Warning to Health Professionals
There is the potential, due to the cardio respiratory complications of the condition, for patients with Alström Syndrome to become hypoxic and rapidly deteriorate when suffering from undercurrent illness. We would therefore recommend that oxygen saturations should be part of the routine monitoring of these patients during any in-patient hospital stay. In addition it is important that any deterioration noted, is reported for assessment by the relevant medical personnel.
Alström Syndrome can be accompanied by a number of inter-related changes which can compromise cardio respiratory function during acute illness particularly chest infection.
These changes include kyphoscoliosis, mild pulmonary fibrosis and cardiomyopathy. It is therefore important to consider intensive oxygen therapy, early intensive care involvement and echocardiography when even borderline hypoxia occurs (oxygen saturation less than 95% on air) in the context of acute illness.