the kidneys, resulting in a "salt-wasting" crisis that manifests as poor feeding, vomiting, dehydration, failure to thrive, weight loss, hypotension, hyponatremia, and hyperkalemia. This condition accompanies the more life-threatening form of CAH, occurring in 75% of patients with 21 OHD CAH The worldwide incidence of the classical type of 21 OHD CAH is between 1 in 15,000 to 1 in 20,000 live births In contrast, the incidence of non-classical 21 OHD CAH is much higher, with 1 in 1,000 live births. Ashkenazi Jews and Hispanics are most afflicted by this disease

Congenital Adrenal Hyperplasia Manifestations

CAH is marked by ambiguous genitalia of the newborn female infant, postnatal virilization in both sexes, and salt-wasting crisis (in the first few weeks of life) with low serum sodium, high serum potassium, hypovolemia, and eventual hypotensive crisis. Simple virilizing CAH is not associated with a salt-wasting crisis and manifests as a muscular body, advanced bone age, and premature pubic hair. Typically, this form is seen later in infancy or early childhood. Untreated or poorly treated CAH can result in an advanced bone age with ultimate adult short stature. A milder form of CAH (3-beta-hydroxysteroid dehydrogenase (3β-HSD) can cause symptoms during childhood or adolescence, with the child exhibiting hirsutism, menstrual irregularities, or delayed menses.

Precocious Puberty Etiology

Central precocious puberty can be idiopathic or caused by CNS tumors (including benign hamartomas), head trauma, infection, septo-optic dysplasia, genetic mutations, or cranial radiation. Central precocious puberty is associated with several genetic syndromes: neurofibromatosis type I, tuberous sclerosis, and Sturge-Weber CNS abnormalities are seen with much greater frequency in boys than girls In girls, precocious puberty is idiopathic in 90% of cases Factors that contribute to precocious puberty in girls include obesity, ethnicity, genetic predisposition, psychosocial stress, and exposure to certain environmental chemicals that disrupt endocrine function The causes of the much less common precocious pseudopuberty (peripheral precocious puberty) include congenital adrenal hyperplasia; other abnormalities or tumors of the adrenal glands, ovaries, or testes; McCune-Albright syndrome; DAX1 genetic mutations; exogenous steroids; and untreated chronic hypothyroidism

Syndrome of Inappropriate Antidiuretic Hormone Etiology

Childhood SIADH is rare and usually related to an underlying cause. The most frequent cause is excessive use of vasopressin in the treatment of central DI Other causes include CNS infections (e.g., encephalitis and meningitis), head trauma, brain tumors, and generalized seizures SIADH is usually transient and resolves when the underlying condition is corrected. However, brain surgery in the region of the hypothalamus or pituitary gland can cause the child to have transient SIADH but permanent DI. A triple response can occur after surgery: the child has DI, then experiences temporary SIADH, finally returning to permanent DI

Diabetes Insipidus

Diabetes insipidus (DI) is an inability to concentrate urine, accompanied by hypernatremia and dehydration. In central DI, the most common form, there is a deficiency of vasopressin, also known as antidiuretic hormone (ADH). In nephrogenic DI, the kidneys are insensitive to vasopressin

Delegating Diabetes Management Responsibilities

Children with diabetes are functionally able to perform diabetes management tasks far sooner than they can cognitively understand the implications of this action or consequences of omitting the action. Transfer of responsibility from the parents to the child should be on a step-by-step basis, according to the child's cognitive understanding and functional abilities. Diabetes management shifts from full parental responsibility to a partnership between parent and child and then to the acceptance of full responsibility by the young adult. Delegation of management responsibility to the child too soon can result in poor diabetes control and frequent DKA episodes. Ongoing parental support and supervision is essential

Acquired Hypothyroidism Pathophysiology

Circulating autoantibodies known as TSH receptor blocking antibodies decrease thyroid gland production of triiodothyronine (T3) and T4. These antibodies bind at the TSH receptor sites on the thyroid gland, resulting in decreased thyroid hormone production. The cause of this antibody production is unknown In contrast to congenital hypothyroidism, adverse effects from hypothyroidism acquired after 2 to 3 years of age are often reversible. Goiter, an enlarged thyroid gland, occurs in response to increased TSH secretion, autoimmune attack of the thyroid gland, or goitrogens.

Hyperthyroidism (Graves' Disease) Pathophysiology

Circulating autoantibodies known as thyroid-stimulating immunoglobulins (TSIs) stimulate the thyroid gland to make T3 and T4. These antibodies bind to the TSH receptor sites on the thyroid gland, resulting in excessive thyroid hormone production. The cause of this antibody production is unknown. In newborns, maternal TSI is transferred through the placenta to the fetus. TSIs bind to the TSH receptor, causing neonatal hyperthyroidism.

Acquired Hypothyroidism Manifestations

Clinical manifestations of hypothyroidism include goiter (one lobe frequently larger than the other); dry, thick skin; coarse, dull hair; fatigue; cold intolerance; constipation; weight gain; decreased linear growth; edema of face, eyes, and hands; and irregular or delayed menses

Congenital Hypothyroidism

Congenital hypothyroidism is a condition in which the thyroid gland does not produce sufficient thyroid hormone to meet the body's metabolic needs. The condition is present from birth and, if not treated, can lead to intellectual impairment.

Congenital Hypothyroidism Etiology

Congenital hypothyroidism is caused by an absent (aplastic), underdeveloped, or ectopic thyroid gland. This group of congenital defects is referred to as thyroid dysgenesis. For unknown reasons, the fetal thyroid gland fails to develop properly or fails to migrate to the appropriate location. Other rare causes are hypothalamic or pituitary disorders in which TSH secretion is insufficient to stimulate the thyroid gland. Biochemical defects in thyroid hormone production also cause congenital hypothyroidism. Maternal intake of medications, such as propylthiouracil (PTU), during pregnancy to control maternal hyperthyroidism can cause transient hypothyroidism in the infant. Transfer of maternal antibodies to the fetus can also cause transient hypothyroidism

Congenital Hypothyroidism Diagnostic Evaluation

Congenital hypothyroidism is detected by elevated TSH (with or without T4 evaluation) on newborn screening that is collected after 24 hours of age, although the best time for testing is between 48 and 72 hours of age Practitioners must be cautious with test interpretation because of the rise in TSH immediately after birth as part of the normal newborn transition. For a newborn with a low T4 value, a TSH level will be obtained. A low T4 level with TSH elevation is indicative of congenital hypothyroidism; further testing is often done to determine the cause Thyroid scans can identify any functioning thyroid tissue. Treatment should never be delayed while waiting for scan results.

DKA Diagnostic Evaluation

Diabetic ketoacidosis is confirmed by the following test results: • Blood glucose ≥ 200 mg/dL • Venous pH < 7.3 • Ketonuria • Ketonemia • Serum potassium: Elevated, normal, or low • Serum phosphorus: Low • White blood cell (WBC) count: Elevated as a result of stress demargination (higher with infection) • Serum carbon dioxide: Low

Growth Hormone Deficiency Diagnostic Evaluation

Diagnosis of GH deficiency begins with careful measurements of growth over an extended period (usually 6 to 12 months). Height should be measured on a consistent scale, preferably with a calibrated stadiometer. Initial screening involves thyroid function tests, electrolytes, blood urea nitrogen (BUN), creatinine, complete blood count, insulin-like growth factor 1 (IGF-1) and IGF binding protein 3 (IGFBP-3), and a bone age radiograph. Normal thyroid function is essential for adequate growth; thyroid studies are essential when evaluating for short stature. Complete blood count and other specific blood screening for any systemic or chronic illness should be done. Electrolytes and renal function studies eliminate primary kidney dysfunction as a cause of poor growth. A karyotype (chromosomes) might be performed for girls to rule out Turner syndrome. Because GH is normally secreted in pulses throughout the day and night, stimulation testing is necessary to confirm the diagnosis of GH deficiency. Agents used in provocative testing to stimulate GH production include insulin, arginine, clonidine, glucagon, and levodopa (L-dopa). Once the stimulating agent is given, serial GH levels are determined. Although diagnostic criteria vary, most clinicians accept a GH level less than 10 ng/mL as indicative of GH deficiency. Generally, two positive tests are required for diagnosis.

Precocious Puberty Diagnostic Evaluation

Diagnosis of precocious puberty begins with a thorough history, including onset of secondary sexual characteristics, and a physical examination. Bilateral testicular enlargement in males, and both bilateral breast development and pubic hair in females will be apparent. Blood tests are performed to evaluate for elevated levels of LH, FSH, testosterone, and estrogen. Unfortunately, because these hormones are released in small bursts during the day, random samples may not be adequate. The gonadotropin-releasing hormone (GnRH) stimulation test is considered the gold standard for diagnosis; however, its lack of availability has led to the use of GnRH analogs (GnRHa) instead. GnRHa is administered intravenously or subcutaneously to stimulate the release of LH and FSH from the pituitary gland. Serial samples of LH and FSH are then obtained over a 2-hour period after IV administration. With subcutaneous administration, a single sample of LH and FSH is obtained using an ultrasensitive assay. Before the onset of puberty, the FSH peak is higher than the LH peak. With the onset of puberty, the LH peak is higher than the FSH peak. An LH/FSH ratio of greater than or equal to 2 or LH level is greater than or equal to 5 IU/L after GnRHa is consistent with central precocious puberty Radiographic studies also support the diagnosis of precocious puberty. Radiographs of the wrist determine bone age and maturation and can assist in predicting final adult height. Skull radiographs screen for CNS lesions, although CT and MRI scans are more accurate in visualizing tumors. Abdominal ultrasound and pelvic ultrasound are beneficial in diagnosing adrenal and ovarian tumors or cysts. Pelvic ultrasound also provides evidence of pubertal changes in the uterus and ovaries. Finally, isolated pubic hair development and elevated androgen hormone levels suggest an adrenal origin for premature hair growth.

Type 1 Diabetes Mellitus Diagnostic Evaluation

Diagnosis of type 1 diabetes mellitus is made on the basis of laboratory data: HbA1c greater than or equal to 6.5% OR a fasting blood glucose (FBG) exceeding 126 mg/dL OR a 2-hour oral glucose tolerance test greater than or equal to 200 mg/dL OR a random serum glucose of 200 mg/dL or more with classic hyperglycemia symptoms Ketonuria, although not diagnostic, is a frequent finding, as is glycosuria. Glucose tolerance testing is rarely used in diagnosing type 1 diabetes mellitus. The glycosylated hemoglobin (HbA1c) value is elevated in response to prolonged elevations of blood glucose.

Diabetes Insipidus Diagnostic Evaluation

Diagnostic criteria include polyuria with associated hypernatremia (greater than 150 mEq/L) low urine specific gravity (less than 1.005), and serum osmolality greater than 300 mOsm/kg in the absence of hyperglycemia, hypokalemia, hypercalcemia, and chronic renal insufficiency. Urine should be checked for glucose to rule out hyperglycemia as a cause of increased urine output A water deprivation test is sometimes necessary to confirm the diagnosis. In this 7- to 8-hour procedure, the child is deprived of all fluid intake. A normal response is decreased urine output with a high urine specific gravity and no change in serum sodium. The child with DI continues to produce large amounts of dilute urine (low urine specific gravity) during fluid restriction. The serum sodium level also increases. To ensure the child's safety, this test is done in a hospital setting with frequent monitoring of serum sodium, hematocrit, and osmolality. Urine osmolality and output are also measured. The child is weighed at the beginning, middle, and conclusion of the water deprivation test. Water deprivation should be stopped if the child loses more than 5% of baseline body weight, has intolerable thirst, becomes dehydrated, or demonstrates a significant change in vital signs or neurologic status

Acquired Hypothyroidism Diagnostic Evaluation/Therapeutic Management

Elevated TSH and low T4 levels are diagnostic of hypothyroidism. Elevated TSH level is the most sensitive indicator of primary hypothyroidism. Thyroiditis is diagnosed by the presence of circulating thyroid antibodies and is usually associated with a firm goiter. Initially TSH is elevated with normal T4 levels, although T4 decreases over time. With secondary or tertiary hypothyroidism, TSH is not elevated; therefore, thyroid-releasing hormone stimulation testing is usually required for diagnosis. Management of the child with hypothyroidism involves thyroid hormone replacement, usually with levothyroxine. The dosage varies according to the child's age and weight and is given as a single daily dose. The dose is titrated to maintain T4 in the upper half of the normal range and to maintain TSH in the normal range for age.

Hyperthyroidism (Graves' Disease) Diagnostic Evaluation

Elevated serum T4 and T3 levels with suppressed TSH levels, associated with signs and symptoms of hyperthyroidism, suggest Graves' disease. Autoantibodies to thyroid tissue usually are positive. Thyroid uptake of radioactive iodine is increased

Syndrome of Inappropriate Antidiuretic Hormone Pathophysiology

Excessive antidiuretic hormone (ADH) results in the kidney reabsorbing too much free water. This causes decreased output of concentrated urine, evidenced by a high urine specific gravity (greater than 1.030). The excess water also causes a slightly expanded intravascular fluid volume and a low serum sodium level. Once the sodium level falls below 120 mEq/L, the child can become symptomatic and have anorexia, nausea, weakness, weight gain, confusion, irritability, and seizures

Type 1 Diabetes Mellitus Therapeutic Management Physical Activity

Exercise is an important aspect of diabetes management. Exercise enhances the action of insulin in lowering blood glucose levels. In addition, exercise promotes a greater sense of well-being, improves physical and cardiovascular fitness, and contributes to an improved lipid profile. The child with diabetes should be encouraged to participate in age-appropriate sports. Early enjoyment of a sport or activity can promote a lifelong active lifestyle. Because exercise lowers glucose levels, the child must be taught how to prevent hypoglycemia. The child should try to schedule activities to avoid exercising when an insulin dose is peaking. Proper hydration must be maintained while exercising.

Nursing Care Plan The Child With Type 1 Diabetes Mellitus in the Community Setting

Focused Assessment • Obtain history from family of signs and symptoms of hyperglycemia: • Polydipsia: requests water through the night • Polyuria: increased frequency of urination • Enuresis (if previously toilet trained) • Polyphagia • Weight loss • Fatigue • Nausea and vomiting • Ask family about all medications the child is taking. • Certain drugs can cause hyperglycemia • Perform a physical assessment and look for signs and symptoms of: • Dehydration • Acidosis • Infection • Assess the child's and family's knowledge and ability to carry out the diabetes home management plan. • Assess the child's and family's ability to cope with the diagnosis of a chronic illness.

Growth Hormone Deficiency Etiology

GH deficiency can be isolated or associated with an underlying cause. Such causes include hypopituitarism, congenital malformations of the pituitary gland, brain tumors (most commonly craniopharyngioma), and cranial irradiation. Other disorders associated with short stature that may respond to GH therapy include Turner syndrome, Prader-Willi syndrome, and chronic illnesses such as renal disease and inflammatory bowel disease.

Precocious Puberty Manifestations

Girls Breast development Enlargement of vagina, uterus, and ovaries Pubic hair Axillary hair Acne Growth spurt Adult body odor Onset of menstrual periods Moodiness Boys Testicular enlargement Penis enlargement Pubic hair Facial hair Acne Deepening of voice Adult body odor Moodiness

Pathophysiology Type 1 Diabetes Mellitus

Glucose is the primary source of energy for body cells. Any extra glucose taken in by the body can be stored as glycogen in muscle or liver cells or in the form of fatty tissues. Glucose can be extracted from glycogen for periods of fasting (e.g., overnight). Once glycogen stores have been depleted, new glucose (gluconeogenesis) is made from amino acids released from muscle into the bloodstream. The energy for gluconeogenesis is supplied by the breakdown of stored fats. Insulin, a hormone, is secreted by the beta cells of the pancreas. Its main function is to regulate the blood glucose level by controlling the rate of glucose uptake by cells. Little or no insulin is secreted by the beta cells when a person is in the fasting state; greater quantities are secreted after the person has eaten a meal. In the fasting state, with relatively small quantities of available insulin, the body mobilizes fats and proteins to be used as fuel sources. The liver then converts the fats into ketoacids, or ketones. With the assistance of insulin, ketones are transported into the cells and are used as an alternative source of fuel for cellular energy. This process ensures an energy source during long periods of fasting. Not all cells in the body are capable of using ketone bodies and require glucose as their primary fuel In the absence of insulin, the metabolism of fats, proteins, and carbohydrates is impaired. Glucose is unable to move into the intracellular space, resulting in hyperglycemia. As blood glucose levels exceed the renal threshold, glucose is "spilled" into the urine, causing osmotic diuresis and subsequent polyuria. Excessive thirst follows in response to fluid loss. Fatigue, hunger, and weight loss also accompany the onset of type 1 diabetes mellitus because cellular starvation continues in the absence of insulin. Ketones (ketoacids), manufactured by the liver from adipose tissue, are produced in response to cellular starvation. In the absence of insulin, ketones are also unavailable to the cell for nourishment. Increasing blood levels of ketones (ketonemia) result in ketoacidosis.

Hyperthyroidism (Graves' Disease) Manifestations

Goiter, increased appetite, weight loss, nervousness, diarrhea, increased perspiration, heat intolerance, increased heart rate, muscle weakness, palpitations, tremors, exophthalmos, poor attention span, and behavior or school problems are common in Graves' disease In the neonate, irritability, tachycardia, hypertension, voracious appetite with poor weight gain, flushing, prominent eyes, and thyroid enlargement are major signs. These signs are self-limiting, but cardiac failure and death can occur if the signs are unrecognized or poorly treated.

Hyperthyroidism (Graves' Disease)

Graves' disease is an autoimmune condition in which excessive thyroid hormones are produced by an enlarged thyroid gland. It is the most common cause of hyperthyroidism in children.

Growth Hormone Deficiency

Growth hormone (GH) deficiency results from inadequate production or secretion of GH, causing poor growth and short stature. Hypoglycemia is sometimes the manifestation of GH deficiency.

Growth Hormone Deficiency Pathophysiology

Growth hormone (GH), thyroxine, cortisol, and sex hormones all influence growth. The hypothalamus secretes GH-releasing factor, which stimulates the pituitary gland to release GH. This hormone is secreted in pulses, with increased secretion during the night. In the presence of hypoglycemia, GH is secreted to counteract insulin and raise the blood glucose level. Many children with GH deficiency have hypoglycemia. Most children with short stature have constitutional growth delay. Children with short stature or poor growth rates may also be deficient in other hormones. Normal thyroid function is essential for growth; therefore, hypothyroidism can cause short stature. Sex hormones are required for the growth spurt and sexual maturation that occur with puberty. Children lacking more than one hormone produced by the pituitary gland are referred to as having hypopituitarism. Rate of growth and final adult height depend on factors such as genetics (family heights), nutrition, and general health. Any child growing less than 5 cm/yr should be referred to an endocrinologist for further evaluation.

Indicators of Hypothyroidism or Hyperthyroidism

Hypothyroidism Fatigue Constipation Cold intolerance Weight gain Dry, thick skin Edema of face, eyes, hands (myxedema) Decreased growth, delayed skeletal maturation and puberty Decreased activity and energy Muscle hypertrophy (pseudodystrophy) Decreased heart rate Increased need for sleep Ataxia Hyperthyroidism Emotional lability, anxiety Diarrhea Heat intolerance Weight loss, increased appetite Smooth, velvety skin Prominent eyes Accelerated linear growth Hyperactivity Muscle weakness Increased heart rate High blood pressure Tremor

Acquired Hypothyroidism

Hypothyroidism is a condition in which the thyroid gland produces an inadequate amount of thyroid hormone to meet the body's metabolic needs. Hashimoto thyroiditis is an autoimmune process and a common cause of acquired hypothyroidism, usually associated with a goiter. Other causes of acquired hypothyroidism include surgical thyroidectomy, radioactive iodine therapy for hyperthyroidism, radiation therapy for malignancies, and excessive iodine ingestion. Less frequently, decreased TSH secretion by the pituitary gland or decreased thyrotropin-releasing hormone (TRH) secretion by the hypothalamus causes hypothyroidism Autoimmune thyroiditis is the most common cause of acquired hypothyroidism in children and adolescents It often occurs in families with a history of thyroid disease. Other family members may test positive for thyroid antibodies. Thyroiditis is more common in girls and most often occurs in chronic lymphocytic thyroiditis

Signs of Hyponatremia

Mild (Early) Anorexia Nausea Headache Vomiting Moderate Confusion Lethargy Irritability Altered level of consciousness Severe Seizures Coma

Congenital Hypothyroidism Therapeutic Management

If capillary TSH on the newborn screen is greater than or equal to 40 mU/L, treatment should be initiated as soon as venous blood sampling can be obtained, without delay to wait for results. Treatment of children with congenital hypothyroidism consists of lifelong thyroid hormone replacement, usually in the form of levothyroxine. It is given as a single daily oral dose that varies with the weight and age of the child or adult The dosage is titrated to maintain TSH and T4 in a normal range.

Inborn Errors of Metabolism

In addition to PAH deficiency, there are other genetically transmitted metabolic diseases that rarely occur in newborns Nurses should create a climate in which parents can express their feelings about the lifelong care of their child, as well as concerns for future pregnancies. Families with affected infants are referred to genetic counseling centers. Many of these infants are identified through universal newborn screening or screening specific for at-risk infants. Additional nursing care is related to the particular disorder but is similar to that for the child with PAH deficiency.

Diabetes Insipidus Manifestations

Increased urination (polyuria) and excessive thirst (polydipsia) are the classic manifestations of DI.

Syndrome of Inappropriate Antidiuretic Hormone Therapeutic Management

Initial treatment is correction of the underlying cause. The healthcare provider orders fluid restriction to correct hyponatremia. A child with severe hyponatremia may need an IV infusion of sodium chloride with slow correction of serum sodium (no faster than 0.5 meq/L/h) to avoid CNS damage such as central pontine myelinolysis. Drug therapy usually is not indicated for transient SIADH. Medications such as lithium and demeclocycline block the action of ADH at the renal collecting tubules and have been used in management; however, its use is discouraged in younger patients because of side effects

Nursing Quality Alert Managing the Child With Type 1 Diabetes Mellitus

Insulin • Store insulin in a cool, dry place. Do not freeze or expose to excessive heat. • Do not shake insulin. It is recommended to roll the vial back and forth to mix. • Check the expiration date on the vial before using. • Once opened, date the vial and discard as recommended. • When mixing two different types of insulin in the same syringe, inject the appropriate amount of air into both vials, withdraw the short-acting (clear) insulin first, and then withdraw the intermediate-acting (cloudy) insulin. Nutrition • Meals and snacks are balanced with insulin action. • Both the timing of the meal or snack and the amount of food are important in avoiding hyperglycemia or hypoglycemia. • Adherence to a daily schedule that maintains a consistent food intake combined with consistent insulin injections aids in achieving metabolic control. Exercise • Avoid exercising during insulin peak-times. • Add extra 15- to 30-g carbohydrate snacks for each 45 to 60 minutes of exercise. • Blood glucose should be ≥100 to 120 mg/dL prior to exercise to decrease risk of hypoglycemia Blood Glucose Monitoring • Record blood glucose results in a diary. • A 3- to 4-day alteration in glucose levels requires an adjustment of insulin doses.

Insulin Therapy Schedule.

Insulin requirements are commonly based on age, body weight, and pubertal status. In general, children who are newly diagnosed with type 1 diabetes typically need an initial total daily dose of approximately 0.5 to 1 unit/kg. Because dosages for infants and toddlers frequently are less than 1 unit, the insulin is diluted with an approved diluent to increase the volume to be administered and improve accuracy in dosing. Many children are managed through administration of insulin by three or more injections per day. Administration involves a combination of intermediate or long-acting insulin (basal) and rapid or short-acting insulin (bolus) injected multiple times per day before meals and snacks. More children are now receiving insulin through a continuous subcutaneous insulin infusion (CSII) pump. The insulin administration schedule is individually prescribed according to the child's age-related glycemic targets. The peak actions of these insulins are timed to correspond to the child's usual meal and snack times to minimize the possibility of hypoglycemia. To prevent hypoglycemia, a child must eat within 15 minutes of administration of rapid-acting insulin. A recent option for the basal/bolus insulin regimen is using both rapid-acting analogs and long-acting insulin that has no peak action time. Glargine (Lantus), a long-acting peakless analog, has been approved for children ages 6 years and older and is given in the evening as the basal insulin and complemented during the day by a rapid-acting insulin (lispro or aspart) when the child eats carbohydrates This schedule provides improved glycemic control Lantus insulin is not mixed in the same syringe with other types of insulin or solutions. Rapid-acting insulin analogs are also beneficial in the management of issues related to insulin resistance during puberty.

Growth Hormone Deficiency Incidence

Isolated growth hormone deficiency occurs in 1 in 4,000 to 1 in 10,000 live births, with 3% to 30% of cases being familial No racial differences in incidence are apparent. Boys are much more likely to be diagnosed and treated for GH deficiency than girls; whether this difference is related to referral bias by parents and practitioners is unknown.

Syndrome of Inappropriate Antidiuretic Hormone Manifestations

Manifestations that occur with SIADH include hyponatremia, decreased urine output, increased urine specific gravity, fluid retention with slightly elevated plasma volume, weight gain, and increased urine osmolality

Growth Hormone Deficiency Manifestations

Manifestations typical of GH deficiency include height below the fifth percentile for age and sex, diminished growth rate (2 or more standard deviations below the mean for age and sex), immature or cherubic facies, delayed puberty, hypoglycemia, diminished muscle mass with relatively increased body fat (adiposity), and micropenis (associated with hypopituitarism).

Precocious Puberty Interventions

Many parents are not comfortable with their child's early development. The nurse explains the stages of puberty and each stage's associated behavioral changes. The nurse teaches parents that the child is experiencing normal changes at an earlier time than expected. Explanations given to the child should be geared to the level of intellectual development. The nurse can direct the parent to books that explain sexual maturation in terms the child can understand. Psychological counseling might be necessary to help the family deal with the sensitive issues of sexuality. The nurse also teaches the family about the prescribed medication regimen. In some instances, the parent is taught how to administer the injections. Injections might be stressful for the young child. The nurse demonstrates appropriate injection technique and teaches the child coping strategies to be used when the injection is given.

Diabetes Insipidus Nursing Considerations

Nursing care involves assessing the understanding of the child and parents about DI. The nurse educates the family about the basic pathophysiology of water metabolism and the cause of DI. This education includes a description of signs and symptoms (increased thirst, polyuria, dehydration) and how they indicate the need for DDAVP, as well as signs and symptoms of excessive DDAVP administration (decreased urine output, headaches, water retention). The child must be closely monitored for these signs and symptoms of dehydration, and the parents must know the appropriate actions to take if dehydration occurs. The family is taught how to correctly administer DDAVP and then provides a return demonstration of medication administration. If appropriate, the nurse instructs the family in the use of a refractometer to measure urine specific gravity. The child should wear a medical alert bracelet noting the diagnosis of DI. School personnel need to be aware of the diagnosis and must allow the child free access to water and toilet facilities.

Pathophysiology PAH Deficiency

PAH deficiency refers to a group of biochemical diseases associated with enzymatic blocks in the conversion of the essential amino acid phenylalanine to tyrosine. Classic PKU consists of the absence of the enzyme phenylalanine hydroxylase. This deficiency results in the toxic accumulation of PHE in the bloodstream after the ingestion of protein containing PHE. Phenylalanine can adversely affect the myelinization process in central nervous system (CNS) development. Most of that process takes place during the first decade of life. Intellectual impairment occurs and progresses if treatment is not implemented.

Precocious Puberty Incidence

Precocious puberty is ten times more likely to occur in girls than boys, affecting 0.2% of girls and less than 0.05% of boys Growing evidence indicates that the number of girls diagnosed with precocious puberty has increased over the years and that Black girls mature at an earlier age than do white girls

Precocious Puberty

Precocious puberty refers to the early onset of puberty, traditionally considered as the onset of puberty before 8 years of age in girls (7.5 years in Hispanics and Blacks) and before 9 years of age in boys Precocious puberty is defined as the premature appearance of secondary sexual characteristics, accelerated growth rate, and advanced bone maturation The major consequence of precocious puberty is rapid bone growth, which causes early growth plate fusion and ultimately short stature in adulthood compared with genetic height potential.

Precocious Puberty Pathophysiology

Puberty occurs when the hypothalamus releases gonadotropin-releasing hormone (GnRH). This stimulates the pituitary gland to release luteinizing-hormone (LH) and follicle-stimulating hormone (FSH). In girls, FSH stimulates formation of ovarian follicles to produce estrogen. Estrogen is necessary for the development of secondary sexual characteristics such as breast development and maturation of the vagina and labia. LH is involved in the process of ovulation. In boys, FSH triggers the testes to support the development of sperm. LH stimulates the production of testosterone, which is necessary for the development of sexual characteristics and sperm production. Puberty development is classified according to Tanner stages 1 through 5 The adrenal glands produce the hormone dehydroepiandrosterone (DHEA), which causes pubic and axillary hair growth. During puberty the growth rate increases, called a "growth spurt," in which a child grows an average of 4 to 6 inches/yr. In precocious puberty the sex hormones that accelerate growth also cause the bone plates (epiphyseal plates) to close early. Usually the epiphyseal plates fuse at 14 years of age for girls and 17 years for boys; premature closure can lead to decreased linear growth with reduced adult height. With true precocious puberty, children have hormonal changes that mimic the onset of normal puberty. These hormonal changes may be central, arising from the hypothalamus, or peripheral, arising from the ovaries, testes, or adrenal glands.

Phenylalanine Hydroxylase Deficiency (Formally Phenylketonuria) Diagnostic Evaluation

Routine neonatal screening for PAH deficiency is mandatory in all 50 states of the United States. With early postpartum discharge, screening is often performed on infants younger than 2 days of age because of the concern that the infant will be lost to follow-up. Previously, when screening was performed before the third day of life there was a higher risk of a false-negative outcome. However, screening now uses tandem mass spectrometry that can quantify PHE concentrations as early as 24 hours after birth. A serum PHE level greater than 130 µmol/L indicates a positive result; however, this result alone is not diagnostic and indicates that the infant should be evaluated further

Syndrome of Inappropriate Antidiuretic Hormone Diagnostic Evaluation

SIADH should be suspected in children with CNS involvement, such as infections or head trauma, who have decreased urine output despite adequate intake. Laboratory diagnosis includes evidence of hyponatremia, hypochloremia, and low serum osmolality. Urine osmolality is usually greater than serum osmolality. Urine specific gravity is more than 1.030. Adrenal, thyroid, and renal function studies can rule out other causes of hyponatremia.

Blood Glucose Monitoring

Self-monitoring of blood glucose (SMBG) provides an objective tool to assist with diabetes control. Monitoring is recommended before meals and before the bedtime snack. Monitoring is more frequent during prolonged exercise, during an illness, or if nighttime hypoglycemia is suspected. Blood glucose goals must be tailored to the abilities of the caregivers and the age of the child. For example, goals for the infant or toddler are usually liberalized to help prevent severe hypoglycemia. The identified goals are a target range; not all glucose levels fall in this range, 1265even in the child with excellent diabetes control. Preprandial (before meal) blood glucose goals are as follows (ADA, 2015): • Non-diabetic: 70 to 110 mg/dL • Children with type 1 diabetes mellitus: 90 to 130 mg/dL • Infants and toddlers with type 1 diabetes mellitus: 100 to 180 mg/dL Glucose test results should be recorded in a diary or record book. Patterns or trends in blood glucose levels outside the target range indicate a need to adjust the insulin dose. Three or 4 days of a consistent pattern of elevated glucose values (e.g., 200 mg/dL before the evening meal for 3 consecutive days) indicates a need to increase the dose of the appropriate insulin. The healthcare team may provide the family with guidelines for increasing insulin doses based on blood glucose patterns. A majority of blood glucose meters can store multiple monitoring results so that blood glucose patterns can be evaluated. Blood glucose meters are accurate only if used according to manufacturer recommendations. Regardless of the brand selected, quality control procedures must be performed as recommended. Test supplies must be stored according to manufacturer specifications and discarded when outdated.

Long-Term Healthcare Needs for the Child With Type 1 Diabetes Mellitus

Serious complications are associated with long-term type 1 diabetes, including retinopathy, nephropathy, neuropathy, and cardiovascular disease. Studies have demonstrated that strict metabolic control of diabetes can decrease the severity of complications and/or delay onset A team approach to diabetes management can best provide the tools to achieve metabolic control. The team includes the physician specialist, nurse educator, dietitian, and behavioral specialist. Regular checkups and frequent telephone communication between the diabetes team; the parents; and the child, if age-appropriate, are essential to address the needs of the growing child.

Type 1 Diabetes Mellitus Developmental Issues Adolescent.

The adolescent's developmental milestones are often in conflict with the recommendations for achieving diabetes control. The young adolescent is concerned with body image and peer-group acceptance and is moving away from the family for support and identity. Clothing, diet, lifestyle, and speech are areas in which the early adolescent strives to conform to peers.

Nursing Care The Child With Hyperthyroidism Interventions

The antithyroid drug PTU is usually given two or three times per day, whereas methimazole can be given once daily A multiple-times-a-day medication regimen is difficult for some children and parents to follow. Use of pill dispensers and a watch with an alarm to remind the child to take the medication at specific times enhances compliance. The endocrinologist should evaluate the child and monitor thyroid function every 2 to 4 months while the child is undergoing treatment. Normal values for thyroid function tests and alleviation of symptoms indicate appropriate responses to therapy. Once the child is euthyroid and asymptomatic, she or he should be evaluated once or twice a year. Medication dosages may be tapered after 2 to 3 years to evaluate for remission. Contact sports should be limited while the child is being treated to decrease the possibility of damage to the liver. Collaboration with the school nurse to facilitate medication administration is an important nursing function.

Patient-Centered Teaching Home Management of Type 1 Diabetes Mellitus

The child and family are understandably overwhelmed with questions and fears about the diagnosis. Encourage all family members to participate in the educational process. Choose a comfortable location subject to few interruptions. Provide appropriate literature and materials for family members. DVDs, booklets, and pamphlets should be developmentally appropriate. Educational materials for the parents should also match the parents' literacy skills. The following checklist of outcomes evaluates the family's understanding of the pertinent information: • General information about type 1 diabetes mellitus • How to administer and store insulin • How to monitor blood glucose levels and use the equipment properly • Signs and management of hypoglycemic episodes • Signs and management of hyperglycemia • Strategies for when the child is ill • Nutrition and exercise principles • Potential long-term complications • Available resources for community services and emotional support All family members should be given the opportunity to practice skills taught. Practicing procedures on themselves or each other helps allay fears and allows the child to supervise as a family member performs the procedure. Help develop problem-solving skills by using various scenarios that encourage decision making. Because education is an ongoing process, the family needs a contact person to whom they can turn for advice and support.

DKA Therapeutic Management

The child in DKA usually is admitted to an intensive care unit. Management includes hourly glucose monitoring, hourly vital signs and neurologic checks, strict intake and output measurements, frequent assessment of fluid and electrolyte status, IV fluid replacement, potassium replacement if needed, and administration of continuous IV insulin

Insulin Therapy

The child with type 1 diabetes mellitus loses the ability to make insulin because of autoimmune destruction of the insulin-producing cells, the beta cells. Symptoms of hyperglycemia become evident when most of the beta cells are destroyed. After initiation of insulin therapy, the child may have a "honeymoon" phase characterized by hypoglycemia and a decreasing need for insulin. This phase usually lasts up until 7 months into therapy but the length is highly variable The nurse should prepare the child and family for the possibility of a honeymoon phase, both to avoid the misconception that the diabetes is "going away" and to provide instruction on recognition and treatment of hypoglycemia. The goal of insulin therapy is to replace the insulin the child is no longer able to make in an acceptable physiologic pattern. Synthetic human insulin, made using recombinant deoxyribonucleic acid (DNA) technology, is free of animal impurities and is recommended for children. Oral hypoglycemic agents, although useful in the treatment of type 2 diabetes, are not effective in the treatment of type 1 diabetes. The choice of insulin types and schedule of injections are determined based on the child's needs Daily self-monitoring of blood glucose aids in defining insulin requirements. The child in the honeymoon phase needs less insulin than the child who makes no endogenous insulin. The pubertal child requires larger insulin dosages.

Pediatric Differences in the Endocrine System

The endocrine system is less developed at birth than any other body system. Hormonal control of many body functions is lacking until 12 to 18 months of age. As a result, infants may manifest imbalances in the concentration of fluids, electrolytes, amino acids, glucose, and trace substances.

Congenital Adrenal Hyperplasia Diagnostic Evaluation

The finding of ambiguous genitalia in the newborn infant should raise concern for the possibility of CAH. The diagnosis is confirmed by elevated levels of 17-hydroxyprogesterone, a glucocorticoid precursor. The corticoptropin stimulation test is the gold standard for diagnosis in non-classical or more ambiguous cases. CAH is a part of newborn screening in all 50 states. An appropriate evaluation includes obtaining serum electrolyte, carbon dioxide, and renin levels and performing a physical examination. Serum sodium levels in the infant suspected of CAH will be low, with elevated serum potassium. Serum renin levels will be elevated, indicating mineralocorticoid deficiency. A karyotype to determine genetic sex may be indicated, depending on the degree of genital ambiguity

Safety Alert Congenital Adrenal Hyperplasia

• Children with salt-wasting congenital adrenal hyperplasia (CAH) require glucocorticoid replacement to survive. • In the event of significant stress, such as fever, broken bone, or surgery, children with CAH will require "stress dose" medical therapy. • If the child with CAH begins to vomit, the glucocorticoid must be administered parenterally. • Mineralocorticoid therapy is required in salt-wasting CAH. • Supplemental sodium occasionally may also be required.

Type 1 Diabetes Mellitus Therapeutic Management Nutrition Therapy

The goal of nutrition therapy is to promote normal growth, encourage healthy nutrition, prevent complications, and maintain near-normal blood glucose levels. Because the insulin dosage is balanced with food intake, the diet plan should stress a consistent intake, particularly of carbohydrate food products. The diet therapy chosen should be easy to understand and help the child and family learn to make healthy food choices. The individualized meal plan is based on the child's diet history and is tailored to food preferences, physical activity, cultural aspects, and schedules. As the child grows, the meal plan is tailored to meet changing dietary needs.

Type 1 Diabetes Mellitus Developmental Issues Infant and toddler.

The infant or toddler with type 1 diabetes poses special challenges for diabetes management. The parents must adapt to the diagnosis and master the daily management needs of the child. Severe hypoglycemia occurs most often in this age group, so glucose target levels are liberalized. Achieving consistency in dietary intake can be difficult. Inconsistent intake, particularly of carbohydrates, contributes to blood glucose variability. Food control issues can easily become a battleground between the child and the parent. A diet strategy that stresses carbohydrate consistency rather than specific food groups offers more flexibility. Allowing the toddler to participate in making food choices (from perhaps two or three options) can provide the child a sense of control. The signs and symptoms of hypoglycemia are difficult to recognize in the infant; in a toddler, they can be mistaken for a temper tantrum. Establishing rituals and routines also helps the toddler feel more in control. Parents are encouraged to have a specific place to perform blood tests and to safely store supplies. Toddlers need to know when care activities will occur as well as participate according to their developmental level

Congenital Hypothyroidism Manifestations

The infant with congenital hypothyroidism often displays signs including skin mottling, a large anterior fontanel, a large tongue, hypotonia, slow reflexes, and a distended abdomen Other signs and symptoms include prolonged jaundice, lethargy, constipation, feeding problems, coldness to touch, umbilical hernia, hoarse cry, and excessive sleeping. The infant with congenital hypothyroidism may have none of these signs or symptoms; the newborn screening test is essential to recognize these infants

Growth Hormone Deficiency Interventions

The nurse reassures the child and parents that adherence to the injections will improve growth rate. Reminders that the injections are temporary and are helping the child to grow are also helpful. Keeping a growth chart at home and noting the need for larger clothing sizes are physical signs the child can use to monitor growth. These indicators also can assist with adherence. The nurse has an important role in educating children and families about the proper dilution and administration of the GH if needed. The nurse demonstrates the injection technique to the parents (and child if age-appropriate) and requests a return demonstration. Effectiveness of therapy is evidenced by the child's growth rate. Children are evaluated approximately every 3 to 4 months by an endocrinologist; accurate measurements of height are essential. The use of a growth chart helps identify growth velocity. GH therapy is continued until the child reaches an acceptable adult height or radiographic evidence shows growth plate fusion.

Syndrome of Inappropriate Antidiuretic Hormone Nursing Considerations

The nurse should assess the child with SIADH for signs and symptoms of fluid overload, including edema, weight gain, and urine specific gravity more than 1.030, and for dilutional hyponatremia by checking serum electrolyte levels frequently. The child with hyponatremia is at risk for injury related to seizures. The child's neurologic status is closely monitored every 2 to 4 hours by assessing and recording level of consciousness and observing for headache, irritability, or seizures The provider is alerted immediately if any changes in neurologic status occur. The nurse initiates seizure precautions if the serum sodium level drops below 125 mEq/L. Additional interventions are directed toward maintaining fluid and electrolyte balance. The child's hydration status is carefully assessed. Intake and output are accurately measured and recorded. The child is weighed at least daily to monitor fluid retention. Fluid restrictions are strictly maintained. The child may have difficulty adhering to a decrease in fluid intake. The nurse explains to the child and parents the reasons for limiting fluids and that the restrictions are temporary. The nurse may give the child hard sugarless candies or apply wet washcloths to help keep mucous membranes moist. Diet for the child with hyponatremia should include foods with high sodium content because extra sodium can help correct this problem. However, salty foods, such as chips, may make the child thirsty. Evaluation of the child with SIADH should address a balanced intake and output, stable weight, and normal serum sodium levels. Urine specific gravity should be maintained between 1.010 and 1.020.

Type 1 Diabetes Mellitus Developmental Issues Preschooler.

The preschool years are characterized by increasing motor maturity, a widening social circle, and magical thinking. The preschooler can understand simple explanations regarding diabetes. Such explanations help allay fears that the diabetes was caused by the child being "bad." Play therapy with dolls and diabetes equipment helps the preschooler express concerns regarding injections and finger sticks. The preschooler has a more predictable appetite than the toddler and is frequently willing to try new foods. Nonetheless, supervision is necessary to ensure that meals and snacks are eaten, especially if the child is in a daycare setting with many distractions. The preschooler may be able to identify the feelings associated with hypoglycemia. It is important to use the child's description as a code word for the onset of hypoglycemia symptoms. Preschoolers' preferences for high-energy activities put them at risk for hypoglycemia. The parents and other caregivers should be prepared with readily available carbohydrate foods, as well as emergency medications.

Maple Syrup Urine Disease

This is a very rare (1 in 250,000-300,000 live births) autosomal recessive inherited condition that affects metabolism of certain amino acids. Buildup of acids causes ketoacidosis, which appears 48-72 hr after birth. The infant is lethargic and can display poor feeding, vomiting, weight loss, seizures, and loss of reflexes. The urine smells like maple syrup. Dialysis is needed to reduce accumulated acids. The child must be on a lifelong low-protein, limited amino acid diet. If untreated, the child can die quickly; children who have been treated can have neurologic deficits. Referral to a genetic counseling center is warranted.

Precocious Puberty Therapeutic Management

The primary goal of treatment of the child with precocious puberty is to preserve final adult height followed by stopping or reversing the development of secondary sexual characteristics. Observation is recommended initially since some patients can achieve normal adult height because they have a form of central precocious puberty that progresses slowly. Treatment in girls age 8 years and older is not indicated since they will not benefit Current therapy for central precocious puberty involves administration of a GnRH agonist, or blocker. GnRH blockers inhibit the binding of GnRH to the pituitary gland, causing decreased production of the pubertal hormones and slowing or reversing sexual development. Several commercially available GnRH agonists can be administered by a monthly or 3-monthly intramuscular injection or by subcutaneous implantation every 1 to 2 years. Once therapy is initiated, GnRH secretion is suppressed within 2 to 4 weeks. The accelerated growth rate and bone maturation will slow, and some secondary sexual characteristics will regress within the first year of treatment. Nonadherence with medication therapy, such as missed or delayed administration of injections, can promote pubertal changes rather than suppress puberty. Oxandralone, growth hormone, and aromatase inhibitors have been studied as adjunctive therapies as well with need for further research No evidence suggests that GnRH agonist therapy interferes with the child's reproductive function in the future. Once therapy is discontinued, pubertal progression resumes. For children with peripheral precocious puberty, treatment is aimed at correcting the underlying cause.

Type 1 Diabetes Mellitus Developmental Issues School-age child.

The school-age child and family face the challenge of incorporating diabetes care within a busy school day. To avoid singling out the child, the diabetes care should be as unobtrusive as possible while still maintaining a safe environment for the child. Children with type 1 diabetes mellitus fall under Section 504 of the Individuals with Disabilities Education Act and, as such, are entitled to services within the school setting The child should have a diabetes management plan that specifically describes the frequency of blood glucose testing, insulin doses, nutrition, and any other therapy or modifications associated with the diabetes The family should communicate with school personnel about the child's diabetes. A school nurse or health aide should be identified to supervise before-lunch blood glucose monitoring, assist with insulin injections, and educate other school personnel in recognizing and treating hypoglycemia. Schools vary on the availability of nursing services. Parents may have to work with school personnel to identify appropriate staff to supervise their child's diabetes care. Planning ahead for field trips, school parties, and athletic events allows the child with diabetes to participate safely in age-appropriate activities. For example, the child who has soccer practice three afternoons per week needs to plan how to prevent hypoglycemia during practice.

Syndrome of Inappropriate Antidiuretic Hormone

The syndrome of inappropriate antidiuretic hormone (SIADH) is the inability to excrete free water resulting from excessive production or release of ADH, or vasopressin.

Hyperthyroidism (Graves' Disease) Therapeutic Management

The three approaches to the management of Graves' disease are antithyroid drug therapy, radioactive iodine, or surgery. Antithyroid drug therapy with methimazole is the treatment of choice for childhood hyperthyroidism Propylthiouracil (PTU) has an unacceptable risk for liver toxicity in children and its use is not recommended unless other therapies are not a suitable option or the child is allergic to methimazole. These drugs act by blocking thyroid hormone production by the thyroid gland. The medications usually are given three times per day, and they lower thyroid hormone levels in several weeks. Minor adverse effects include arthralgia, skin rash, pruritus, and gastric intolerance. Major adverse effects include neutropenia, hepatotoxicity, and hypothyroidism A second approach to management is oral radioactive iodine treatment. Radioactive iodine is given as an oral solution. It is typically used in children older than 10 years. With this therapy, the radioactive iodine is absorbed and concentrated by the thyroid gland, destroying the thyroid tissue in approximately 6 to 18 weeks. Hyperthyroid symptoms may intensify briefly after treatment. Hypothyroidism can result once the thyroid gland is irradiated, necessitating thyroid replacement therapy. Subtotal or partial thyroidectomy, the surgical removal of thyroid gland tissue, is the third form of management. Inorganic iodine, given 7 to 10 days before surgery, decreases the gland's vascularity. Surgery carries the risk of bleeding, wound infection, vocal cord paralysis, and injury to the parathyroid glands, resulting in hypoparathyroidism including hypocalcemia. Calcium levels should be monitored after surgery Recurrence of hyperthyroidism is uncommon but possible. Affected children also have a 60% to 80% chance for developing hypothyroidism, which can be treated with thyroid replacement therapy. Follow-up evaluations correlate with response to therapy. As thyroid functions normalize, follow-up endocrine evaluations are recommended once or twice per year.

Congenital Hypothyroidism Pathophysiology

The thyroid gland is a butterfly shaped gland located in front of the neck. Thyroid-stimulating hormone (TSH), secreted by the pituitary, induces the thyroid to produce thyroxine (T4) and triiodothyronine (T3). The thyroid traps iodine and produces T4, which is essential for normal growth and development, especially brain development, in the first 2 years of life. Immediately after delivery, TSH increases dramatically, likely related to the stress of the birth process. Within the first week of life, the TSH level gradually falls. Underdevelopment of the thyroid gland or a hypothalamic or pituitary disorder causes inadequate production of T4, which is essential for brain development. If not treated, this condition can cause intellectual impairment in the developing child. An infant with congenital hypothyroidism has elevated TSH and low T4 levels.

Nursing Care The Child With Hyperthyroidism Assessment

The treatment goals consist of normalizing thyroid hormone levels, alleviating symptoms of hyperthyroidism, and decreasing the goiter. The nurse should assess for adherence to medical therapy and determine if the family understands that medical therapy might take several weeks to decrease thyroid hormone action. The child is closely monitored for adrenergic signs and symptoms. Propranolol, a beta-adrenergic blocker, may be prescribed to decrease adrenergic signs and symptoms (tachycardia, heat intolerance, tremor) until the antithyroid medication takes effect. A child being treated with PTU has an increased risk of neutropenia and hepatotoxicity; regular blood counts and liver function studies are done to assess these risks. The nurse assesses the child for fever, joint pain, edema, rash, and excessive bruising. A child who acquires a fever or sore throat while receiving PTU should be evaluated by a healthcare provider and a complete blood count obtained to evaluate for agranulocytosis.

Phenylalanine Hydroxylase Deficiency (Formally Phenylketonuria) Manifestations

The underlying metabolic alterations begin to have an immediate effect on the infant, although signs may not be apparent until the infant is approximately 3 months old. The first sign may be digestive problems with vomiting. These infants also can have a musty or mousy odor to the urine, infantile eczema, hypertonia, seizures, and hyperactive behavior. Older children can have hypopigmentation of the hair, skin, and irises; they are commonly blond with light blue eyes. Intellectual impairment is a long-term consequence of untreated PAH deficiency

Diabetes Insipidus Therapeutic Management

Treatment for central DI involves maintaining fluid balance and administering synthetic vasopressin (1-deamino-8-D-arginine-vasopressin [DDAVP]). The dose of DDAVP ranges from 5 to 20 mcg/day 1254(intranasal) or 100 to 400 mcg/day (oral), divided into one or two doses per day. The oral form of DDAVP is also used for treatment of nocturnal enuresis in generally smaller doses. The half-life of DDAVP is 3.5 hours, the peak concentration is reached at 50 minutes, and the duration of action is 6 to 18 hours. Decreased urine output is seen 1 to 2 hours after administration. Excessive fluid intake should be avoided while on chronic DDAVP therapy due to the fixed urine osmolality of about 1000 mOsm/kg and risk of developing hyponatremia. Other treatments include thiazide diuretics, chlorpropamide, clofibrate, and carbamazepine; and hypo-osmolar with low sodium diets Dosage is individualized on the basis of the child's age, size, urine output, and urine specific gravity. Doses are timed so that before the next dose, the child is allowed to have mildly increased urination to help prevent overtreatment and water retention. Parents are often taught to measure urine specific gravity at home to monitor the effectiveness of treatment The goals for the management of nephrogenic DI are to prevent severe dehydration and provide adequate calories for growth. With acquired disease, treatment is focused on eliminating the underlying cause; congenital nephrogenic DI is very difficult to treat

Diabetes Mellitus

Type 1 and type 2 diabetes mellitus (DM) are chronic diseases requiring life-long management and care Both types of DM involve abnormal carbohydrate metabolism and hyperglycemia. While the incidence of DM increases with age until mid-puberty, type 1 diabetes mellitus can occur at any age, including infancy. The increasing prevalence of both types of diabetes mellitus worldwide is of concern. A study of 3300 adolescents ages 12 to 19 years revealed that the prevalence of prediabetes/diabetes (fasting blood glucose >99 mg/dL) increased significantly from 9% in 1999 to 23% in 2008

Type 1 Diabetes Mellitus Etiology

Type 1 diabetes mellitus is an inflammatory process in the insulin-secreting islet cells of the pancreas resulting from an autoimmune process that causes their eventual destruction. Although multiple genes are thought to play a role in the genetic predisposition to type 1 diabetes, an environmental trigger is thought to initiate the autoimmune destructive process. Possible triggers include viral and bacterial infections, dietary toxins, history of obesity, and certain chemicals Current research is focused on identifying factors that increase susceptibility and exploring methods of interrupting or preventing the autoimmune response in susceptible individuals (first-degree relatives of a diabetic person). At this time, no prevention or cure is available; however, transplantation of islet cells and the pancreas is being explored. Children with type 1 diabetes mellitus are prone to developing other autoimmune conditions such as Graves disease, Hashimoto thyroiditis, and celiac disease

Type 1 Diabetes Mellitus Manifestations

Type 1 diabetes mellitus is manifested by the classic initial signs of hyperglycemia known as the three Ps: polyuria (or enuresis in a toilet-trained child), polydipsia, and polyphagia. The child's other symptoms include weight loss (despite increased food intake), fatigue, and blurred vision. If the condition progresses without intervention, the child can exhibit signs and symptoms of DKA: nausea and vomiting, abdominal pain, acetone (fruity) odor to breath, dehydration, increasing lethargy, Kussmaul respirations, and coma. Children who receive insulin for treatment of type 1 diabetes mellitus can have hypoglycemia.

Type 1 Diabetes Mellitus

Type 1 diabetes mellitus results when the pancreas is unable to produce and secrete an adequate amount of insulin. This form of diabetes, the most common childhood endocrine disorder, presents challenges in the areas of teaching, management, and adherence. Because of recent changes in the healthcare delivery system, meeting the needs associated with management of type 1 diabetes mellitus has become more complicated. Unless the newly diagnosed child is in diabetic ketoacidosis (DKA), hospitalization is not always necessary. The nurse must develop a plan of care that involves child and family education and support in either an inpatient or outpatient setting.

Phenylalanine Hydroxylase Deficiency (Formally Phenylketonuria)

a genetic metabolic disorder that results in central nervous system (CNS) damage from toxic levels of phenylalanine (PHE) in the blood. PAH deficiency is characterized by a deficiency of phenylalanine hydroxylase, the enzyme needed to convert PHE to tyrosine. PAH deficiency is an autosomal recessive disorder and is manifested only in the homozygote (individual who inherited two identical genes for a specific trait). With both parents carrying the recessive gene, each pregnancy has a 25% chance that the child will have PAH deficiency. PAH deficiency occurs most commonly in whites, with an overall incidence of 1 in 10,000 live births. It is more prevalent in some European countries, including Turkey and Ireland

Congenital Adrenal Hyperplasia

a group of autosomal recessive disorders in which the adrenal gland is not able to manufacture adequate glucocorticoid (cortisol), and while working to make glucocorticoid, produces excess androgens (sex hormones). Mineralocorticoid (aldosterone) production can be either normal or low.

Diabetic Ketoacidosis Manifestations

abdominal and chest pain, nausea and vomiting, fruity breath smell, decreased level of consciousness (LOC), Kussmaul respirations, and symptoms of dehydration.

Type 1 Diabetes Mellitus Therapeutic Management

children newly diagnosed with diabetes mellitus be managed and educated by a multidisciplinary team of experts in pediatric diabetes. Children diagnosed with type 1 diabetes will be started on insulin therapy to reverse metabolic imbalances At the time of their initial type 1 diagnosis, one third of children present with DKA and require management in a pediatric intensive care unit. The goals of diabetes management for children with type 1 diabetes mellitus include the following: • Facilitating appropriate growth (height, weight) • Maintaining an age-appropriate lifestyle • Achieving age-related near-normal HbA1c with minimal episodes of hypoglycemia • Preventing acute complications (hypoglycemia, hyperglycemia, DKA)

Nursing Quality Alert Precocious Puberty

• Children with precocious puberty appear older than their chronologic age. Although they tend to be treated as older children, they should be treated according to their chronologic age. • Other children often tease children with precocious puberty. • Precocious puberty may occur in infancy or childhood.

Nursing Quality Alert Diabetes Insipidus

• Hypernatremia (sodium > 150 mEq/L) and low urine specific gravity in the absence of hyperglycemia are diagnostic of diabetes insipidus. • 1-Deamino-8-D-arginine-vasopressin (DDAVP) is the treatment of choice for central diabetes insipidus. • Overtreatment with DDAVP will result in fluid retention and dilutional hyponatremia. If the hyponatremia is severe enough, seizures may occur.

Nursing Quality Alert Syndrome of Inappropriate Antidiuretic Hormone (SIADH)

• SIADH is characterized by low serum sodium (125 mEq/L or lower) and high urine specific gravity, as well as decreased serum osmolality and increased urine osmolality. • Seizures may develop with hyponatremia. • Treatment depends on strict fluid restriction to maintain serum sodium in a near-normal range. Frequent and precise measurements and recording of intake and output along with daily weights are critical to the evaluation and management of the child with SIADH.

Nursing Quality Alert Criteria for Suspecting Growth Hormone Deficiency*

• Severe short stature: height < −3 standard deviations (SD) below mean • Height < −1.5 below mid-parental height • Height < −2 SD below mean + height velocity < −1 SD below mean over year OR decrease in height SD more than 0.5 SD over year • If not short stature, height velocity < −2 SD below mean over year OR < −1.5 below mean over 2 years • Neonatal hypoglycemia, microphallus, prolonged jaundice, or craniofacial midline abnormalities • Consistently poor growth (less than 5 cm/yr) • Signs of intracranial lesion • Signs of multiple pituitary hormone deficiency

Nursing Quality Alert Autoimmune Thyroid Disorders

• Treatment for Graves disease is either medical (antithyroid medications) or ablative (radioactive iodine or surgery). • Adherence to medical therapy is problematic because of the requirement for twice-daily or three-times-daily dosing for protracted periods (2 to 3 years). • Goiter can be present with either hypothyroidism or hyperthyroidism. • Autoimmune thyroiditis resulting in either hypothyroidism or hyperthyroidism can be permanent or transient.

Nursing Quality Alert The Child With Congenital Hypothyroidism

• Untreated hypothyroidism leads to intellectual impairment. • Thyroxine (T4) and thyroid-stimulating hormone (TSH) levels vary with age, but any infant with a low free T4 OR an elevated TSH > 20 mU/L (even if normal free T4) is considered to have primary hypothyroidism; treatment should be initiated immediately*