Prognosis
Fibrothorax complicating another condition, such as tuberculous pleuritis, empyema, or acute haemothorax often spontaneously resolves in 3-6 months.The prognosis after surgical decortication is variable and depends on the health of the underlying lung before the procedure takes place. If the lung was otherwise healthy, then certain aspects of lung function, such as vital capacity, may improve after decortication. If, however, the lung had significant disease, then lung function often does not improve and may even deteriorate after such intervention. The duration of fibrothorax does not affect prognosis.The mortality of surgery is less than 1% overall, but rises to 4-6% in the elderly. Other factors predicting poorer surgical outcomes include intraoperative complications, incomplete surgery, lung disease beyond the fibrothorax being treated, and specific causes of fibrothorax such as asbestosis. Epidemiology
Sporadic cases are rarely reported in the medical literature, for example, due to iatrogenic or postoperative complications. Fibrothorax is rare in developed countries, mainly due to a lower incidence of tuberculosis. The condition is far more common in workers exposed to asbestos, with 5–13.5% of those exposed subsequently developing some degree of pleural fibrosis, sometimes not diagnosed until decades after the initial exposure. == References ==
Patients who received successful bone marrow transplants had a 2-year survival rate of 68% and a 10-year survival rate of 64%. Patients who did not receive bone marrow transplants had a significantly reduced lifespan, with a median age of 6.8 years. Epidemiology
Hurler syndrome has an overall frequency of one per 100,000. Combined, all of the mucopolysaccharidoses have a frequency of approximately one in every 25,000 births in the United States. Research
Gene therapy
A great deal of interest exists in treating MPS I with gene therapy. In animal models, delivery of the iduronidase gene has been accomplished with retrovirus, adenovirus, adeno-associated virus, and plasmid vectors. Mice and dogs with MPS I have been successfully treated with gene therapy. Most vectors can correct the disease in the liver and spleen, and can correct brain effects with a high dosage. Gene therapy has improved survival, neurological, and physical symptoms; however, some animals have developed unexplained liver tumors. If safety issues can be resolved, gene therapy may provide an alternative human treatment for MPS disorders in the future.Sangamo Therapeutics, headquartered in Richmond, California, is currently conducting a clinical trial involving gene editing using Zinc Finger Nuclease (ZFN) for the treatment of MPS I. History
In 1919, Gertrud Hurler, a German pediatrician, described a syndrome involving corneal clouding, skeletal abnormalities, and mental retardation. A similar disease of "gargoylism" had been described in 1917 by Charles A. Hunter. Hurler did not mention Hunters paper. Because of the communications interruptions caused by World War I, it is likely that she was unaware of his study.