**Core Concept**
Albright's syndrome, also known as McCune-Albright syndrome, is a rare genetic disorder characterized by the triad of polyostotic fibrous dysplasia, café-au-lait skin spots, and precocious puberty. It is caused by mutations in the GNAS1 gene, which encodes the stimulatory G-protein alpha subunit (Gsα).
**Why the Correct Answer is Right**
The Gsα subunit plays a crucial role in activating adenylate cyclase and increasing cyclic AMP (cAMP) levels in various tissues, including the hypothalamus and pituitary gland. This leads to the premature activation of the hypothalamic-pituitary-gonadal axis, resulting in precocious puberty. The café-au-lait skin spots are thought to result from the increased activity of melanocortin 1 receptor (MC1R) due to the Gsα mutation.
**Why Each Wrong Option is Incorrect**
**Option A:** Polyostotic fibrous dysostosis is a key feature of Albright's syndrome, characterized by the replacement of bone marrow with fibrous tissue, leading to bone deformities and pain.
**Option B:** Precocious puberty is a hallmark of Albright's syndrome, manifesting as early onset of puberty, often before the age of 8 in girls and 9 in boys.
**Option C:** Café-au-lait skin spots are a distinctive feature of Albright's syndrome, typically appearing as light to dark brown patches on the skin.
**Clinical Pearl / High-Yield Fact**
Albright's syndrome is a rare genetic disorder that highlights the importance of G-protein signaling in regulating various physiological processes, including hormone secretion and melanogenesis.
**Correct Answer: D.**
Free Medical MCQs · NEET PG · USMLE · AIIMS
Access thousands of free MCQs, ebooks and daily exams.
By signing in you agree to our Privacy Policy.