January 25, 2015
Re: Obstructive Trachea, breaking news!
Trachea obstruction in Morquio patient
Chest CT suggests the tortuous and redundant trachea with buckling of main trachea, and the stretched and abnormally shaped lumen of the bronchus. Sagittal section shows narrowing trachea.
Trachea: black, Red arrows: abnormal trachea.
Yasuda E, Fushimi K, Suzuki Y, Shimizu K, Takami T, Zustin J, Patel P, Ruhnke K, Shimada T, Boyce B, Kokas T, Barone C, Theroux M, Mackenzie W, Nagel B, Ryerse JS, Orii KE, Iida H, Orii T, Tomatsu S. Pathogenesis of Morquio A syndrome: An autopsied case reveals systemic storage disorder, Mol. Genet. Metab. 2013109(3):301-11.
Dear Morquio Community,
We had an urgent incidence on obstructive trachea in a 16-year-old Morquio patient. His respiratory situation went down and was at a high risk. He needed an artificial ventilation and had an urgent operation at Nemours/Alfred I. duPont Hospital for Children in Wilmington, Delaware. The operation was successfully done by cardiologist, Dr. Pizarro and his team. The child's life was saved. We are very pleased to hear this great news since many Morquio patients have died in the past due to airway complications.
Dr. Pizarro will make a presentation of this new surgical procedure at the up coming Morquio Conference (July 17-19, 2015). Dr. Tomatsu and his research team are now researching the mechanism of trachea obstruction with Dr. Pizarro.
Please let us know if you have any concern about trachea obstruction.
Dr. Shunji Tomatsu