Narrow trachea in mucopolysaccharidoses
| Autor: | W. Adkins, Mary Ellen Peters, S. Arya, Leonard O. Langer, Carlson Ra, Gilbert Ef |
|---|---|
| Rok vydání: | 1985 |
| Předmět: |
congenital
hereditary and neonatal diseases and abnormalities Pathology medicine.medical_specialty Adolescent Autopsy Glycosaminoglycan Mucolipidoses medicine Humans Radiology Nuclear Medicine and imaging Respiratory system Child skin and connective tissue diseases X ray radiography Tracheal Diseases Mucolipidosis business.industry nutritional and metabolic diseases Mucopolysaccharidoses respiratory system medicine.disease Radiography Trachea Storage material Dysplasia Child Preschool Pediatrics Perinatology and Child Health business Laryngotracheal stenosis |
| Zdroj: | Pediatric Radiology. 15:225-228 |
| ISSN: | 1432-1998 0301-0449 |
| Popis: | Nine of 56 patients with mucopolysaccharidoses (MPS) showed small tracheal diameters on their frontal chest radiographs. Autopsy of an MPS I-H (Hurler disease) patient demonstrated that the small calibre was secondary to deposition of glycosaminoglycan (mucopolysaccharide). Autopsies of two patients with other storage diseases, one with geleophysic dysplasia and one with mucolipidosis II, also exhibited compromise of their airways because of storage material accumulation. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Full text from SpringerLink