After a short break I am back with Avery intresting Disease.Before introducing this disease I would say something about it ..How you will feel that all of sudden ur bone is missing from the body?,Or just out of the blue your x-ray reveals that ur bone is fractured? .As if a ghost in your body...The disease is called GORHAM'S STOUT DISEASE OR simply GORHAM'S DISEASE.
Now I will share some detailed knowledge about this rare but fear causing abnormal condition.
GORHAM'S DISEASE also known as BONE DISAPPEARING DISEASE.Itis an extremely uncommon medical condition of the skeletal system which does not have a specific etiology in which there is an uncontrolled proliferation of lymphatic channels within the bone causing resorption and replacement of bone fibrosis. Gorham’s Disease or Disappearing Bone Disease does not have any clear cut treatment and only treatments conducted are clinical trials which mixed results.
GORHAM'S DISEASE is an extremely a rare medical condition with unknown etiology.But it has been diagnosed in males,females and all races of the world.It has been oftenly recognized in children and young people.T he medical reports have been found from each and every continent.
As the disease is not common and commonly misdiagnosed exactly how many people are diagnosed with this disease is not clearly known. But a figure of about 200 cases has been found by literature.The risk factor is unknown but it has been suggested that trauma or any sort of injury is a basic risk factor
CAUSES- The main cause is unknown.But the mechanism has been found.Actually in our body continuously wear and tear of bone goes on under osteoblasts and osteoclasts.And a proper balance maintains the homeostasis of the body but in this bone disappearing disease there is an imbalance of the enzymatic activity of these bone forming and bone destructing cells. Gorham and Stout found that vascular anomalies always occupied space that normally would be filled with new bone and speculated that the presence of angiomatosis may lead to chemical changes in the bone. The Gorham-Stout syndrome may be, essentially, a monocentric bone disease with a focally increased bone resorption due to an increased number of paracrine or autocrine stimulated hyperactive osteoclasts. The resorbed bone is replaced by a markedly vascularized fibrous tissue.
SYMPTOMS- The initial phase is very aggressive and active followed by slow relapsing phase.But few symptoms have been observed such as
Acute onset of localized pain and swelling
Reduced range of motion and generalized weakness
Increased risk of fracture
Difficulty breathing and chest pain may be present if the disease is present in the ribs.
When Gorham’s disease affects the maxillofacial (jaw) bones, symptoms can include jaw pain, loose teeth, fractures, and facial deformity
Some people with Gorham’s disease may develop pleural effusion, a condition where there is ‘water on the lungs’ due to the build-up of excess fluid between the pleura or thin membranes that line the lungs and the inside of the chest cavity. Because the pleura help to lubricate and are important for healthy respiratory function, Gorham’s disease can lead to respiratory distress (i.e. breathing problems) and even respiratory failure
COMPLICATIONS-Pleural effusion, haemaengioma, pathological fracture, pleural carditis.
DIAGNOSIS-MRI,CT SCAN AND BIOPSY
TREATMENT-Antivascular and immunosuppressant medications have been proved effective in slowing down the progression and even reversing the damage effects caused due to this disease and at last stabilizing the body.The medicines are sirolimus,interferon alpha-2b ,Bisphosphinates.
SURGERY- In this case the affected part is being removed but surgery is not so effective, as it cannot cure the disease.
Radiation therapy is recommended for treating pain and other symptoms but it's delayed complications makes it 2nd line of treatment.
PREVENTION-Still not clear but research is going on.
My motto to share this knowledge disease was to make people aware and understand thaat regular check up complete body us must as anything can disturb your body.And any change in daily routine is felt or observed so be serious and go for tests as most of the people either takes body tests to be lightly or just ignore which can be hazardous for life..As it is very well said TIME NEVER COMES BACK.