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Literature DB >> 3923421

Narrow trachea in mucopolysaccharidoses.

M E Peters, S Arya, L O Langer, E F Gilbert, R Carlson, W Adkins.

Abstract

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.

Entities: Chemical Disease Species

Mesh：

Year: 1985 PMID： 3923421 DOI： 10.1007/bf02388760

Source DB: PubMed Journal: Pediatr Radiol ISSN： 0301-0449

10 in total

1. Long-term complications in Hunter's syndrome.

Authors: I D Young; P S Harper
Journal: Clin Genet Date: 1979-08 Impact factor: 4.438

2. Gargoylism; lipochondrodystrophy; a review of clinical observations in 18 cases.

Authors: W A REILLY; S LINDSAY
Journal: Am J Dis Child Date: 1948-04

3. I-cell disease, mucolipidosis II. Pathological, histochemical, ultrastructural and biochemical observations in four cases.

Authors: E F Gilbert; G Dawson; G M zu Rhein; J M Opitz
Journal: Z Kinderheilkd Date: 1973

4. Two cases of Hunter's syndrome--the anaesthetic and operative difficulties in oral surgery.

Authors: R Hopkins; J A Watson; J H Jones; M Walker
Journal: Br J Oral Surg Date: 1973-03

5. Determination of the normal transverse diameter of the trachea in childhood.

Authors: Y Menu; D Lallemand
Journal: Ann Radiol (Paris) Date: 1981-01

6. Geleophysic dysplasia.

Authors: J Spranger; E F Gilbert; S Arya; G M Hoganson; J M Opitz
Journal: Am J Med Genet Date: 1984-11

7. Pulmonary disability in the Hurler syndrome (lipochondrodystrophy): a study of two cases.

Authors: J F MURRAY
Journal: N Engl J Med Date: 1959-08-20 Impact factor: 91.245

8. Anaesthetic implications of the mucopolysaccharidoses: a fifteen-year experience in a children's hospital.

Authors: D Baines; J Keneally
Journal: Anaesth Intensive Care Date: 1983-08 Impact factor: 1.669

9. Myelopathy in mucopolysaccharidosis type II (Hunter syndrome).

Authors: C E Ballenger; T R Swift; R T Leshner; T A El Gammal; T F McDonald
Journal: Ann Neurol Date: 1980-04 Impact factor: 10.422

10. Mild form of Hunter's syndrome: clinical delineation based on 31 cases.

Authors: I D Young; P S Harper
Journal: Arch Dis Child Date: 1982-11 Impact factor: 3.791

10 in total

1. Mucolipidosis type III α/β: the first characterization of this rare disease by autopsy.

Authors: Darcy A Kerr; Vincent A Memoli; Sara S Cathey; Brent T Harris
Journal: Arch Pathol Lab Med Date: 2011-04 Impact factor: 5.534

2. Anaesthesia-Relevant Disease Manifestations and Perianaesthetic Complications in Patients with Mucolipidosis-A Retrospective Analysis of 44 Anaesthetic Cases in 12 Patients.

Authors: Luise Sophie Ammer; Nicole Maria Muschol; René Santer; Annika Lang; Sandra Rafaela Breyer; Phillip Brenya Sasu; Martin Petzoldt; Thorsten Dohrmann
Journal: J Clin Med Date: 2022-06-24 Impact factor: 4.964

3. I-Cell Disease (Mucolipidosis II): A Case Series from a Tertiary Paediatric Centre Reviewing the Airway and Respiratory Consequences of the Disease.

Authors: Rachel Edmiston; Stuart Wilkinson; Simon Jones; Karen Tylee; Alexander Broomfield; Iain A Bruce
Journal: JIMD Rep Date: 2018-09-13

Narrow trachea in mucopolysaccharidoses.

1. Long-term complications in Hunter's syndrome.

2. Gargoylism; lipochondrodystrophy; a review of clinical observations in 18 cases.

3. I-cell disease, mucolipidosis II. Pathological, histochemical, ultrastructural and biochemical observations in four cases.

4. Two cases of Hunter's syndrome--the anaesthetic and operative difficulties in oral surgery.

5. Determination of the normal transverse diameter of the trachea in childhood.

6. Geleophysic dysplasia.

7. Pulmonary disability in the Hurler syndrome (lipochondrodystrophy): a study of two cases.

8. Anaesthetic implications of the mucopolysaccharidoses: a fifteen-year experience in a children's hospital.

9. Myelopathy in mucopolysaccharidosis type II (Hunter syndrome).

10. Mild form of Hunter's syndrome: clinical delineation based on 31 cases.

1. Mucolipidosis type III α/β: the first characterization of this rare disease by autopsy.

2. Anaesthesia-Relevant Disease Manifestations and Perianaesthetic Complications in Patients with Mucolipidosis-A Retrospective Analysis of 44 Anaesthetic Cases in 12 Patients.

3. I-Cell Disease (Mucolipidosis II): A Case Series from a Tertiary Paediatric Centre Reviewing the Airway and Respiratory Consequences of the Disease.

Review 4. Clinical overview and treatment options for non-skeletal manifestations of mucopolysaccharidosis type IVA.

5. Anesthesia in a child with Maroteaux-Lamy syndrome undergoing mitral valve replacement.

6. Mannose 6 phosphorylation of lysosomal enzymes controls B cell functions.

7. The characterisation of pulmonary function in patients with mucopolysaccharidoses IVA: A longitudinal analysis.

8. Humoral immune response in adult Brazilian patients with Mucolipidosis III gamma.

9. Severe tracheal and bronchial collapse in adults with type II mucopolysaccharidosis.

Review 10. Mucopolysaccharidosis Type I: A Review of the Natural History and Molecular Pathology.