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.
机构:
Childrens Hosp, Dept Surg, Boston, MA 02115 USA
Childrens Hosp, Vasc Biol Program, Boston, MA 02115 USAChildrens Hosp, Dept Surg, Boston, MA 02115 USA
Le, Hau D.
Fallon, Erica M.
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Childrens Hosp, Dept Surg, Boston, MA 02115 USA
Childrens Hosp, Vasc Biol Program, Boston, MA 02115 USAChildrens Hosp, Dept Surg, Boston, MA 02115 USA
Fallon, Erica M.
de Meijer, Vincent E.
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Childrens Hosp, Dept Surg, Boston, MA 02115 USA
Childrens Hosp, Vasc Biol Program, Boston, MA 02115 USAChildrens Hosp, Dept Surg, Boston, MA 02115 USA
de Meijer, Vincent E.
Malkan, Alpin D.
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Childrens Hosp, Dept Surg, Boston, MA 02115 USA
Childrens Hosp, Vasc Biol Program, Boston, MA 02115 USAChildrens Hosp, Dept Surg, Boston, MA 02115 USA
Malkan, Alpin D.
Puder, Mark
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Childrens Hosp, Dept Surg, Boston, MA 02115 USA
Childrens Hosp, Vasc Biol Program, Boston, MA 02115 USAChildrens Hosp, Dept Surg, Boston, MA 02115 USA
Puder, Mark
Gura, Kathleen M.
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Childrens Hosp, Dept Pharm, Boston, MA 02115 USAChildrens Hosp, Dept Surg, Boston, MA 02115 USA
机构:
Univ Calif Los Angeles, David Geffen Sch Med, Pediat Gastroenterol Hepatol & Nutr, Los Angeles, CA 90095 USAUniv Calif Los Angeles, David Geffen Sch Med, Pediat Gastroenterol Hepatol & Nutr, Los Angeles, CA 90095 USA
Namjoshi, Shweta S.
Muradian, Sarah
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机构:
Childrens Natl Hlth Syst, Washington, DC USAUniv Calif Los Angeles, David Geffen Sch Med, Pediat Gastroenterol Hepatol & Nutr, Los Angeles, CA 90095 USA
Muradian, Sarah
Bechtold, Hannah
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Stanford Med Ctr, Palo Alto, CA USAUniv Calif Los Angeles, David Geffen Sch Med, Pediat Gastroenterol Hepatol & Nutr, Los Angeles, CA 90095 USA
Bechtold, Hannah
Reyen, Laurie
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Ronald Reagan UCLA Med Ctr, Dept Nursing, Los Angeles, CA USAUniv Calif Los Angeles, David Geffen Sch Med, Pediat Gastroenterol Hepatol & Nutr, Los Angeles, CA 90095 USA
Reyen, Laurie
Venick, Robert S.
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Univ Calif Los Angeles, David Geffen Sch Med, Pediat Gastroenterol Hepatol & Nutr, Los Angeles, CA 90095 USAUniv Calif Los Angeles, David Geffen Sch Med, Pediat Gastroenterol Hepatol & Nutr, Los Angeles, CA 90095 USA
Venick, Robert S.
Marcus, Elizabeth A.
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Univ Calif Los Angeles, David Geffen Sch Med, Pediat Gastroenterol Hepatol & Nutr, Los Angeles, CA 90095 USA
VA Greater Los Angeles Hlth Care Syst, Los Angeles, CA USAUniv Calif Los Angeles, David Geffen Sch Med, Pediat Gastroenterol Hepatol & Nutr, Los Angeles, CA 90095 USA
Marcus, Elizabeth A.
Vargas, Jorge H.
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Univ Calif Los Angeles, David Geffen Sch Med, Pediat Gastroenterol Hepatol & Nutr, Los Angeles, CA 90095 USAUniv Calif Los Angeles, David Geffen Sch Med, Pediat Gastroenterol Hepatol & Nutr, Los Angeles, CA 90095 USA
Vargas, Jorge H.
Wozniak, Laura J.
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Univ Calif Los Angeles, David Geffen Sch Med, Pediat Gastroenterol Hepatol & Nutr, Los Angeles, CA 90095 USAUniv Calif Los Angeles, David Geffen Sch Med, Pediat Gastroenterol Hepatol & Nutr, Los Angeles, CA 90095 USA
机构:
Univ Paris VII, Hosp Beaujon, Dept Gastroenterol & Nutr Support, Paris, FranceUniv Paris VII, Hosp Beaujon, Dept Gastroenterol & Nutr Support, Paris, France
Joly, Francisca
Baxter, Janet
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NHS Tayside, Nutr & Dietet Serv, Dundee, ScotlandUniv Paris VII, Hosp Beaujon, Dept Gastroenterol & Nutr Support, Paris, France
Baxter, Janet
Staun, Michael
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Rigshosp, Dept Med Gastroenterol CA, Blegdamsvej 9, DK-2100 Copenhagen O, DenmarkUniv Paris VII, Hosp Beaujon, Dept Gastroenterol & Nutr Support, Paris, France
Staun, Michael
Kelly, Darlene G.
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St Marys Hosp, Mayo Clin Rochester, Rochester, MN 55905 USA
Methodist Hosp, Mayo Clin Rochester, Rochester, MN 55905 USAUniv Paris VII, Hosp Beaujon, Dept Gastroenterol & Nutr Support, Paris, France
Kelly, Darlene G.
Hwa, Yi Lisa
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机构:
St Marys Hosp, Mayo Clin Rochester, Rochester, MN 55905 USA
Methodist Hosp, Mayo Clin Rochester, Rochester, MN 55905 USAUniv Paris VII, Hosp Beaujon, Dept Gastroenterol & Nutr Support, Paris, France
Hwa, Yi Lisa
Corcos, Olivier
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Univ Paris VII, Hosp Beaujon, Dept Gastroenterol & Nutr Support, Paris, FranceUniv Paris VII, Hosp Beaujon, Dept Gastroenterol & Nutr Support, Paris, France
机构:
Fdn IRCCS Ca Granda Osped Maggiore Policlin, Neonatal Intens Care Unit, Milan, ItalyFdn IRCCS Ca Granda Osped Maggiore Policlin, Neonatal Intens Care Unit, Milan, Italy
Roggero, Paola
Liotto, Nadia
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Fdn IRCCS Ca Granda Osped Maggiore Policlin, Neonatal Intens Care Unit, Milan, Italy
Fdn IRCCS Ca Granda Osped Maggiore Policlin, Ctr Nutr Partenzaneonatale, Clin Mangiagalli, Via Commenda 12, I-20122 Milan, ItalyFdn IRCCS Ca Granda Osped Maggiore Policlin, Neonatal Intens Care Unit, Milan, Italy
Liotto, Nadia
Piemontese, Pasqua
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Fdn IRCCS Ca Granda Osped Maggiore Policlin, Neonatal Intens Care Unit, Milan, ItalyFdn IRCCS Ca Granda Osped Maggiore Policlin, Neonatal Intens Care Unit, Milan, Italy
Piemontese, Pasqua
Menis, Camilla
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Fdn IRCCS Ca Granda Osped Maggiore Policlin, Neonatal Intens Care Unit, Milan, ItalyFdn IRCCS Ca Granda Osped Maggiore Policlin, Neonatal Intens Care Unit, Milan, Italy
Menis, Camilla
Perrone, Michela
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Fdn IRCCS Ca Granda Osped Maggiore Policlin, Neonatal Intens Care Unit, Milan, ItalyFdn IRCCS Ca Granda Osped Maggiore Policlin, Neonatal Intens Care Unit, Milan, Italy
Perrone, Michela
Tabasso, Chiara
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Fdn IRCCS Ca Granda Osped Maggiore Policlin, Neonatal Intens Care Unit, Milan, ItalyFdn IRCCS Ca Granda Osped Maggiore Policlin, Neonatal Intens Care Unit, Milan, Italy
Tabasso, Chiara
Amato, Orsola
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Fdn IRCCS Ca Granda Osped Maggiore Policlin, Neonatal Intens Care Unit, Milan, ItalyFdn IRCCS Ca Granda Osped Maggiore Policlin, Neonatal Intens Care Unit, Milan, Italy
Amato, Orsola
Orsi, Anna
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Fdn IRCCS Ca Granda Osped Maggiore Policlin, Neonatal Intens Care Unit, Milan, ItalyFdn IRCCS Ca Granda Osped Maggiore Policlin, Neonatal Intens Care Unit, Milan, Italy
Orsi, Anna
Pesenti, Nicola
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Univ Milano Bicocca, Dept Stat & Quantitat Methods, Div Biostat Epidemiol & Publ Hlth, Milan, ItalyFdn IRCCS Ca Granda Osped Maggiore Policlin, Neonatal Intens Care Unit, Milan, Italy
Pesenti, Nicola
Leva, Ernesto
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Fdn IRCCS Ca Granda Osped Maggiore Policlin, Dept Pediat Surg, Milan, Italy
Univ Milan, Dept Clin Sci & Community Hlth, Milan, ItalyFdn IRCCS Ca Granda Osped Maggiore Policlin, Neonatal Intens Care Unit, Milan, Italy
Leva, Ernesto
Mosca, Fabio
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Fdn IRCCS Ca Granda Osped Maggiore Policlin, Neonatal Intens Care Unit, Milan, Italy
Univ Milan, Dept Clin Sci & Community Hlth, Milan, ItalyFdn IRCCS Ca Granda Osped Maggiore Policlin, Neonatal Intens Care Unit, Milan, Italy
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Shiraz Univ Med Sci, Thorac & Vasc Surg Res Ctr, Shiraz, Iran
Shiraz Univ Med Sci, Student Res Comm, Shiraz, IranShiraz Univ Med Sci, Abu Ali Sina Hosp, Shiraz Transplant Ctr, Shiraz 7193711351, Iran
Shahriarirad, Reza
Shojazadeh, Alireza
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Shiraz Univ Med Sci, Student Res Comm, Shiraz, IranShiraz Univ Med Sci, Abu Ali Sina Hosp, Shiraz Transplant Ctr, Shiraz 7193711351, Iran
Shojazadeh, Alireza
Ranjbar, Keivan
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Shiraz Univ Med Sci, Thorac & Vasc Surg Res Ctr, Shiraz, Iran
Shiraz Univ Med Sci, Student Res Comm, Shiraz, IranShiraz Univ Med Sci, Abu Ali Sina Hosp, Shiraz Transplant Ctr, Shiraz 7193711351, Iran
Ranjbar, Keivan
Darabi, Mohammad Hasan
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Shiraz Univ Med Sci, Student Res Comm, Shiraz, IranShiraz Univ Med Sci, Abu Ali Sina Hosp, Shiraz Transplant Ctr, Shiraz 7193711351, Iran
Darabi, Mohammad Hasan
Tangestanipour, Sina
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Shiraz Univ Med Sci, Student Res Comm, Shiraz, IranShiraz Univ Med Sci, Abu Ali Sina Hosp, Shiraz Transplant Ctr, Shiraz 7193711351, Iran
Tangestanipour, Sina
Hosseini, Seyed Morteza
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Shiraz Univ Med Sci, Abu Ali Sina Hosp, Shiraz Transplant Ctr, Shiraz 7193711351, IranShiraz Univ Med Sci, Abu Ali Sina Hosp, Shiraz Transplant Ctr, Shiraz 7193711351, Iran
Hosseini, Seyed Morteza
Zahiri, Leila
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Shiraz Univ Med Sci, Dept Internal Med, Shiraz, IranShiraz Univ Med Sci, Abu Ali Sina Hosp, Shiraz Transplant Ctr, Shiraz 7193711351, Iran
Zahiri, Leila
Nikeghbalian, Saman
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Shiraz Univ Med Sci, Abu Ali Sina Hosp, Shiraz Transplant Ctr, Shiraz 7193711351, IranShiraz Univ Med Sci, Abu Ali Sina Hosp, Shiraz Transplant Ctr, Shiraz 7193711351, Iran
机构:
Salford Royal NHS Fdn Trust, Intestinal Failure Unit, Manchester M6 8HD, EnglandSalford Royal NHS Fdn Trust, Intestinal Failure Unit, Manchester M6 8HD, England
Harrison, Elizabeth
Allan, Philip
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Salford Royal NHS Fdn Trust, Intestinal Failure Unit, Manchester M6 8HD, England
Oxford Univ Hosp NHS Trust, Translat Gastroenterol Unit, Oxford OX3 6RP, EnglandSalford Royal NHS Fdn Trust, Intestinal Failure Unit, Manchester M6 8HD, England
Allan, Philip
Ramu, Amrutha
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Salford Royal NHS Fdn Trust, Intestinal Failure Unit, Manchester M6 8HD, England
Oxford Univ Hosp NHS Trust, Translat Gastroenterol Unit, Oxford OX3 6RP, EnglandSalford Royal NHS Fdn Trust, Intestinal Failure Unit, Manchester M6 8HD, England