Pediatric Endocrinology Fellowship MCQs 2009-2010

Dr. Abdulmoein Al-Agha

Consultant Pediatric Endocrinologist
abdulmoein@dr-agha.com

1. Which one of the following G-Protein–Coupled receptor does not match

with the clinical conditions associated with their receptor mutations?

a) Inactivating mutation of Melanocortin-4 receptor leads to obesity in

childhood
b) Inactivating mutation of LH receptor in males lead to Leydig cell
Hypoplasia
c) Activating mutation of Calcium-sensing receptor associated with
Autosomal-dominant hypocalcemic hypocalciuria
d) Inactivation mutation of GnRH receptor leads to Isolated
Hypogonadotrophic hypogonadism
e) Activation mutation of TSH receptor associated with non – autoimmune
hypothyroidism

Answer is E
Leads to hyperthyroidism rather than hypothyroidism

2. The parents of 14 year old boy are concerned about his short stature. BW= 3
kg, His physical examination was normal. US/LS ratio =0.98. Pre-pubertal
with small amount of fine axillary and pubic hair were present. In this
situation. Which one of the following is least indicated to do?

a) Measure FSH/LH
b) Obtain information regarding parental heights
c) Compare bone age with height age and chronological age
d) Obtain records of previous measurements and plot on growth curve
e) Measure TSH / fT4

Answer is (A)

3. Which one of the following syndromes is not associated with small

penis/ Cryptorchidism?

a) Aarskog syndrome
b) Holoprosencephaly
c) Myotonic dystrophy
d) Prader-Willi syndrome
e) Fragile X syndrome

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Answer is (E)
Fragile X syndrome is not associated with cryptorchidism, is usually associated
with large testis

4. Thirteen - year old a symptomatic girl found to have an enlarged thyroid which
was growing slowly for more than a year. Of the following is not in the
differential diagnosis?

a) Hashimoto’s thyroiditis
b) Grave’s disease
c) Non-toxic multinodular goiter
d) Toxic multinodular goiter
e) Acute Supportive thyroiditis

Answer is E: Acute Suppurative thyroiditis

5. A 12 year old girl with thyroid nodule. Technetium scan revealed “cold
nodule”. Child is euthyroid. Which is the following is least likely?

a) Simple adenoma
b) Follicular carcinoma
c) Papillary carcinoma
d) Thyroid cyst
e) Dysgenetic thyroid gland

Answer is E

6. Which one of the following is a rare cause of hypothyroidism in

neonatal period?

a) Thyroid Dysgenesis
b) Thyroid Dyshormonogenesis
c) Hypothalamic-Pituitary Hypothyroidism
d) Thyroid Hormone Resistance
e) Transient Hypothyroidism
Answer is D

7. Which one of the following is least Clinical Features Suggesting

Malignancy in Children presenting with thyroid nodule?
a) History of neck radiation
b) Family history of medullary carcinoma
c) Slow growth of nodule
d) Vocal cord paralysis
e) Enlarged regional lymph nodes
Answer is C

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8. Which one of the following syndromes is associated with short stature
and normal mentality?

a) Russell-Silver syndrome
b) Seckel syndrome
c) Noonan syndrome
d) Bloom syndrome
e) Prader-Willi syndrome
Answer is A
Russell-Silver syndrome is usually have normal mentality

9. Which one of the following established transcription factors is not

resulting in GH Deficiency?

a) Pit -1
b) PROP1
c) HESX1
d) SOX3
e) DAX -1

Answer is E
DAX-1 is associated with congenital adrenal Hypoplasia

10. Which one of the following is not true regarding vitamin D–Resistant
(Hypophosphatemic) Rickets?

a) X-linked dominant condition results from decreased renal tubular

reabsorption of phosphate
b) Clinical features are generally more severe in females
c) The metabolic and skeletal abnormalities cannot be overcome by vitamin
D therapy alone
d) Preliminary studies with GH therapy have indicated (at least in the short
term) an enhancement of skeletal growth
e) Skeletal deformities are usually severe and needs surgical correction

Answer is B
Clinical features are more severe in males not in females

11. Which one of the following is not Clinical Features of growth

hormone insensitivity?
a) Birth weight: near-normal
b) Genitalia: micropenis in childhood; normal for body size in adults
c) Forehead: prominent; frontal bossing
d) Dentition: delayed eruption
e) Voice: low-pitched
Answer is E, Voice: High-pitched

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12. Which one of the following is not true regarding psychosocial
dwarfism?

a) Most cases of failure to thrive due to poor home environment and

inadequate parenting
b) Behavior is characterized by bizarre eating and drinking habits, such as
drinking from toilets, social withdrawal, and primitive speech
c) GH secretion is abnormally low after provocative testing but returned to
normal on removal from the home.
d) Even though GH secretion is reduced, treatment with GH is not usually of
benefit
e) In the developing communities, it is a common cause

Answer is E
Fortunately, rare cause in the developing countries

13. Forty - days old boy, product of term pregnancy delivered by normal vaginal
delivery, the antenatal period was normal. Birth weight was 1.7kg with
symmetrical IUGR. Family History revealed healthy consanguineous parents.
He was found to have high blood sugar level >250mg/dl no ketonuria. Which
one of the following is not true regarding neonatal diabetes?

a) Caused by mutations in genes by impairing the ability to secrete adequate

amounts of insulin to overcome insulin resistance
b) Most affected infants are intrauterine growth retarded (IUGR), reflecting
the critical role of insulin as an in utero growth factor
c) Clinical features include polyuria with Glucosuria, dehydration, failure to
thrive, and occasionally diabetic ketoacidosis
d) When measured, IGF-1 levels are high
e) About 50% of cases of neonatal DM have the permanent form from the
outset

Answer is D, when measured; IGF-1 levels are Low

14. Which of the following investigations is least common to order during

hypoglycemia?

a) Glucose level
b) Insulin
c) Cortisol
d) Ketone
e) Catecholamine

Answer is E, Catecholamine levels not necessary investigation during

hypoglycemia

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15. Which one of the following is not true in the diagnostic criteria of
hyperinsulinemia?

a) Detectable insulin when glucose less than 50 mg/dl

b) Low free fatty acid (1.5 mmol/L)
c) High plasma -hyrdoxybutyrate
d) glucose rise 30 mg/dL in response to 1 mg intravenous Glucagon
e) In some cases high serum ammonia level
Answer is C : Low plasma -hyrdoxybutyrate

16. True sexual precocity in girls is most likely caused by?

a) Feminizing ovarian tumor

b) Gonadotropin producing tumor
c) CNS lesion
d) Exogenous estrogens
e) Idiopathic
Answer is E, the most common cause is idiopathic

17. Ten year old boy with Cushing's syndrome, which is the following, is
likely cause?

a) Bilateral adrenal hyperplasia

b) Adrenal adenoma
c) Craniopharyngioma
d) Adrenal carcinoma
e) Ectopic ACTH –secreting tumors
Answer is A., Bilateral adrenal hyperplasia

18. one day old infants develop tetany and convulsions. Serum calcium was 1.7
mmol/l. which one of the following is least likely diagnosis?

a) Perinatal asphyxia
b) High phosphate intake
c) Maternal diabetes
d) Maternal hyperparathyroidism
e) prematurity

19. Which one of the following is not recognized laboratory findings in

primary adrenocortical failure?
a) Elevation of serum ACTH.
b) Antibodies to 21-Hydroxylase.
c) Serum ACTH suppression with glucocorticoid therapy.
d) Decreased serum osmolality.
e) Elevated serum angiotensin converting enzyme.
Answer is E

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20. Which one of the following is not true regarding lymphocytic
Hypophysitis?

a) Has an equal incidence in males and females

b) Child could presents with diabetes insipidus
c) Can only be diagnosed on biopsy
d) Should be suspected in a peripartum females
presenting with a pituitary mass
e) May be managed conservatively
Answer is A

21. Which one of the following couldn’t cause inappropriate secretion of

vasopressin from posterior pituitary gland?

a) Pulmonary TB
b) Addison’s disease
c) Post operative
d) Bronchial ca
e) Lymphoma
Answer is B

22. Regarding thyroid Hormone tests, which one is true?

a) Serum thyroxin is the most useful test for screening thyroid function
b) Elevated thyroid hormones, together with inappropriately raised TSH levels is
suspected of a TSH’oma
c) Heterophilic antibodies may result in spuriously low serum TSH levels
d) Heparin can cause spuriously low serum fT4 levels
e) Sick euthyroid syndrome is characterized by low serum T4 and high serum
TSH levels
Answer is B

Whishing you the success