Short bowel syndrome – characterisation of an orphan condition with many phenotypes

Abstract

Introduction: In this review, the orphan condition of short bowel syndrome (SBS) is described thereby providing the theoretical premises for the non-surgical management and treatment.

Areas covered: After a brief summary of the normal human intestinal assimilation, this review focuses on the characterisation of adult SBS patients. It is evident, that adult patients SBS are truly heterogeneous based on their individual pathophysiological characteristics. SBS patients with inadequate absorption of macronutrients, fluid, electrolytes, trace elements and vitamins to meet the metabolic requirements, suffer from intestinal failure and depend on parenteral support (PS, i.e., parenteral nutrition and/or intravenous fluids and electrolytes). Although PS can be provided at home, the symptoms of SBS and the burden and potential complications of PS significantly impair the quality of life in these patients. In order to best target the treatment and prevent or minimize complications of long-term PS, the types, groups, grading and psychosocial characteristics of the adult patients with SBS are presented.

Expert opinion: Due to the heterogeneity and complexity of the SBS patients, the expert opinion is, that the successful long-term management of SBS patients requires the setup of an organisational framework and preferably ‘Centres of Excellence', as it is routine in some countries in Europe, with knowledge, facilities, and treatments to diminish the human distress and health care burden associated with this rare condition. With the increasing numbers of IF patients, such clinical care centres for these complex patients need to be increasingly developed within academic medical centres around the world.

Keywords::

• home parenteral nutrition
• intestinal failure
• intestinal insufficiency
• short bowel syndrome

Notes

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