Chapter 402 Hypopituitarism

Question 1

Hypothalamic dysfunction and hypopituitarism may result from dysgenesis of the

Answer 1

septum pellucidum or corpus

calosum

Question 2

These children exhibit variable combinations of cleft palate, syndactyly, ear deformities, hypertelorism, optic nerve hypoplasia, micropenis, and anosmia. Pituitary dysfunction leads to diabetes insipidus, growth hor- mone (GH) deficiency and short stature, and, occasionally, thyroid- stimulating hormone (TSH) deficiency.

Answer 2

Septic optic dysplasia

Question 3

results from defective hypothalamic gonadotropin-releasing hormone (GnRH) synthesis and is associated with anosmia or hyposmia due to olfactory bulb agenesis or hypoplasia

Answer 3

Kallmann syndrome

Question 4

Classically, the syndrome may also be associated with color blindness, optic atrophy, nerve deafness, cleft palate, renal abnormalities, cryptorchidism, and neurologic abnormalities such as mirror movements.

Answer 4

Kallmann syndrome

Question 5

Males present with delayed puberty and pronounced hypogonadal features, including micropenis, probably the result of low testosterone levels during infancy. Females present with primary amenorrhea and failure of secondary sexual development.

Answer 5

Kallmann syndrome

Question 6

Kallmann syndrome and other causes of congenital GnRH deficiency are characterized by

Answer 6

low luteinizing hormone (LH) and follicle-stimulating hormone (FSH) levels and low concentrations of sex steroids (testosterone or estradiol).

Question 7

Long-term treatment of males with ______ restores pubertal development and secondary sex characteristics; women can be treated with cyclic ____

Answer 7

1: human chorionic gonadotropin (hCG) or testosterone

2. estrogen and progestin.

Question 8

Fertility also may be restored by the administration of _____ or by using a portable infusion pump to deliver subcutaneous, pulsatile ____.

Answer 8

gonadotropins / GnRH

Question 9

This very rare genetically heterogeneous disorder is characterized by mental retardation, renal abnormalities, obesity, and hexadactyly, brachydactyly, or syndactyly

Answer 9

Bardet-Biedl Syndrome

Question 10

In Bardet-Biedl Syndrome, GnRH deficiency occurs in ___of males and half of affected females. Retinal degeneration begins in early childhood, and most patients are blind by age

Answer 10

75%

30.

Question 11

Deficiencies of leptin or its recep- tor cause a broad spectrum of hypothalamic abnormalities, includ- ing

Answer 11

hyperphagia, obesity, and central hypogonadism

Question 12

This is a contiguous gene syndrome that results from deletion of the paternal copies of the imprinted SNRPN gene, the NECDIN gene, and possibly other genes on chromosome 15q.

Answer 12

prader-Willi Syndrome

Question 13

Is associated with hypogonadotropic hypogonadism, hyperphagia-obesity, chronic muscle hypotonia, mental retardation, and adult-onset diabetes mellitus

Answer 13

Prader- Willi syndrome

Question 14

This occurs most often in postpartum women; it usually presents with hyperprolactinemia and MRI evidence of a prominent pituitary mass that often resembles an adenoma, with mildly elevated PRL levels.

Answer 14

Lymphocytic Hypophysitis

Question 15

In lymphocytic hypophysitis, The inflammatory process often resolves after several months of _____ treatment

Answer 15

glucocorticoid

Question 16

Acute intrapituitary hemorrhagic vascular events can cause substantial damage to the pituitary and surrounding sellar structures

Answer 16

Pituitary Apoplexy

Question 17

is an endocrine emergency that may result in severe hypoglycemia, hypotension and shock, central nervous system (CNS) hemorrhage, and death. Acute symptoms may include severe headache with signs of meningeal irritation, bilateral visual changes, ophthalmoplegia, and, in severe cases, cardiovascular collapse and loss of consciousness.

Answer 17

Apoplexy

Question 18

are indications for early surgery in pituitary apoplexy

Answer 18

severe ophthalmoplegia or visual deficits

Question 19

causes men- strual disorders and infertility in women and decreased sexual function, infertility, and loss of secondary sexual characteristics in men.

Answer 19

Gonadotropin deficiency

Question 20

causes growth disorders in children and leads to abnormal body com- position in adults

Answer 20

GH deficiency

Question 21

causes growth retardation in children and features of hypothyroidism in children and adults.

Answer 21

TSH deficiency

Question 22

leads to hypocortisolism with relative preservation of mineralocorticoid production

Answer 22

ACTH deficiency

Question 23

Peak growth rates occur during midpuberty when bone age is

Answer 23

12 (girls) or 13 (boys)

Question 24

Mean growth velocity is____ in later childhood and usually is maintained within a given range on a standardized percentile chart.

Answer 24

~6 cm/year

Question 25

deficiency is characterized by short stature, micropenis, increased fat, high-pitched voice, and a propensity to hypoglycemia due to relatively unopposed insulin action

Answer 25

GH deficiency

Question 26

Replacement therapy with recombinant GH ____ restores growth velocity in GH-deficient children to___

Answer 26

(0.02–0.05 mg/kg per day SC)

~10 cm/year.

Question 27

Go replacement Contraindications to therapy include the presence of an

Answer 27

active neoplasm, intracranial hypertension, and uncontrolled diabetes and retinopathy.

Question 28

is the most common presenting feature of adult hypo- pituitarism even when other pituitary hormones are also deficient.

Answer 28

Hypogonadism