Endocrine & Metabolic
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What does the endocrine system do and what is it comprised of?
- The endocrine system is composed of
- various tissues that produce and secrete hormones
What is feedback control?
- Feedback control is how the tissues know
- when and how much hormone to release
What is negative feedback?
- Negative feedback—when the system detects
- increasing amounts of hormone and limits their release
What is positive Feedback?
- Positive feedback when the system detects
- hormone levels are too low and secretes the hormone
What do hormons do?
Stimulate and regulate hte actions of other tissues-- the target tissues.
What does the Endocrine system work in tandum with to regulate growth, metabolism and reproduction?
Autonomic nervous system.
What controls the activites of the Endo and auto nerv system?
The hypothalamic-pituitary axis.
What are some diffrences in the pediatric endocrine system?
- It is less developed at birth than any other body system
- Hormonal control of many body functions is lacking until 12 to 18 months of age
- as a result infant ma manifest imbalances in the concentration of fluids electrolytes amino acids glucose and trace substances.
What happens when too little circulating hormone is present?
The target gland is stimulated ot secrete additonal hormon.
What is the pituiatry gland composed of?
Anterior and posterior lobes.
What does the anterior lobe secrete?
Adrenocorticotropic hormone (ACTH)
Thyroid-stimulating hormone (TSH)
Follicle-stimulating hormone (FSH)
Luteinizing hormone (LH)
Growth hormone (GH)
Which four anterior hormones stimulate their target glands to secrete hormone?
ACTH, TSH, LH, FSH
What hormones does the posterior gland deal with?
IT stores and releases antidiureteic hormone (ADH) and oxytocin. Which are synthesized by the hypothalamus.
What Posterior lobe hormones are synthesized by the hypothalamus?
ADH and oxytocin
What are normal hormone levels related to?
THe childs age and stage of puberty.
When assessing a childs endocrine system what do you need an accurate measurment of?
- and sexual development using the Tanner stages
What is the Pancrease responsible for?
- Islets of Langerhans
- Beta cells
- responsible for insulin
What hormones come from the adrenal glands?
Which babies are at risk for Neonatal Hypoglycemia?
- The SGA babies
- 1.3-3 per 1000 births
Symptoms of neonatal hypoglycemia?
- poor feeding, lethargy, seizures
Treatment for Neonatal Hypglycemia?
- When asymptomatic treat with breast milk,
- formula or D5W po
lIf lethargic, may require gavage feedings
- lIf glucose < 20-25 mg, IV bolus of D10
- W at 2 ml/kg and continuous IV replacement
lGlucose goal—50 mg or above
- lIf condition persists, a total workup is
What is level for hypocalcemia?
- serum calcium below 7.0
- risk are babies born to diabetic mothers, SGA, or with history of birth
Symptoms of Hypocalcemia?
Managment of hypocalcemia?
- feeding may correct
- or parenteral supplement
- calcium is very irritating to veins
What is Phenylketonuria (PKU)
Genetic metabolic disorder that results in CNS damage from toxic levels of phenylalanine in the blood.
What is the etiology of PKU?
An autosomal recessive disorder and is manifested in the homozygote (individual who inherited tow identical genes foe a specific trait).
How soon is the infant affected by PKU?
Metabolic alterations begin immediately but signs do not appear until 3 MONTHS
What is the first sign of PKU?
Digestive problems with vomiting!! they may also have a musty or mousy oder to the urine also infatile eczema, hypertonis, hyperactive.
What may older children with PKU have?
Hypopigmentation of hair skin and irises (commonly blond with light blue eyes) also MR is a long term consequence.
What is a long term consequence of untreated PKU?
Intellectual impairment (MR)
How do you diagnosis PKU?
- baby is less than 3 days old, may have a false negative test
- results are presumptive
Why is PKU screening done on infants younger that 2 days
For fear of the infant will not have in follow up.
Why should the PKU test be done at 3 days old?
Because any earlier might have a false negative result. If it is done before 3 days than you should repeat at several days old.
What tests are used to screen for PKU?
- Guthrie test
- fluorometric assay
Management of PKU
Immediate restriction of phenylalanine
lLife-long dietary restrictions
- lRESTRICTS protein, avoids meats, cheese,
- fish, eggs, milk, and legumes.
lLow protein bread, cereal, and pasta
- lStaples are vegetables, starches, and
- lMay take phenylalanine-free protein
What is the goal of PKU therapy?
Serum Phenylalanine level at 2 to 6 in infants and young children. 2 to 15mg/dl in children older than 12 years
What is Cofactor tetrahydrobiopterin (sapropterin)?
A medication that can lower blood phenylalnine levels.
What is congenital adrenal Hyperplasia?
- cannot produce glucocorticoid
- androgen excess resulting in decreased glucocorticoid and increased androgen
- in ambiguous genitalia of newborn
- wasting” or low sodium
What causes CAH?
a defect in the enzymateic pathway of adrenal steroid productions, Diminished glucocorticod production prompts increased ACTH produciton further increasing adrenal androgen excess
Managment of CAH?
- glucocorticoid replacement
- require mineralocorticoid with salt wasting features
- the child is sick, the replacement dose may be increased and require a
- different mode of delivery
- may require reconstructive surgery for genitalia
- bone density evaluations
What happens to the salt in infants with dimihsed mineralocorticoid production?
Will waste salt through the kidneys. "Salt-wating" crissis.
What can happen with Salt wasting crisis?
- 21-hydroxylase deficiency.
What can untreated or poorley treated CAH result?
Advanced bone age with ultimatie adult short stature.
How is CAH diagnosis confirmed?
What are special sick day instructions for CAH?
Teh glucocorticoid dosage is usually double or tripled when the child is ill, has a broken bone or is undergoing a surgical procedure.
If a child with CAH starts to vomit you should?
admin glucocorticoid parentaerally
What do you look assess for in an older child taking glucocorticoid replacement?
Liner growth and signs of early puberty.
What is congenital hypothyroidism?
a condition in which teh thyroid gland does not produce sufficent thyroid hormoneto meet the bodys metabolic needs.
- deficit at birth due to absent, underdeveloped, or ectopic thyroid gland
- by mandatory newborn screening
- undetected or untreated profound mental retardation
What causes congenital hypothyroidism?
an absent (Aplastic) underdevelped, or ectopic thyroid gland this group of congential defects is reffered to as Thyriod dysgenesis
What can happen if there is a maternal intake of medications such as pylthiouracil (PTU)?
can cause transient hypothyrodism
What else can cause transiet hypothryoidism?
Transfer of maternal antibodies to the fetus.
Symptoms of congenital
Managment of CH?
- suspected, do not delay treatment
- parents symptoms of hypothyroid and hyperthyroid to help with medication
What can CH cause if untreated?
How is CH diagnosed?
By testing for T4in newborn screening. at 2-6 days old.
What med is used in life long treatment of CH?
What is acquired hypothyroidism?
a condition in which the thyroid galand produces an inadequate amount of thyroid hormone to meet the body's metabolic needs
acquired hypothyroidism is?
- child’s thyroid is underproductive
- causes but the most common is Hashimoto’s thyroiditis--an autoimmune response
- cause decreased thyroid production and TSH levels rise—a goiter is common
What is a common cause of AH?
Hashimoto's thyroiditis and is usually associated with a goiter it is a result of an autoimmune process.
Who is thyroiditis most common in?
In contrast to congential hypothyroidism when do AR's present in AH?
2-3 years and are often reversible.
What is a goiter?
An enlarged thyroid gland that occure in response to increased TSH secretion autoimmune attack of the thyroid gland.
What is hyperthyroidism (grave's disease)?
an autoimmune condition in which excessive thryoid hormones are produced by an enlarged thyroid gland. it is most common cause of hyperthyroidism in children.
- condition causing excessive thyroid hormone production
- undetected or untreated may result in cardiac failure and death
Hypothyroid versus Hyperthyroid
Lab findings for thyroid disorders?
- thyroid antibodies
- iodine uptake with scans
Treatment of thyroid disorders
Antithyroid drugs: propylthiouracil, methimazole
Why is methimazole used over PTU in graves disease?
Can cause liver toxicity.
THe three approches to treating graves disease?
- Antithyroid drug therapy
- radioactive iodine
Radioactive Iodine is given to children older than?
10 years old
What is give to the patient prior to a thyroidectomy?
Lugols solution (Potassium Iodine) give 10-14 dAYS prior to decrease glands vascularity. Calcium levels are monitored after surgery.
What is Diabetes insipidous?
an inablitiy to concentrate urine.
In central DI what is there a deficiency of?
Vasopressin also known as Antidiurtic hormone (ADH)
in Nephrogenic DI what are the kidneys insensitive to?
Diabetes Insipidous vs. Syndrome of Inappropriate
Diabetes Insipidus (high and dry)
- concentrate urine vasopressin—ADH deficit
- polyuria, nocturia,
lExcessive release of ADH or vasopressin
- urination, increased urine sp. gravity, fluid retention, weight gain.
Managment of DI
- specific gravity at 1.1010-1.020
Management of SIADH?
- precautions if sodium < 125
What do you admin for central DI?
- synthetic vasopressin DDAVP
Levels for hypernatremia?
- sodium >150 meq/l
- low urin specific gravity in the abscence of hyperglycemia
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