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Syndromes: Rapid Recognition and Perioperative Implications
At a Glance, Incidence and Genetic Inheritance, Pathophysiology, Clinical Aspects, Anesthetic Considerations, Other Conditions to Be Considered, References
Very rare disorder characterized by a child with happy face (
= happy, and
= nature). This condition is believed to be a "focal" mucopolysaccharidosis. The presence of dysostosis-multiplex-like changes, predominantly in the hands and feet, associated with cardioand hepatomegaly has been reported. Right ventricular hypertrophy, severe mitral valve stenosis and aortic regurgitation as the patient ages has been observed...."
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