Endocrine Disorders in Children
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| Absence of the hepatic enzyme phenylalanine hydroxylase needed to metabolize the essential amino acid phenylalanine to tyrosine. | Phenylketonuria (PKU) not an endocrine disease
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| ____ is needed to form melanin, epinephrine and thyoxine | throsine - missing in pt with PKU
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| If phenylanine is not metabolized, as seen in pt with PKU, it accumulates in the blood stream and causes damage to the developing ____ Mental retardation will occur if not corrected. | brains and CNS
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| PKU primarily affects ____ children | caucasian
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| Diagnostic test for PKU includes ___ blood test | Guthrie blood test
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| The Guthrie blood test Determines phenylalanine levels in blood (greater than ____ mg/dl) Normal new born levels are 0.5-1 mg/dl. | 4
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| clinical manifestations of PKU | FTT, vomiting, irritable, developmental delay, unpredictable erratic behaviros, MR, mousy or musty odor to urine in older infant
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| T/F restriciting dietary protein can maintain phenylalanine levels within the safe range 2-8mg/dl | True
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| How much phenylalanine per kg per day is allowed to keep blood levels within normal range? | 20-30mg of phenylalanine
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| T/F an infant with PKU should not be breast fed | False - as long as the mother is careful about monitoring her diet
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| Foods with the lowest amount of phenylalanine include | low-protein breads and pasta, veggies, fruits and juices
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| Foods with the highest amount of phenylalanine include | chicken, steak, pork chops, hamburger, milk, eggs, cheese, refried beans, nuts and peanutbutter
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| Since aspartame is converted to phenylanine in the GI tract, pt with PKU should avoid ____ | NutraSweet, Equal and some meds with artificial sweetner
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| T/F it is recommended for PKU pt to stay on low phenylalanine diet for life | True - even after brain is fully developed
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| ____ is the inability to synthesize adequate amounts of Thyroid hormone | congenital hypothyroidism
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| Most congenital hypothyroidism is due to ____ | thyroid digenesis (defective thyroid gland development)
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| Congenital hypothyroidism is due to ____ recessive trait | autosomal
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| clinical manifestations of congenital hypothyroidism right after birth | poor feding, lethargy, prolonged neonatal jaundice, resp difficulty and bradycardias, constipation, hoarse cry, and large fontanels
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| manifestations of congenital hypothyroidism after 6 weeks of age: | depressed nasal bridge, short forehead, puffy eyelids, large tongue, course dry lusterless hair, large fontanels and wide cranial sutures, umbilical hernia, abdominal distension and hypothermia
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| Diagnosis of congenital hypothyroidism includes a blood test to measure ___ levels | T4 blood levels - usually taken within first 24-48 hrs
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| T/F diagnosis of congenital hypothyroidism and PKU are mandatory in all 50 states | True
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| Reason for early diagnosis for congenital hypothyroidism | prevent progressive deterioration of the nervous system and MR
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| Tx of congenital hypothyroidism is life long replacement of ___ | thyroid hormone - Synthetic Levothyroxine Sodium (Synthroid or Levothyroid)
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| Prognosis of congenital hypothyroidism if hormone replacement is started shortly after birth | normal physical growth and intelligence
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| Signs of overdosage of Synthroid or Levothyroid to treat congenital hypothyroidism include | irritability or nervous, tremor or insomnia, loose stools, wt loss, increase appetite, fever, sweating, tachycardia and palpitations
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| Signs of underdosage of Synthroid or Levothyroid to treat congenital hypothyroidism include | fatigue, sleepiness, decreased appetite and constipation
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| Cause of growth hormone deficiency include disfunction of the ____ gland | pituitary
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| ____ is the total lack of all hormones | panhypopituitarism - result of trauma, hypoplasia, tumor, irradiation and all hormones of the pituitary need to be replaced
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| What is the chief complaint with idiopathaic hypopituitarism is ____ caused by decreased secretion of growth hormone | short stature
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| Diagnosis of Growth Hormone Deficiency - Any child who is only as tall as children ____ or more years younger or who falls away from previously normal growth curve should be evaluated by a doctor preferable a pediatric endocrinologist. | two
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| The diagnostic evaluation of growth hormone deficiency is aimed at identifying any ____ cause for the problem. | organic - like family history, child's nutrition and past illnesses, and growth patterns
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| A diagnostic evaluation for short stature includes ____ surveys including epiphyseal maturation and bone age | radiographic
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| What percentage of growth hormone is produced during sleep | 66%
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| Growth hormone deficiency is established when several tests show that ____ | no growth hormone is present or that the amount of growth hormone is not enough
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| Tx of growth hormone deficiency is effective ____% of children | 80%
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| ____ closure is definite criterion to stop therapy | epiphyseal
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| How much of a synthetic growth hromone is used as daily injections for growth hromone deficiency | 25-50mcg/kg
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| How much is a child expected to grow after receiving the synthetic growth hormone | 4-4.8" 1st yr then 2.7-3.5" following year
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| ____ puberty is sexual development has been before the age of 9 in boys and 8 in girls | precocious puberty - being reevaluated for girls to age 7 caucasian and 6 for african american
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| Goal of precocious puberty is to ____ | hault the progress of sexual development
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| Drug used to block the body's production of the sex hormone to treat precocious puberty | LHRH and synthetic hormones given once every 4 wks IM
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| LHRH and synthetic hormones to treat precocious puberty is discontinued when | at a chronological appropriate time allowing pubertal changes to resume
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| T/F a child with precocious puberty should dress appropriate for their development | False - should dress appropriate for age
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| ____ interest is usually not advanced beyond the child's chronological age for children with precocious puberty | sexual, even though the child is fertile
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| A child with precocious puberty is fertile but should not take____ | contraception - unless sexually active
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| Congenital Adrenal Hyperplasia causes increases of what three hormones from the adrenal gland | Mineralocorticoids (produce aldosterone) Androgens (produce testosterone) and gluccocorticoids (produce cortisol)
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| Adrenals normally synthesize ____ from cholesterol to secrete the hormones | steroids
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| With congenital adrenal hyperplasia there is a lack of steriods (cortisol) and the pituitary senses this and attempts to force the adrenals to produce cortisol - but there is no cortisol being made by the fetal adrenal gland so what happens | The only thing being produced by the fetal adrenal gland is androgens so an excess is produced resulting with femal ambiguous genitalia
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| ____ is when the clitoris is hypertrophied and resembles a penis with hypospadius and the labia is fused resembles a scrotum | ambiguous genitalia from congenital adrenal hyperplasia
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| Classic salt wasting of congenital adrenal hyperplasia manifestations are: | females present with ambiguous genitalia at birth, both male and female present with a salt-losing adrenal crisis in the first weeks - shock, dehydration, and elevated K+ levels
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| Non-classic salt wasting of congenital adrenal hyperplasia signs include | females present with varying degree of genital virilization, both males and females do not manifest the salt-losing probs as they have normal aldosterone, they lack cortisol.
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| nonclassical congenital adrenal hyperplasia signs | girls do not have ambiguous genitalia, less severly affected infants with variable manifestations
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| ____ can trigger adrenal insufficiency episode | stress
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| lab results for congenital adrenal hyperplasia include a 24 hr urine for presence of ____ levels | 17-ketosteroid
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| Tx for congenital adrenal hyperplasia | hydrocortisone (cortisol) replacement in the form of hydrocortisone tablets
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| Hydrocortisone (cortisol) treats congenital adrenal hyperplasia by | supression of ACTH by the pituitary, androgen production is decreased, and virilization is halted
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| lifelong replacement of hydrocortisone (cortisol) is neeed with increased doses during periods of ___, ____, ____, and ____ | stress, surgery, infection, and illness
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| Medications for Salt-Losers with congenital adrenal hyperplasia | aldosterone replacment, Florinef Acetate, cortisone (Solu-cortef) when under stress, increased Na+ intake
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| If no replacement medications are given for kids with congenital adrenal hyperplasia ____ of female will continue | masculization
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| If treatment is not given for kids with congenital adrenal hyperplasia both sexes will have premature epiphyseal closure resulting in ____ adults | dwarfed adults
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| males without tx for congenita adrenal hyperplasia will be ____ as an adult | sterile
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| surgical tx in girls for congenital adrenal hyperplasia should be done before ____ months of age | 17 months (old enough to experience surgery but before aware of abnormal genitalia)
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| Most common endocrine disease in children peaks in incidence between 10-15 years but can occure anytime usually b4 age 20 | Type 1 diabetes
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| Type 1 diabetes is more prominent in ____ and an absolute ____ deficience | caucasians with absolute insulin deficiency
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| factors behind type 1 diabetes is ____ predisposition and ____ factors such as viral illness, toxins, & cows milk which trigger an autoimmune response | genetic predisposition and environmental factors
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| Incidence of type 2 diabetes has increased in recent years due to | environmental factors of obesity, inactivity and diets high in fat and refined carbs
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| type 2 diabetes is more prevelent in ____ ancestery | Non-european
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| Manifestations of type 1 diabetes | 3P's (polyuria, polydipsia, polyphasia) weight loss, ketonuria, hyperglycemia, fatigue, and irritability
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| clinical manifestations of type 2 diabetes | insidious onset, overweight, may have 3 p's weakness, fatigue, may have frequent infections due to year (vaginal and UTI)
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| 90% of children with type 2 diabetes have ____ nigricans which is a velvety hyperpigmented area in skin folds | Acanthosis Nigricans
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| ___ is the only oral diabetic agent approved for youths | Metformin
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| Tx plan for children with type 2 diabetes should be addressed at | life style and risk factors of the entire family
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| Diagnosis of diabetes in children fasting blood sugar on two occasions | 126mg/dl - the perfered method of diagnosis
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| ____ - administration of glucose to determine how quickly it is cleared from the blood is one of the diagnosis used for type 1 diabetes | Oral glucose tolorance test OGTT
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| Goals for management of diabetes in children | achieve glycemic control, promote normal growth and development, and prevent or delay complications
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| Infant desired blood glucose levels | 100-200
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| Toddler and Preschool blood glucose levels b4 meals and bedtime and A1c | B4 meals = 100-180, Bedtime 110-200, A1c 7.5% - 8.5%
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| School age desired blood glucose (age 6-12) B4 meals, Bed time and A1c | B4 meals 90-180, Bedtime 100-180, A1c less than 8%
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| Adolescent and young adults desired blood glucose B4 meals, Bedtime and A1c | B4 meals 90-130, Bedtime 90-150, A1c less than 7.5%
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| The younger a pt is with diabetes the higher the rates of desired blood glucose levels due to thier ____ | vulnerability to hypoglycemia
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| Shortly after insulin therapy, the pancrease kicks in one last time and releases exogenous insulin for a period of time is called | Honeymoon Period
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| ___ destruction will continue during the honeymoon period and continue to give low doses of insulin | Beta cells destruction
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| Rapid acting insulin - onset peak and duration | Lispro (Humalog) (Novalog) - onset 5-15 min, peak 30-90 minutes, duration 4-5 hours
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| Short acting insulin - onset, peak and duration | Regular insulin (Humulin R) (Novolin R) Onset30-90 minutes, Peak 2-4 hours, Duration 5-7 hours
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| Intermediate-acting insulin - onset, peak, duration | NPH (Humulin N), onset 1-2 hours, peak6-14 hours and duration 16-20 hours.
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| Long-acting insulin - onset, peak and duration | Insulin glargine (Lantus) onset 90 minutes, Peakless, duration 24 hours
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| Which insulin do you not mix with ohter insulins | Long-acting Insulin glargine (Lantus)
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| Insulin is given ____ injection at least ___ tims a day | subQ - BID minimum
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| Tight control of diabetes involves ____ dose injections. | multiple to cover food intake and once at night (Lantis) for bedtime basal control of 24 hours
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| T/F the insulin pump improves the A1c levels | True
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| Advantages of the insulin pump | allows for more physiologic insulin delivery, flexible with meal and snack time, decreased amount of injections
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| The insulin pump allows for ____ baseline infusion with intermittent boluses as determined by user | continuous
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| Boluses given via insulin pump are determined by ____, ____, and ____ | blood sugar, activity and diet
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| How many times must blood sugar be monitored for a pt with an insulin pump | At least 5-6X a day
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| An insulin pump delivers insulin by a small needle injected in ____ tissue of the abdomen | subcutaneous
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| How often are needles changed in an insulin pump | every 48 hours
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| How long can an insulin pump be removed | One hour per day
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| ____ is the main food that raises blood sugar no matter its source and must be counted to determine how much insulin is needed | Carbohydrate
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| ____ helps establish total grams of carbohydrates to eat at meal's and snacks | dietitian
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| One serving of starch, fruit, or milk is equal to ____ grams of carbohydrates | 12-15
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| T/F vegetables are counted as carbs | False - they are not counted
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| How much fat and protein are recommended for children with type 1 diabetes | reasonable amounts
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| concentrated sweets for type 1 diabetics are discouraged by not ____ | prohibited
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| How many grams of carbohydrates equals one carb | 15
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| Cells utilize blood glucose more effectively when the child engages in ____ | exercise/Physical Activity
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| Exercise ____ blood sugar and helps ____ to enter the cell more readily | lowers blood sugar and helps insulin ener cell
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| Take carbohydrate source before exercise to prevent ____ | hypoglycemia
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| Do not exercise if blood glucose is greater than ____ | 240, splitting ketones
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| How long after activity can glucose drop | 6-12 hours so check glucose at bedtime and or during the night
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| Alcohol increases or decreases blood glucose | decreases = hypoglycemia
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| Mild symptoms of hypoglycemia | sweating, pallor, nervous, palpitations, tremulousness, hunger, cold, clammy
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| Moderate reactions of hypoglycemia | weakness, dizziness, A, drowsiness, irritable, loss of coordination, and disorientation
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| severe reaction of hypoglycemia | seizures, semi comatose or coma, slurred speech, staggering gait, combative, confused
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| Mild to Moderate tx of hypoglycemia | 10-15 grams of simple carbohydrates followed by complex carbs and retest blood sugar in 15-30 minutes
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| Severe tx of hypoglycemia | Adminster glucagon, 911 to get 50% IV glucose
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| Too much stress and stressful events such as illness/surgery or emotional upsets cause hyper or hypoglycemia | Hyperglycemia - b/c stress can cause the cells to reduce their sensitivity to insulin and the sugars can not be carried into the cells
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| The increase of growth hormone at puberty causes hyper or hypoglycemia | Hyperglycemia
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| Gradual onset of hyperglycemia includes | lethargy, weakness, 3P's, N/V, abdominal pain, dehydration and warm and flushed
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| Quick reference between manifestations of hyper and hypo glucemia | warm and dry blood sugar is high, cool and clammy need more candy
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| At which stage may a child need a major readjustment of insulin dosages | Puberty
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| Occures most frequently in adolescents due to noncompliance or changs in hromone levels | diabetic ketoacidosis
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| Often the first indication of presence of diabetes and child ends up in the hospital | diabetic ketoacidosis
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| Blood sugar above 240 and pH less than 7.3 is an indication of | diabetic ketoacidosis
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| Manifestations of diabetic ketoacidosis | ketosis, ketonuria, impaired consciousness, fruity odor to breath, Kussmal's respirations (quick and shallow), dehydration and electrolyte embalances
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| Do not stop taking ____ when a child with type 1 diabetes is sick | insulin
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| How much fluid should a type 1 diabetic who is ill try to take | 8oz per hour
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| How often should a type 1 diabetic check blood sugar when ill | every 2-4 hours
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| Notify healthcare provider if type 1 diabetic ill patient has blood sugar over ____ and is vomiting | BS over 240 and vomiting
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| diagnosis is difficult at which growth and development stage | Infancy -
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| ____ issues can mask the polyuria sign of type 1 diaetics in toddlers | Toilet training
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| At which stage is it a problem to correctly identify and treat hypoglycemic episodes | Mostly Toddler - preschoolers as well
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| Factors that make it difficult to treat toddlers with type 1 diabetes | fear of sticks, finicky eating patterns and poor appitite, and temper tantrums
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| During the preschool stage the child may interpret diagnosis of diaetes as ____ for real or imagined offenses | punishment
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| Preschool children may suffer a set back in ____ and need positive reinforcement | self-esteem
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| Preschoolers also fear injections, and have unpredictable ____ and food intake which lead to hypoglycemic episodes | appitite
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| preschoolers need to be involved in diabetic activity but lack ____ and cognitive development to perform most tasks | coordination
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| school age children are capable of ____ with adult supervision with their diabetes | active role in his own care with adult supervision
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| School age children have ____ issues | dietary
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| Adolescense with diabetes deal with body image concerns and ____ pressure as well as behavioral experimentation, loss of control and ___ skills | body image concerns, peer pressure, behavioral experimentation, loss of control and coping skill
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| education in diabetic management is a major responsibility of ___ | nurses
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| The more a family understands about diabetes the better they will be able to maintain a high degree of ____ | control
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| Phenylketonuria is a genetic disease that results in the body's inability to correctly metabolize? A. glucose B. thyroxine C. Phenylalanine D. Phenylketones | Phenylketonuria (PKU) is an inborn error of metabolism caused by a deficiency or absence of the enzyme needed to metabolize the essential amino acid phenylalanine. Phenyketones are metabolites of phenylalanine, excreted in the urine.
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| Early diagnosis of CH and PKU is essential to prevent which of the following? A. Obesity B. Diabetes C. Mental retardation D. Respiratory distress | Untreated both PKU and CH cause mental retardation. With newborn screening and early intervention, mental retardation from these two disorders can be prevented.
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| Which of the following is characteristic of type 1 diabetes mellitus? A. Onset is usually gradual B. Ketoacidosis is infrequent C. Peak age incidence is 10-15 years D. Oral agents are available for treatment | Type 1 has an abrupt onset unlike type 2. Ketoacidosis is more common in type 1 rather than in type 2. Type 1 diabetes typically has its onset before the age of 20 with a peak incidence between ages 10 & 15 yrs old. Oral agents are only used for type 2.
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| Which of the following is considered a cardinal sign of diabetes mellitus? A. Nausea B. Seizures C. Impaired vision D. Frequent urination | Hallmarks of diabetes mellitus are polyuria, polydipsia, and polyphagia.
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| Glucocorticoids, mineralcorticoids, and sex steroids are secreted by the? A. Thyroid gland B. Adrenal cortex C. Anterior pituitary D. Parathyroid glands | B. Adrenal cortex
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| A child eats some sugar cubes after experiencing syptoms of hypoglycemia. This rapid releasing sugar should be followed by which of the following? A. Glucose tabs & protein B. Glass of water & crackers C. Milk & peanut butter on bread | S/S of hypoglycemia are treated with a rapid-releasing sugar source followed by a complex carbohydrate & protein. Milk supplies lactose & prolonged action from the protein. The bread is a complex carbohydrate, peanut butter provides a sustained action
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| what should the nurse explain a bout exercise to a newly Dx type 1 diabetes fam? A. Exercise increases BS B. extra insulin is required during exercise C. additional snacks are needed before exercise D. excessive physical activity should be restricted. | Exercise lowers blood glucose, reducing the need for insulin. Extra snacks are provided to maintain the blood glucose levels.
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