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N404 Peds Endocrine Lecture
Transcript of N404 Peds Endocrine Lecture
Clinical Manifestations: few signs in the first weeks of life
cretinoid features - thickened protuberant tongue, thick lips, and dull appearance
prolonged neonatal jaundice
bradycardia and decreased pulse pressure
hypothermia and cool extremities
large posterior fontanel
Obesity (related more to dropping age of puberty?)
Exposure to endocrine disrupting chemicals (EDCs) - can delay accelerate or delay puberty
DDT (bug spray) - estrogenic effects
PCBs (coolents and lubricants) - persistent estrogenic effects
Isofavones in soy - weak form of estrogen
phthalates (plastics, medical equipment, cosmetics, and beauty products) - mimics estrogen
Hormone containing skin and hair care products
Hormones in assisted reproductive technologies
Early exposure to increasingly sexualized society (very controversial connection)
Central / gonadotropin-dependent precocious puberty
2/3 of cases in girls are idiopathic
hypothalmus is activated to secrete gonadotropin-releasing hormone (GnRH)
congenital anomaly (e.g. hydrocephalus)
Peripheral / gonadotropin-independent precocious puberty
McCune-Albright syndrome (mostly females)
ovarian follicular cyst
HCG secreting tumor
Incomplete Precocious Puberty
premature thelarche, adrenarche, menarche (look for gyn cause)
Tami Bland, DNP, CPNP
Endocrine Disorders/ Inborn Errors of Metabolism
fetal differentiation of reproductive and CNS
regulation of growth and development (along with CNS)
coordination of male and female reproductive systems
optimal level of hormones for body functioning
homeostatisis in presence of ever-changing external environment
Functions of the
Thyroid-stimulating hormone (TSH)
Adreno-corticotropic hormone (ACTH)
Follicle-stimulating hormone (FSH)
Luteinizing hormone (LH)
Melanocyte-stimulating hormone (MSH)
Secondary sex characteristics before age 8 or onset of menarche before 9.
Lack of enzyme needed for synthesis of cortisol and aldosterone - 95% of these are 21-hydroxlyase (21-OH)
autosomal recessive inheritance pattern
Leading cause of pseudoheraphroditism (ambiguous genitalia)
Disturbance of adrenal steroid pathway
overproduction of androgen
decreased cortisol production
Virilization of female external genitalia begins in week 10 of gestation
If untreated, accelerated height, early closure of epiphyseal plates, and premature sexual development
decreased or no production
males are generally normal in appearance
Adrenal crisis: salt loss, hypovolemic shock, and death
Virilization (in females)
failure to thrive
Virilization (in females)
Early beard growth and enlarged hallus with small testes in males
Accelerated growth, advanced bone age, premature adrenarche
Significant cystic acne and adult body odor
Linear growth must be monitored
closely - a direct reflection of
Glucocorticoid therapy can cause obesity
Development should be normal with appropriate treatment, including puberital, but late diagnosis and inadequately treated children may look taller and more mature than peers until they stop growing prematurely and then are shorter than their peers.
Start of therapy may trigger precocious puberty.
Allow for adequate sodium intake - can salt to taste
Avoid calorie dense foods to prevent obesity
Increase in cortisol levels
vomiting or unable to feed
school or emotional stress
Medical information should always be on the child
Emergency supply of IM hydrocortisone or glucocorticoid suppositories - medication available within 15 minutes for trips and high risk activities
Symptoms typically present within few weeks of birth, but nonclassic CAH is often diagnosed later in life when growth is affected.
Girls with common urogenital canals may be prone to vaginal voiding and vaginitis.
Normal behavior and discipline should be expected.
Encourage active participation in care in preparation for transition to self-care during adolescence.
Prepare for rebellious periods by teens.
Personnel must understand nature of diagnosis, need for timely medication, safety precautions, and signs of serious illness.
Monitor for premature sexual
Menstrual irregularities are common
Fertility rates are low
elevated androgens leading to ovarian dysfunction
psychosexual behaviors around gender identity and selection of sexual partners
Focus changes from growth and
development to long-term health preservation.
See patient without parents as much as possible.
Primary care often needs to assist family in finding adult specialists to replace pediatric specialists.
Studies show that too early assumption of self-management for diabetes results in poorer psychological and metabolic outcomes. At the same time, adolescents transitioning want maternal oversight to decrease with a knowledge transfer to the teen.
Help regarding insurance, medical benefits, and medication funding is often needed, particularly for expensive medications such as growth hormone.
Assist with focusing on the normalcy of child.
Case management assistance may be needed.
Genetic counseling is often needed if there is an increased risk for other children to have the same diagnosis.
Cultural implications must be considered. For example, discussions about sexual issues for child with CAH may be difficult for some families.
Goal is to suppress adrenal secretions by replacing deficient hormones
Prenatal steroids used to suppress mutations
Lifelong use of oral glucocorticoids (individualized by monitoring growth parameters, bone age, and hormone levels)
Reconstructive surgery of enlarged clitoris during first year of life common
University of Wisconsin Hospitals and Clinics Authority. (2011). Guidelines for the Child Who Is Cortisol or Steroid Dependent. Retrieved from http://www.uwhealth.org/healthfacts/B_EXTRANET_HEALTH_INFORMATION-FlexMember-Show_Public_HFFY_1105646182511.html
CARES (Congenital Adrenal Hyperplasia Research, Education, and Support) Foundation Inc. www.caresfoundation.org
Congential Adrenal Hyperplasia Education and Support Network. http://www.congenitaladrenalhyperplasia.org/
Ratio of girls to boys 10:1
Age limits set 30 years ago. Decreasing age of menarche in girls is spurring debate about lowering normal ages to 7 in white and 6 in African American girls.
Other Risk Factors
Queen Helene Placenta cream hair conditioner
Placenta revitalizing shampoo
Perm Repair with placenta
Proline Perm Repair with placenta
Hormone hair food Jojoba oil
Triple action super grow
Luster's Sur Glo Hormone
B & B Super Gro
Lekair natural Super Glo
Lekair Hormone hair treatment with Vitamin E
Isoplus Hormone hair treatment wit Quinine
Fermodyl with Placenta hair conditioner
Supreme Vita-Gro with allantoin and estrogen plus TEA-COCO
Hask Placenta Hair conditioner
Nu Skin body smoother and
Nu Skin Enhancer
associated with an increased risk for breast cancer later in life
LH & FSH
60% of premature thelarche are identified between 6 and 24 months
normal growth velocity
minimal breast development progression
10% of girls eventually develop true central precocious puberty needing treatment
Central precocious puberty generally treated with needed surgery and/or GnRH agonist. When treatment is stopped, puberty usually resumes and progresses at normal rate.
Non-pituitary related cases are managed by removal of agonist or tincture of time.
Be aware that children are not cognitively or emotionally prepared for pubertal development.
Young girls are more likely to be depressed, moody aggressive, or socially withdrawn.
Assist the child as much as possible in dealing with social stigma associated with condition
Older girls are more likely to participate in high risk behaviors
Open discussion and education regarding sexual decisions
Assist family as needed for sexual discussions with child
Premature pubertal growth and accelerated bone maturation often leads to reduced adult height
Although initially taller than their peers, children usually finalize at a normal height, if appropriately treated.
Thyroid hormones influence
growth and development
metabolism of lipids, carbs, and proteins
Most common cause (in U.S.) is Hashimoto - autoimmune destruction of the thyroid gland
Deficiency of TSH secondary to pituitary disease
Dysfunction of the hypothalamus
of all states newborn screening
increased weight for height
drop in school performance
T4 T3 TSH
T4 T3 TSH
Synthetic thyroid hormone levothyroxine (Synthroid) needed by most for life. Some congenital cases do not need medication for life. Some teens with idiopathic thyroiditis have spontaneous remission.
Congenital cases diagnosed and treated before 3 months have best change at optimal mental development.
Frequent monitoring of lab values and medication adjustment is needed for optimal growth and development.
Most common cause of hyperthyroidism
Greatest incidence in girls 12-14 years
Autoimmune disorder that causes thyroid stimulating immunoglobulin to bind to TSH receptors causing increased thyroid hormone production.
Maternal antibodies (persist for about 3 months) can cause Neonatal Graves Disease
increased appetite with weight loss
goiter (almost 100%)
wide pulse pressure
underweight for height
eyelid lag or exophthalmus
nodules in the thyroid
warm, smooth skin
tremor or hyperreflexia
Soy-based products interfere with Synthroid absorption. Higher dosing and close monitoring for infants on soy formula may be needed.
Often multiple medications (managed by endocrinology)
Radiation Therapy (if medication is ineffective
Nutritional needs may be greater until hyperactive thyroid is controlled. Then prevention of over-eating.
May have significant school impact due to concentration problems, easy fatigue, and increased body temperature.
May have poor self-image secondary to exophthalmus. Eye protection may be needed. Sleep with head of bed elevated to prevent optic nerve damage.
Insomnia and emotional lability may be significant problems.
Thyrotoxicosis (thyroid crisis or storm) - medical emergency
TH levels extremely high
Can be life threatening
muscle weakness, tremor, diarrhea, sweating, palpitations, cardiac failure, and coma
Growth Hormone Deficiency
Caused by decreased activity of the pituitary gland (source may be hypothalmus)
Infarction of the pituitary gland (sickle cell disease can do this)
Pituitary gland, hypothalamus tumors, and other brain tumors
Genetic syndrome such as Prader-Willi, Turner syndrome, and Down syndrome
Chronic renal failure/Cushing syndrome
Other significant disorder (e.g. cardiac or GI)
late pubertal growth spurt caused by delayed pubertal hormone secretion
Constitutional Growth Delay
Disorder of girls in which one X chromosome is present instead of two.
Normal birth weights and lengths
Delays sometimes seen by 1 year with continued slow growth
Infants may present with hypoglycemic seizures, hyponatremia, neonatal jaundice, pale optic discs, micropenis,and undescended testes
Children appear "cherubic" with youthful facial features
higher pitched voices
decreased muscle mass and skeletal maturation
delayed sexual maturation
Associated with slipped capital femoral epiphysis
Always consider familial short stature!
GH replacement therapy
Continued until child achieves acceptable height or growth velocity drops to less than 1 inch per year
Onset of puberty may be delayed with gonadotropin-releasing hormone analogs
Partner with child, parents, and schools to identify any academic problems.
Encourage parents to treat child in age-appropriate manner.
Suggest sports and activities that are not size dependent.
Help child to develop positive coping mechanisms for problems such as teasing and inappropriate treatment by adults.
Promote positive self-image.
Support for child and family.
The Magic Foundation. http://www.magicfoundation.org/www
Incidence is 1:2500 girls
Absence of ovarian function
lymphedema of hands and feet as newborns
cubitus valgus (increased angle at elbow)
congenital heart disease
urinary tract anomalies
lack of breast development
high risk for learning disabilities but usually normal intelligence
Treatment consists of growth hormone therapy, low-dose estrogen, and progesterone
Sexual development concerns
Being treated according to age and not physical development
Type 1 and Type 2 in Young People
Only 20% of Type 1 have a close relative with Type 1.
Incidence in monozygotic twins is 30%-50%.
Incidence in first-degree family members is 4%-7%.
Numbers are fast growing for Type 1 AND Type 2
In 2011, about 1 in 400 children
Healthcare costs for diabetics is more than twice that for others.
Unlike adults, 60%-80% of diabetics in children are Type 1- statistic is changing!
Data for Type 2 is still not good. New CDC SEARCH study begun in 2004 should give us better data.
A1C > 6.5%
FPG >= 126
OGTT with 2 hour PG >=200
Random PG >=200 and classic hyperglycemic symptoms
Nonalcoholic fatty liver
Complications slower to develop in Type 1.
Follow a more rapid path in Type 2 than Type 1 – hyperinsulinemia and obesity are both risk factors for cardiovascular disease
Complications have been found in early twenties.
Must be managed well for readiness to learn and avoidance of absences
Hypoglycemia is more of a threat
Age of onset of type 1 and history of severe hypoglycemia correlated with decreased memory capability
Normal glucose restoration does not mean normal thinking – may take a little longer
Impact on Learning
“At least once a day, I’ll feel bad, from either a low or a high. What really kills me is working with these four-year-olds at …camp, and knowing they’re going to feel like shit once a day for the rest of their lives.” quote from Diabetes Rising by Dan Hurley
Eating disorders common
Joslin researchers found 30% of young women had practiced “diabulimia” – triples risk of death over an 11-year period
Much greater problem with self-esteem and depression – especially girls
The poorer the glucose control is, the more likely the depression
More likely to have risk-taking behaviors
Depression / Psychosocial Complications
New exogenous insulin causes beta cells to start again
Insulin needs decrease
Some never have one
Some last for months (maybe even years)
Can be difficult to regulate glucose and insulin
Often only need 2 shots a day of intermediate acting
May have polyphagia
Initial ketoacidosis in 30-40%
Short duration of symptoms
Obese, little or no weight loss, or significant weight loss
Long duration of symptoms
Polyuria, polydipsia (mild or absent)
Glycosuria without ketonuria in 33% initial cases
Androgen-mediated problems (acne, hirsutism, menstrual disturbances, POS)
Excessive weight gain and fatigue secondary to insulin resistance
Continuous Glucose Monitoring
(cc) photo by medhead on Flickr
control and poor body image due to attached technology
< 6 yrs: 7.5% < A1c < 8.5%
6-12 yrs: < 8%
>12 yrs: <7.5%
Helping child and families with Type 2 diabetes requires intensive nutrition and lifestyle modifications. There are few studies showing successful approach.
Monitor growth closely.
Tight insulin control is associated
with increased risk for being overweight.
May be delays in puberty and menarche. Loss of menses may indicate poor metabolic control.
In Type 2, obesity is associated with early puberty and fast growth.
Insure family understands how to prepare for, be alert for, and manage hypoglycemic episodes.
Routine immunizations, especially flu vaccine.
Increased risk for dental carries
Annual eye exams including retinal exams.
Celiac disease and Hashimoto's thyroiditis (other autoimmune disorders) are frequent comorbidities in Type 1 diabetics.
Be aware that illness can significantly raise glucose.
Endocrinologists generally give "sick day" guidelines for monitoring and dosing.
Hydration is very important.
Be sure that annual lipid profiles and urine screens are done.
Diabetes can have major affect on academic performance - frequent absences, glucose swings at school.
Work with family and school in development of 504 plan.
Birth control is very important!!
American Diabetes Association www.diabetes.org
Impacts growth and development, leading to fast cognitive impairment and even death
Treated children bounce back quickly and can lead normal lives
Problems develop quickly in children
uterine contraction & milk letdown
temperature, thirst & hunger
Regulates glucocorticoids & androgens
Stimulates estrogen, ovarian follicle maturation, sperm production
stimulates androgens and progesterone
metabolic process of growth
The MASTER Gland
stimulates thyroid (metabolism and growth)
releasing and inhibiting hormones that regulate many endocrine glands (thyroid, adrenal, gonads)
Some glands work with nervous system for hormone regulation
islets of Langerhans and parathyroid
Cellular level of hormone secretion
pineal gland, some GI tract epithelial cells, thymus
Target organ (gland)
Most growth hormone is released during sleep
1 in 3500 children
What you talkin' 'bout?
CT or MRI
Pituitary function testing
Nursing Management Goals:
enhancing child's self-esteem
Syndrome of Inappropriate ADH
Central or neurogenic - treatment but no cure
inadequate production or secretion of ADH
Brain tumors and treatment; brain trauma; CNS infection
inability of the renal collecting tubules to respond to ADH
Sometimes a transient form caused by drug toxicity or adverse drug reaction
Rare in children
Brain tumors and trauma
Side effects of some meds and chemo
Tubules do not reabsorb water
Irritable if fluids withheld
Desmopressin acetate (DDAVP)
I&O in infants
keep fluids available
monitor sodium intake
medic alert id
communication with school
acute illness warrant evaluation for sudden hypernatremia onset
"low and wet"
Suppression of renin-angiotension mechanism and sodium excretion
distended jugular veins
lung field crackles
non-edematous weight gain
decreased and concentrated UO
lethargy, confusion, headache
Altered level consciousness
Seizure and coma
ADH blocker (demeclocycline
Lifelong med may be needed
Most cases are idiopathic,
not an endocrine
Most common cause of preventable mental retardation.
Congenital hypothyroidism usually unexpected - family support important
Daily at same time
Signs of too high or low dose (tachycardia or fatigue)
Relatively rare in children
Parents bring their 1 week old to the clinic. Her newborn screening test was positive for hypothroidism. Her parents are shocked and upset by the news. Her mother states, "My daughter's been doing so well since she came home from the hospital. She seems to be doing everything she should be. she's a great sleeper. I just can't believe anything's wrong with her."
1. How would you address the parents' concerns?
2. What teaching will be appropriate for this family?
Family education regarding the importance of medications and emergency kit
Monitor blood pressure carefully - indicator of efficacy and usage of medications
Sleep patterns should not differ, but unusual fatigue may indicate a need for cortisol replacement
Do not assume sex of baby, wait for genetic screen.
Excess cortisol leads to Cushing's (very rare in children)
Deficiency of cortisone and aldosterone
Most cases are autoimmune, may be infectious or trauma source
weakness, lethargy, anorexia, salt craving, poor weight gain or loss, hyperpigmentation, abdominal pain, N&V, diarrhea, hypoglycemia
Stress can bring on adrenal crisis
Estrogen:Testosterone ratio imbalence
Associated with drugs that increase prolactin levels (THC, TCA's, Ca channel blockers
Primary: absence of menarche by 14.5 years with no secondary sex characteristics or age 16 with
reproductive system structural defects; chromosomal abnormalities; pituitary tumors; thyroid dysfunction; POS
Secondary: cessation of menses for 6 months or 3 cycles
May be due to excessive sports, malnutrition
Inborn Errors of Metabolism
Significant problem during infancy
Metabolic error causes harmful by-product to accumulate and cause damage
Most related to some protein intolerance
Newborn screening finds most
Not all are symptomatic immediately
Defect in protein utilization
Leads to severe mental retardation, seizures, and death
Life-long diet low in phenylalanine
Defect in carb metabolism
Rapid organ damage and life-threatening problems
Death within 1 month without treatment
Life-long galactose-free diet
More common error
Involves a breakdown in pathway that converts fat to energy
Can lead to cardiomyopathy, hepatomegaly, muscle weakness, coma, and death
Most diagnosed due to symptoms
Must avoid fasting, some need carnitine supplementation
Rare disorder of amino acid metabolism
Causes cerebral edema, progressive neurological impairment, and death
The build up of amino acids and metabolites cause the body fluids to have the sweet odor of maple syrup
Special formulas and life-long special diet
99% of babies with Turner's miscarry
end up a normal height!