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Pediatric Board Review Endocrine, Part 4

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Title: Pediatric Board Review Endocrine, Part 4


1
Pediatric Board ReviewEndocrine, Part 4
  • Anne-Marie Kaulfers, MD
  • Pediatric Endocrinology
  • University of South Alabama
  • August 25, 2011

2
Overview of calcium metabolism
Gut 1,25 OH Vit D Pulls Ca and
Phos from food into blood
3
Overview of calcium metabolism
Gut 1,25 OH Vit D Pulls Ca and
Phos from food into blood
4
Recognize typical lab findings of
hypoparathyroidism
  • Parathyroid hormone (PTH)
  • Wants to keep blood calcium levels normal (PTH
    couldnt care less about the bones)
  • Tells kidney to hold onto calcium and pee out
    Phos
  • Tells kidney to make activated Vit D (1,25-OH Vit
    D), which goes to the gut and gets the calcium
    from your food and puts it into your blood
  • Lab findings
  • Low PTH, Low Calcium, HIGH Phos

5
Signs and Symptoms of low Ca
  • Chvosteks sign
  • Tap on cheekbone, and the upper lip will twitch
  • Trousseaus sign
  • Hand spasm with prolonged BP cuff inflation
  • Perioral tingling
  • Seizures/Psychiatric changes
  • Papilledema, cataracts, tetany, laryngospasm
  • Prolonged QT interval on EKG
  • Low calcium with low phos vitamin D deficiency

6
DiGeorge syndrome and low Ca
  • Microdeletion of 22q11.2, detected on FISH
  • CATCH-22
  • Cardiac abnormalities (aortic arch, etc)
  • Abnormal facies (small jaw, funky ears)
  • Thymic aplasia (T-cell related infections)
  • Cleft palate
  • Hypocalcemia (no parathyroid gland, so have low
    Ca, HIGH Phos, low PTH)

7
Recognize a child with Pseudohypoparathyroidism
  • Resistance of kidney to PTH
  • (You can make PTH but you cant use it)
  • Low Ca, HIGH Phos, but also HIGH PTH
  • Type 1A is the most common
  • You inherit this defect from mom
  • Also have resistance to other hormones
  • High TSH, ACTH, LH, and FSH

8
Recognize a child with Pseudohypoparathyroidism
  • Wont have low calcium until after age 3 years
  • Seizures in elementary school
  • Subcutaneous calcifications
  • Will look fine until after age 1 year, then will
    develop Short stature, obesity, round face, bad
    teeth, short fingers and toes, joint problems
    bowleg, can have MR
  • Albright Hereditary Osteodystrophy

9
Causes of low Ca in neonate
  • In first 2 days of life
  • Preemies
  • Infants with birth asphyxia/sepsis
  • Infant of a diabetic mother
  • Large for gestational age
  • Low blood sugar
  • After first week of life
  • High Phosphate load
  • Low Magnesium
  • Moms hyperparathyroidism turning off neonatal
    PTH secretion (check moms PTH level)
  • Neonatal Hypoparathyroidism

10
X-linked (familial) hypophosphatemic rickets
clinical and lab findings
  • Bow-legged!!!
  • Poor growth, bad teeth
  • Problem the kidney pees out too much phosphorus
    and kidney is never told to make 1,25 OH Vitamin
    D (aka calcitriol)
  • Remember PTH is only sensitive to calcium
  • Labs normal calcium, therefore normal PTH
  • Labs low Phos and high Alk Phos
  • Rickets on Xray

11
X-linked (familial) hypophosphatemic rickets
treatment
  • Phosphorus (Neutra-Phos)
  • (Cholecalciferol/Ergocalciferol/Calcidiol are all
    names of Vitamin D3 or D2, aka 25-OH D)
  • 1,25 OH Vitamin D (aka calcitriol)
  • This goes to the gut and takes the calcium and
    Phos from your food and puts it into your blood
  • Routine monitoring
  • Renal U/S looking for stones (from too much Ca)
  • Serum Alk Phos, goal is to normalize levels

12
General Causes of Rickets
25-OH Vit D 1,25-OH Vit D Calcium Phos Alk Phos PTH
Vitamin D Deficiency (nutritional) ? ?
Vitamin D dependent rickets Type 1, 1 alpha - hydroxylase def (cant make 1,25 OH Vit D) ? ?
Vitamin D dependent rickets type 2, aka Vitamin D resistant rickets, aka (resistant to 1,25-OH Vit D) ? ?
X-linked (famlial) hypo-phosphatemic rickets ? Nl
Renal disease ? ?
13
General Causes of Rickets
25-OH Vit D 1,25-OH Vit D Calcium Phos Alk Phos PTH
Vitamin D Deficiency (nutritional) ? NL/?/? ? ? ? ?
Vitamin D dependent rickets Type 1, 1 alpha - hydroxylase def (cant make 1,25 OH Vit D) ? ? ? ? ? ?
Vitamin D dependent rickets type 2, aka Vitamin D resistant rickets, aka (resistant to 1,25-OH Vit D) NL ? ? ? ? ?
X-linked (famlial) hypo-phosphatemic rickets NL NL NL ? ? NL
Renal disease NL ? ? ? ? ?
14
General Causes of Rickets
  • Vit D Deficiency (nutritional) 25OH D is your
    bodys stores of Vit D. Your liver gives it to
    the kidney and it gets activated, then it goes to
    the gut and takes the calcium and phos out of
    your food and gives it to the blood. So without
    25OH, you cant activate very much, so the blood
    levels of Ca and Phos stay low. Because Ca is
    low, PTH goes up.

15
General Causes of Rickets
  • Vitamin D Dependent Rickets Type 1, or 1 alpha
    hydroxylase deficiency You cant make 1,25 OH
    D, but your bones are sick and hurting so it
    keeps trying. It stores all the 25OH D that it
    can, but none of it can get activated, so the 25
    OH D levels are high, and 1,25OH D levels are
    low. You have no activated D to get the Ca and
    Phos out of your gut and into your blood, so the
    Ca and Phos levels are low. Because Ca is low,
    PTH goes up.

16
General Causes of Rickets
  • Vitamin D dependent rickets type 2, aka Vitamin D
    resistant rickets, aka (resistant to 1,25-OH Vit
    D) These kids have alopecia areata, weird.
    Anyway, you can store 25-OH D fine, and your
    kidneys can activate it into 1,25 OH D, but your
    gut wont recognize it. So you get high levels
    of 1,25 OH D, but the Ca and Phos cant get out
    of your gut and into your blood, so the blood
    levels of Ca and Phos are low. Because Ca is
    low, PTH goes up.

17
Signs and Symptoms of Hypercalcemia
  • Bones, groans, and stones
  • Weakness, fatigue, decreased appetite, nausea,
    vomiting, constipation (abd pain)
  • Polyuria and polydipsia
  • Prolonged can lead to kidney stones, arms and
    legs and back pain
  • Can happen after prolonged immobilization, too
    much vitamin A or D, gramulomatous dz

18
Hypoglycemia in a Young child
  • Normal healthy 2-8 year old child
  • Has symptomatic low blood sugar
  • Responds easily to IV or oral dextrose
  • Diagnosis Idiopathic ketotic hypoglycemia
  • Test Check urine for ketones

19
Hypoglycemia in a young child
  • Most common cause ketotic hypoglycemia
  • Other causes/tests
  • High insulin but low C-peptide got an insulin
    shot
  • High insulin and high C-peptide has an insulin
    tumor or ate grandmas sulfonylurea medicine
  • (Insulin turns off ketones, so a positive urine
    test for ketones means that insulin levels are
    not too high)
  • Proinsulin Tells you nothing
  • Cortisol or growth hormone deficiency

20
Natural History of type 1 Diabetes
  • At diagnosis, still have 20 of beta cells
  • But they are sick (glucose toxicity prevents them
    making insulin)
  • After a few weeks, these 20 wake up
  • All blood sugars look stable for next weeks to
    months to a year the Honeymoon period. Diabetes
    seems to be in remission, patient may think
    they are cured.
  • Then these 20 of insulin cells finally die off
    and blood sugars start to get really high, really
    fast

21
Clinical Example
  • At diagnosis A1c is 12, all BG in hospital are
    200s to 500s
  • 3 months later All BG are between 60 and 90
    mg/dl, even when they drink coke or juice or eat
    candy or skip shots, A1c is under 6, patient
    only needs small amount of insulin
  • 6 months later A1c is 12, all BG are 200s to
    500s honeymoon is over. Increase the insulin
    doses to normal. STOP DRINKING SWEET TEA

22
Type 1 diabetesScreening guidelines
  • Hypothyroidism (Chronis Lymphocytic Thyroiditis,
    or Hashimotos disease)
  • TSH every 2-3 years after diagnosis
  • Weight gain and fatigue
  • 10 of all kids with type 1 diabetes
  • Celiac Disease
  • TTG antibody at least once after diagnosis
  • Weight loss, poor growth, big liver
  • 5 of all kids with type 1 diabetes

23
Type 1 diabetes mellitusLong Term complications
  • Hemoglobin A1c average BG over 3 months
  • Microvascular Retinopathy and Nephropathy
  • Neuropathy Peripheral and autonomic
  • Macrovascular CAD, stroke, PVD
  • How to prevent these
  • Good glycemic control
  • Blood pressure control
  • No smoking

24
Basal/Bolus Insulin Dosing
  • Basal insulin needs when you arent eating
  • Lantus (Glargine)
  • Bolus insulin to cover carbohydrates
  • Humalog (Lispro), Novolog (Aspart)
  • Half the daily dose is basal, half is bolus
  • Insulin pump uses only Lispro or Aspart
  • Never use Regular insulin in kids
  • Except in insulin gtts in the PICU

25
Sick Day Management
  • ALWAYS NEED INSULIN. Always give insulin.
  • Dont hold the insulin!!
  • When ill, you need energy and electrolytes
    (carbs). Need to give insulin for the carbs.
  • If pump or pump site malfunctions, need to give a
    SQ injection of lispro/aspart
  • If patient is on Glargine and is vomiting but can
    sip on Sprite give insulin for the Sprite

26
Sick day Management
  • Glargine
  • Lantus
  • lasts 24 hours
  • Slow release
  • Never give it more than once every 24 hours.
  • Ok to give even with low blood sugars
  • Lispro/Aspart
  • Humalog/Novolog
  • works immediately
  • Dont give if blood sugar is low
  • Last 3-4 hours
  • Can give this as a bolus every 3-4 hours

Dont give Regular insulin! If one of the
choices on the test asks you to Give regular
insulin, it is the wrong answer!
27
Type 1 Diabetes DKA
  • Electrolyte imbalance before treatment
  • High blood glucose
  • High potassium
  • High Phos
  • Low Bicarb (CO2)
  • Electrolyte changes after treatment with IVF and
    insulin gtt
  • Low blood glucose
  • Low potassium
  • Low Phos
  • Higher bicarb

28
Type 1 diabetes Cerebral edemaHow to NOT Kill
a Diabetic
Brain
Blood vessel
Correct treatment for DKA slow rehydration with
½ NS or NS over 24-48 hours
29
Type 1 diabetes DKA
Brain
Blood vessel
30
Type 1 diabetes DKA
Brain
Blood vessel
31
Type 1 diabetes DKA
Brain
Blood vessel
32
Type 1 diabetes DKA
Brain
Blood vessel
33
Type 1 diabetes DKATreatment with a big IVF
bolus
Brain
Blood vessel
34
Type 1 diabetes DKATreatment with a big IVF
bolus
Brain
Blood vessel
35
Type 1 diabetes DKATreatment with lots of
insulin
Brain
Blood vessel
36
Type 1 diabetes DKATreatment with lots of
insulin
Brain
Blood vessel
37
Type 1 diabetes DKA
  • You are taking care of a new-onset diabetic she
    is 18 months old, initial ph was 6.9 (very low).
    She is on IVF and an insulin gtt and is doing
    fine. 6 hours into treatment, mom runs to you
    panicking that she cant wake her up, whats
    going on and what do you do?
  • 1 Decrease the IVF rate and decrease the
    insulin gtt rate.
  • 2 Administer Mannitol

38
Type 1 diabetes DKA
  • DONT
  • EVER
  • GIVE
  • BICARB
  • !!!!!!!!!!

Unless Dr. Vidal tells you to do it, then it is
ok ?
39
Type 1 vs Type 2 diabetes
  • Type 1
  • Positive antibodies
  • GAD Ab
  • Anti-islet cell Ab
  • Insulin autoantibody
  • Insulinoma 2 associated Ab
  • Low C-peptide
  • urine ketones/DKA
  • No acanthosis
  • Type 2
  • NO antibodies
  • C-peptide /-
  • Usually high, can be low
  • Family history /-
  • Certain ethnic origin /-
  • Urine ketones/DKA /-
  • Acanthosis nigricans /-

40
Screening for Type 2 diabetes
  • It is now ok to use HgbA1c to diagnose diabetes,
    on board exams and clinically
  • Insulin and C-peptide can be variable and high
    insulin levels dont mean you have diabetes
  • Screen with fasting plasma glucose every 2 years
    in overwt kids with fam history or certain
    ethnic group or AN
  • If fasting glucose is over 110, do a 2 hr OGTT

41
Treatment for Type 2 diabetes
  • Lifestyle modification Diet and exercise
  • If no success in a few months, start Metformin
  • If BG are over 300 and pt also has ketones or
    presented in DKA, consider starting insulin
  • Dont use any other diabetes drugs they are not
    approved for kids
  • It is normal for kids with type 2 to already have
    CV problems at diagnosis high BP, low HDL, high
    LDL, etc

42
Metabolic Syndrome
  • Obesity, high blood pressure, low HDL, high
    triglycerides, and insulin resistance.
  • Acanthosis Nigricans (AN) can be a sign of
    insulin resistance. It does NOT mean that the
    patient has high blood sugar or diabetes.
  • (PCOS abnormal menses, clinical signs of excess
    androgens(hirsute), and cysts on ovary, only need
    2 of these)

43
Metabolic Syndrome
  • If your patient has obesity, high BP, low HDL,
    high triglycerides, and signs of insulin
    resistance, check for other medical problems
  • Fatty liver check ALT (transaminase)
  • PCOS in females ask about hirsutism, check free
    testosterone and DHEA
  • Look for sleep apnea
  • Look for microalbuminuria

44
Recognize hypopituitarism
  • Most common acquired cause craniopharyngioma
  • Clinically will see growth failure from GH
    deficiency and hypothyroid, but it is often
    missed
  • First signs of cranio visual field defects and
    headaches. Then growth failure is noticed.
    Usually patient will be overweight, and have
    delayed puberty.

45
Pediatric EndocrinologyPotpourri, Board Review
  • Anne-Marie Kaulfers, MD
  • Pediatric Endocrinology
  • March 18, 2010

46
General Growth and Development
  • 1A normal newborn weighs 3 kg. How much will he
    weigh at 3 months of life?
  • Actual Numbers
  • 0-3 mo 2 lb and 3.5 cm/month
  • 3-6 mo 1.25 lb and 2 cm/month
  • 6-9 mo 1 lb and 1.5 cm/month
  • Shortcut
  • A little less than 1 kg and a little more than 2
    cm/month
  • After age 2 years 5-8 cm/year is normal

47
Puberty
  • 2A 14 year old boy has testes that are 8 ml. Is
    this normal, early, or late?
  • Ages
  • 10 years old testes are 5 ml
  • 12 years old testes are 10 ml
  • 14 years old testes are 20 ml

48
Puberty
  • 3A 10 year old boy is Tanner 2. What size are his
    testes?
  • Tricks to remember
  • Multiply by 3
  • Tanner 2 size 6 ml
  • Tanner 3 size 9 ml
  • Tanner 4 size 12 ml
  • Tanner 5 size 15 ml

49
Puberty
  • 4A 16 year old boy is really short, testes are 15
    ml. Has he had a growth spurt yet/will he get
    much taller?
  • Testicular Size
  • Sizes 1-25 ml
  • 4 start of puberty
  • 10 start of growth spurt
  • 20 adult size

50
Puberty Gynecomastia
  • 5A very tall 17 y/o boy has bilateral breast
    tissue and small testes. What genetic syndrome
    does he have?
  • Other features
  • Learning disability
  • Behavior disorder/ADHD
  • 47, XXY
  • Low testosterone, High LH and FSH

51
Delayed puberty
  • 6A 15 y/o boy is seen for delayed puberty. He has
    a normal height and has Tanner 5 body hair, but
    his testes are only 2 ml. He has a history of a
    cleft lip repair, and only has one kidney. He
    gets extra help in school because of mild MR.
    You check labs and his LH, FSH, and testosterone
    levels are all low. What genetic syndrome does
    he have?
  • What if I tell you he also cant smell?

52
Growth
  • 7They show a picture of a normal appearing girl
    who they tell you is short. What is the
    diagnosis?
  • 8What autoimmune disease is she at risk for?
  • 9What imaging studies are needed at diagnosis?
  • Other things to consider
  • Can start GH after age 9 months
  • Needs hearing and eye evaluation
  • May develop scoliosis, teeth problems
  • May have a nonverbal learning disorder
  • Start estrogen at 12 years
  • If has a mosaic karyotype including a Y (XO/XYY),
    need to remove the gonads due to risk for
    gonadoblastoma

53
Brain abnormalities
  • 10A newborn baby has nystagmus. MRI reveals
    optic nerve hypoplasia with absent septum
    pellucidum. What endocrine problem do you
    consider?
  • 11A newborn baby has a micropenis. What diagnosis
    do you consider?
  • 12A baby has a single central incisor. What
    diagnosis do you consider?

54
Adrenoleukodystrophy
  • Only happens in boys (X-linked)
  • At age 3-4 years, they get progressive behavior
    problems aggression, hyperactivity, poor memory,
    poor handwriting.
  • Then it progresses to ataxia, increased
    spasticity, blind, deaf, then cant speak or
    swallow. Interval from 1st sign to complete
    vegetative state is 2 years, followed by death.
  • They also get N/V, fatigue, low BG, muscle
    weakness from adrenal insufficiency
  • Test VLFCA (very long chain fatty acids)

55
Hypo/Hyperphosphatasia
  • Hypophosphatasia
  • Low levels of Alk Phos
  • Usually fatal in infancy
  • Leads to osteomalacia and bad periodontal dz
  • Hyperphosphatasia
  • Transient
  • very high Alk Phos with no liver or bone disease,
  • resolves in months with no tx.
  • usually kid is under age 5 years and is healthy
  • Permanent look for underlying bone and liver dz

56
Lab tests
  • 13A known diabetic comes into the ER in DKA.
    Initial BG is 600 mg/dl, and the sodium level on
    the blood work reads 140 mmol/L. What is the
    real sodium level?
  • High glucose falsely lowers the sodium. For
    every 100 mg/dl the glucose is over normal, the
    sodium level decreases by 1.6 mmol/l.

57
Lab tests - Growth
  • 14What lab tests do you use to screen for growth
    hormone deficiency?
  • 15If IGF-1 and IGF-BP3 are low, what test do you
    do to confirm growth hormone deficiency?
  • 16Which lab test do you use to monitor the
    patient once they are on GH therapy?
  • 17What are some side effects of GH therapy?
  • 18What agents are used for GH stimulation testing?

58
Other tests for growth
  • If overweight
  • Hypothyroid
  • TSH and free thyroxine
  • GH deficiency
  • IGF1 and IGF-BP3
  • Cushings syndrome
  • 24 hr urine free cortisol
  • If underweight
  • GI disease
  • Crohns
  • ESR
  • Renal disease (RTA)
  • Chem-8
  • Celiac disease
  • Tissue tranglutaminase

59
Indications for Growth Hormone
  • Growth Hormone deficiency
  • Hypopituitarism
  • Panhypopituitarism
  • Turners syndrome
  • Noonans syndrome
  • Prader Willi syndrome
  • AIDS wasting
  • Chronic renal insufficiency/failure
  • Born SGA or IUGR with failure of catch-up growth
    by age 2 years
  • Idiopathic Short Stature
  • SHOX gene deficiency

60
Lab tests - Puberty
  • 19A little 5 y/o girl has breast development and
    accelerated height velocity. What stimulation
    test do you use to evaluate for precocious
    puberty?
  • 20Once central precocious puberty is confirmed,
    what imaging study do you do next?
  • 21What brain lesion causes precocious puberty and
    gelastic seizures (aka laughing fits)?

61
Pre-Puberty
GnRH pulses
Hypothalamus
Pituitary
LH, FSH
Ovary/ Testes
62
Puberty
Hypothalamus
GnRH pulses
Pituitary
LH, FSH
Ovary/ Testes
Early Puberty Before age 7 in black girls Before
age 8 in white girls Before age 9 in boys
63
Precocious puberty
  • 22A 15 y/o girl moves to the USA from an
    underdeveloped country with poor health care.
    She is very short and she started her periods
    when she was 4 years old. On physical exam, you
    notice many café-au-lait spots with irregular
    borders a coast of Maine appearance. One arm
    is longer than the other and she has a strange
    bony overgrowths on her face. What syndrome does
    she have?

64
McCune-Albright syndrome
  • Peripheral precocious puberty
  • Café-au-lait spots
  • Polyostotic fibrous dysplasia
  • Bone is replaced by fibers

65
Polycystic ovarian syndrome
  • Oligo or an- ovulation
  • Clinical or biochemical evidence of
    hyperandrogenism
  • Polycystic ovaries on ultrasound
  • Need 2 out of these 3 to make a diagnosis
  • Increased risk of metabolic syndrome
  • Obesity, high trig, low HDL, high BP, high
    fasting BG

66
PCOS clinical picture
Mostly obese some have metabolic syndrome Some
have polycystic ovaries on U/S
  • High
  • Free Testosterone
  • Androstendione
  • DHEA
  • DHEA-S
  • Insulin
  • Total Testosterone (or nl)
  • LH
  • LH/FSH ratio gt 3 (or nl)
  • Low
  • Sex-hormone binding globulin
  • FSH

67
Hirsutism(Clinical evidence of
hyperandrogenism/PCOS)
  • Male pattern hair in women
  • Hairs are long, dark, curly terminal
  • On Upper lip, chin, upper chest, abd, back
  • The degree of hirsutism is NOT directly related
    to the levels of androgens in the blood
  • Hirsutism doesnt indicate pathology
  • Diff from virilization
  • Clitiromegaly, male body shape, male-pattern
    baldness, voice deepening
  • Needs a more complete workup

68
Amenorrhea
  • No menses 4 yrs after breast dev
  • No menarche by age 16 years
  • Delayed puberty no signs by age 14 yrs
  • Secondary missing 3 cycles of menses
  • Anovulation
  • Major cause of amenorrhea
  • Happens to most girls in 1st 2 yrs of menses
  • Can still be happening at 5 yrs post-menses

69
Hypothalamic amenorrhea
  • Low GnRH leads to low LH and low FSH
  • Stress
  • Exercise
  • Poor nutrition (eating disorders)
  • Athlete triad disordered eating, amenorrhea,
    osteoporosis
  • Weight changes
  • Extreme emotion
  • Drugs heroin, methadone
  • Chronic Disease CF, renal dz
  • Genetic Kallmans, Prader-Willi, Bardet-Biedl
  • High Prolactin
  • Thyroid dysfunction

70
Prolactin
  • Clinical picture of a prolactinoma
  • Galactorrhea (variable)
  • Headache
  • Visual field deficit
  • Infertility/irregular periods
  • Osteopenia (from low estrogen levels)
  • Only ½ will have a mass on MRI
  • Microadenoma lt10 mm (Macro gt10 mm)

71
Lab tests - Adrenal
  • Newborn baby has congenital adrenal hyperplasia.
  • First lab to check
  • 17-hydroxyprogesterone
  • Most likely adrenal enzyme deficiency
  • 21 alpha hydroxylase
  • Test to confirm the diagnosis
  • ACTH stimulation test

72
Tests - Thyroid
  • Different names
  • TSH and thyrotropin
  • T4 and thyroxine and levothyroxine
  • T3 and triiodothyronine and Cytomel
  • Dont treat with T3 or measure T3

73
Water Hormones
  • Diabetes Insipidus
  • Cant make ADH
  • Pee out all your water
  • Only thing left in blood is salt
  • Only thing in your pee is water
  • Cant concentrate the urine
  • Patient pees too much, so therefore they drink

74
Diabetes Insipidus - Labs
  • High serum Na
  • High serum Osm
  • Low spec grav in urine
  • Low urine osmolarity
  • polyuria/polydipsia, even overnight
  • Patient prefers only water, cold water
  • Confirm with water deprivation test
  • Treat with DDAVP (vasopressin)

75
Diabetes Insipidus - Testing
  • Confirm with a water deprivation test
  • Admit kid to hospital, no eating or drinking
  • When serum Na goes above 140 and serum osm goes
    above 300 and urine is still dilute, you have
    confirmed DI
  • Then give vasopressin (DDAVP)
  • Central DI urine will concentrate (urine osm
    gt600)
  • Nephrogenic DI urine will stay dilute
  • Treatment low salt diet and a thiazide diuretic

76
SIADH
  • You make too much ADH, so you cant pee out your
    water.
  • Only thing in the pee is salt
  • The blood is mostly water
  • Happens after brain injury
  • Urine high sodium, high osm, high SG
  • Blood low Na, low osm
  • Treatment water restriction

77
23What heart lesion do these girls have?
I see full cheeks and a large mouth
78
24What heart lesion do these girls have?
79
Answers
  • 1 He will weigh 5.4 kg
  • 2 Late
  • 3 Testes are 6 ml.
  • 4 No, he has finished growing.
  • 5 Klinefelters
  • 6 Kallmanns syndrome
  • 7 Turners syndrome
  • 8 Hypothyroidism, and also celiac
  • 9 Get a heart Echo and renal ultrasound
  • 10-12 hypopituitarism
  • 13 Sodium level is 148.

80
Answers
  • 14 IGF-1 and IGF-BP3
  • 15 growth hormone stimulation test, with two
    agents arginine, clonidine, L-dopa, or insulin
  • 16 IGF-1
  • 17 headache (increased ICP), SCFE, high blood
    sugar
  • 18 Clonidine, Arginine, L-Dopa, Glucagon,
    Insulin
  • 19 leuprolide stimulation test
  • 20 MRI of the brain
  • 21 hypothalamic hamartoma
  • 22 McCune-Albright syndrome
  • 23 Williams syndrome supravalvular aortic
    stenosis
  • 24 Turners syndrome coarctation of the aorta
    and bicuspis aortic valve
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