Short Bowel Syndrome and Biliary System Flashcards

Comprehensive vocabulary flashcards covering Short Bowel Syndrome, intestinal failure, lipid management, and biliary metabolism based on lecture notes.

Short Bowel Syndrome (Adults)

Surgical removal or functional loss resulting in less than $180-200\,cm$ of remaining small bowel (normal length is $275-150\,cm$).

Intestinal Failure (IF)

A malabsorptive disorder caused by congenital defects, disease-associated loss of absorption, obstruction, dysmotility, or surgical resection, requiring PN for more than $60\,d$.

Short Bowel Syndrome (Neonate)

Remaining small bowel of less than $40\,cm$ ($12.6\%$ of expected bowel length) or the need for PN for more than $60\,d$ after intestinal resection.

Gastroschisis

A birth defect in which a baby's intestines develop outside of the abdomen.

Enteral Autonomy (EA)

Adequate growth for $12\,weeks$ after the discontinuation of PN, IV fluids, and IV electrolyte support.

Ileocecal Valve (ICV)

A structure that decreases transit time and prevents reflux of colonic bacteria; its presence is a predictor for achieving EA.

Glucagon-like Peptide 2 (GLP-2)

A hormone released by the terminal ileum that is crucial for intestinal growth and adaptation.

Ileal Brake

A negative feedback mechanism where undigested nutrients in the ileum are sensed by L-cells, leading to increased GLP-1 and Peptide YY, which slows gastric emptying and gut motility.

Hypergastrinemia

A condition resulting from stomach resection involving hyperacidity that impairs digestion of amino acids/starch and inhibits micelle formation.

Cyclic PN

A method for clinically stable infants where PN is provided for $2-4\,hours/d$ to help decrease cholestasis.

SO-IVFE

Soy-Based Intravenous Lipid Emulsions containing Omega-6 and phytosterols, associated with inflammation, oxidative stress, and cholestasis.

SMOFlipid

A composite IVFE (fish oil, soybean oil, MCT, olive oil) that improves lipid tolerance and decreases inflammation, but does not 100% prevent PNALD.

FO-IVFE (Omegaven)

Fish oil-based intravenous lipid emulsion used in patients with PNAC; associated with decreased mortality and transplant rates.

Cholestasis

A condition of reduced or blocked bile flow from the liver, leading to the accumulation of bile acids, bilirubin, and cholesterol in the liver and blood.

Bile Salts

The key active ingredient in bile that acts as a natural detergent to emulsify and break down fats.

Amphipathic

The property of bile salts having a hydrophobic side that binds fat and a hydrophilic side that faces the water environment of the gut.

Ampulla of Vater

Also known as the hepatopancreatic ampulla, it is the dilated reservoir where the common bile duct and pancreatic duct meet.

Sphincter of Oddi

A muscular valve surrounding the ampulla of Vater that controls the release of bile and pancreatic juices into the small intestine.

Unconjugated Bilirubin

A lipid-soluble, water-insoluble byproduct of red blood cell breakdown that must bind to serum albumin to travel to the liver.

Glucuronidation

The process in the liver where UGT attaches glucuronic acid to bilirubin to make it water-soluble (conjugated).

Stercobilin

The substance converted from urobilinogen by gut bacteria that gives stool its brown color.

Urobilin

The substance formed when a small amount of reabsorbed urobilinogen is oxidized in the kidneys, giving urine its yellow color.