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Short bowel syndrome
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== '''Late phase management''' == * Medical ** TPN/fluids/micronutrients as required at steady state *** B12 *** Fat-soluble vitamins *** Calcium, magnesium, zinc, selenium ** Teduglutide (GLP-2 analogue) - if unable to wean from TPN despite aggressive therapy - initiates and maintains small bowel adaptive response to resection and improves nutrient absorption ** Prevent and treat bone disease and other complications * Surgical strategies - limited success ** Preserve existing bowel length and restore continuity ** Delay intestinal transit time *** Construction of various valves and sphincters *** Anti-peristaltic segments - moderate success *** Abandoned techniques: **** Colonic interposition **** Recirculating loops of bowel **** Retrograde electrical pacing ** Increase absorptive area *** Bianchi procedure - intestinal tapering and lengthening procedure - serial transverse enteroplasty - possibly gets good results in well-selected patients- 90% show improved nutrition, and can lengthen by up to 55% in some patients - but high-risk of complications. Need a distended gut for this to work, which is not commonly seen in adults. Increase length but more importantly slow transit. Starkey has never seen it. ** Intestinal transplantation *** Standard of care for patients in whom intestinal rehabilitation has failed and are at risk of life-threatening TPN complications (impending liver failure, thrombosis of more than two major access veins, frequent severe line infections, dehydration) *** One year survival of 77%; 5 year survival 58%, even in modern times. Lots of perioperative mortality but then ongoing attrition. *** Can be done in combination with liver - 5 year survival 54% *** No role for transplantation in the acute setting - wait for extent of disease to become clear *** Recognised by USA medicare system in 2000 - progressive uptake since then. Most of the initial indications were related to complications of home TPN, 'failure of TPN', see above. *** There are about 5000 people worldwide, as of June 2023, who have had a transplant at a recognised centre, based on registrary data, and only 100-200 per year nowadays. 13 Australians transplanted as of June 2023.
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