Narrow trachea in mucopolysaccharidoses
Tóm tắt
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.
Tài liệu tham khảo
Baines D, Keneally J (1983) Anaesthetic implications of the mucopolysaccharoidoses: A fifteen-year experience in a children's hospital. Anaesth Intensive Care 11(3): 198
Ballenger CE, Swift TR, Leshner RT, El Gammal TA, McDonald TF (1980) Myelopathy in mucopolysaccharidoses type II (Hunter syndrome) Ann Neurol 7: 382
Bornstein P (1974) Disorder of connective tissue. In: Bondy PK, Rosenberg LE (eds) Duncan's diseases of metabolism, 7th ed. WB Saunders, Philadelphia, p 916
Gilbert EF, Dawson G, ZuRhein GM, Opitz JM, Spranger (1973) Z Kinderheilk 114: 259
Hopkins R, Watson JA, Jones JH, Walker M (1973) Two cases of Hunter's syndrome — the anaesthetic and operative difficulties in oral surgery. Br J Oral Surg 10: 286
Lindsay S, Reilly WA, Gotham TJ, Skahen R (1948) Gargoylism II. study of pathologic lesions and clinical review of twelve cases. Am J Dis Child 75: 595
McKusick VA, Neufeld EF, Kelly TE (1978) The mucopolysaccharide storage disease. In: Stanbury JB, Wyngaarden JB, Fredrickson DS (eds) The metabolic basis of inherited diseases, 4th eds. p 1295
Menu Y, Lallemand D (1981) Détermination du diamétre transversal normal de la trachée chez l'enfant. (Determination of the normal transverse diameter of the trachea in childhood.) Ann Radiol 24: 73
Murray JF (1959) Pulmonary disability in the Hurler syndrome (Lipochondrodystrophy): a study of two cases. N Engl J Med 261: 378
Sly WS (1980) The mucopolysaccharidoses. In: Bondy PK, Rosenberg (eds) Metabolic control and disease, 8th eds. WB Saunders, Philadelphia, p 562
Spranger J, Gilbert E, Arya S, Hoganson G, Opitz J (1984) Geleophysic dwarfism. Am J Med Genetics 19: 497
Young ID, Harper (1982) Mild form of Hunter's syndrome: clinical delineation based on 31 cases. Arch Dis Child 57: 828
Young ID, Harper PS (1979) Long term complications in Hunter's syndrome. Clin Genet 16: 125