Hormone Resistance Syndromes Flashcards Preview

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Flashcards in Hormone Resistance Syndromes Deck (8)
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1
Q

How does LH-R manifest in men vs. women?

A

Male: pseudohermaphraditism (low testosterone–>failure to develop male structures)
Female: Ovarian resistance

2
Q

How does FSH-R manifest in men vs. women?

A

Male: infertility (testosterone; but no Sertoli cells for spermatogenesis)
Female: ovarian resistance (no follicles to produce estrogen)

3
Q

GH-R Restance: clinical features (4), Hormone levels and Rx

A

Short stature, frontal bossing, high pitched voice, obesity
High GH, low IGF-1
Rx: IGF-1

4
Q

Androgen Insensitivity Syndrome

A

XY genotype with female external phenotype
Inguinal testes
Absent uterus (testes still secrete AMH)
High testosterone, LH

5
Q

AIS: Key Points

A

Wide phenotypic spectrum
Management involves psychosocial as well as hormonal issues
Distinguishes roles of T, MIS, DHT (5a-reductase)

6
Q

Estrogen-R Resistance in Male: Clinical features (6)

A

Tall stature, prolonged linear growth, bone mineral density low, impaired glucose tolerance, abnormal lipid profile, early coronary atherosclerosis

7
Q

ER Restistance in Females: Cliical features (5)

A
Tanner 1 breasts
Tanner 4 pubic hair
Cystic ovaries
Low BMI/BMD
High estradiol, nl LH/FSH
8
Q

RTH Resistance:

A

Dominant negative: mutated receptor messes with normal receptor leading to pathogenesis

T4 builds up and is converted to TRa–>tachycardia