Ch. 557 - Hypopituitarism

Question 1

Denotes underproduction of GH ALONE or in combination with deficiencies of other pituitary hormones

Answer 1

Hypopituitarism

Question 2

Cause of acquired hypopituitarism wherein GH deficiency characteristically precedes other deficiencies

Answer 2

Irradiation

Question 3

Mutations of this gene are the MC explanation for recessive MPHD and are 10 times as common as the combined total mutations in other pituitary transcription factor genes

Answer 3

PROP1

Question 4

Mutations in this gene result in defect in incomplete development of the septum pellucidum in association with optic nerve hypoplasia and isolated GH deficiency

Answer 4

HESX1

Question 5

Patients with mutations in this gene often show the triad of a small anterior pituitary gland, an attenuated pituitary stalk, and an ectopic posterior pituitary bright spot

Answer 5

HESX1

Question 6

T/F DI is more frequent in acquired rather than in congenital hypopituitarism

Answer 6

T

Question 7

MC acquired lesion associated with DI

Answer 7

Craniopharyngioma

Question 8

Hormone axis that is more susceptible to disruption by acquired conditions than are other hypothalamic-pituitary axes

Answer 8

GH axis

Question 9

Recognized causes of acquired GH deficiency

Answer 9

1) Radiotherapy 2) Malignancy 3) Meningitis 4) Histiocytosis 5) Trauma

Question 10

GH deficiency is almost universal ___ years after radiotherapy with a total dose of ___ Gy

Answer 10

5 years; ≥35 Gy

Question 11

In contrast to other forms of hypopituitarism, acquired type brought about by radiotherapy manifests with

Answer 11

Early rather than delayed puberty

Question 12

Syndrome that involves mutation of the GH receptor

Answer 12

Laron syndrome

Question 13

T/F Child with congenital hypopituitarism is usually of normal size and weight at birth

Answer 13

T

Question 14

Children with severe defects in GH production or action typically fall more than 4 SD below the mean for length by ___ y/o

Answer 14

1

Question 15

Infants with congenital defects of the pituitary or hypothalamus may present with prolonged jaundice, particularly with elevated ___ bilirubin

Answer 15

Conjugated and unconjugated

Question 16

T/F On PE, patients with congenital hypopituitarism present with underdeveloped mandible and chin

Answer 16

T

Question 17

T/F On PE, patients with congenital hypopituitarism present with deep set eyes

Answer 17

F, eyes that are somewhat bulging

Question 18

T/F On PE, patients with congenital hypopituitarism present with late teeth eruption and crowded teeth

Answer 18

T

Question 19

T/F On PE, patients with congenital hypopituitarism present with short neck and small larynx

Answer 19

T

Question 20

T/F On PE, patients with congenital hypopituitarism present with low-pitched voice

Answer 20

F, high-pitched and remains high after puberty

Question 21

T/F On PE, patients with congenital hypopituitarism present with well-proportioned extremities, small hands and feet

Answer 21

T

Question 22

T/F On PE, patients with congenital hypopituitarism present with normal weight for height

Answer 22

T

Question 23

T/F On PE, patients with congenital hypopituitarism present with excess body fat and deficiency of muscle mass

Answer 23

T

Question 24

T/F On PE, patients with congenital hypopituitarism present with large genitals

Answer 24

F, genitals that are small for age

Question 25

T/F On PE, patients with congenital hypopituitarism present with delayed or absent sexual maturation

Answer 25

T

Question 26

T/F On PE, patients with congenital hypopituitarism present with scanty scalp hair

Answer 26

F, lacking facial, axillary, and pubic hair but scalp hair is fine

Question 27

T/F On PE, patients with congenital hypopituitarism present with intelligence that is normal for age

Answer 27

T

Question 28

T/F Acquired hypopituitarism is accompanied by atrophy of the adrenal cortex, thyroid, and gonads

Answer 28

T

Question 29

T/F In acquired hypopituitarism, sexual maturation fails to take place or regresses if already present

Answer 29

T

Question 30

Acquired hypopituitarism presents with slowing of growth and this can antedate neurologic signs and symptoms, especially with what lesion

Answer 30

Craniopharyngiomas

Question 31

Criteria for growth failure

Answer 31

1) Height below the 1st percentile for age and sex, OR 2) Height >2 SD below sex-adjusted mid-parent height

Question 32

T/F Laboratory measures of GH sufficiency is highly specific

Answer 32

F, lack specificity

Question 33

Definitive diagnosis of GH deficiency traditionally requires demonstration of

Answer 33

Absent or low levels of GH in response to stimulation

Question 34

Administration of these substances should rapidly increase GH level in normal children

Answer 34

Insulin, arginine, clonidine, or glucagon

Question 35

Signs of chronic GH deficiency

Answer 35

1) Subnormal linear growth 2) Delayed skeletal age, and 3) Low peak levels of GH (<10 ng/mL) in each of 2 provocative tests

Question 36

Imaging modality appropriate for recognizing suprasellar calcification associated with craniopharyngiomas and bony changes accompanying histiocytosis

Answer 36

CT

Question 37

Severe early-onset MPHD show the triad of

Answer 37

1) A small anterior pituitary gland 2) A missing or attenuated pituitary stalk, and 3) An ectopic posterior pituitary bright spot at the base of the hypothalamus

Question 38

T/F Craniopharyngiomas are common and pituitary adenomas are rare in children with hypopituitarism

Answer 38

T

Question 39

T/F Skeletal maturation is delayed in patients with IGHD

Answer 39

T

Question 40

Patients with systemic conditions other than GH deficiency often have greater loss of length than weight

Answer 40

F, weight than length

Question 41

T/F Likelihood of finding mutations that result in hypopituitarism is increased by consanguinity and occurrence in siblings across generations

Answer 41

T

Question 42

Mutations in ___ and ___ genes result in hypopituitarism without anomalies of other organ systems

Answer 42

T

Question 43

Classically the MCC of hypopituitarism

Answer 43

Pituitary tumors

Question 44

Any lesion that damages the ___, ___ or ___ can cause pituitary hormone deficiency

Answer 44

Hypothalamus, pituitary stalk, anterior pituitary gland

Question 45

___ cells normally account for >50% of pituitary volume

Answer 45

Somatotropes