Biomaterial Database

Upper airway obstruction in Hunter syndrome. 1986

I Brama, and I Gay, and R Feinmesser, and C Springer

Patients with Hunter syndrome may have symptoms of hoarseness, stridor and breathing difficulties as a result of laryngeal and tracheal involvement. In planning their evaluation, we must prefer non-invasive methods as X-ray or CT scan, and avoid doing endotracheal intubation or bronchoscopies. Review of adult cases in the literature and description of the only case of a child with Hunter syndrome having life-threatening complications of his upper airways is discussed in this report. In this case and in the literature we cannot exclude intubation or bronchoscopy as a serious aggravating factor, causing further narrowing of the larynx.

UI	MeSH Term	Description	Entries
D007829	Laryngostenosis	Developmental or acquired stricture or narrowing of the LARYNX. Symptoms of respiratory difficulty depend on the degree of laryngeal narrowing.	Laryngeal Stenosis,Acquired Laryngeal Stenosis,Acquired Subglottic Stenosis,Congenital Subglottic Stenosis,Acquired Laryngeal Stenoses,Acquired Subglottic Stenoses,Congenital Subglottic Stenoses,Laryngeal Stenoses,Laryngeal Stenoses, Acquired,Laryngeal Stenosis, Acquired,Laryngostenoses,Stenoses, Acquired Laryngeal,Stenoses, Acquired Subglottic,Stenoses, Congenital Subglottic,Stenoses, Laryngeal,Stenosis, Acquired Laryngeal,Stenosis, Acquired Subglottic,Stenosis, Congenital Subglottic,Stenosis, Laryngeal,Subglottic Stenoses, Acquired,Subglottic Stenoses, Congenital,Subglottic Stenosis, Acquired,Subglottic Stenosis, Congenital
D008173	Lung Diseases, Obstructive	Any disorder marked by obstruction of conducting airways of the lung. AIRWAY OBSTRUCTION may be acute, chronic, intermittent, or persistent.	Obstructive Lung Diseases,Obstructive Pulmonary Diseases,Lung Disease, Obstructive,Obstructive Lung Disease,Obstructive Pulmonary Disease,Pulmonary Disease, Obstructive,Pulmonary Diseases, Obstructive
D008297	Male		Males
D001999	Bronchoscopy	Endoscopic examination, therapy or surgery of the bronchi.	Bronchoscopic Surgical Procedures,Surgical Procedures, Bronchoscopic,Bronchoscopic Surgery,Surgery, Bronchoscopic,Bronchoscopic Surgeries,Bronchoscopic Surgical Procedure,Bronchoscopies,Surgeries, Bronchoscopic,Surgical Procedure, Bronchoscopic
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D014057	Tomography, X-Ray Computed	Tomography using x-ray transmission and a computer algorithm to reconstruct the image.	CAT Scan, X-Ray,CT Scan, X-Ray,Cine-CT,Computerized Tomography, X-Ray,Electron Beam Computed Tomography,Tomodensitometry,Tomography, Transmission Computed,X-Ray Tomography, Computed,CAT Scan, X Ray,CT X Ray,Computed Tomography, X-Ray,Computed X Ray Tomography,Computerized Tomography, X Ray,Electron Beam Tomography,Tomography, X Ray Computed,Tomography, X-Ray Computer Assisted,Tomography, X-Ray Computerized,Tomography, X-Ray Computerized Axial,Tomography, Xray Computed,X Ray Computerized Tomography,X Ray Tomography, Computed,X-Ray Computer Assisted Tomography,X-Ray Computerized Axial Tomography,Beam Tomography, Electron,CAT Scans, X-Ray,CT Scan, X Ray,CT Scans, X-Ray,CT X Rays,Cine CT,Computed Tomography, Transmission,Computed Tomography, X Ray,Computed Tomography, Xray,Computed X-Ray Tomography,Scan, X-Ray CAT,Scan, X-Ray CT,Scans, X-Ray CAT,Scans, X-Ray CT,Tomographies, Computed X-Ray,Tomography, Computed X-Ray,Tomography, Electron Beam,Tomography, X Ray Computer Assisted,Tomography, X Ray Computerized,Tomography, X Ray Computerized Axial,Transmission Computed Tomography,X Ray Computer Assisted Tomography,X Ray Computerized Axial Tomography,X Ray, CT,X Rays, CT,X-Ray CAT Scan,X-Ray CAT Scans,X-Ray CT Scan,X-Ray CT Scans,X-Ray Computed Tomography,X-Ray Computerized Tomography,Xray Computed Tomography
D014135	Tracheal Stenosis	A pathological narrowing of the TRACHEA.	Stenoses, Tracheal,Stenosis, Tracheal,Tracheal Stenoses
D016532	Mucopolysaccharidosis II	Systemic lysosomal storage disease marked by progressive physical deterioration and caused by a deficiency of L-sulfoiduronate sulfatase. This disease differs from MUCOPOLYSACCHARIDOSIS I by slower progression, lack of corneal clouding, and X-linked rather than autosomal recessive inheritance. The mild form produces near-normal intelligence and life span. The severe form usually causes death by age 15.	Gargoylism, Hunter Syndrome,Hunter's Syndrome,Iduronate 2-Sulfatase Deficiency,Iduronate Sulfatase Deficiency,Sulfoiduronate Sulfatase Deficiency,Hunter Syndrome,Hunter Syndrome Gargoylism,I2S Deficiency,Mucopolysaccharidosis 2,Mucopolysaccharidosis Type 2,Mucopolysaccharidosis Type II,Deficiency, I2S,Deficiency, Iduronate 2-Sulfatase,Deficiency, Iduronate Sulfatase,Deficiency, Sulfoiduronate Sulfatase,Hunters Syndrome,Iduronate 2 Sulfatase Deficiency,Syndrome, Hunter,Syndrome, Hunter's