Pediatric Endocrine Disorders - Student Summer 2024 (PDF)

Pediatric Endocrine Disorders Dr. Meadows-Oliver  What are Hormones? A complex chemical substances produced and secreted into body fluids by a cell or group of cells that exerts a physiologic controlling effect on other cells ○ Messengers Local or S...

Pediatric Endocrine Disorders Dr. Meadows-Oliver  What are Hormones? A complex chemical substances produced and secreted into body fluids by a cell or group of cells that exerts a physiologic controlling effect on other cells ○ Messengers Local or Systemic hormones Two types of Glands- ○ Exocrine secretions reach epithelial cells may pass thru a duct ○ Endocrine secrete into the blood UN.1255: Pediatric Nursing Major Organs and Glands of the Endocrine System UN.1255: Pediatric Nursing  Neuroendocrine Interrelationships Homeostasis is maintained by two regulatory systems ○ Endocrine system Pace of growth & development controlled, including brain, reproductive system and body Maintain optimal levels for body functioning ○ Autonomic nervous system Sympathetic system – fight or flight Parasympathetic system- rest & digestion UN.1255: Pediatric Nursing Review: Hypothalamic- Pituitary Axis system Hypothalamus - secretes trophic releasing hormones Then triggers Anterior pituitary gland (“master gland”) Releases Stimulating hormones Gland releases hormone Controlled via Negative Feedback Loop Once brain receives feedback that sufficient hormone has been released, stops secreting releasing hormone UN.1255: Pediatric Nursing Pituitary Hormones and their Target organs UN.1255: Pediatric Nursing Endocrine Glands and Their Functions UN.1255: Pediatric Nursing Endocrine Glands and Their Functions UN.1255: Pediatric Nursing Important Childhood Hormones For Proper Growth and Maturation ○ Growth Hormone ○ Thyroid ○ Adrenal ○ Gonadal For Proper Metabolic Function- Na, Ca, Glucose, ○ Antidiuretic hormone (ADH) ○ Parathyroid ○ Insulin UN.1255: Pediatric Nursing  Pediatric Differences Endocrine System Responsible for: ○ Sexual differentiation in fetal development 7-8 weeks gestation, male embryo begins to secrete testosterone which causes the gonads to differentiate into testes Female embryo secretes estrogen causing the gonads to differentiate into ovaries UN.1255: Pediatric Nursing  Pediatric Differences Stimulating growth and development in childhood and adolescence Skeletal maturity can be detected by bone age (stage of bone ossification). UN.1255: Pediatric Nursing  Pediatric Differences Production of sex hormones (estrogen progesterone, testosterone) is low during early childhood Adrenarche—onset of adrenal androgen production—leads to the development of acne, body hair, and adult body odor Puberty--sexual maturation occurs when gonads secrete increased amounts of sex hormones (estrogens and androgens) ○ Girls 11 to 13 ○ Boys 13 to 15 UN.1255: Pediatric Nursing  Endocrine Disorder Signs Changes in ○ Growth rate ○ Vital signs- BP, pulse ○ Facial characteristics (moon face, protuberant eyes, hirsutism) ○ Muscle tone ○ Sexual development (Tanner stage?) ○ Metabolic regulation (fluid & electrolyte balance) UN.1255: Pediatric Nursing Disorders Related to the Reproductive System Primary amenorrhea ○ No secondary sex characteristics and no menarche by 14-15 years ○ Secondary sex characteristics present, no menarche by age 16-16.5 years Secondary amenorrhea ○ Absence of menses for 6 months in first two years ○ Pregnancy ○ Disordered eating - Athletes UN.1255: Pediatric Nursing Hypopituitarism or Growth Hormone (GH) Deficiency Inhibits somatic growth Primary site of dysfunction appears to be in the hypothalamus (releases somatostatin) Decreased release of Growth Hormone from anterior pituitary Results in slower linear growth UN.1255: Pediatric Nursing  Causes of Short Stature Growth Hormone Deficiency Familial Short Stature Hypothyroidism Constitutional Growth Delay Chronic Renal Failure Cushing’s Syndrome Inborn Metabolism Errors Genetic disorders (Turner’s syndrome) Cardiac, GI, Respiratory Disease (CF) UN.1255: Pediatric Nursing Therapeutic Management of GH Deficiency— Treatment Biosynthetic growth hormone (injections) Other hormone replacements as needed Thyroid extract Cortisone Testosterone or estrogens and progesterone UN.1255: Pediatric Nursing  Prognosis GH replacement successful in 80% of affected children Growth rate of 3.5 to 4 cm/yr (~2 inches) before treatment and increase to 8 to 9 cm/yr (~ 4 inches) after treatment Response varies based on age, length of treatment, frequency of doses, dosage, weight, and GH receptor amount Continued only until child reaches acceptable height or velocity slows (less than 2cm per year), then stopped UN.1255: Pediatric Nursing Nursing Considerations for GH Replacement Family members need support Child’s body image – Bullying? Encourage regular physical activity May help to release growth hormone Preparing child for daily SQ injections Injections given at bedtime for best results Can use topical anesthetic prior to subcutaneous injection Treatment very expensive ($20,000 to $30,000 per year) UN.1255: Pediatric Nursing  Growth Hormone Excess (Hyperpituitarism) Excessive secretion of growth hormone increases growth rate Grow to 7-8 feet before closure of epiphyseal plates If disorder occurs after closure of growth plates, acromegaly occurs Diagnosis of male children often delayed because of tall stature is valued in our society Increased insulin-like growth factor (IGF-1) noted Treatment—high dose sex hormones to close epiphyseal plates; radiation, surgery UN.1255: Pediatric Nursing Thyroid Function UN.1255: Pediatric Nursing  Hypothyroidism Thyroid hormone production is decreased Labs will show elevated TSH levels (compensatory) Congenital—tested for on newborn screening test Acquired ○ Rarely occurs from dietary insufficiency of iodine in United States Autoimmune disease UN.1255: Pediatric Nursing  Congenital Hypothyroidism Jaundice Thick tongue Hypotonia Umbilical hernia Hoarse cry Dry skin Large fontanelles UN.1255: Pediatric Nursing Clinical Manifestations of Hypothyroidism Decelerated growth Constipation Sleepiness Skin changes ○Cool, dry skin ○ Sparse hair Periorbital edema If untreated- develop cretinoid features (thick, protuberant tongue, and intellectual impairment) UN.1255: Pediatric Nursing  Therapeutic Management Thyroid hormone important for growth and development and metabolizing nutrients and hormones. Oral thyroid hormone replacement for life Prompt treatment needed for brain growth in infant May administer in increasing amounts over 4 to 8 weeks to reach euthyroidism Compliance with medication regimen is crucial to prevent cognitive impairment! UN.1255: Pediatric Nursing Administration of Medication Safety Specific dosage per kilogram/body weight Determination of drug dosage Narrow therapeutic range Ability to metabolize medication For infants & toddlers ○ Mixing oral medication with sweet foods – applesauce, pudding ○ Do NOT add to bottle or use household spoon For infants, squirt small amount in cheek while sitting upright ○ Give while calm ○ Crush pills or offer in liquid form UN.1255: Pediatric Nursing  Nursing Considerations Identify children with hypothyroidism EARLY Alert for signs and symptoms of need for dosage readjustments Follow labs UN.1255: Pediatric Nursing  Congenital Hyperthyroidism Occurs in infants of mothers with Graves Disease Transplacental transfer of immunoglobulins Usually resolves by 12 weeks of age UN.1255: Pediatric Nursing  Hyperthyroidism Thyroid levels are increased Graves disease--Immunoglobulins (produced by b-lymphocytes) stimulate over-secretion of thyroid hormones Most often in females High familial incidence UN.1255: Pediatric Nursing  Clinical Manifestations Enlarged, tender thyroid (goiter) Prominent or bulging eyes (exophthalmos) Eyelid lag Tachycardia Increased appetite with weight loss Moodiness Heat intolerance Irregular menses Insomnia UN.1255: Pediatric Nursing  School Difficulties Difficulty concentrating Behavioral problems Declining school performance Easily frustrated Overheated and fatigued during physical education class Tired because of insomnia UN.1255: Pediatric Nursing  Diagnosis and Treatment Serum TSH (decreased), T3 and T4 levels (elevated) Thyroid scan Medication Therapy Methimazole (Tapazole) Propylthiouracil (PTU)—not recommended for children because of concern of liver disease. Radiation Surgery (thyroidectomy)—which results in hypothyroidism UN.1255: Pediatric Nursing Congenital Adrenal Hyperplasia (CAH) Autosomal recessive May see at birth or “late- onset” Caused by decreased enzyme activity required for cortisol production in the adrenal cortex Results in low cortisol levels Elevated ACTH (Adrenocorticotropic hormone) levels leading to increased release of androgens Results in over expression of testosterone UN.1255: Pediatric Nursing  Clinical manifestations of CAH Low cortisol levels (adrenal insufficiency) Weakness, fatigue, poor growth, frequent infections Nausea & vomiting, salt craving Can lead to hypotension, hypoglycemia, dehydration, hyponatremia Overexpression of adrenal androgens and virilization UN.1255: Pediatric Nursing  Clinical manifestations of CAH Males—precocious genital development Females—may be born with varying degrees of ambiguous genitalia ○ Enlarged clitoris appears as a small phallus ○ Fused labia produce saclike structure without testes ○ Internal female sexual organs are intact Female child- hirsutism, delayed menses Both- acne, tall stature, precocious puberty UN.1255: Pediatric Nursing  Clinical manifestations of CAH Ultrasonography to visualize pelvic organs Chromosome typing for positive sex determination and to rule out any other genetic anomalies Labs- ○ Cortisol levels ○ ACTH levels ○ Testosterone levels UN.1255: Pediatric Nursing  Therapeutic Management of CAH Confirm diagnosis and assign a sex to the infant according to genotype Lifelong Cortisone Replacement(glucocorticoid) ○ To suppress abnormally high secretion of ACTH that is triggering excessive androgen release ○ Oral dosing when well, injections prn ○ Given in am, with meals ○ Need more if ill or stressed Replace Aldosterone if salt wasting Reconstructive surgery as required once clear UN.1255: Pediatric Nursing  Precocious Puberty Defined as sexual development before age 9 in boys or before age 7 - 8 in girls ○ Thelarche- breasts ○ Adrenarche- pubic or axillary hair Occurs more frequently in girls Potential causes ○ Disorder of the gonads, adrenal glands, or hypothalamic-pituitary gonadal axis ○ No causative factor in 80% to 90% of girls and 50% boys UN.1255: Pediatric Nursing Therapeutic Management of Precocious Puberty Treatment of specific cause if known ○ Overproduction of hormones ○ Tumor (brain, ovarian) ○ Creams/chemicals in the environment May be treated with a gonadotropin-releasing hormone agonists (GRHA) such as Lupron or Supprelin LA ○ Supprelin LA—Implantable, lasts up to 12 months ○ Lupron: IM injections monthly Slows prepubertal growth to normal rates to PREVENT premature closure of epiphyseal plates and SHORT stature Treatment is discontinued at “normal" age for pubertal changes to resume UN.1255: Pediatric Nursing  Therapeutic Management Psychological support for child and family ○ May see mood swings and body image issues ○ Role play response to bullying ○ Age appropriate dress ○ Age appropriate expectations UN.1255: Pediatric Nursing Diabetes UN.1255: Pediatric Nursing Pathophysiology of Diabetes UN.1255: Pediatric Nursing  Type I Diabetes Caused by autoimmune destruction of pancreatic islet cells Results in beta cell failure and insulin deficiency (symptomatic when 80% lost) Only small percentage have family hx in close relative Rarely see acanthosis nigricans See DKA in 35-40 % Same rate of obesity as the general population UN.1255: Pediatric Nursing  Type 2 Diabetes Physiological ○ Caused by insulin resistance in tissues ○ Lack of insulin to maintain glycemic control ○ Highly associated with obesity ○ Reversal possible with wt loss More prevalent in ethnic minority populations UN.1255: Pediatric Nursing  More Differences Type 2 More common in females (rates 60% higher) ○ Irregular menses ○ Hirsutism in girls (PCOS) Acanthosis nigricans is common Family Hx seen in 90% Lower socioeconomic status May see DKA in a small percentage UN.1255: Pediatric Nursing  Diagnostic Criteria Same for all types Fasting glucose ≥ 126 mg/dL or Random glucose ≥ 200 mg/dL or 2hr glucose during an OGTT ≥ 200 mg/dL OR HA1C ≥ 6.5 *More commonly used now UN.1255: Pediatric Nursing  Diagnostic Tests Needed *HA1c (captures BS values over previous 3 months) Blood Glucose Urinalysis ○ Glycosuria (when BS>180) ○ Ketonuria, if metabolizing fats/protein BMI vs BMI percentile ○ In adults, overweight if BMI >25, Obese if BMI >30 th ○ In kids, at risk if > 85 %ile, overweight if >95th%ile UN.1255: Pediatric Nursing  BMI UN.1255: Pediatric Nursing  Current Diagnostic Criteria: Prediabetes ○ Fasting ≥ 100 = Impaired Fasting Glucose (IFG) ○ 2 hour PP>140 = Impaired Glucose Tolerance (IGT) ○ HA1c 5.7- 6.4 % Recommendations- 1. Encourage lifestyle modification 2. Consider use of metformin to prevent progression to Type 2 DM UN.1255: Pediatric Nursing Common Clinical Manifestations What are the most common clinical manifestations of diabetes? ○ Hyperglycemia ○ 3 P’s - Polyuria, polydipsia, polyphagia ○ Bedwetting/nocturia ○ Weight loss – not good! ○ Fatigue ○ Blurry vision ○ Candidiasis infection (thrush or vaginal) UN.1255: Pediatric Nursing Therapeutic Management of Type 1 DM Insulin therapy—types of insulin, duration, onset and peak action, mixing and administration, rotation of injection sites Insulin pump therapy Glucose monitoring—goal range ○ 80 to 120 mg/dl Lab measurement of hemoglobin A1C Urine testing for ketones ○ Not routinely used except to test q 3 h during illness and whenever glucose is ≥240 mg/dl when illness not present UN.1255: Pediatric Nursing Therapeutic Management of Type 1 DM—cont’d Meal planning Exercise Teach patient and family how to manage hypoglycemic episodes ○ Simple vs complex carbohydrates Illness management ○ Take insulin as prescribed Prevention of DKA UN.1255: Pediatric Nursing  Patient Education—cont’d Recognition and treatment of hypoglycemia ○ Give rapid source of glucose (15 gms) followed by a complex carb and protein, such as milk or peanut butter ○ Glucagon IM if unconscious or unable to swallow Management of “minor” illnesses Record keeping Hygiene & foot care Eye care Family support UN.1255: Pediatric Nursing Hypoglycemia happen quickly Physical Shakiness/Weakness/ fatigue Sweating, chills and clamminess Hunger and nausea Rapid/fast heartbeat Lightheadedness or dizziness Blurred/impaired vision Tingling or numbness in the lips or tongue Headaches Lack of coordination Seizures UN.1255: Pediatric Nursing Hypoglycemia can happen quickly LOC change Irritability or impatience Nervousness or anxiety Confusion, including delirium Anger, stubbornness, or sadness Sleepiness Nightmares or crying out during sleep Unconsciousness UN.1255: Pediatric Nursing  Ketones When glucose is unavailable for cellular metabolism, the body breaks down alternate sources of energy Ketones released and excess ketones eliminated in urine (ketonuria) or by the lungs (acetone breath) Ketones in blood are strong acids that lower serum pH and produce ketoacidosis UN.1255: Pediatric Nursing  Diabetic Ketoacidosis (DKA) Pediatric emergency Results in progressive deterioration with dehydration, electrolyte imbalance, acidosis, coma, and may cause death UN.1255: Pediatric Nursing Clinical presentation of type two diabetes Nausea & vomiting (ketosis causes ileus) Abdominal pain Dry mouth Deep Kussmaul respirations Fruity breath Shortness of breath Lethargy, may progress to coma d/t cerebral edema Dehydration Possible in both types!- must treat as Type 1 with insulin until ruled out UN.1255: Pediatric Nursing  Kussmaul Respirations Hyperventilation characteristic of metabolic acidosis resulting from the respiratory system’s attempt to eliminate excess CO by increased depth and rate 2 UN.1255: Pediatric Nursing  Medical Management of Type 2 Three FDA approved medications for children: Metformin (oral; pill--twice daily with meals) ○ Increases sensitivity of insulin receptors in liver, muscles & fat ○ Decreases glucose production by liver ○ SE: abd pain, nausea, diarrhea ○ Low risk of hypoglycemia episodes Liraglutide (daily injection) ○ Helps release insulin from pancreas after meals. Insulin if HA1c > 8 or 9 - necessary to restore Beta cell function; used to assist glucose entry into cells. ○ Used with metformin to decrease insulin resistance Blood glucose monitoring at home Lifestyle modifications (nutrition and activity) UN.1255: Pediatric Nursing  Nursing Management of Type 2 Educate & support in self care Encourage dietary changes: ○ Eliminate sugary drinks ○ Portion control ○ Encourage weight loss/maintenance- stabilize curve velocity if still growing ○ Encourage to eat breakfast ○ Encourage reduced fat and high fiber foods UN.1255: Pediatric Nursing  Nursing Management of Type 2 Support & encourage lifestyle changes: ○ Limit screen time (tv, ipad, video games) to 2 hrs ○ Encourage 60 minutes of exercise/day ○ Encourage regular family meals and healthier choices in all family members Advocate for changes in schools and communities that encourage healthy options UN.1255: Pediatric Nursing  Road Blocks Encountered —Teenagers ○ Trying to be independent & fit in ○ Susceptible to peer influence ○ Enjoy fast food, sedentary activities (e.g. video games) High risk population- minorities, low income Family history of non-adherence with healthy lifestyle recommendations Mental health issues UN.1255: Pediatric Nursing Insulin Injection Sites UN.1255: Pediatric Nursing  Nursing Implications: Obesity Prevention Screen for increasing weight gain at all primary care visits, follow growth curve and intervene early to reduce risk if velocity increases Plot BMI and follow %ile. A child with obese parents have a greater risk of becoming obese UN.1255: Pediatric Nursing  Nursing Implications: Use nonjudgmental language ○ Glucose “in target/out of target” range NOT “good/bad” Focus on BMI and waist circumference vs only weight (less emotionally charged) ○ BMI is an imperfect measure because it does not directly assess body fat (AMA). ○ Should be used in conjunction with other measures Important to establish a rapport Use Motivational Interviewing to set patient centered goals and find solutions that work for each individual UN.1255: Pediatric Nursing American Medical Association and BMI https://www.ama-assn.org/delivering-care/public-health/ama-use-bmi-alone-imperfect-clinical-measure The AMA policy also states that: BMI is significantly correlated with the amount of fat mass in the general population but loses predictability when applied on the individual level. The policy says, there are significant limitations associated with the widespread use of BMI in clinical settings and suggests its use be in a conjunction with other valid measures of risk such as, but not limited to, measurements of: Visceral fat. Body adiposity index. Body composition. Relative fat mass. Waist circumference. Genetic or metabolic factors. Body Roundness Index (BRI) May be a more precise way to estimate obesity BMI estimates a person’s body mass index using just two measurements, height and weight BRI incorporates hip and waist circumferences to estimate how much total fat and visceral fat someone has ○ Waist to hip ratio [WHR] divide waist/hip circumference ○ WHR males: obesity defined as greater than 0.9 ○ WHR females: obesity defined as greater than 0.85 BRI is not able to calculate a person’s muscle mass UN.1255: Pediatric Nursing Questions?