Details of Disease

General Information of Disease (ID: DISMB5FU)

• Disease Name: Short bowel syndrome
• Synonyms: acquired short bowel syndrome; short gut syndrome
• Disease Class: KB89: Neonatal malabsorption syndrome
• Definition: Short bowel syndrome is an intestinal failure due to either a congenital defect, intestinal infarction or extensive surgical resection of the intestinal tract that results in a functional small intestine of less than 200cm in length and is characterized by diarrhea, nutrient malabsoption, bowel dilation and dysmobility.
• Disease Hierarchy:
  DISGPMUQ: Intestinal disorder
  DISMB5FU: Short bowel syndrome
• ICD Code:
  ICD-11: ICD-11: KB89.1
  ICD-9: ICD-9: 579.3
  Expand ICD-11: 'KB89.1
  Expand ICD-9: 579.3
• Disease Identifiers:
  MONDO ID: MONDO_0015183
  MESH ID: D012778
  UMLS CUI: C0036992
  MedGen ID: 19966
  Orphanet ID: 104008
  SNOMED CT ID: 235724004

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 3 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Glucagon recombinant	DMFLUTR	Approved	Small molecular drug	[1]
L-glutamine	DM69G8X	Approved	Small molecular drug	[2]
Teduglutide	DMYOAKS	Approved	Small molecular drug	[3]

This Disease is Treated as An Indication in 2 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Apraglutide	DM5MVWR	Phase 3	Peptide	[4]
Glepaglutide	DMUEQ4J	Phase 3	Peptide	[5]

This Disease is Treated as An Indication in 1 Investigative Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
AMX-256	DMGHXQE	Investigative	NA	[6]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 6 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
GCG	TT6Y4PN	Limited	Biomarker	[7]
SLC15A1	TT5LF3C	Limited	Altered Expression	[8]
GLP2R	TT1YWO5	Strong	Biomarker	[9]
INSR	TTCBFJO	Strong	Therapeutic	[10]
LEP	TTBJEZ5	Strong	Biomarker	[11]
LEPR	TT0HD6V	Strong	Biomarker	[11]

This Disease Is Related to 3 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
SPINK4	OTHTCW94	Limited	Biomarker	[12]
PTPN5	OT2H1KDK	Definitive	Biomarker	[13]
SLFN12	OTTBXM25	Definitive	Biomarker	[14]

References

• [1] Where are we at with short bowel syndrome and small bowel transplant. World J Transplant. 2012 Dec 24;2(6):95-103.
• [2] URL: http://www.guidetopharmacology.org Nucleic Acids Res. 2015 Oct 12. pii: gkv1037. The IUPHAR/BPS Guide to PHARMACOLOGY in 2016: towards curated quantitative interactions between 1300 protein targets and 6000 ligands. (Ligand id: 723).
• [3] URL: http://www.guidetopharmacology.org Nucleic Acids Res. 2015 Oct 12. pii: gkv1037. The IUPHAR/BPS Guide to PHARMACOLOGY in 2016: towards curated quantitative interactions between 1300 protein targets and 6000 ligands. (Ligand id: 7049).
• [4] ClinicalTrials.gov (NCT04627025) Trial to Evaluate Efficacy and Safety of Apraglutide in SBS-IF (STARS). U.S. National Institutes of Health.
• [5] ClinicalTrials.gov (NCT04881825) Evaluation of Long Term Safety and Efficacy of Glepaglutide in Treatment of SBS - Extension Trial (EASE SBS 3). U.S. National Institutes of Health.
• [6] URL: http://www.guidetopharmacology.org Nucleic Acids Res. 2015 Oct 12. pii: gkv1037. The IUPHAR/BPS Guide to PHARMACOLOGY in 2016: towards curated quantitative interactions between 1300 protein targets and 6000 ligands. (Target id: 250).
• [7] Differential action of TGR5 agonists on GLP-2 secretion and promotion of intestinal adaptation in a piglet short bowel model.Am J Physiol Gastrointest Liver Physiol. 2019 May 1;316(5):G641-G652. doi: 10.1152/ajpgi.00360.2018. Epub 2019 Mar 28.
• [8] Distribution of the H+/peptide transporter PepT1 in human intestine: up-regulated expression in the colonic mucosa of patients with short-bowel syndrome.Am J Clin Nutr. 2002 May;75(5):922-30. doi: 10.1093/ajcn/75.5.922.
• [9] Glucagon-like peptide-2 promotes gallbladder refilling via a TGR5-independent, GLP-2R-dependent pathway.Mol Metab. 2017 Mar 31;6(6):503-511. doi: 10.1016/j.molmet.2017.03.006. eCollection 2017 Jun.
• [10] Oral insulin stimulates intestinal epithelial cell turnover in correlation with insulin-receptor expression along the villus-crypt axis in a rat model of short bowel syndrome.Pediatr Surg Int. 2010 Jan;26(1):37-44. doi: 10.1007/s00383-009-2520-x.
• [11] Leptin affects intestinal epithelial cell turnover in correlation with leptin receptor expression along the villus-crypt axis after massive small bowel resection in a rat.Pediatr Res. 2009 Dec;66(6):648-53. doi: 10.1203/PDR.0b013e3181be9f84.
• [12] Novel GLP-1/GLP-2 co-agonists display marked effects on gut volume and improves glycemic control in mice.Physiol Behav. 2018 Aug 1;192:72-81. doi: 10.1016/j.physbeh.2018.03.004. Epub 2018 Mar 11.
• [13] Retracing our STEPs: Four decades of progress in intestinal lengthening procedures for short bowel syndrome.Am J Surg. 2019 Apr;217(4):772-782. doi: 10.1016/j.amjsurg.2018.11.025. Epub 2018 Nov 27.
• [14] Regulation of epithelial differentiation in rat intestine by intraluminal delivery of an adenoviral vector or silencing RNA coding for Schlafen 3.PLoS One. 2013 Nov 11;8(11):e79745. doi: 10.1371/journal.pone.0079745. eCollection 2013.