DERANGED SMOOTH MUSCLE α-ACTIN: A BIO-MARKER OF INTESTINAL PSEUDO-OBSTRUCTION. A CONTROLLED MULTINATIONAL CASE SERIES AND ONTOLOGICAL STUDY.

Chronic idiopathic intestinal pseudo-obstruction (CIIP) is a severe motility disorder associated with significant morbidity. Several histopathological phenotypes have been described, but only a single adult with jejunal smooth muscle α-actin deficiency. This article present a prospective multinational case series of 131 fully clinically and physiologically characterised CIIP patients from 3 European centres investigating smooth muscle α-actin deficiency as a biomarker of this disease.