Normal stature with minimal or absent pubeal development may be seen in:
Correct Answer: Kallmann syndrome
Description: Testicular feminization is a syndrome of androgen insensitivity, characterized by a normal 46 XY genotype; normal female phenotype during childhood; tall stature; and "normal" breast development with absence of axillary and pubic hair. Gonadal dysgenesis (e.g., 45X Turner syndrome) is characterized by sho stature and absence of pubeal development; in these girls, the ovaries are either absent or streak gonads that are non-functional. Kallmann syndrome (hypogonadotropic hypogonadism) should be suspected in patients of normal stature with delayed or absent pubeal development, especially when associated with the classic finding of anosmia. These individuals have a structural defect of the CNS involving the hypothalamus and the olfactory bulbs. The hypothalamus does not secrete GnRH in normal pulsatile fashion, if at all.
Category:
Gynaecology & Obstetrics
Get More
Subject Mock Tests
Practice with over 200,000 questions from various medical subjects and improve your knowledge.
Attempt a mock test nowMock Exam
Take an exam with 100 random questions selected from all subjects to test your knowledge.
Coming SoonGet More
Subject Mock Tests
Try practicing mock tests with over 200,000 questions from various medical subjects.
Attempt a mock test now