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Endocrine Disorders in Children

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Question
Answer
Absence of the hepatic enzyme phenylalanine hydroxylase needed to metabolize the essential amino acid phenylalanine to tyrosine.   show
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show 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.   show
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show caucasian  
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show Guthrie blood test  
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show 4  
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show 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   show
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show 20-30mg of phenylalanine  
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T/F an infant with PKU should not be breast fed   show
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show low-protein breads and pasta, veggies, fruits and juices  
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Foods with the highest amount of phenylalanine include   show
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Since aspartame is converted to phenylanine in the GI tract, pt with PKU should avoid ____   show
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show True - even after brain is fully developed  
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____ is the inability to synthesize adequate amounts of Thyroid hormone   show
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show thyroid digenesis (defective thyroid gland development)  
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Congenital hypothyroidism is due to ____ recessive trait   show
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show 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:   show
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show 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   show
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show prevent progressive deterioration of the nervous system and MR  
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show thyroid hormone - Synthetic Levothyroxine Sodium (Synthroid or Levothyroid)  
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Prognosis of congenital hypothyroidism if hormone replacement is started shortly after birth   show
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Signs of overdosage of Synthroid or Levothyroid to treat congenital hypothyroidism include   show
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show fatigue, sleepiness, decreased appetite and constipation  
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Cause of growth hormone deficiency include disfunction of the ____ gland   show
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show panhypopituitarism - result of trauma, hypoplasia, tumor, irradiation and all hormones of the pituitary need to be replaced  
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show short stature  
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show two  
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show 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   show
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What percentage of growth hormone is produced during sleep   show
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show no growth hormone is present or that the amount of growth hormone is not enough  
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show 80%  
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____ closure is definite criterion to stop therapy   show
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show 25-50mcg/kg  
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How much is a child expected to grow after receiving the synthetic growth hormone   show
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____ puberty is sexual development has been before the age of 9 in boys and 8 in girls   show
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show 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   show
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show 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   show
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show sexual, even though the child is fertile  
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show contraception - unless sexually active  
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Congenital Adrenal Hyperplasia causes increases of what three hormones from the adrenal gland   show
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Adrenals normally synthesize ____ from cholesterol to secrete the hormones   show
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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   show
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show ambiguous genitalia from congenital adrenal hyperplasia  
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show 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   show
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show girls do not have ambiguous genitalia, less severly affected infants with variable manifestations  
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show stress  
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show 17-ketosteroid  
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Tx for congenital adrenal hyperplasia   show
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show 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 ____   show
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Medications for Salt-Losers with congenital adrenal hyperplasia   show
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show masculization  
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show dwarfed adults  
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show sterile  
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surgical tx in girls for congenital adrenal hyperplasia should be done before ____ months of age   show
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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   show
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show 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   show
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Incidence of type 2 diabetes has increased in recent years due to   show
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type 2 diabetes is more prevelent in ____ ancestery   show
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show 3P's (polyuria, polydipsia, polyphasia) weight loss, ketonuria, hyperglycemia, fatigue, and irritability  
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clinical manifestations of type 2 diabetes   show
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90% of children with type 2 diabetes have ____ nigricans which is a velvety hyperpigmented area in skin folds   show
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show Metformin  
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Tx plan for children with type 2 diabetes should be addressed at   show
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show 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   show
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Goals for management of diabetes in children   show
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Infant desired blood glucose levels   show
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Toddler and Preschool blood glucose levels b4 meals and bedtime and A1c   show
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School age desired blood glucose (age 6-12) B4 meals, Bed time and A1c   show
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Adolescent and young adults desired blood glucose B4 meals, Bedtime and A1c   show
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The younger a pt is with diabetes the higher the rates of desired blood glucose levels due to thier ____   show
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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   show
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show Beta cells destruction  
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Rapid acting insulin - onset peak and duration   show
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Short acting insulin - onset, peak and duration   show
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Intermediate-acting insulin - onset, peak, duration   show
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Long-acting insulin - onset, peak and duration   show
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Which insulin do you not mix with ohter insulins   show
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show subQ - BID minimum  
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Tight control of diabetes involves ____ dose injections.   show
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T/F the insulin pump improves the A1c levels   show
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Advantages of the insulin pump   show
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The insulin pump allows for ____ baseline infusion with intermittent boluses as determined by user   show
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Boluses given via insulin pump are determined by ____, ____, and ____   show
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show At least 5-6X a day  
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show subcutaneous  
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show every 48 hours  
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show 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   show
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____ helps establish total grams of carbohydrates to eat at meal's and snacks   show
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One serving of starch, fruit, or milk is equal to ____ grams of carbohydrates   show
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T/F vegetables are counted as carbs   show
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How much fat and protein are recommended for children with type 1 diabetes   show
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show prohibited  
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show 15  
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show exercise/Physical Activity  
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show lowers blood sugar and helps insulin ener cell  
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show hypoglycemia  
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Do not exercise if blood glucose is greater than ____   show
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How long after activity can glucose drop   show
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Alcohol increases or decreases blood glucose   show
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show sweating, pallor, nervous, palpitations, tremulousness, hunger, cold, clammy  
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Moderate reactions of hypoglycemia   show
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severe reaction of hypoglycemia   show
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show 10-15 grams of simple carbohydrates followed by complex carbs and retest blood sugar in 15-30 minutes  
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show 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   show
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show Hyperglycemia  
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Gradual onset of hyperglycemia includes   show
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show 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   show
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Occures most frequently in adolescents due to noncompliance or changs in hromone levels   show
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show diabetic ketoacidosis  
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Blood sugar above 240 and pH less than 7.3 is an indication of   show
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Manifestations of diabetic ketoacidosis   show
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show insulin  
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show 8oz per hour  
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show every 2-4 hours  
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Notify healthcare provider if type 1 diabetic ill patient has blood sugar over ____ and is vomiting   show
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diagnosis is difficult at which growth and development stage   show
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show Toilet training  
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show Mostly Toddler - preschoolers as well  
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Factors that make it difficult to treat toddlers with type 1 diabetes   show
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During the preschool stage the child may interpret diagnosis of diaetes as ____ for real or imagined offenses   show
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Preschool children may suffer a set back in ____ and need positive reinforcement   show
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Preschoolers also fear injections, and have unpredictable ____ and food intake which lead to hypoglycemic episodes   show
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show coordination  
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show active role in his own care with adult supervision  
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School age children have ____ issues   show
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show 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 ___   show
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The more a family understands about diabetes the better they will be able to maintain a high degree of ____   show
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show 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   show
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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   show
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show 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   show
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show 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.   show
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