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What is Precocious Puberty?
Refers to early onset of puberty usually before the age of 8 years in gils and 9 years in boys.
- Early onset of puberty in girls before
- age 8 and boys before 9 years
- The child will have secondary sex
- characteristics, accelerated growth rate, and advanced bone maturation
Diagnosed through H&P, labs, xrays
- Treated with GnRH
- agonists monthly IM or intranasally to suppress
- At risk for child sexual abuse—encourage
- parents to promote friendships with peers of the same age
What causes premature appearance of secondary sexual charactristics accelerated growth rate and advanced bone maturation?
What is the major consequence of precocious puberty?
Rapid bone growth which causes early growth plate fusionn and ultimately short stature in adulthood.
What is the definitive test to diagnose between central and peripheral Precocious puberty?
The gonadotrpoin releasing hormone (GnRH)
What is the aim of treatment in PP?
To stop or reverse the development of secondary sexual characteristics and to maximize adult height.
Why are girls with PP at greater risk for sexual abuse?
Because of their mature apperance.
Even though a child with PP looks older how should they be treated?
According to their chronologic age.
What is growth hormone deficiency?
Deficiency that results from inadequate production or secretion of GH. Causing poor growth and short stature
What may manifest in GH deficiency?
Growth hormone deficiency?
Due to inadequate GH
- Symptoms include short stature (below 5th percentile), poor growth,
- immature/cherubic faces, delayed puberty, hypoglycemia, diminished muscle mass
Incidence in US is 1:4000
- Confirmed over 6-12 months and if
- persists, treatment with synthetic GH
What are some other disorders associated with short stature that may respond to GH therapy?
- Turner syndrome
- Prader-Willis syndrome
- Renal disease
- inflammatory bowel disease.
What is the criteria for suspecting growth hormone Deficiency?
Consistently poor growth (less than 5 cm per year)
Growth rate more that 2 standard deviations below the mean for age
Downward deciation from the previous growth rate.
at the time of Diagnosis what percentage of beta cells are still producing insulin in DM2?
Type 2 diabetes Mellitus
- to adult
- presents with
- obesity and insulin
How do you manage DM2?
- Nutritional interventions
- regular exercise
- insulin therapy- may switch to oral agents.
- Glucose monitoring
WHat are the goals for treating DM2?
Achieving near normal glycemic control
reasonable weight for height
normal blood glucose levels
prevention of hyperlipidemia/hypertension
decrease the frequency of microvascular and cardiovascular complications.
What is the only oral drug approved for children with DM2 under 10 years old?
What is Type 1 DM?
results when the pancrease is unable to produce and secrete and adequate amount of insulin.
WHat is the most common childhood endocrine disorder?
type 1 DM
Type 1 diabetes?
- cannot produce and secrete insulin
- from autoimmune process that destroys insulin-secreting cells of pancreas
- predisposition plus environmental or viral trigger initiate process
Incidence of type 1 DM
Increase of 40% from 1997-2010
- Diagnosed in 1:400-500
Median age of onset 7-15 yrs
- Average age of onset--school-aged
- Presents with polyuria, polydipsia,
- polyphagia, weight loss, fatigue, and/or blurred vision
Management goals for Type 1 DM?
- normal glycosated
- hemoglobin (HgbA1c)
- complications especially with hypo and hyperglycemia
What is the Etiology of Type 1 DM?
an inflammatory process in the insulin-secreting islet cells of the pancreas and result from and autoimmune process that cause their eventual destruction.
- disease course is known as the “honeymoon phase” since very little insulin is
- required This may last from a few weeks to 1 year or longer.
The three classic signs of type 1 Dm?
Polyuria (or enursis in toilet trained child)
other signs- weight loss (despite increased food intake)
What is the goal of insulin therapy?
To replace teh insulin the child is no longer able to make in an acceptable physiologic pattern.
Are oral DM agents useful in type 1 DM?
What is intesive insulin treatment?
- or more injections daily
- mimic physiology
Admin of Insulin injections
- degree angle with ½ inch needle
- degree with 5/16 needle
WHy should you rotate insulin injections?
Prevent adipose hypertrophy
Where is the best absorption of insulin?
- increases absorption
Excercise precations with DM?
- working out when insulin is peaking
- carbohydrates pre-workout (15-30 grams)
- glucose frequently
- continue to burn energy post exercise—HS snack
When to monintor Glucose?
- prolonged or new exercise program
- 0200 if hypoglycemia is suspected
- for children 80-180mg/dl pre-meal and for infants/toddlers 80-200mg/dl
considerations in DM
- with carbohydrates
- of control with food choices
- tantrum or hypoglycemia?
School aged considrations in DM?
Can understand simple explanations
- Diabetes didn’t happen “because you were
Willing to try new foods
Often distracted when eating
- More at risk for hypoglycemia due to
- activity level
Adolescence with Dm?
Want to be in control
- Middle adolescence more prone toward
- risk-taking and challenging parents—this could mean missed injections, skipped
- testing, irregular meals, splurging on food
Late adolescence with DM?
- may have trouble relinquishing care
- motivated by predictions of complications
- on the here and now
Sick day care in DM?
Never skip insulin
Test more often
Test for ketones
Hydrate with no calorie drinks
- If unable to eat, use simple
- carbohydrates in liquid form
- ER warnings—”fruity breath”, N/V, deep and rapid respirations, decreased LOC, +
- persistent hyperglycemia.
Diabetic ketoacidosis (DKA
- onset due to too little insulin
- and chest pain, N/V, fruity breath, dehydration, lethargic, decreased LOC, Kussmaul’s
- respirations, and coma
- serum glucose > 300, positive ketones, and serum pH < 7.25
- with IV fluids, insulin drip, electrolyte replacement, and supportive intensive
- onset due to too much insulin
- with 15 g carbohydrate
- loss of consciousness, give glucagon or IV glucose
Signs and symptoms of
Hyopglycemia in the neonate is defined as a plasma glucose concetraion of less than
The neonates who are most likley to experience hypoglycemis are ____infants and infants who are_____?
Premature/small for gestational age.
List five signs that indicate a neonte is hypoglycemic?
- poor feeding
- high pitched cry
- temp instability
- resp alterations
What is teh intervention for a neonate who is hypoglycemcic but asymptomatic?
feed the infant formula breast milk or d5w monitor response by doing glucose checks.
What assessments are indicated if a neonate is at increased risk for hypoglycemia?
Gestational age assessment infants weight lenght and head circumfrence plotted on growht curve blood glucose level by 2 hours of age.
What complication can occur when a neonate is receiving IV glucose
Infiltraion of IV can cause sever extravasation.
Neonatal hypocalcemia is definedas total serum calcium concentration of less than_____?
Why does neonatal hypocalcemia occur most often in infants of diabetic mother?
Maternal diabetes causes immature parathyroid function in the neonate.
What is the best time to admin oral calcium supplement?
Oral supplements are given with feedings because they can cause gastric irritation.
What is the genetic transmission pattern of phenylketonuria?
Phenylketonuria results in damage to which body system?
When should a neonate be screened for PKU?
After 2 days of birth.
What is the treatment for PKU?
Special diet that restricts phenylalanine intake
T/F- THe child with galactosemia must be on a lifelong low protein limited amino acid diet?
T/F- Maple syrup urine disease casuse ketoacidosis 2-3 days after birth.
T/F- Tay-sachs disease can be treated through dietary modifications?
In congenital adrenal hyperplasia the adrenal gland is not able to manufacture_____but instead producess excess___?
What finding in the newborn infant should raise suspicion of congenital adrenal hyperplasia?
The treatment of congential adrenal hyperplasia involves lifelong________?
T/F- Newborn screening for hypothyroidism should be done between 10-14 days of age?
T/F- If untreated congenital hypothyroidism can result in intellectual disability?
T/F- Treatment of congenital hypothyroidism requires lifelong thyroid homeone replacement
T/F- The most common cause of acquired hypothyroidism in children is an autoimmune process?
A decreased TSH level is the most sensitive indicator of primary hypothyroidism?
Decreased activity is related to?
Nervousness is related to?
Increased appetite is related to?ro
Weight gain is related to?
Edema of face hands and eyes is related to?
Cold intolerance is related to?
THe preferred treatment for a child with hyperthyroidism is___?
Antithyroid therapy with propylthiouracil or methimazole
THe child receiving propylthiouracil must be monitored for which three significant AR's
Why may treatment of graves disease be tapered after a few years.
To determine whether a child has gone into remission.
List two classic manifestations of diabetes insipidus
Increased urination (polyuria)
excessive thrist (polydipsia)
Define precocious puberty?
Premature apperance of secondary sexual characteristics accelerated growth rate and advanced bone maturation.
WhAT is the major consequence of precocious puberty?
Rapid bone growth whic causes early fusion and ultimately results in short adult stature.
The treatment of precocious puberty?
Admin of a gonadotropin-releasing hormone GnRH agonist or blocker.
How does the treatment for precocious pueberty inhibit prcocious puberty?
It inhibits binding of GnRH to the pituitary gland causing a decrease in hormone production this stops sexual development from progressing and slows down bone age advancement