Severe short bowel syndrome (SBS) is a major cause of chronic (Type 3) intestinal failure (IF) where structural and functional changes contribute to malabsorption and risk of micronutrient deficiencies. Chronic IF may be reversible, depending on anatomy and intestinal adaptation, but most patients require long-term nutritional support, generally in the form of parenteral nutrition (PN). SBS management begins with dietary changes and pharmacologic therapies taking into account individual anatomy and physiology, but these are rarely sufficient to avoid PN. New hormonal therapies targeting intestinal adaptation hold promise. Surgical options for SBS including intestinal transplant are available, but have significant limitations. Home PN (HPN) is therefore the mainstay of treatment for severe SBS. HPN involves chronic administration of macronutrients, micronutrients, fluid, and electrolytes via central venous access in the patient's home. HPN requires careful clinical and biochemical monitoring. Main complications of HPN are related to venous access (infection, thrombosis) and metabolic complications including intestinal failure associated liver disease (IFALD). Although HPN significantly impacts quality of life, outcomes are generally good and survival is mostly determined by the underlying disease. As chronic intestinal failure is a rare disease, registries are a promising strategy for studying HPN patients to improve outcomes.
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North West London Hosp NHS Trust, Dept Oral & Maxillofacial Surg, Harrow HA1 3UJ, Middx, EnglandNorth West London Hosp NHS Trust, Dept Oral & Maxillofacial Surg, Harrow HA1 3UJ, Middx, England
Lee, A. M.
Gabe, S. M.
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St Marks Hosp, Lennard Jones Intestinal Failure Unit, Harrow HA1 3UJ, Middx, EnglandNorth West London Hosp NHS Trust, Dept Oral & Maxillofacial Surg, Harrow HA1 3UJ, Middx, England
Gabe, S. M.
Nightingale, J. M.
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St Marks Hosp, Lennard Jones Intestinal Failure Unit, Harrow HA1 3UJ, Middx, EnglandNorth West London Hosp NHS Trust, Dept Oral & Maxillofacial Surg, Harrow HA1 3UJ, Middx, England
Nightingale, J. M.
Fiske, J.
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Special Care Dent, London SE1 9RT, EnglandNorth West London Hosp NHS Trust, Dept Oral & Maxillofacial Surg, Harrow HA1 3UJ, Middx, England
Fiske, J.
Burke, M.
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Guys & St Thomas NHS Fdn Trust, London SE1 9RT, EnglandNorth West London Hosp NHS Trust, Dept Oral & Maxillofacial Surg, Harrow HA1 3UJ, Middx, England
机构:
Univ Ljubljana, Fac Med, Vrazov Trg 2, Ljubljana 1000, SloveniaUniv Ljubljana, Fac Med, Vrazov Trg 2, Ljubljana 1000, Slovenia
Rondaij, Tadej
Kozjek, Nada Rotovnik
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Univ Ljubljana, Fac Med, Vrazov Trg 2, Ljubljana 1000, Slovenia
Inst Oncol, Dept Clin Nutr, Zaloska Cesta 2, Ljubljana 1000, SloveniaUniv Ljubljana, Fac Med, Vrazov Trg 2, Ljubljana 1000, Slovenia
Kozjek, Nada Rotovnik
Popovic, Peter
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Univ Ljubljana, Fac Med, Vrazov Trg 2, Ljubljana 1000, Slovenia
UMC Ljubljana, Inst Radiol, Zaloska Cesta 7, Ljubljana 1000, SloveniaUniv Ljubljana, Fac Med, Vrazov Trg 2, Ljubljana 1000, Slovenia
Popovic, Peter
Jordan, Taja
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Univ Ljubljana, Fac Med, Vrazov Trg 2, Ljubljana 1000, Slovenia
UMC Ljubljana, Inst Radiol, Zaloska Cesta 7, Ljubljana 1000, SloveniaUniv Ljubljana, Fac Med, Vrazov Trg 2, Ljubljana 1000, Slovenia
机构:
North West London Hosp NHS Trust, SDO NHS Hillingdon, Dept Oral & Maxillofacial Surg, Harrow HA1 3UJ, Middx, EnglandNorth West London Hosp NHS Trust, SDO NHS Hillingdon, Dept Oral & Maxillofacial Surg, Harrow HA1 3UJ, Middx, England
Lee, A. M.
Gabe, S. M.
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St Marks Hosp, Lennard Jones Intestinal Failure Unit, London, EnglandNorth West London Hosp NHS Trust, SDO NHS Hillingdon, Dept Oral & Maxillofacial Surg, Harrow HA1 3UJ, Middx, England
Gabe, S. M.
Nightingale, J. M.
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St Marks Hosp, Lennard Jones Intestinal Failure Unit, Imperial Coll, London, EnglandNorth West London Hosp NHS Trust, SDO NHS Hillingdon, Dept Oral & Maxillofacial Surg, Harrow HA1 3UJ, Middx, England
Nightingale, J. M.
Burke, M.
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Guys & St Thomas NHS Fdn Trust, London, EnglandNorth West London Hosp NHS Trust, SDO NHS Hillingdon, Dept Oral & Maxillofacial Surg, Harrow HA1 3UJ, Middx, England