Endocrinology Flashcards
1
Q
2 year old child is adopted by obese parents. What can be said about this child?
a) Child will almost certainly be obese, irrespective of biological parents.
b) Child will be thin if biological parents are thin
c) Obesity associated with higher socioeconomic status
d) Obese as infant = 90% chance of being obese adult
A
b) Child will be thin if biological parents are thin
Obesity RF:
- higher maternal education protective
- low SES increases risk
- risk more strongly conferred by biological than adoptive parents
2
Q
A 12-year-old girl has a bone age of 11 years. All of the following might be expected EXCEPT:
a) menses
b) increased growth velocity
c) breast development
d) axillary hair
e) acne
A
a) menses
- stages of puberty (females): thelarche (breast bud development; 10-11 years) —> pubarche (pubic hair;
6-12 months later) —> increased growth velocity (always precedes menarche) —> menarche (2-2.5 years,
may be up to 6 years)
- stage of puberty is more accurately predicted by bone age than chronological age
3
Q
Which of the following is true in puberty?
a) menstruation at Tanner stage IV
b) maximum penile growth at Tanner stage II
c) axillary hair in males at Tanner stage III
d) voice change at Tanner Stage III
e) Double breast contour in girls at Tanner stage III
A
a) menstruation at Tanner stage IV
4
Q
Which of the following has the latest onset of pubertal changes in girls:
a. Axillary hair
b. Menarche
c. Breast development
d. Increased growth velocity
A
b. Menarche
5
Q
Mechanism of action for insulin:
a) glycogen synthesis
b) gluconeogenesis
c) lipolysis
d) ketogenesis
e) cAMP
A
a) glycogen synthesis
gluconeogenesis triggered by glucagon
6
Q
Picture of growth curve tracking parallel but below 5 th percentile. Most likely:
a. Russel-Silver syndrome
b. constitutional growth delay
c. familial short stature
d. CF
e. chronic renal failure
A
c. familial short stature
7
Q
The use of growth hormone has yielded positive results in which condition:
a) familial short stature with height <2 standard deviations below the mean for age
b) constitutional short stature with height <2 standard deviations below the mean for age
c) growth hormone deficiency in girls after epiphyseal closure
d) Turner syndrome
e) Down syndrome
A
ANSWER: d) Turner syndrome
Indications for GH therapy:
- GH deficiency
- Turner
- idiopathic <2.25SD below mean, corrected for bone age
- SGA without catch up by 2 years
- Prader-Willi
- Noonan
- chronic renal failure before transplant
8
Q
9-year-old girl with growth velocity 2 cm/yr for the past 2 years. Best diagnostic investigation:
a) TSH
b) CT head
c) AM and PM cortisol
d) nocturnal growth hormone level
A
ANSWER: a) TSH - hypothyroidism is the most common endocrinopathy
- lowest normal prepubertal growth is 5cm/year
Note: b) CT head - to assess for craniopharyngioma (growth pattern is a normally growing kid who growth then falls off but weight is preserved)
c) AM and PM cortisol - Cushing disease; would usually just screen with an AM cortisol
d) nocturnal growth hormone level - recommended test for GH deficiency is IGF-1 as a screen and then GH stimulation test
9
Q
Features of constitutional short stature include all EXCEPT:
a) adequate nutrition
b) normal CT of sella turcica
c) growth velocity < 3 cm/yr
d) bone age delayed by 1.5-4 years
A
c) growth velocity < 3 cm/yr
should have normal growth velocity (parallel curve, just below it)
10
Q
27 mos girl bone age 17 mos, short stature. Graphs show N BW now 3rd for ht and wt a constitutional b familial c growth hormone deficiency d malnutrition
A
a constitutional
11
Q
Who should get GH studies?
a. Short kid with tall parents
b. All children below 3 rd percentile
c. Children with > 3SD below the mean
d. Growth of 6cm/year
A
c. Children with > 3SD below the mean
12
Q
A 9 year old boy who used to be about average height in his class is now becoming one of the smallest. His height has dropped for the 25 th to the 10 th percentile and his weight has dropped from the 25 th to the 5 th percentile. Which of the following tests is most likely to help in making the diagnosis:
a. TSH
b. IGF-1 level
c. Anti-tissue transglutaminase antibody
A
c. Anti-tissue transglutaminase antibody
13
Q
14 yo boy with short stature. Both parents are normal height. He is 5 th %ile for both ht and wt. He is tanner 2. What is your next test. 1. random growth hormone 2. bone age 3. cortisol level 4. testosterone level
A
- bone age
14
Q
9 year old girl has just had the onset of her first menses. Parents are concerned about her final height. What do you do?
a. Tell them that she will grow another 5 cm
b. calculate mid-parental height
c. do a karyotype
d. do a bone age
A
a. Tell them that she will grow another 5 cm
15
Q
In which of the following situations is growth hormone testing indicated:
a. All children below the 3 rd /5 th percentile
b. Children with growth velocity less than 6 cm/year
c. Children with a height is greater than 3 SD below the mean
A
c. Children with a height is greater than 3 SD below the mean
16
Q
6 year old child who was growing 2 cm/year, height now <5th percentile and 50% for weight. Bone age 4 years. Diagnosis?
a. Celiac disease
b. GH deficiency
c. Turner syndrome
A
b. GH deficiency
c. Turner syndrome
Could be either - Turner usually starts to fall off around age 6 so if ongoing for longer may be more likely GH deficiency; if female may be more likely Turners
17
Q
6 year old Short child growing along the third. Bone age is 4 years. No change in his growth velocity. Otherwise well. Gaining weight normally. What is the cause of his short stature? Mom is 160 cm dad is 180 cm calculate the mid parental height.
A
- cause: constitutional growth delay
2. MPH = 180+160+13/2 = 176.5cm
18
Q
Child born term, bw normal, down to 3 rd percentile by 12 mos, growth chart shown with ht and wt both following 3 rd percentile. Parents heights given - both short. Diagnosis?
A
familial short stature
19
Q
Kid who has bone age of 12. Calculate mid parental height. Mom was 157cm, dad was 180 cm.
A
MPH = 162 if female, 175 if male
20
Q
Picture of a growth curve showing a 9 year old girl who has maintained the 25 th percentile for weight
from ages 3-9 years but who has fallen off the height curve from the 25 th percentile (from ages 3-7 years)
to below the 3 rd percentile (at 9 years). What is the most likely cause?
A
Hypothyroidism (most common endocrinopathy), could also be GH deficiency or Turners. Screen with TSH AND fT4
When growing normally and then height falls off while weight is preserved, think about and endocrinopathy. Constitutional delay, growth starts to slow at around 3 years
21
Q
Child with micropenis. Best test to determine sex of rearing
a. Y chromosome (karyotype)
b. testosterone level
c. hypospadias
d. palpable gonads
e. size of phallus
A
a. Y chromosome (karyotype)
Investigations for micropenis:
- karyotype
- assess pituitary function
- assess testicular function
- MRI (hypothalamic or pituitary lesions)
22
Q
Which of the following is most important in assigning sex of rearing:
a. level of testosterone
b. chromosomes
c. if testes are descended
d. gonadotropin levels
A
b. chromosomes
23
Q
Which deficiency might be found in a female infant with clitoromegaly:
a) 21-hydroxylase
b) alpha-1-antitrypsin
c) phenylalanine hydroxylase
d) pyruvate kinase
e) cystathionine
A
a) 21-hydroxylase
- most common enzyme deficiency in CAH (test 17-OHP to diagnose)
24
Q
A patient with XX, absence of testosterone, absence of SRY, and absence of Mullerian inhibiting hormone will have what gender phenotype?
A
female (SRY leads to formation of testes, MIS prevents formation of uterus, fallopian tubes and proximal vagina so if you DO NOT have it you’ll be a normal female)
25
A patient with XY, androgen production, SRY and anti-mullerian hormone production will have what gender phenotype?
male
26
Androgen insensitivity syndrome occurs due to:
a) decreased peripheral sensitivity to testosterone
b) decreased production of testosterone
c) impaired conversion of testosterone precursors
a) decreased peripheral sensitivity to testosterone
| All have testes and normal or elevated testosterone and LH
27
A 1 week old female infant has ambiguous genitalia, vomiting and lethargy. Which of the following abnormalities are you likely to find on bloodwork:
a. Metabolic acidosis
b. Isolated hyperkalemia
c. Hypokalemia and metabolic alkalosis
d. Hyponatremia, hyperkalemia and metabolic alkalosis
a. Metabolic acidosis
CAH blood work: hyponatremia, hyperkalemia, metabolic acidosis
- deficiency in cortisol and aldosterone, while having too much androgens
28
A mother had amniocentesis due to advanced maternal age, with a karyotype of XY. The baby is born and is a normal female. What is the most likely etiology of this:
a. Testicular feminization (aka AIS)
b. Chimera
c. Impossible situation
d. Maternal karyotype
a. Testicular feminization (aka AIS)
29
A 3 week old female presents with ambiguous genitalia, vomiting and lethargy. Which of the
following tests do you recommend for diagnosis:
a. Aldosterone
b. 17-OHP
c. Cortisol
b. 17-OHP
30
What lab test would most likely be elevated in a child with the genitalia pictured. – picture of
ambiguous genitalia, looked like female virilization.
1. sodium
2. potassium
3. cortisol
4. aldosterone
5. glucose
2. potassium
31
Child with perineoscrotal hypospadias , enlarged phallus, non-palpable testicles.
a. congenital adrenal hyperplasia
b. 5-alpha reductase deficiency
c. partial androgen insensitivity
d. normal male
Could probably be any of:
b. 5-alpha reductase deficiency
OMIM/Orphanet seem to associate perineoscrotal hypospadias with 5-alpha reductase specifically; need functional 5a to fuse scrotum
32
Newborn with ambiguous genitalia. Phallus is 2 cm, labia are fused. What is the most likely diagnosis
(1) ? What test would give you the answer (1) What acute complication is this child susceptible to?
1. CAH
2. 17-OHP
3. salt wasting crisis
33
Which is true regarding lymphocytic thyroiditis:
a) intrathyroidal infiltration primarily by B cells
b) anti-thyroglobulin antibodies are commonly present
c) TSH is always elevated early in the course
d) thyroid microsomal antibodies (now called antiTPO abs) are present in the majority of children
e) TSH antibody is present
d) thyroid microsomal antibodies (now called antiTPO abs) are present in the majority of children (90%)
lymphocytic thyroiditis = AKA hashimoto or autoimmune thyroiditis; infiltration 60% T cells, 30% B cells
antithyroglobulin abs positive in less than half of cases; TSH antibody in 18%
34
What is the best way to monitor effectiveness of thyroid replacement in autoimmune thyroiditis?
a. T4
b. Free T4
c. T3
d. TSH
e. Antibody
d. TSH
35
Which is used to determine the initial dose of replacement therapy in lymphocytic thyroiditis:
a) TSH
b) T3
c) T4
d) free T4
e) thyroid antibodies
d) free T4
36
A 10-year-old girl has an enlarged asymptomatic thyroid. Most likely:
a) Hashimoto's thyroiditis
b) Graves' disease
c) congenital goiter
d) carcinoma
a) Hashimoto's thyroiditis
37
Family with hypothyroidism on Synthroid bring daughter with growth 5 cm/year. Normal exam. What to do?
a) TSH, T4
b) T4, T3
c) fT4, T4
d) Nothing
d) Nothing
38
All are seen in hypoparathyroidism EXCEPT:
a) hyporeflexia
b) increased intracranial pressure
c) carpopedal spasm
d) candidiasis
e) nonspecific EEG changes
```
a) hyporeflexia
hypopara leads to hypoCa = hyperreflexia
- muscular pain and cramps - progressing numbness, stiffness, tingling hands and feet
- carpopedal spasm, seizures
- mucocutaneous candidiasis
- headache, vomiting, increased ICP, papilledema
- cataracts
- keratoconjunctivitis
```
39
3 mo with constipation since birth, now FTT. What hormonal test would be best?
TSH (congenital hypothyroidism)
40
```
A 12 year old girl is falling of her height curve. She has also gained weight recently and is cold-intolerant.
What test would you order?
a) TSH
b) GH
c) a.m. cortisol
d) TTG
```
a) TSH
41
Newborn with a TSH of 25. What do you do?
a. start thyroxine
b. repeat neonatal screen
c. bring them into the office as soon as possible
d. thyroid ultrasound
c. bring them into the office as soon as possible
need to have confirmatory testing done and be started on thyroxine, but do need to clinically assess first; if TSH >40 start thyroxine immediately after confirmatory testing is drawn (do not wait for test results)
42
Newborn's TSH screen comes back as 25 ( N less than 20). What is the most likely cause of a false positive screen ( l line). What is the most likely cause of a false negative screen (1 line). List 3 investigations to work up congenital hypothyroidism.
1. false positives: prematurity, screening before 24 hours of age
2. false negatives: critical illness, post-transfusion
3. investigations:
o TSH + free T4
o US or thyroid scan to determine location and size of thyroid tissue
o TPO antibodies
43
Asymptomatic goiter. What is your management.
a. See in 6 monhts
b. Thyroid ultrasound
c. TSH
c. TSH
44
What is best test to determine initial dose of thyroid replacement in Hashimoto’s
a) TSH
b) T4
c) fT4
a) TSH
subclinical (high TSH, normal T4)= controversial= most tx until growth and puberty and R/A so no
impact on brain or growth; if TSH high and T4 low, definitely treat
45
Teenaged girl has decreased growth and weight gain, and goitre. What is her specific diagnosis? (1) What test
would you do that is specific to the diagnosis (1)?
1. Hypothyroidism, likely secondary to hashimoto thyroiditis
| 2. antiTPO antibodies
46
Child diagnosed with Graves disease, symptomatic with hand tremors. Enjoys painting! Medications x2
- propranolol - antithyroid medications take weeks to work. so beta blockers can control symptoms in the meantime
- methimazole
- radioactive iodine or surgical resection are curative but will leave lasting hypothyroidism
47
15 yo obese black male presents with polyuria, abdominal pain, and vomiting. Blood glucose is 40, bicarb 21,
and Ketone 1+. What is the most likely diagnosis?
a) DM1
b) DM2
c) Hypercortisolemia
b) DM2
48
Diabetic with microalbuminuria. What would you prescribe?
a. Enalapril
b. Hydrochlorothiazide
c. Nifedipine
d. Salt and water restriction
a. Enalapril
| ACE inhibitors halt progression of diabetic nephropathy; microalbuminuria is a good early screen for renal dysfunction
49
Diabetic going to OR. NPO since midnight. BS is now 5. To get IV D5NS. What are your insulin orders?
a. Keep same NPH, R
b. Don’t give R, give 50% NPH
c. Give 50% of R, no NPH
b. Don’t give R, give 50% NPH
- important to give IV insulin infusion and fluids with dextrose in OR to prevent hypoglycaemia and DKA
- if have long acting basal (lentos) give regular dose the night before
- if use NPH/lente as nasal then give half of usual dose the mooning of surgery
50
Picture of acanthosis nigricans in obese Native adolescent.
A) Identify picture.
B) What condition causes this?
B) acanthosis is sign of insulin resistance and is part of metabolic syndrome (may have T2DM)
51
15 y.o. male presents with two week history of polyuria, polydypsia and weight loss. You diagnose diabetes
mellitus.
A) If the child has DM1, what is required for diagnosis?
B) What is the BEST method to screen for diabetic nephropathy? First morning urine for albumin to creatinine
ratio (12y old or 5y after diagnosis, annually)
C) When do you send this child for ophthalmology assessment to screen for retinopathy? 15y old if >5y from
diagnosis, annually
A) If the child has DM1, what is required for diagnosis? Fasting blood sugar of >7.0, random blood sugar of
>11, or HbA1c >6.5
B) What is the BEST method to screen for diabetic nephropathy? First morning urine for albumin to creatinine
ratio (12y old or 5y after diagnosis, annually)
C) When do you send this child for ophthalmology assessment to screen for retinopathy? 15y old if >5y from
diagnosis, annually
52
You are managing a child who presented to your emergency department in DKA. Suddenly she develops a
decreasing level of consciousness, and on CT scan there is cerebral edema noted.
a. Name 2 errors that you could have made in your management that would have contributed to this
complication
b. Name 2 risk factors for this complication.
a. - Early insulin infusion (within 1st hour of starting fluids), insulin bolus, high volumes of fluid/fluid boluses and use of bicarbonate
b. RF for cerebral edema:
- age <5 years
- new onset diabetes
- high initial urea
- low initial CO2
53
Child with type 1 diabetes. Ate supper but missed pre-supper insulin (5R and 8N). About to take evening snack.
Glucose 23.5. Mom calling for advice (4 lines).
- Check for ketones
- Drink fluids
- Take insulin, (needs to correct for current BG and amount of carbs about to be consumed)
- repeat BG in 1 hour
54
15 year old boy with T1DM. You can only follow him in your clinic until 18 years of age. 5 things you would do to try to help him transition to adult care.
- ensure follow-up during transition - continue seeing while he is waiting for initial assessment by adult service
- organized transition services (possible joint clinic)
- adolescent involvement in management (increasing levels of responsibility and information, see youth alone to
promote autonomy)
- adolescent and family understanding of diabetes and reasons for treatment
- teach skills of negotiation and communication required in adult system
- formal graduation
- transition letter about new location, staff and what to expect
55
10 year old 30kg girl presents in DKA. PH<7.25, Glucose 40, 10 % dehydrated. Current sodium is 120. What type
of initial fluid would you give her? What would be the rate? What initial insulin dose/type would you start her on?
- NaCl at 130cc/hr (maintenance plus deficit over 48 hours; 4ml/kg/h)
- Insulin (rapid acting) infusion 0.1 U/kg/h 1-2 hours after IV fluid rehydration started
56
Hypoparathyroidism can be seen in all EXCEPT:
a) primary proximal renal tubular acidosis
b) vitamin D-deficient rickets
c) rickets with liver failure
d) rickets with anticonvulsants
b) vitamin D-deficient rickets - can mimic hypopara but there is no actual impact on parathyroid hormones or glands
57
3 week old has hypocalcemia. Most likely diagnosis?
- transient hypoparathyroidism (due to natural fall)
58
Child takes grandfather's glyburide pills. Looks well but glu 1.7. What is your initial management (1 line)? How often would you check the glucose (1 line)? How long would you observe in hospital (1 line)?
Glyburide is sulfonylurea - increases release of endogenous insulin
1. give simple sugar PO (juice, dextrose fast tab), start IV, bolus 5ml/kg D10W; ECG - can prolong QT
2. Check glucose q1-2 until stabilized
3. observe x24h (duration of effect of glyburide)- D/C if maintaining glucose without support
59
18 month old child with hypoglycemia and no ketonemia or ketonuria. What are the two most likely diagnoses?
Fatty acid oxidation defects
| Hyperinsulinism
60
6 y.o. female with 6 month history of pubic hair development. No other secondary sexual characteristics. Bone age is 6 yr 6 months . Urine ketosteroids markedly
increased. Urine hydroxysteroids normal. Serum gonadotropins, testosterone, 17-hydroxyprogesterone and ketosteroids normal. No change with dexamethasone
challenge. Most likely diagnosis:
a. CAH
b. adrenal tumour
c. physiologic adrenarche
d. undisclosed exogenous source of steroids
e. Cushing’s
c. physiologic adrenarche
61
```
A 6 year old female with pubic hair. The is increased ketosteroids, normal 17-OHP, normal testosterone. After dexamethasone stimulation there is no increase in
17-OHP. The diagnosis is:
a. CAH
b. Premature pubarche
c. Adrenal tumour
d. Cushing syndrome
e. Hyperthyroidism
```
b. Premature pubarche
| - increased basal and ACTH stimulated levels of DHEA, 17-hydroxypregnenolone and androstenedione
ketosteroids
62
A 6y F has pubic and axillary hair. No other signs of puberty. Where should you look for pathology
a. Hypothalamus
b. Pituitary
c. Adrenals
d. Ovaries
c. Adrenals
63
2 y.o that presents with premature thelarchy. Bone age 3 yrs, 3 mos. How will she progress to puberty?
a) Slow progression
b) Fast progression
c) Resolution at 3 yrs
c) Resolution at 3 yrs
Benign premature thelarche can present with bone age advanced in 30% of kids >2 years beyond chronologic age
64
Which is true regarding precocious puberty:
a) it is more common in boys than girls
b) final height is not affected
c) the most common etiology in girls is idiopathic
d) gonadotropins are invariably increased
e) the sella turcica is often abnormal
c) the most common etiology in girls is idiopathic
65
Which of the following investigations would you do in an 8 year old boy with the onset of puberty:
a. Cranial imaging
b. LH/FSH levels
c. Testicular ultrasound
b. LH/FSH levels
LH (basal in a.m.), FSH, either estradiol or testosterone (results help differentiate central and peripheral precocity)
- central puberty implies activation of HPG axis and therefore LH and FSH are high
- if central precocious puberty confirmed - get MRI
66
6 year old girl with breast development, pubic hair development, advanced bone age of 10 years. What two
investigations do you do for the diagnosis?
LH, FSH, estradiol - confirm central precocious puberty
67
6 year old child with pubic hair. Bone age 6 1/2 years. Most likely dx?
a. craniopharyngioma
b. benign premature adrenarche
b. benign premature adrenarche
Premature Adrenarche:
● ISOLATED development of pubic hair or body odour, mild acne with NO other signs of puberty (rapid growth or advancement in bone age aka no vaginal d/c or breast in F and no testicular enlargement in M)
68
Pubertal gynecomastia is most likely associated with:
1. Tanner I
2. breast size < 4 cm
3. nontender breasts
1. breasts lasting for 3 years
2. testes < 3.5 cm
2. breast size < 4 cm (this is talking about males obvi)
| gynecomastia prior to puberty PATHOLOGICAL (measure testicular volume. If < 4 cc= not in puberty).
69
6 year old child with vaginal bleeding, no foreign body, no exogenous estrogen sources. Has bone age of 7.5 years, 17-OPH normal, what is dx
a. CAH
b. craniopharyngioma
c. premature adrenarche
b. craniopharyngioma
70
Which is an indication of delayed puberty?
a) 15 yo girl with amenorrhea
b) 12 year old girl with no axillary hair
c) 13 yo boy with no pubic hair
d) 15 yo boy with no voice change
e) 13 yo girl with no menarche
a) 15 yo girl with amenorrhea
Delayed Puberty: no menarche by 15 y.o. OR no secondary sexual traits by 13 y.o. for F or 14 y.o. for M.
71
A 14 y.o. girl presents with concerns about delayed puberty. She has no breast development or pubic hair, and
has never had a period. Her height is below the 3rd percentile for age, and her bone age is normal. Most likely
diagnosis:
a) Hypothyroidism
b) Turner syndrome
c) Androgen insensitivity syndrome
d) Constitutional growth delay
b) Turner syndrome
72
15 yo female with amenorrhea and falling off growth curve. List Ddx (4)
```
● Turner’s Syndrome
● Hypothyroidism
● Anorexia Nervosa (Functional hypothalamic amenorrhea)
● Athletic Triad
● Craniopharyngioma
● Chronic illness
```
73
Delayed sexual behaviour in adolescence is associated with?
a) precocious puberty
b) poverty
c) sexual abuse
d) strict parenting
d) strict parenting
74
A 12 year old boy notices a mass on one of his testicles. He has no other systemic symptoms. Which of the
following investigations do you do first:
a. Testicular scan
b. Ultrasound
c. Beta HCG & AFP
b. Ultrasound
● Serum tumour markers (i.e. alpha fetoprotein, beta HCG, chorionic gonadotropin) should also be drawn.
75
An 8 month old boy is referred for unilateral cryptorchidism Why is an orchidopexy recommended?
a) to prevent cancer
b) to allow for easier examination of the testis
c) to optimize fertility
c) to optimize fertility
76
```
16 yo boy has admitted to using anabolic steroids. His testes appear small. Why are his testes small? (1 line)
What test(s) would you do to confirm your suspicion of why testes are small.
```
1. Negative feedback of anabolic/androgens on HPA axis
| 2. Testosterone, LH, FSH
77
Girl with PCOS. what lab abnormalities would be expected? What clinical complaints would be expected?
1. increased LH, decreased FSH; high androgens (serum testosterone, DHEAS; decreased sex hormone binding globulin)
2. increased LH = increased ovarian androgen production = acne, balding, hirsutism, rapid weight gain, insulin resistance, metabolic syndrome
- decreased FSH = impaired folliculogenesis = oligomenorrhea (irregular periods or secondary amenorrhea)
78
Mediterranean teen with coarse hair on chest, bum, legs, arms. Mod-severe acne. Most likely dx?
a. CAH
b. PCOS
c. Familial hirsutism
b. PCOS
Late onset 21-hydroxylase deficiency rare, but possible and needs to be ruled out in diagnostic work up
79
The estrogen part of the OCP causes what?
a. moodiness
b. salt and water retention
c. acne
b. salt and water retention
80
Girl described…obese, irregular periods, acanthosis nigricans, increased facial hair. What is her most
likely diagnosis?
PCOS
81
What is the diagnostic criteria for PCOS?
2 of 3:
- menstrual irregularities - oligomenorrhea (<8 periods per year)
- physical or biochemical hyperandrogenism
- polycystic ovaries
82
16 year old girl with secondary amenorrhea. She was having normal periods since 13. She has not been on any medications. What are 6 possible causes of her secondary amenorrhea (3) What are two tests that can help in the investigation
```
Causes of secondary amenorrhea:
1. PCOS
2. anorexia/weight loss
3. pregnancy
4. hyperthyroidism
5. prolactinoma
6. autoimmune ovarian failure
7. Cushings
8. stress
9. exercise
Ix:
-beta-hCG, TSH, PRL, FSH, LH, ovarian U/S
```
83
14 year old girl has severe dysmenorrhea and has missed 2-3 days of school with each period. What are three causes of dysmennorhea(3)? What two classes of medications would you prescribe for her (2)?
1. Causes of dysmenorrhea:
- idiopathic
- endometriosis
- uterine polyps
- ovarian cyst
- PID
- IUD
2. Medications
- combined OCP
- NSAIDs
84
Child presents to ER. Has septic optic dysplasia. Is in shock. Na 148 K 6.2 Glucose 3.2 After fluid resuscitation, what is your next step.
a. Antibiotics
b. IV hydrocortisone
c. Glucose bolus
d. D5 ½ NS at some rate
b. IV hydrocortisone
Septo-optic dysplasia can have panhypopit so would need steroids for adrenal insufficiency (no release of ACTH from pituitary)
85
McCune Albright question (description of girl with café o lait, short stature, etc.), what would you do? what are some typical presenting complaints of McCune-Albright?
A. NF1 testing,
B. endocrinopathy work up
B. endocrinopathy work up
Café-Au-Lait spots (coast of Maine - large, do not cross midline); fibrous dysplasia of bones; precocious puberty
endo findings: precocious puberty, hyperthyroid, hyperparathyroid, pitutary adenomas - increased GH, Cushing
86
```
Kid presents with vaginal bleeding. You note she is Tanner 3, has cafe-au-lait patches and has a bump on her leg. Xray confirms fibrous dysplasia.
What do you look for?
a. neurofibromas
b. other endocrinopathies
c. cardiac echography
```
b. other endocrinopathies
McCune-Albright
- expect menarche with SMR 4
87
Which of the following is a feature of primary nephrogenic DI?
a. Mental retardation
b. Male predominance
b. Male predominance
88
Teenaged boy who has just had orthopedic surgery. Has been in the casts and on bedrest for 11 days. Suddenly develops anorexia, polydypsia and polyuria. Glucose is normal on admission, BMI 29. What is the diagnosis? What one test can give you the diagnosis (1) How is this managed?
1. immobilization hypercalcemia
2. test: ionized calcium
Basically you're lying in bed doing nothing so your body decides to start breaking your bones down - you get high calcium which makes your stomach hurt and decreases your kidney's ability to concentrate your urine - so you get dehydrated and feel thirsty and drink all the time
3. mgmt: IVF at 1.5-2x maintenance; lasix to help get rid of calcium
89
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3 wk male infant vx, lethargy, poor po intake, Na 118, K8, most important lab test:
a vasopressin
b 17 OHP
c calcium
d renin
e cortisol
```
b 17 OHP
CAH - salt wasting form - hyponatremia, hyperkalemia, metabolic acidosis
90
Child fatigued and tanned, K 5.2, Na 132, glucose 2.6, shocky, vomiting and has diarrhea. What is used to treat the underlying condition?
a. D5 0.25NS
b. nothing
c. NS 20 cc/kg
d. IV hydrocortisone
d. IV hydrocortisone
High K and low Na; shock + tan (melanocyte stimulating hormone - stimulated by ACTH production - is elevated) = Adrenal crisis
91
Approach to treatment of adrenal insufficiency crisis (5 things):
```
Salt: restore volume - NS okay
Sugar: treat hypoglycaemia
Steroids: IV hydrocortisone
Support: maintain BP (pressors PRN), PICU admission
Search: look for underlying etiology
```
92
Babe with CAH. Likely clinical picture?
a . Non ambigious boy with low sodium and high K
b . Virilized boy
c. Virilized female with high sodium and high K
a . Non ambigious boy with low sodium and high K
93
Kid with midline defect of septo optic dysplasia presents shocky, mottled, normal wccc, glc, Na 138, K 6, After fluids what is your next Rx?
a) Abx
b) IV Hydrocortisone
c) hypotonic saline
b) IV Hydrocortisone (the point is adrenal crisis + midline defects; see below)
94
Babe in shock with Na 131, K 5.9. What is the BEST thing to do right now?
a. IV hydrocortisone
b. NS bolus
c. D10W
d. D5W
b. NS bolus - bolus first given shock, then steroids
95
Kid with salt wasting CAH. Has gastro. She takes hydrocortisone and florinef. Tolerated oral rehydration solution. Ready for D/C lytes are normal. What would be your suggestion on discharge.
Stress Dosing (30mg/m2) until well
(§ HC 8-10mg/m2 for maintenance
§ Stress dose 20-30mg/m2 for illness
§ Stress dose 50-100mg/m2 for surgery/crisis)
96
Male 3 weeks old. Presents with shock, hyperpigmented scrotum, low NA 115, K 6.8. What is your most important investigation? What is your immediate management?
1. 17-OH-P
| 2. NS Bolus, Check glucose, IV Hydrocortisone
97
Young child presents to the emergency room looking unwell with a sodium of 132 and potassium of 6.2. What is the diagnosis?
Adrenal Insufficiency
98
When to intervene and counsel on risk of obesity:
a. BMI > 75 kg/m2
b. BMI > 85 kg/m2
c. BMI > 90 kg/m2
d. BMI > 95 kg/m2
b. BMI > 85 kg/m2
99
Obese teenager. What is she at risk for?
1. type 2 diabetes mellitus
2. delayed puberty
3. AVN of the femoral head
4. Thyroid problems
1. type 2 diabetes mellitus
100
FHX of hypercholesterolemia. Has high total cholesterol (6) and high LDL. What do you advise?
a. lifestyle modification
b. lifestyle modification + low fat diet
c. lifestyle modification + statin
d. lifestyle modification + cholestyrine.
b. lifestyle modification + low fat diet
101
14 yo female with BMI of 31. List 4 things that you should screen her for.
- Glucose intolerance
- Hypertension
- Hyperlipidemia
- Fatty Liver
- OSA
- Depression/anxiety
- Hypothyroidism (as cause)
102
Child who is 120 cm and 28.8 kg. In your office. He complains of being tired during the day. Has occasional headaches at school. Snores at night. Calculate his BMI. BMI chart provided for him. What do you think of his BMI? (he plots directly on the 97 percentile). What would you be concerned about IN THIS PARTICULAR PATIENT?
BMI= 28.8/1.2x1.2= 20= 97%ile= obesity, OSA
103
A description is given of an early-adolescent girl who is overweight. She is wondering what her ideal
body weight should be.
BMI < 85%ile
| Could also look at weight for heigh graph - 50th percentile
104
Child with BMI over 31. What are 5 diseases that
| he is at risk for (3)?
- OSA
- hyperlipidemia
- hypertension
- T2DM/glucose intolerance
- MSK issues - Blount's disease, SCFE
- Fatty liver disease
- (PCOS - female)
- pseudotumour cerebri
- psychosocial difficulties
105
What are the diagnostic criteria for metabolic syndrome?
3 of:
- Tg >1.7mmol/L
- HDL <1.03 mmol/L
- BP >90%ile or >130/85
- Glucose >5.6 mmol/L or Diabetes 2
- Waist circumference >90%ile
106
Vitamin D deficient Rickets, what will be low?
a. ALP
b. PTH
c. Vit 1-25 OH vitamin D
d. Vit 25 OH vitamin D
ANSWER: d. Vit 25 OH - Low in vitamin D deficiency Rickets (due to low endogenous vitamin D)
a. ALP (UP because lots of bone turnover)
c. Vit 1-25 OH - Low in vitamin D dependent rickets (which is reduced activity of 25 (oh) 1-alpha-hydroxylase)
107
Vitamin D dependent Rickets, what will be low?
a. ALP
b. PTH
c. Vit 1-25 OH vitamin D
d. Vit 25 OH vitamin D
c. Vit 1-25 OH vitamin D
108
1 yo child with clinical symptoms of rickets. Ca and PO4 are low normal. He has widened physes. You start treatment with D3 for suspected vitamin D deficient rickets. When would you have the child return to assess the response to treatment (1 line). If he's not responding, what other diagnoses would you consider (2). What blood test would you monitor to assess when you could decrease the vit D supplements.
1. BW q2 weeks and xray q4 weeks (should normalize by 3 months)
2. Adherence, Enzyme deficiency, receptor malfunction, malabsorption, renal disease
3. 25, OHD
109
18 mo immigrant child from Somalia. Bowed legs and wide wrists. What does he have? X ray given of wrist. What 3 other clinical signs
1. Rickets
| 2. Rachetic Rosary, Craniotabes, frontal bossing, FTT, delayed dentition
110
Description of a child with rickets (bowed legs, wide wrists). What are three laboratory tests that you would order?
Ca, PO4, ALP, PTH, 25OHD
111
X-linked familial Rickets, what will be low?
a. ALP
b. PTH
c. Vit 1-25 OH vitamin D
d. calcium
e. phosphate
e. phosphate
| also called hypophosphatemic Rickets
112
4 effects of excess vitamin D
Excess vitamin D leads to hypercalcemia (hypervitaminosis D):
- nausea, vomiting, anorexia
- constipation and abdominal pain
- HTN, short QT, torsades
- hypotonia, lethargy, psychosis
- polyuria, dehydration, nephrocalcinosis
