Short bowel syndrome (SBS) is defined as the presence of <200 cm of small intestine in continuity.1 SBS is frequently associated with malabsorption, diarrhea, steatorrhea, malnutrition, and dehydration. SBS represents the primary pathophysiological mechanism underlying chronic intestinal failure, which is characterized by a reduction in intestinal function below the minimum necessary for the absorption of macronutrients and/or water and electrolytes, thus necessitating IV supplementation to maintain health and/or growth.1 In patients with SBS, a rare form of acidosis—known as d-lactic acidosis—may occur, which is precipitated by bacterial overgrowth.

Teduglutide, an analog of human glucagon-like peptide-2 (GLP-2), is the first long-term non-symptomatic treatment approved for SBS. GLP-2 is a peptide secreted by the intestinal L cells located in the ileum, which enhances portal and intestinal blood flow, inhibits gastric acid secretion, and reduces intestinal motility.2 Teduglutide preserves mucosal integrity by promoting intestinal repair and growth by increasing of villus height to crypt depth ratio.2 Its approval is based on the STEPS studies,3 which observed a significant reduction in the need for parenteral nutrition (PN) and even complete independence from PN in up to 20% of patients in the teduglutide-treated group vs placebo 2 years after starting therapy.2

This article describes a case of d-lactic acidosis in a patient on teduglutide therapy for SBS.