Severe Intestinal Dysmotility (SD)

Severe intestinal dysmotility (SD) is a rare and severe clinical condition that significantly impacts patients' quality of life and life expectancy. SD is characterized by a marked impairment of intestinal propulsion due to alterations in one or more cellular components of the enteric system, including neurons, glial cells, interstitial cells of Cajal, and muscle cells.

SD is divided into two main clinical phenotypes:

• Chronic Intestinal Pseudo-Obstruction (CIPO): Characterized by severe and recurrent episodes of sub-occlusion, resembling mechanical obstruction, with radiologically visible intestinal dilation but without lesions occluding the lumen.
• Enteric Dysmotility (ED): Characterized by altered intestinal motility detected via manometry but without recurrent sub-occlusive episodes or dilation of the intestinal loops.

Despite advances in understanding structural alterations, clinical manifestations, and symptoms, the underlying mechanisms of SD remain largely unknown. For this reason, our laboratory is currently conducting research projects aimed at addressing unresolved questions.

Question 1:

Do other factors play a pathological role in SD? Could they be targeted in future therapies?

To answer this question, we refer to two projects that go specifically to investigate these issues:

• The vascular component in SD: a new pathophysiological and therapeutic target.
• Extracellular Matrix Abnormalities in the Digestive Tract: Pathological Implications of Collagen Alterations in SD.

Question 2:

Is it possible to define a universally recognized gold standard for evaluating the cellular components of the enteric nervous system (ENS) that would ensure uniformity in data collected across laboratories worldwide?

The answer to this question is sought with the project on morphometric studies aimed at defining a standardized quantification method for ENS impairment.