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Pediatric

Endocrine Disorders in Children

QuestionAnswer
Absence of the hepatic enzyme phenylalanine hydroxylase needed to metabolize the essential amino acid phenylalanine to tyrosine. Phenylketonuria (PKU) not an endocrine disease
____ is needed to form melanin, epinephrine and thyoxine throsine - missing in pt with PKU
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
PKU primarily affects ____ children caucasian
Diagnostic test for PKU includes ___ blood test Guthrie blood test
The Guthrie blood test Determines phenylalanine levels in blood (greater than ____ mg/dl) Normal new born levels are 0.5-1 mg/dl. 4
clinical manifestations of PKU FTT, vomiting, irritable, developmental delay, unpredictable erratic behaviros, MR, mousy or musty odor to urine in older infant
T/F restriciting dietary protein can maintain phenylalanine levels within the safe range 2-8mg/dl True
How much phenylalanine per kg per day is allowed to keep blood levels within normal range? 20-30mg of phenylalanine
T/F an infant with PKU should not be breast fed False - as long as the mother is careful about monitoring her diet
Foods with the lowest amount of phenylalanine include low-protein breads and pasta, veggies, fruits and juices
Foods with the highest amount of phenylalanine include chicken, steak, pork chops, hamburger, milk, eggs, cheese, refried beans, nuts and peanutbutter
Since aspartame is converted to phenylanine in the GI tract, pt with PKU should avoid ____ NutraSweet, Equal and some meds with artificial sweetner
T/F it is recommended for PKU pt to stay on low phenylalanine diet for life True - even after brain is fully developed
____ is the inability to synthesize adequate amounts of Thyroid hormone congenital hypothyroidism
Most congenital hypothyroidism is due to ____ thyroid digenesis (defective thyroid gland development)
Congenital hypothyroidism is due to ____ recessive trait autosomal
clinical manifestations of congenital hypothyroidism right after birth poor feding, lethargy, prolonged neonatal jaundice, resp difficulty and bradycardias, constipation, hoarse cry, and large fontanels
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
Diagnosis of congenital hypothyroidism includes a blood test to measure ___ levels T4 blood levels - usually taken within first 24-48 hrs
T/F diagnosis of congenital hypothyroidism and PKU are mandatory in all 50 states True
Reason for early diagnosis for congenital hypothyroidism prevent progressive deterioration of the nervous system and MR
Tx of congenital hypothyroidism is life long replacement of ___ thyroid hormone - Synthetic Levothyroxine Sodium (Synthroid or Levothyroid)
Prognosis of congenital hypothyroidism if hormone replacement is started shortly after birth normal physical growth and intelligence
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
Signs of underdosage of Synthroid or Levothyroid to treat congenital hypothyroidism include fatigue, sleepiness, decreased appetite and constipation
Cause of growth hormone deficiency include disfunction of the ____ gland pituitary
____ is the total lack of all hormones panhypopituitarism - result of trauma, hypoplasia, tumor, irradiation and all hormones of the pituitary need to be replaced
What is the chief complaint with idiopathaic hypopituitarism is ____ caused by decreased secretion of growth hormone short stature
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
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
A diagnostic evaluation for short stature includes ____ surveys including epiphyseal maturation and bone age radiographic
What percentage of growth hormone is produced during sleep 66%
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
Tx of growth hormone deficiency is effective ____% of children 80%
____ closure is definite criterion to stop therapy epiphyseal
How much of a synthetic growth hromone is used as daily injections for growth hromone deficiency 25-50mcg/kg
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
____ 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
Goal of precocious puberty is to ____ hault the progress of sexual development
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
LHRH and synthetic hormones to treat precocious puberty is discontinued when at a chronological appropriate time allowing pubertal changes to resume
T/F a child with precocious puberty should dress appropriate for their development False - should dress appropriate for age
____ interest is usually not advanced beyond the child's chronological age for children with precocious puberty sexual, even though the child is fertile
A child with precocious puberty is fertile but should not take____ contraception - unless sexually active
Congenital Adrenal Hyperplasia causes increases of what three hormones from the adrenal gland Mineralocorticoids (produce aldosterone) Androgens (produce testosterone) and gluccocorticoids (produce cortisol)
Adrenals normally synthesize ____ from cholesterol to secrete the hormones steroids
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
____ 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
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
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.
nonclassical congenital adrenal hyperplasia signs girls do not have ambiguous genitalia, less severly affected infants with variable manifestations
____ can trigger adrenal insufficiency episode stress
lab results for congenital adrenal hyperplasia include a 24 hr urine for presence of ____ levels 17-ketosteroid
Tx for congenital adrenal hyperplasia hydrocortisone (cortisol) replacement in the form of hydrocortisone tablets
Hydrocortisone (cortisol) treats congenital adrenal hyperplasia by supression of ACTH by the pituitary, androgen production is decreased, and virilization is halted
lifelong replacement of hydrocortisone (cortisol) is neeed with increased doses during periods of ___, ____, ____, and ____ stress, surgery, infection, and illness
Medications for Salt-Losers with congenital adrenal hyperplasia aldosterone replacment, Florinef Acetate, cortisone (Solu-cortef) when under stress, increased Na+ intake
If no replacement medications are given for kids with congenital adrenal hyperplasia ____ of female will continue masculization
If treatment is not given for kids with congenital adrenal hyperplasia both sexes will have premature epiphyseal closure resulting in ____ adults dwarfed adults
males without tx for congenita adrenal hyperplasia will be ____ as an adult sterile
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)
Most common endocrine disease in children peaks in incidence between 10-15 years but can occure anytime usually b4 age 20 Type 1 diabetes
Type 1 diabetes is more prominent in ____ and an absolute ____ deficience caucasians with absolute insulin deficiency
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
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
type 2 diabetes is more prevelent in ____ ancestery Non-european
Manifestations of type 1 diabetes 3P's (polyuria, polydipsia, polyphasia) weight loss, ketonuria, hyperglycemia, fatigue, and irritability
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)
90% of children with type 2 diabetes have ____ nigricans which is a velvety hyperpigmented area in skin folds Acanthosis Nigricans
___ is the only oral diabetic agent approved for youths Metformin
Tx plan for children with type 2 diabetes should be addressed at life style and risk factors of the entire family
Diagnosis of diabetes in children fasting blood sugar on two occasions 126mg/dl - the perfered method of diagnosis
____ - 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
Goals for management of diabetes in children achieve glycemic control, promote normal growth and development, and prevent or delay complications
Infant desired blood glucose levels 100-200
Toddler and Preschool blood glucose levels b4 meals and bedtime and A1c B4 meals = 100-180, Bedtime 110-200, A1c 7.5% - 8.5%
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%
Adolescent and young adults desired blood glucose B4 meals, Bedtime and A1c B4 meals 90-130, Bedtime 90-150, A1c less than 7.5%
The younger a pt is with diabetes the higher the rates of desired blood glucose levels due to thier ____ vulnerability to hypoglycemia
Shortly after insulin therapy, the pancrease kicks in one last time and releases exogenous insulin for a period of time is called Honeymoon Period
___ destruction will continue during the honeymoon period and continue to give low doses of insulin Beta cells destruction
Rapid acting insulin - onset peak and duration Lispro (Humalog) (Novalog) - onset 5-15 min, peak 30-90 minutes, duration 4-5 hours
Short acting insulin - onset, peak and duration Regular insulin (Humulin R) (Novolin R) Onset30-90 minutes, Peak 2-4 hours, Duration 5-7 hours
Intermediate-acting insulin - onset, peak, duration NPH (Humulin N), onset 1-2 hours, peak6-14 hours and duration 16-20 hours.
Long-acting insulin - onset, peak and duration Insulin glargine (Lantus) onset 90 minutes, Peakless, duration 24 hours
Which insulin do you not mix with ohter insulins Long-acting Insulin glargine (Lantus)
Insulin is given ____ injection at least ___ tims a day subQ - BID minimum
Tight control of diabetes involves ____ dose injections. multiple to cover food intake and once at night (Lantis) for bedtime basal control of 24 hours
T/F the insulin pump improves the A1c levels True
Advantages of the insulin pump allows for more physiologic insulin delivery, flexible with meal and snack time, decreased amount of injections
The insulin pump allows for ____ baseline infusion with intermittent boluses as determined by user continuous
Boluses given via insulin pump are determined by ____, ____, and ____ blood sugar, activity and diet
How many times must blood sugar be monitored for a pt with an insulin pump At least 5-6X a day
An insulin pump delivers insulin by a small needle injected in ____ tissue of the abdomen subcutaneous
How often are needles changed in an insulin pump every 48 hours
How long can an insulin pump be removed One hour per day
____ is the main food that raises blood sugar no matter its source and must be counted to determine how much insulin is needed Carbohydrate
____ helps establish total grams of carbohydrates to eat at meal's and snacks dietitian
One serving of starch, fruit, or milk is equal to ____ grams of carbohydrates 12-15
T/F vegetables are counted as carbs False - they are not counted
How much fat and protein are recommended for children with type 1 diabetes reasonable amounts
concentrated sweets for type 1 diabetics are discouraged by not ____ prohibited
How many grams of carbohydrates equals one carb 15
Cells utilize blood glucose more effectively when the child engages in ____ exercise/Physical Activity
Exercise ____ blood sugar and helps ____ to enter the cell more readily lowers blood sugar and helps insulin ener cell
Take carbohydrate source before exercise to prevent ____ hypoglycemia
Do not exercise if blood glucose is greater than ____ 240, splitting ketones
How long after activity can glucose drop 6-12 hours so check glucose at bedtime and or during the night
Alcohol increases or decreases blood glucose decreases = hypoglycemia
Mild symptoms of hypoglycemia sweating, pallor, nervous, palpitations, tremulousness, hunger, cold, clammy
Moderate reactions of hypoglycemia weakness, dizziness, A, drowsiness, irritable, loss of coordination, and disorientation
severe reaction of hypoglycemia seizures, semi comatose or coma, slurred speech, staggering gait, combative, confused
Mild to Moderate tx of hypoglycemia 10-15 grams of simple carbohydrates followed by complex carbs and retest blood sugar in 15-30 minutes
Severe tx of hypoglycemia Adminster glucagon, 911 to get 50% IV glucose
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
The increase of growth hormone at puberty causes hyper or hypoglycemia Hyperglycemia
Gradual onset of hyperglycemia includes lethargy, weakness, 3P's, N/V, abdominal pain, dehydration and warm and flushed
Quick reference between manifestations of hyper and hypo glucemia warm and dry blood sugar is high, cool and clammy need more candy
At which stage may a child need a major readjustment of insulin dosages Puberty
Occures most frequently in adolescents due to noncompliance or changs in hromone levels diabetic ketoacidosis
Often the first indication of presence of diabetes and child ends up in the hospital diabetic ketoacidosis
Blood sugar above 240 and pH less than 7.3 is an indication of diabetic ketoacidosis
Manifestations of diabetic ketoacidosis ketosis, ketonuria, impaired consciousness, fruity odor to breath, Kussmal's respirations (quick and shallow), dehydration and electrolyte embalances
Do not stop taking ____ when a child with type 1 diabetes is sick insulin
How much fluid should a type 1 diabetic who is ill try to take 8oz per hour
How often should a type 1 diabetic check blood sugar when ill every 2-4 hours
Notify healthcare provider if type 1 diabetic ill patient has blood sugar over ____ and is vomiting BS over 240 and vomiting
diagnosis is difficult at which growth and development stage Infancy -
____ issues can mask the polyuria sign of type 1 diaetics in toddlers Toilet training
At which stage is it a problem to correctly identify and treat hypoglycemic episodes Mostly Toddler - preschoolers as well
Factors that make it difficult to treat toddlers with type 1 diabetes fear of sticks, finicky eating patterns and poor appitite, and temper tantrums
During the preschool stage the child may interpret diagnosis of diaetes as ____ for real or imagined offenses punishment
Preschool children may suffer a set back in ____ and need positive reinforcement self-esteem
Preschoolers also fear injections, and have unpredictable ____ and food intake which lead to hypoglycemic episodes appitite
preschoolers need to be involved in diabetic activity but lack ____ and cognitive development to perform most tasks coordination
school age children are capable of ____ with adult supervision with their diabetes active role in his own care with adult supervision
School age children have ____ issues dietary
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
education in diabetic management is a major responsibility of ___ nurses
The more a family understands about diabetes the better they will be able to maintain a high degree of ____ control
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.
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.
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.
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.
Glucocorticoids, mineralcorticoids, and sex steroids are secreted by the? A. Thyroid gland B. Adrenal cortex C. Anterior pituitary D. Parathyroid glands B. Adrenal cortex
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
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.
Created by: cgwayland