Is McCune-Albright syndrome the same as fibrous dysplasia?

Is McCune-Albright syndrome the same as fibrous dysplasia?

McCune-Albright syndrome is a disorder that affects the bones, skin, and several hormone-producing (endocrine) tissues. People with McCune-Albright syndrome develop areas of abnormal scar-like (fibrous) tissue in their bones, a condition called polyostotic fibrous dysplasia.

Can McCune-Albright syndrome be cured?

There is no cure for Fibrous dysplasia/McCune-Albright Syndrome. However, many aspects of the disease can be managed with medications or surgery.

Can boys get McCune-Albright?

Children with McCune-Albright condition have weakened bones that break easily. The disorder is present at birth, but symptoms may not appear until later in childhood. McCune-Albright affects boys and girls equally.

Why does McCune-Albright syndrome cause precocious puberty?

Precocious puberty in McCune-Albright syndrome is gonadotropin-independent. This means that it is not caused by early release of gonadotropins (luteinizing hormone and follicle-stimulating hormone), but, instead, the cause is the early secretion of high levels of sex hormones (male androgens and female estrogens).

Is Fibrous dysplasia inherited?

Although fibrous dysplasia is a genetic disorder, it’s caused by a gene mutation that’s not passed from parent to child. There’s no cure for the disorder.

What is the cause of McCune-Albright syndrome?

McCune-Albright syndrome is caused by a mutation in a gene called GNAS1. This gene mutation occurs after fertilization of the embryo (somatic mutation) and is therefore not inherited, nor will affected individuals pass the mutation on to their children.

Is McCune-Albright rare?

McCune-Albright syndrome (MAS) is an extremely rare disorder that classically affects the bones, skin, and endocrine system.

What hormone causes fibrous dysplasia?

The mechanism by which growth hormone stimulates fibrous dysplasia of bone is unknown but may be related to increased proliferation of bone marrow stromal cells. Impaired differentiation of these cells is a primary effect of the activation of Gαs, driving the formation and expansion of fibrous dysplasia lesions.

Can you have café au lait spots without neurofibromatosis?

Indeed, anyone can have 1 or 2 cafe-au-lait spots without having neurofibromatosis. The only significance to the cafe-au-lait spot is that it suggests the possibility that a person might have NF1. People with NF1 usually have many cafe-au-lait spots, sometimes hundreds, and almost always more than 6.

Does fibrous dysplasia get worse with age?

Pain is more likely to occur if the bone affected is one of the weight-bearing bones of the leg or pelvis. Pain caused by fibrous dysplasia generally begins as a dull ache that worsens with activity and lessens with rest. It can get progressively worse over time.

Can fibrous dysplasia be cured?

Although fibrous dysplasia is a genetic disorder, it’s caused by a gene mutation that’s not passed from parent to child. There’s no cure for the disorder. Treatment, which may include surgery, focuses on relieving pain and repairing or stabilizing bones.

How common is McCune-Albright syndrome?

McCune-Albright syndrome affects males and females in equal numbers. Precocious puberty is more common in females. The disorder is estimated to affect 1 in 100,000 to 1 in 1,000,000 individuals in the general population.