Pulmonary Disability in the Hurler Syndrome (Lipochondrodystrophy)

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Abstract
THE Hurler syndrome (lipochondrodystrophy, or gargoylism) is a rare disease with striking manifestations in the fully developed case. Typical features include numerous skeletal deformities, deafness, hepatosplenomegaly and cardiac involvement. Variants of the disease are probably more common, the So-Called formes frustes,1 and may be difficult to distinguish from the large group of eponymic connective-tissue disorders.2 The 2 cases of Hurler's disease reported below present pulmonary-function data on 1 patient and autopsy findings on another for the purpose of emphasizing the respiratory aspects of this disease; pulmonary insufficiency is an important component of the Hurler syndrome and has not received . . .

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