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A genetic metabolic disorder that causes CNS damage. Homozygote, autosomal recessive disorder.
PKU
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What enzyme is deficient in PKU?
phenylalnine hydroxlase
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What is commonly the first sign of PKU?
Digestive problems with vomiting
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Name the signs and symptoms of PKU
- Digestive problems
- Musty odor to urine
- Infantile eczema
- Hypertonia
- Hyperactivity
- Common in blonde hair/blue eyes
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This disorder can adversely affect the myelinization process in CNS development
PKU
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Patients with PKU should avoid what foods?
Meet, fish, eggs, cheese, milk, legumes, aspartame
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Foods children with PKU can eat
Veggies, fruits, starches, milk substitutes
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What is a common manifestation associated with GH deficiency?
Hypoglycemia
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Possible underlying causes to GH deficiency
- Hypopituitarism
- Brain Tumors
- Turner Syndrome
- Prader-Willi Syndrome
- Renal Disease
- Inflammatory Bowel Disease
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Manifestations of GH deficiency
- Height less than 5th percentile of age and sex
- Delayed Puberty
- Hypoglycemia
- Diminished muscle mass
- Increased adiposity
- Micropenis
- Hyponatremia
- Jaundice
- High-pitched voice
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Children lacking more than one hormone produces by the pituitary gland are referred to as having what?
Hypopituitarism
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Children growing less than ________ per year should be referred to an endocrinologist.
5 cm
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How is synthetic growth hormone administered?
- Sub Q
- 6-7 times per week
- Usually at bedtime
- Done for 1 year
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How often should a child with GH deficiency be evaluated?
Every 3-4 months
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When should GH therapy be discontinued?
When child reaches appropriate adult height or when growth plates close
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An autoimmune condition in which excessive thyroid hormones are produced by an enlarged thyroid gland.
Graves Disease
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Which gender is more likely to have Grave's Disease?
Girls are 5x more likely
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What is the peak age for acquiring Grave's Disease?
11-15 years old
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Manifestations of Grave's Disease
- Goiter
- Increased appetite
- Wight loss
- Nervousness
- Heat intolerance
- Perspiration
- Increased HR
- Muscle weakness
- Exopthalmos
- Poor attention span
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In Graves Disease, Serum T4 will be ___ and TSH will be ___.
- Elevated T4
- Suppressed TSH
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What is the treatment of choice for children with Grave's?
- Antithyroid Drug Therapy
- -propylthiouracil
- -methimazole
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Name key points in Radioactive Iodine treatment of Grave.
- Oral solution
- Typically used in children older than 10
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What is given to a patient having a thyroidectomy 10-14 days before the surgery to decrease gland's vascularity?
Lugol's Solution (potassium iodine)
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What level should be specifically monitored after removal of thyroid gland?
Calcium. Injury to the parathyroid gland can result in hypocalcemia
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Important considerations for children taking propylthiouracil.
- Taken 2-3 times a day
- Monitor for fever, rash,joint pain, edema, and excessive bruising
- Increased risk for neutropenia and hepatotoxicity
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How often should child be evaluted while undergoing treatment for Graves?
Every 2-4 months
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Due to possible liver damage, contact sports should be limited during treatment of this disease.
Graves
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S/S of Congenital Hypothyroidism
- Skin mottling
- Large fontanel
- Large tongue
- Hypotonia
- Slow reflexes
- Distended ab
- Prolonged Jaundice
- Cool to touch
- Lethargy
- Umbilical hernia
- Hoarse cry
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With congenital hypothyroidism, the T4 levels will be ___ and the TSH will be ___
- T4 will be low
- TSH will be high
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How often are children with Hypothyroidism evaluated during their first year of life?
every 1-2 months
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What measurements should the nurse obtain at every visit in the child with congenital hypothyroidism?
Height, Weight, Head circumference
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What should the parent do if a child vomits within one hour of administering levothyroxine?
Readminister
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