Pulmonary Disability in the Hurler Syndrome (Lipochondrodystrophy)

Murray, John F.

doi:10.1056/nejm195908202610804

Cited by 23 publications

(5 citation statements)

References 26 publications

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“…This potentially lethal complication is poorly documented. A 43-year-old man with alleged Hurler's syndrome, who in retrospect probably had Hunter's syndrome, has been described (Murray 1959) as benefiting from an elective tracheostomy inserted a t the age of 32 years. The findings in this individual do not correspond exactly to those in our patient, for he is reported as having a "small irregular larynx".…”

Section: Discussionmentioning

confidence: 99%

Long‐term complications in Hunter's syndrome

Young

Harper

1979

Clinical Genetics

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This report describes the clinical and investigative findings in a 31-year-old man with Hunter's syndrome, showing evidence of severe multisystem involvement. Papilloedema has been present for at least 8 years with no evidence of progressive visual impairment. His most serious and life-threatening complications are laryngeal oedema and tracheal narrowing, which both compromise his respiratory function and constitute a major anaesthetic risk.

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Section: Discussionmentioning

confidence: 99%

Long‐term complications in Hunter's syndrome

Young

Harper

1979

Clinical Genetics

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“…However, the restricted pulmonary function seen in this patient has been reported in other chest wall deformities, such as kyphoscoliosis, pectus excavatum, ankylosing spondylitis, mucopolysaccharidosis, and obesity, as well as in experimental chest strapping (12)(13)(14)(15)(16). Although neuromuscular weakness can explain the functional abnormalities, this is probably not true in this patient because she can generate greater than 20 mm Hg mouth pressure both on inspiration and expiration.…”

Section: Discussionmentioning

confidence: 54%

Pulmonary Function in Myositis Ossificans Progressiva¹

Buhain¹,

Gumpeni²,

Berger³

1974

Am Rev Respir Dis

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______________________________________________________ __Pulmonary function was evaluated in a patient with myositis ossificans progressiva, a rare disorder characterized by ossification of muscles,. fascia, tendons, and ligaments, and associated with congenital digital anomalies. Results showed diminished lung volumes with normal airway dynamics and lung elastic recoil, suggesting a restricted chest cage disorder.

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“…The respiratory problems which may occur in patients with mucopolysaccharidoses have been reported previously (Murray 1959;Semenza & Pyeritz 1988). lJpper airway findings include airway narrowing due to hypoplasia or infiltration with storage material, and there may be narrowing of the tracheobronchial tree.…”

Section: Discussionmentioning

confidence: 99%

Anaesthesia and the mucopolysaccharidoses: a review of patients treated by bone marrow transplantation

FCAnaes

Ffaracs

Mrcp

1992

Pediatric Anesthesia

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Summa yThe mucopolysaccharidoses present many potential difficulties to the anaesthetist. A review was undertaken of the last 10 years' experience at a paediatric hospital which has a special interest in the treatment of these conditions using bone marrow transplantation. Thirty-five patients underwent 150 general anaesthetics. There was a high incidence of airway problems and cardiorespiratory disease. Laryngoscopy and tracheal intubation was difficult in 40% of patients with Hurler's syndrome and 71% of those with Hunter's syndrome. A tracheal tube of smaller than predicted internal diameter was commonly required. Cardiorespiratory problems included both myocardial and valvular disease which could often be identified preoperatively as could kyphoscoliosis. The implications for anaesthesia are discussed.

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Pulmonary Disability in the Hurler Syndrome (Lipochondrodystrophy)

Cited by 23 publications

References 26 publications

Long‐term complications in Hunter's syndrome

Long‐term complications in Hunter's syndrome

Pulmonary Function in Myositis Ossificans Progressiva¹

Anaesthesia and the mucopolysaccharidoses: a review of patients treated by bone marrow transplantation

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Pulmonary Disability in the Hurler Syndrome (Lipochondrodystrophy)

Cited by 23 publications

References 26 publications

Long‐term complications in Hunter's syndrome

Long‐term complications in Hunter's syndrome

Pulmonary Function in Myositis Ossificans Progressiva1

Anaesthesia and the mucopolysaccharidoses: a review of patients treated by bone marrow transplantation

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Product

Resources

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Pulmonary Function in Myositis Ossificans Progressiva¹