[09.17a] Recognizing Common Pediatric Endocrine Disorders (Part 1) V2

Glands that release hormones directly into the bloodstream

What is the endocrine system composed of?

Chemical signaling

How does the endocrine system communicate with target cells?

Pituitary, Hypothalamus, Thyroid, Parathyroid, Adrenal, Pancreas, Ovaries, and Testes

Name the eight endocrine components mentioned in the introductory diagram.

Neuroendocrine system

What is the collective name for the endocrine and nervous systems due to their close association?

Neural control center

What part of the brain controls endocrine glands through feedback?

Feedback loop

What mechanism works to control hormone release and maintain homeostasis?

Homeostasis

What is the state of steady condition vital for life?

Releasing hormones

What type of hormones does the hypothalamus secrete to stimulate the pituitary?

Anterior pituitary

Which gland secretes stimulating hormones in response to the hypothalamus?

Target gland hormones

What hormones send feedback signals to the hypothalamus and anterior pituitary to increase or decrease secretion?

Too little or too much secretion

What is the fundamental cause of endocrine disorders?

More than 2 standard deviations below the mean

What is the definition of short stature using WHO z-scores?

Less than 3rd percentile

What is the definition of short stature according to CDC population standards?

Short since young, shortest in class, small compared to peers, and younger sibling is taller

List the four clinical recognition signs of short stature.

Nutritional status

What is height measurement used to assess in a child?

Recumbent length

How is height measured for children under 2 years old?

Standing height

How is height measured for children over 2 years old?

Height velocity

What is the rate of increase in height over a specific period of time?

Height T2 minus Height T1 divided by months between measurements times 12

What is the formula for height velocity in cm/yr?

First year

When is normal height velocity usually at its fastest?

Pubertal growth spurt

When does height velocity accelerate again after the childhood slowdown?

18 to 25 cm

What is the normal growth rate in centimeters from birth to 1 year?

10 to 13 cm

What is the normal growth rate in centimeters for children aged 1 to 2 years?

5 to 6 cm

What is the normal growth rate in centimeters from 2 years to puberty?

6 to 11 cm

What is the normal growth rate for girls during the pubertal growth spurt?

7 to 13 cm

What is the normal growth rate for boys during the pubertal growth spurt?

Less than 5 cm per year

What is the general abnormal height velocity threshold from age 2 to puberty?

Less than 5.5 cm per year

What is decreased height velocity for a child aged 2 to 4?

Less than 5 cm per year

What is decreased height velocity for a child aged 4 to 6?

Less than 4 cm per year

What is decreased height velocity for boys from age 6 to puberty?

Less than 4.5 cm per year

What is decreased height velocity for girls from age 6 to puberty?

Parental growth genes

What primarily influences a child's height potential?

13.0 cm

What is the standard sex difference value used in midparental height calculations?

Father height plus (Mother height plus 13) divided by 2

What is the adjusted MPH formula for a boy?

Mother height plus (Father height minus 13) divided by 2

What is the adjusted MPH formula for a girl?

Target height

What is another name for adjusted midparental height?

MPH plus or minus 5.0 cm

How is the target height range calculated?

10th to 90th percentile

The target range of +/- 5 cm corresponds to which percentile range?

Pathologic condition

What does a projected height differing from MPH by more than 10 cm suggest?

X-ray of the left hand and wrist

How is bone age determined?

Skeletal maturity

What does bone age specifically reflect?

Sexual maturity rating or puberty onset

With what does bone age correlate closely?

Epiphyseal growth plates

What structures in the hand x-ray are checked to see if growth is still possible?

Atlas of Greulich and Pyle

What reference is used to compare a child's x-ray to normal standards?

80 percent

What percentage of short stature cases are normal variants?

Familial Short Stature and Constitutional Growth Delay

Name the two normal variant causes of short stature.

20 percent

What percentage of short stature cases are pathologic?

Disproportionate and Proportionate

What are the two categories of pathologic short stature?

Prenatal and Postnatal

Proportionate short stature is divided into which two types based on onset?

Bone age

What is the primary differentiator between FSS and CGD?

Bone age equals chronologic age

What is the bone age relationship in familial short stature?

Bone age is less than chronologic age

What is the bone age relationship in constitutional growth delay?

Normal

What is the height velocity in familial short stature?

Normal

What is the height velocity in constitutional growth delay?

Short stature

What is the typical family history for familial short stature?

Delayed puberty

What is the typical family history for constitutional growth delay?

Normal

What is the timing of puberty in familial short stature?

Delayed

What is the timing of puberty in constitutional growth delay?

Low but normal for target height

What is the final height in familial short stature?

Normal

What is the final height in constitutional growth delay?

18 months

At what age does the height of an FSS child typically decelerate to pattern after parental genes?

Catch up growth

What allows a child with CGD to eventually reach a normal final height?

Upper segment, Lower segment, and Arm span

What three measurements establish body proportions?

Axial skeleton

What does the upper segment measurement reflect?

Limb growth

What do the lower segment and arm span reflect?

Disorders affecting long bones

Which disproportionate category has a decreased arm span and increased US/LS ratio?

Disorders affecting the spine

Which disproportionate category has a normal arm span and decreased US/LS ratio?

Short limb dwarf

What is another term for a child with achondroplasia?

Short trunk dwarf

What is another term for a child with spondyloepiphyseal dysplasia?

Achondroplasia

Which condition shows an increased upper to lower segment ratio and affects long bones?

Vitamin D resistant Rickets

Which condition presents with bowing knees, alopecia, and flaring of the wrist and ankle?

Osteogenesis Imperfecta

Which condition is characterized by visible bone deformities?

Pseudohypoparathyroidism

Which condition presents with short 4th fingers or toes and increased US/LS ratio?

Leri-Weill Dyschondrodysplasia

Which condition presents with short upper and lower extremities?

Genetic defect in the parathyroid hormone receptor

What causes Jansen metaphyseal chondrodysplasia?

Turner Syndrome

Which chromosomal disorder causes proportionate short stature with webbed neck and low-set ears?

Noonan Syndrome

Which syndrome looks like Turner syndrome but has normal chromosomal analysis?

Webbed neck, low hairline, and wide spaced nipples

Name three physical signs shared by Turner and Noonan syndromes.

Russell Silver

Which syndrome is characterized by triangular facies and failure to thrive?

Prader-Willi Syndrome

Which syndrome involves being floppy and difficult to feed as an infant?

Voracious eaters

What describes the eating habits of Prader-Willi children after 1 year of age?

Almond shaped eyes, hypogonadism, mental retardation, and short stature

List four features of Prader-Willi syndrome.

Chronic systemic disease, Endocrine disorder, and Psycho-social dwarfism

Name the three categories of postnatal proportionate short stature.

Growth hormone deficiency, Hypothyroidism, and Cushing syndrome

Name three endocrine causes of postnatal short stature.

Lack of care

What is the cause of psycho-social dwarfism?

Change in environment

What can reverse psycho-social dwarfism?

Truncal obesity, buffalo hump, and moon facies

Name three signs of Cushing syndrome.

Congenital Hypothyroidism

Which condition, if untreated, leads to short stature and can become disproportionate?

Empty Sella Syndrome

What condition involves an MRI finding of an empty pituitary area and causes GHD?

Simple Virilizing CAH

Which condition in an untreated girl leads to masculine features and short stature?

Testosterone

Which hormone is in excess in simple virilizing CAH?

Renal Osteodystrophy

Which pathologic condition is labeled F in the endocrine disorder photos?

Below 5 cm per year

What is the threshold for abnormally slow growth rate?

51.0 cm

What is the normal mean length at birth after 9 months in utero?

25.0 cm per year

What is the normal mean height velocity during infancy?

12.5 cm per year

What is the normal mean height velocity for a toddler?

6.3 cm per year

What is the normal mean height velocity during childhood?

9.4 cm per year

What is the normal mean height velocity for males in puberty?

8.3 cm per year

What is the normal mean height velocity for females in puberty?

18 months

At what age is downward crossing of percentile channels considered abnormal?