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Short bowel syndrome
Short-bowel syndrome is a disorder clinically defined by malabsorption, diarrhea, steatorrhea, fluid and electrolyte disturbances, and malnutrition.
Prevalence
Age of Onset
ICD-10
D81.8
Inheritance
Autosomal dominant
Autosomal recessive
Mitochondrial/Multigenic
X-linked dominant
X-linked recessive
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Short bowel syndrome (SBS, or simply short gut) is a rare malabsorption disorder caused by a lack of functional small intestine. The primary symptom is diarrhea, which can result in dehydration, malnutrition, and weight loss. Other symptoms may include bloating, heartburn, feeling tired, lactose intolerance, and foul-smelling stool. Complications can include anemia and kidney stones. Most cases are due to the surgical removal of a large portion of the small intestine.
5 Facts you should know
FACT
1
Short bowel syndrome (SBS, or simply short gut) is a malabsorption disorder caused by a lack of functional small intestine.
FACT
2
Most cases are due to the surgical removal of a large portion of the small intestine.
FACT
3
In children the main causes include necrotizing enterocolitis, intestinal atresias, and intestinal volvulus.
FACT
4
Various nonoperative procedures, such as radiation enteritis, can leave patients with a functional short-bowel syndrome.
FACT
5
Disease, traumatic injury, vascular accident, or other pathology that leaves less than 200 cm of viable small bowel or results in a loss of 50% or more of the small intestine places the patient at risk for developing short-bowel syndrome.
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Common Signs & Symptoms
Bronchiectasis
Permanent enlargement of the airways of the lungs
Decreased circulating IgG2 level
Decreased proportion of class-switched memory B cells
Decreased specific pneumococcal antibody level
Increased circulating IgM level
Increased proportion of transitional B cells
Lymphadenopathy
Swollen lymph nodes
Recurrent ear infections
Frequent ear infections
Current Treatments
Currently, the main treatments for Short Bowel Syndrome include:
Parenteral nutrition (PN)
This is a method of delivering nutrients directly into the bloodstream via a vein. PN can be used as a temporary or long-term treatment for SBS, and is often used in combination with other treatments.
Decreased circulating IgG2 levelEnteral nutrition
This is a method of delivering nutrients directly into the small intestine via a tube.
Examples:
- Jejunal feeding tube (J-tube)
- Gastrostomy tube (G-tube)
Medications
The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for the treatment of this condition.
- Teduglutide [rDNA origin](Brand name: Gattex) Manufactured by NPS Pharmaceuticals, Inc. FDA-approved indication: Treatment of adult patients with short bowel syndrome (SBS) who are dependent on parenteral support National Library of Medicine Drug Information Portal.
- Glutamine(Brand name: NutreStore™) Manufactured by Emmaus Medical Inc. FDA-approved indication: Treatment of short bowel syndrome in patients receiving specialized nutritional support when used in conjunction with a recombinant human growth hormone that is approved for this indication. National Library of Medicine Drug Information Portal Follitropin alfa, recombinant(Brand name: Zorbtive) Manufactured by Emmaus Medical, Inc.
- FDA-approved indication: Treatment of short bowel syndrome in patients receiving specialized nutritional support. National Library of Medicine Drug Information Portal
Surgery
Some surgical procedures can help to increase the absorptive surface area of the small intestine, such as:
- Lengthening procedures (for example, serial transverse enteroplasty (STEP))
- Bowel transplantation
It is important to note that the treatment plan for SBS will vary for each individual and will depend on the cause and severity of their condition.
Source:
UpToDate: Short bowel syndrome in adults
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK): Short Bowel Syndrome
Top Clinical Trials
| Title | Description | Phases | Status | Interventions | Locations | More Information |
|---|---|---|---|---|---|---|
| VIBRANT Study of Vurolenatide in Adult Patients With Short Bowel Syndrome. | A Phase 2 study of Vurolenatide in adult patients with SBS. A total of 22 patients are planned to be dosed in four treatment groups with placebo and/or active Vurolenatide. The study is 13 weeks (3 weeks screening, 4 weeks SC study drug administration, and 6 weeks of follow up). Safety and efficacy will be analyzed. Information on patients reported outcomes (ePRO) will be collected. | Phase 2 | Recruiting | Drug: Vurolenatide 50mg/PBO|Drug: Vurolenatide 100mg/PBO|Drug: Vurolenatide 50/50 mg|Drug: Placebo | Research Site, Nashville, Tennessee, United States | More Information |
| Proof of Concept Study in Patients With Short Bowel Syndrome | This is a Phase I/II. proof of concept, open label, two-dose, dose escalation study of NM-002 in adult patients with SBS who previously responded to exenatide. NM-002 is planned to be administered twice, at up to 3 different dose levels, in up to 3 cohorts, each consisting of 3-4 patients. Doses will be administered on Days 1 and 15 by subcutaneous injection. Patients will be monitored for their usage of parenteral supplementation, and will fill out a daily diary for their symptoms of SBS. Urine output will be measured on a daily basis. Patients will be followed for 6 weeks after the second dose. | Phase 1|Phase 2 | Active, not recruiting | Drug: NM-002 | Research Facility One, Los Angeles, California, United States | More Information |
| Functional Sucrase Deficiency in Short Bowel Syndrome Patients With Intestinal Failure | Short gut syndrome with intestinal failure patients may have decreased production of disaccharidases, like sucrase, an enzyme responsible for digesting sugar in foods. This can happen due to loss of bowel length from surgery or from loss of cellular function in the intestines due to use of parenteral nutrition intravenously. Therefore, patients with these conditions may not be able to digest sucrose (sugar) fully. Patients might experience abdominal distension/pain, vomiting and diarrhea when sugar is taken in orally or through the g-tube, which can limit patients' ability to increase oral or g-tube feeds in short gut syndrome patients with intestinal failure. In patients with short gut syndrome and intestinal failure, the administration of exogenous sucrase (enzyme) may improve sucrose (sugar) digestion and thus the ability to tolerate more oral or g-tube feeds. | Phase 2 | Not yet recruiting | Drug: Sucrase|Other: Placebo | Jackson Memorial Hospital, Miami, Florida, United States|University of Miami, Miami, Florida, United States | More Information |
| Trial to Evaluate Efficacy and Safety of Apraglutide in SBS-IF | The primary objective of the trial is the confirmation of the efficacy of apraglutide to evaluate the efficacy of weekly subcutaneous apraglutide in reducing parenteral support dependency. | Phase 3 | Recruiting | Drug: apraglutide | Ronald Reagan UCLA Medical Center, Los Angeles, California, United States|Gastroenterology Group of Naples, Naples, Florida, United States|University of Illinois, Outpatient Care Center, Chicago, Illinois, United States|Massachusetts General Hospital, Boston, Massachusetts, United States|Boston Children's Hospital, Boston, Massachusetts, United States|Henry Ford Medical Center - Columbus, Novi, Michigan, United States|Mayo Clinic, Rochester, Minnesota, United States|University of Nebraska Medical Center, Omaha, Nebraska, United States|Mount Sinai Medical Center, New York, New York, United States|Duke University Hospital, Durham, North Carolina, United States|Cleveland Clinic Foundation, Cleveland, Ohio, United States|Vanderbilt University Medical Center, Center for Human Nutrition, Nashville, Tennessee, United States|Universitair Ziekenhuis Leuven - Gasthuisberg, Leuven, Vlaams Brabant, Belgium|Hospital Novy Jicin, Nový Jičín, Czechia|University Hospital Plzen, Pilsen, Czechia|Fakultní nemocnice Královské Vinohrady, II. Interní klinika, Prague, Czechia|Thomayer University Hospital, Prague, Czechia|CHU de Caen Normandie, Caen, France|Hôpital Beaujon, Clichy, France|CHU Hôtel Dieu, Nantes, France|Hôpital ARCHET II, Nice, France|Hôpital Haut-Lévèque, Pessac, France|Hospices Civils de Lyon, Pierre Benite, France|Rennes University Hospital Center - Hospital Pontchaillou, Rennes, France|Charité - Universitätsmedizin Berlin, Berlin, Germany|Asklepios Klinik St. Georg, Hamburg, Germany|University Hospital Muenster, Münster, Germany|Polyclinic S. Orsola-Malpighi, Bologna, Italy|Fukuoka University Chikushi Hospital, Fukuoka, Japan|Yokohama Municipal Citizen's Hospital, Kanagawa, Japan|Yokohama City University Medical Center, Kanagawa, Japan|National University Corportation Tohoku University Tohoku University Hospital, Miyagi, Japan|Osaka University Hospital, Osaka, Japan|Keimyung University Dongsan Medical Center, Daegu, Korea, Republic of|The Catholic University of Korea, Seoul St. Mary's Hospital, Seoul, Korea, Republic of|Asan Medical Center, Soeul, Korea, Republic of|Samsung Medical Center, Soeul, Korea, Republic of|Stadmedica, Non-Public Healthcare Facility, Bydgoszcz, Poland|COPERNICUS Limited Liability Company, Gdansk, Poland|Gastromed Poland Sp. z o.o., Lublin, Poland|SOLUMED Medical Center, Poznań, Poland|Stanley Dudrick Multispecialty Hospital, Skawina, Poland|M. Pirogow Provincial Specialized Hospital, Łódź, Poland|Far Eastern Memorial Hospital, New Taipei City, Taiwan|China Medical University Hospital (CMUH), Taichung, Taiwan|Taichung Veterans General Hospital, Taichung, Taiwan | More Information |
| Evaluation of Long Term Safety and Efficacy of Glepaglutide in Treatment of SBS | The primary objective of the trial is to evaluate the long-term safety of glepaglutide treatment in patients with short bowel syndrome (SBS). Glepaglutide is the International Nonproprietary Name and USAN for ZP1848. | Phase 3 | Recruiting | Drug: glepaglutide | Mayo Clinic, Scottsdale, Arizona, United States|Georgetown University Medical Center, Washington, District of Columbia, United States|Miami Transplant Institute, Miami, Florida, United States|Division of Gastroenterology and Hepatology, Chicago, Illinois, United States|University of Chicago Children's Hospital, Chicago, Illinois, United States|University of Louisville, Louisville, Kentucky, United States|Mayo Clinic College of Medicine, Rochester, Minnesota, United States|University of Nebraska Medical Center, Omaha, Nebraska, United States|Mount Sinai Hospital, New York, New York, United States|Cleveland Clinic, Cleveland, Ohio, United States|Vanderbilt University Medical Center, Nashville, Nashville, Tennessee, United States|Houston Methodist Hospital, Houston, Texas, United States|Hôpital Erasme, Bruxelles, Belgium|UZ Leuven, Leuven, Belgium|The Royal Alexandra Hospital, Edmonton, Canada|Western University, London, Canada|University Health Network - Toronto General Hospital, Toronto, Canada|Rigshospitalet, Copenhagen, Denmark|Hôpital Beaujon, Clichy, France|CHRU de Lille, Lille, France|CHRU Nancy, Nancy, France|Hôpital de l'Archet, Nice, France|Centre Hospitalier Lyon-Sud, Pierre-Bénite, France|Charité - Universitätsmedizin Berlin, Berlin, Germany|Universitätsklinikum Bonn, Bonn, Germany|Universitätsklinikum Frankfurt - Med. Klinik I, Frankfurt, Germany|Asklepios Kliniken Hamburg GmbH, Hamburg, Germany|Universitätsmedizin Rostock, Rostock, Germany|Universitätsklinikum Tübingen - Innere Medizin I, Tübingen, Germany|Azienda Ospedaliero-Universitaria, Bologna, Italy|Azienda Ospedaliero Universitaria Federico II, Napoli, Italy|UMC Radboud Nijmegen, Nijmegen, Netherlands|Solumed, Poznań, Poland|Szpital Skawina sp. z o.o. im. Stanley Dudricka, Skawina, Poland|Wojewodzki Specjalistyczny Szpital im. M. Pirogowa w Lodzi, Łódź, Poland|St Mark's Hospital, Harrow, United Kingdom|UCLH Foundation NHS Trust, London, United Kingdom|Salford Royal NHS Foundation Trust, Manchester, United Kingdom|University of East Anglia, Norwich, United Kingdom|University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom | More Information |
| Efficacy And Safety Evaluation of Glepaglutide in Treatment of SBS | The primary objective of the trial is to confirm the efficacy of glepaglutide in reducing parenteral support volume in patients with short bowel syndrome. Glepaglutide is the International Nonproprietary Name and USAN for ZP1848. | Phase 3 | Recruiting | Drug: glepaglutide|Drug: Placebo | Mayo Clinic, Scottsdale, Arizona, United States|Georgetown University Medical Center, Washington, District of Columbia, United States|Miami Transplant Institute, Miami, Florida, United States|Division of Gastroenterology and Hepatology, Chicago, Illinois, United States|University of Chicago Children's Hospital, Chicago, Illinois, United States|University of Louisville, Louisville, Kentucky, United States|Mayo Clinic College of Medicine, Rochester, Minnesota, United States|University of Nebraska Medical Center, Omaha, Nebraska, United States|Mount Sinai Hospital, New York, New York, United States|Cleveland Clinic, Cleveland, Ohio, United States|Vanderbilt University Medical Center, Nashville, Nashville, Tennessee, United States|Houston Methodist Hospital, Houston, Texas, United States|Hôpital Erasme, Bruxelles, Belgium|UZ Leuven, Leuven, Belgium|The Royal Alexandra Hospital, Edmonton, Canada|Western University, London, Canada|University Health Network - Toronto General Hospital, Toronto, Canada|Aalborg University Hospital, Aalborg, Denmark|Rigshospitalet, Copenhagen, Denmark|Hôpital Beaujon, Clichy, France|CHRU de Lille, Lille, France|CHRU Nancy, Nancy, France|Hôpital de l'Archet, Nice, France|Centre Hospitalier Lyon-Sud, Pierre-Bénite, France|Charité - Universitätsmedizin Berlin, Berlin, Germany|Universitätsklinikum Bonn, Bonn, Germany|Universitätsklinikum Frankfurt - Med. Klinik I, Frankfurt, Germany|Asklepios Kliniken Hamburg GmbH, Hamburg, Germany|Universitätsmedizin Rostock, Rostock, Germany|Universitätsklinikum Tübingen - Innere Medizin I, Tübingen, Germany|Azienda Ospedaliero-Universitaria, Bologna, Italy|Azienda Ospedaliero Universitaria Federico II, Napoli, Italy|UMC Radboud Nijmegen, Nijmegen, Netherlands|Solumed, Poznań, Poland|Szpital Skawina sp. z o.o. im. Stanley Dudricka, Skawina, Poland|Wojewodzki Specjalistyczny Szpital im. M. Pirogowa w Lodzi, Łódź, Poland|St Mark's Hospital, Harrow, United Kingdom|UCLH Foundation NHS Trust, London, United Kingdom|Salford Royal NHS Foundation Trust, Manchester, United Kingdom|University of East Anglia, Norwich, United Kingdom|University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom | More Information |
Top Treatments in Research
| Agent | Class/Mechanism of Action | Development Status | Company | Company Contact | Clinical Studies | More Information |
|---|---|---|---|---|---|---|
| Vurolenatide | Long-acting injectable GLP-1 analogue, vurolenatide is designed specifically to slow digestive transit, thereby mitigating the SBS hallmark of excessive gastric motility due to deficient GLP-1 in the shortened intestine. This mechanism is intended to improve intestinal absorption of nutrients and water and reduce diarrhea for all patients with any type of post surgical SBS, regardless of their parenteral support requirements. Owing to its extended half-life, vurolenatide only needs to be dosed twice monthly at most, thus considerably improving convenience for patients and their caregivers. | Phase 2 | 9 Meters Biopharma, Inc. | Mary E Layton 919-275-1933 [email protected] | More Info | More Information |
| Apraglutide | A next-generation, long-acting, synthetic GLP-2 analog that acts as a selective, full agonist of the GLP 2 receptor. | Phase 3 | VectivBio AG | Fritze 41615513030 [email protected] | More Info | More Information |
| Glepaglutide | Glepaglutide is a long-acting GLP-2 analog in development for the treatment of short bowel syndrome (SBS). | Phase 3 | Zealand Pharma | Medical Director +4588773600 [email protected] Senior Clinical Trial Manager +4588773600 [email protected] | More Info | More Information |