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Endocrine > Pituitary- pediatrics > Flashcards

Pituitary- pediatrics Flashcards

1
Q

Rathkes pouch

A

Part of pituitary formed from mouth epithelium

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2
Q

Hypothalamus blood supply

A

Superior hypophysis L artery

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3
Q

Anterior pituitary blood supply

A

Long portal vein from hypothalamus (tearing)

Short portal vein (from posterior pituitary)

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4
Q

What are the investigatory results of growth hormone deficiency?

A

Delayed bone age
Low IGF-1
Abnormal response to GH stimulation test

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5
Q

What tests show central thyroid issues?

A

Low free t3/4

With abnormally normal TSH

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6
Q

Symptoms of low cortisol?

A

Fatigue
Vomiting
Hypoglycemia
Dilutional hyponatremia

Can be life threatening

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7
Q

Septo-optic dysplasia

A

Cause of hypopituitarism

Two of three: optic nerve hypoplasia, cerebral dysgenesis, hypopituitarism

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8
Q

Prader Willi

A

Cause of hypopituitarism
SX: hyperphagia, hypotonia, hypogonadism, hypomentation, obesity
Initially failure to feed/thrive, later reverses because of lack of satiety

Cause: genomic imprinting

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9
Q

Kallman syndrome

A

Associated with gonadotropin deficiency

Cause: x linked KAL mutation

SX: anosmia (no smell), microphallus,

IX: MRI shows changes to olfactory bulb

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10
Q

Laurence moon Bardet beidl

A

Rental degradation
Extra digit

Cause of gonadotropin deficiency

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11
Q

Acquired causes of hypopituitarism

A

Radiation to head (leukemia and brain tumours)
Trauma
Tumours

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12
Q

Craniopharyngioma

A

Benign pituitary tumor but malignant by position

SX: hydrocephalus, visual field defects, anterior or posterior pituitary defects

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13
Q

Symptoms of hypopituitarism

A
  1. Hormonal deficits
  2. Space lesion effects:
    - headaches
    - visual field changes
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14
Q

How are height targets estimated for children?

A

By calculating mid parental heights

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15
Q

Causes of normal variant short stature

A
  1. Familial: normal growth velocity/bone age/puberty but
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16
Q

Causes of disproportionate short stature?

A

Skeletal dysplasia:

achondroplasia: short proximal limbs
hypochondroplasia: milder version

Spinal radiation
Rickets

17
Q

Prenatal causes of short stature?

A

IUGR (low birth weight)

can be EARLY (end up short, ie. down's)
or LATE (low birthweight but usually normal as adults)

Chromosomal Disorder:
ie. Turner’s Syndrome (screen in short girls)

18
Q

Postnatal (non-endocrine) causes of short stature?

A

Malnutrition/chronic disease: ie Celiac’s
Drugs/radiation: ie steroids
Psychosocial: neglect/abuse

19
Q

Post-natal endocrine causes of short stature?

A
  1. Hypothyroid
  2. Cushing’s Syndrome (exogenous source common)
  3. GH deficiency
  4. IGF-1 deficiency (Laron dwarfism)
  5. IGF-1 resistance
20
Q

Testing for short stature

A
  1. Growth charts
  2. Midparental height
  3. CBC & differential, lytes, Cr (rule out dz)
  4. Celiac screen
  5. Thyroid screen
  6. Bone age (wrist xray)

Maybe:
karyotype
serum IGF-1
GH testing

21
Q

Microphallus

A

Sign of low gonadotropin or combined hypopituitarism

22
Q

Do this before giving thyroxine

A

Assess for ACTH deficiency, because if this is present thyroxine can be very dangerous

23
Q

Sydromic causes of tall stature

A

Klinefelter

Marian

24
Q

Consider this cause in short girls

A

Turners syndrome

25
Q

Indications for growth hormone treatment

A 
GH deficiency
chronic renal failure
turner syndrome
IUGR
prader-willi
normal variant short stature
adult GH deficiency
26
Q

Ddx tall stature

A

Non-Pathologic: familial, constitutional
Obesity
Syndromes: Klinefelter, Marfan’s
GH excess

27
Q

Genetic causes of hypopituitarism

A

PROP-1
PIT-1
HESX-1

28
Q

Profile of Turner’s Syndrome

A

Females with short stature (X0)
Wide nipples, small fingernails, aortic constriction, elbow deformity, MSK/KIDNEY/HEART MALFORMATIONS
Visual spatial leaning disability
Primary ovarian failure: delayed puberty

COMPLICATIONS: HEART & T2DM

29
Q

Klinefelter Syndrome

A

Males (XXY) with tall stature
Feminine characteristics with hair & body
Cognitive impairment
Small hard testicles

Endocrine (12 decks)

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