Peds Metabolid disorders
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What is Diabetes Inspitus?
- • An inability to concentrate urine because of a deficiency of vasopressin (ADH)
- Frequently results from head trauma, tumors, or infections within the hypothalmus
- Also results from cranial irradiation for tumors
- • Many cases considered idiopathic
SnSs of diabetes insipitus?
- • Increased urination (polyuria)
- • Excessive thirst (polydipsia)
- • Other: nocturia and dehydration
- • Diagnostics: Hypernatremia and low urine specific gravity without hyperglycemia
Tx of diabetes insipitus?
- • Maintain fluid balance
- • Administer synthetic vasopressin (DDAVP)
- • Overtreatment will result in fluid retention and dilutional hyponatremia
Whats the diff between diabetes insipitus and SIADH?
What is Cushing's Syndrome?
- • Group of manifestations caused by excessive circulating free cortisol
- • Can result from a variety of causes
- • Clinical Manifestations:
- Moon facie, pendulous abdomen, poor wound healing, weight gain, excessive hair growth (hirsutism)
What is congenital adrenal hyperplasia?
- • Group of disorders
- • Adrenal gland is not able to produce adequate glucocorticoid
- However produces excess androgens during process of attempting to make adequate glucocorticoid
- Caused by a defect in the enzymatic pathway
What are SnSs of congenital adrenal hyperplasia?
- • Ambiguous genitalia of the newborn female
- • Salt-wasting crisis in the first few weeks of life with low serum Na+, high serum K+, hypovolemia, and hypotensive crisis
- • May manifest with a muscular body, advanced bone age, and premature pubic hair
Tx of congenital adrenal hyperplasia?
- • Life-long treatment with glucocorticoid therapy
- Hydrocortisone TID
- Mineralocorticoid (Aldosterone) replacements if “salt wasting”
- Monitor growth and assess for signs of early puberty
What is congenital hypothyroidism?
SnSs of congenital hypothyroidism?
- Skin mottling, large fontanel, large tongue, hypotonia, slow reflexes, distended abdomen
- Other: lethargy, constipation, feeding problems, coldness to touch, excessive sleeping
- Usually id’d by newborn screening
- Thryoid scan can identify any functioning thyroid tissue
- Requires life-long thyroid replacement (Levothyroxine)
How is aquired hypothyroidism usually aquired?
- From Tx for cancer, ie Hashimoto's thyroiditis.
- • Usually presents with a goiter
- • Result of an autoimmune process
- • Most common cause of hypothyroidism in children and adolescents
What is the pathophys of aquired autoimmune hypothyroidism
- Circulating autoantibodies decrease thyroid gland production of T3 and T4
- These autoantibodies bind at TSH sites on the thyroid gland, resulting in decrease of TH production
- Cause of autoimmune reaction is unknown
- • Diagnosis is made by noting elevated levels
- of TSH and low T4 levels
SnSs of aquired hypothyroidism?
- • Goiter
- • Dry, thick skin
- • Coarse dull hair
- • Fatigue, cold intolerance
- • Constipation
- • Weight gain
- • Decreased linear growth
- • Edema of the face, eyes, and hands
- • Irregular or delayed menses
Tx for aquired hypothyroidism?
- • Oral Thyroid hormone replacement
- • Prompt treatment needed for brain growth in infants
- • Compliance with medication regime is crucial
What is hyperthyroidism?
- AKA Grave's disease
- • Most common cause of hyperthyroidism in childhood is Graves Disease
- Autoimmune condition in which excess thyroid hormones are produced by an enlarged thyroid gland
Tx for Grave's Disease?
- • Manifestations develop gradually, often over 6 to 12 months
- • Diagnosis based on increased levels of T3, T4 with suppressed TSH
- • Treatment: anti-thyroid drug therapy, radioactive iodine, or surgery
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