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Endocrine > Pediatric Endocrinology > Flashcards

Pediatric Endocrinology Flashcards

1
Q

Growth hormone Deficiency in children

A

Usually begins in children between 5-8 yrs

  • child is normal initially but then falls off the growth curve
  • children have no other medical conditions usually and is normal on examination other than weight and height
  • children’s parents will both be taller than average usually and they have no reported developmental delays
  • growth rate is delayed**

**bone ages are delayed

Screen tests:

  • CBC, ESR, electrolytes
  • IGF-1/3 levels, somatomedin C and GH levels
  • bone age radiographs
  • *if a female = get karotype to look for Turner syndrome if present**

Replacement therapy = recombinant GH injections several times per week (need to go to endocrinologist)

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2
Q

Calculation for a rough mid-parental height

A
  • *calculations correct for theopposite gender parents height**
  • gives a general estimate for how tall the child should be

Boys [paternal height + (maternal height + 5 inches)]/2 (+/- 2 inches)

Girls [maternal height + (paternal height - 5 inches)]/2 (+/- 2 inches)

if they are way off then check for potential GH deficiency

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3
Q

Bone age definition

A

Compare the left wrist radiographs in children older than 2 yrs (knee if younger than 2 yrs) to published “normals”
- this determines how old the bones appear compared to chronological age and thus provides an estimate of remaining growth potential of bones

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4
Q

Constitutional growth delay

A

A condition in which the child’s growth is slower than expected but one or more parents also demonstrated a pubertal developmental delay
- bone age = height age

There is no history or examination of abnormalities
- the growth rate is NORMAL**

Family history is usually positive for at least one “late bloomer” who developed at normal adult height eventually

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5
Q

Familial short statue definition

A

A short child who is born to short parents who had normal timing of their pubertal development

Kids grow around 3rd-5th percentile at all time, but nothing is technically wrong with them
- bone age = chronological age which means no extra growth potential so no GH replacement fixes this

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6
Q

Growth velocity definition

A

Increase in measured height or length over time as compared to standardized growth curves

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7
Q

Height age definition

A

The age at which a child’s measured height is at the 50th percentile

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8
Q

Idiopathic short stature

A

Condition in which short stature diagnosis cant be reaches

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9
Q

How much growth does someone typically see each year?

A

1st year of life = 9-11 inches

1-3 yr = 3-5 inch per year

3-puberty = 2-2.75 inch per year

Puberty = 4 inch for guys and 3 inch for girls per year

only time its bad when a kid”falls off the curve” (VERY common in early onset celiac disease also)

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10
Q

Precocious puberty

A

Excessive growth hormone which produces onset of secondary sexual development before 8 years in girls and 9 years in boys

Can be true = central cause usually pituitary adenomas or CNS lesions
- must rule this out in girls younger than 6-8 yrs and any boy under 9 yrs

Can be pseudopuberty = non-central causes
- gonadotropin independent (usually OCP’s, estrogen/testosterone cream or adrenal/ovarian tumors

Very rare = incomplete
- you get some puberty symptoms but some not

Signs are:

  • girls = breast, pubic hair and tall stature for age
  • boys = very tall for age and get acne and deep voice quicker
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11
Q

Racial variation of puberty

A

African Americans = hit puberty earlier

Caucasian’s and Asians = hit puberty later

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12
Q

Delayed puberty definition

A

No signs of puberty in

  • girls by the age of 13
  • boys by the age of 14

May be caused by gonadal failure, chromosomal abnormalities, hypopituitarism, chronic disease, malnutrition

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13
Q

Clinical approach to precocious puberty

A

True precocious puberty overall is more common in girls

  • excess secretion of GnRH with normal appearing but early progression of pubertal events
  • growth is accelerated and bone age is advanced

However boys are more likely to have a CNS tumor (still like 25% only)

Idiopathic cases are 90% girls

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14
Q

What is the most urgent symptom for precocious puberty?

A

Vision changes
- can signal mass effect symptoms from somatotropin adenomas

must get an urgent head MRI and initiate prompt referral to pediatric neurosurgeon

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15
Q

Most common Causes of peripheral precocious puberty (no central)

A

Isolated ovarian cysts/tumors

Exposure to exogenous strokes or androgens

McCune-Albright syndrome

Severe untreated hypothyroidism

CAH

  • *abnormal pattern of development of secondary sex characteristics**
  • growth is accelerated and bone age is advanced
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16
Q

Benign premature thelarche and adrenarche

A

Thelarche = breast development without rapid linear growth or other signs of pubertal development observed over a 4-6 month period
- often occurs in girls younger than 2 years

Adrenarche = pubic hair and/or body odor without rapid linear growth

  • clitoromegaly, penile growth testicular enlargement. Is observed before age 8 in girls and 9 in boys
  • often occurs in African American females
17
Q

What is estrogenization of the vaginal mucosa?

A

Becomes dull gray-pink compared to shiny red

18
Q

Laboratory testing for precocious puberty

A

Serum estradiol (elevated in girls

Serum testosterone (elevated in boys)

Serum LH

  • in noncentral = undetectable
  • in central = high

GnRH stimulation test

Bone age radiographs

Consider pelvic UA to check for ovarian tumors/cysts/adrenal tumors

Consider a CT or MRI if central

  • pretty much mandatory in all boys
  • almost mandatory in all girls under 6 years
19
Q

Treatment for precocious puberty

A

goal is to treat this quick enough to prevent premature closure of the epiphyses

GnRH agonists are used for central as first line and often is a good choice for noncentral also

  • purpose is to desensitize the gonadotrophic cells in the pituitary to GnRH
  • need a pediatric endocrinologist to sign off though
GnRH agonist examples 
- Leuprorelin 
- Goserelin 
- Buserelin 
“Erelin’s”
20
Q

Congenital hypothyroidism

A

Abnormal formation of the thyroid gland (thyroid dysgenesis is the majority of the causes)

Most are detected at within 1st few weeks of birth via screening programs (mandated in all 50 states)

  • often asymptomatic at the time but if they are symptomatic
  • if severely affected though will manifest symptoms within 1st week of life

Occurs in 1/4000 or 1/2000 births (variable)

21
Q

Congenital adrenal hyperplasia

A

Most common cause of ambiguous genitalia

Autosomal recessive disorder that the majority shows 21-hydroxylase deficiency

  • causes NO HTN
  • causes VIRULIZATION
  • also causes electrolyte abnormalities which can be problematic (no aldosterone)

Need to get a karyotype and serum electrolyte levels
- also get serum 17-hydroxyprogesterone levels (this will be high)

Three subtypes:

1) salt wasting (classical form)
- also shows deficiency in aldosterone and is measured by low sodium and high potassium levels

2) simple virilizing form
- mild Deficiency of aldosterone but not enough to show electrolyte abnormalities

3) non classical form
- no ambiguous genitalia and no salt wasting, but there is still cortisol deficiency
- shows late onset premature pubarche and advanced bone age
- females show hirsutism

Girls with CAH are always fertile and therefore must be assigned female sex at birth

need to have an endocrinologist, geneticist, urologist and psychiatrist to help treat

22
Q

True hermaphodism

A

About 70% are 46,XX (30% are 46, XY)
- however the majority of these are assigned male gender at birth (75%)

Comprise less than 10% of all intersex cases
- 75% are classified as male at birth because the external genitalia is virilized

Shows:

  • bilateral ovotestes (both ovaries and testies)
  • testicular tissue that varies in degree of Virilization
23
Q

Mixed gonadal dysgenesis

A

Most are 46,XY/45,XO karyotype

  • produces testies with sertoli and leydig cells but there is no germinal elements and streak gonads are present (aplastic gonads)
  • also produces hypospadias and partial labioscrotal fusion with undescended testes

25% of streak gonads develop malignancy

24
Q

Female and male pseudohermaphroditism

A

Female = Produces a 46,XX karyotype and is the largest neonatal group with ambiguous genitalia**
- predominant etiology is CAH

This will show male external genitalia and virulization but ovaries inside and female internal genitalia

Male = produces 46,XY

  • predominant etiology is androgen insensitivity syndrome**
  • produces female external characteristics with functional testicular tissue and have undescended testes
  • diagnosed with primary amenorrhea occurs
25
Q

What is the serum marker for CAH?

A

Elevated 17-hydroxyprogesterone

26
Q

What imaging is needed for androgen and sex disorders?

A

Pelvic US and pelvic MRI are usually first line
- can give urogential sinus xray with injection if needed

Laparoscopy is usually not needed

27
Q

Treatment for ambiguous genitalia

A

First thing needed is to get functionally normal external genitalia by surgery and/or hormones
- emphasis on cosmetic appearance since this can reinforce doubt about sexual identity as early as 6 months

Then work up internal and actual disorder and treat that

28
Q

Gender assignment

A
  • *in cases in which fertility is not found, depends upon**
  • appearance of external genitalia
  • potential of unambiguous appearance
  • potential for normal sexual functioning

if fertility is determined, then its based on that

29
Q

Should you do circumsicion on hypospadias patients?

A

NEVER

30
Q

Treatment for CAH

A

Monitor glucose and electrolytes

- give hydrocortisone 10-20 mg/M2 divided 3 doses daily for pretty much life

31
Q

What are the required physical examination parts to conduct in a patient with precocious puberty

A

Growth velocity

Skin (examine for cafe-au-lait spots, oiliness and acne)

Presence of axillary hair, body odor and amount of breast tissue

Characteristic of pubic hair via tanner staging

Abdomen for palpable masses

Enlargement of penicillin and thinning of scrotum

Testicle sizes
- if unilateral enlargement = get testicular transillumination to check for hydrocele vs cyst

Neurological exam and potential MRI of th Brian

32
Q

What are the cut off levels for congenital hypothyroidism screening on infants?

A

Get a heel stick test on the 2nd or 3rd day of life

TSH normal measurement = <15

TSH 15-29 = measure T4 levels
- if T4 is low = immediate referral to pediatric endocrinologist

TSH 30 or greater = immediate referrals

Endocrine (46 decks)

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