Introduction: Short bowel syndrome (SBS) is generally defined as the loss of an extensive length of the small intestine resulting in an inadequate absorption of enteral nutrients. Teduglutide is a recombinant analog of native human GLP-2 with resistance to in vivo degradation, enhanced intestinal adaptation and increased the absorptive capacity of the remnant intestine in patients with SBS.

Methods: A premature girl (33 weeks) with necrotizing enterocolitis (NEC) underwent extensive intestinal resection (only 30 cm of jejunum anastomosed to the sigmoid colon preserved). Intestinal dilation and the need for PN forced to perform an intestinal lengthening with a modified serial transverse enteroplasty procedure (STEP) twice, at 19 months and 3.5 years (final small bowel length 175 cm).

At the age of 7 years old, the patient still required PN 25 Kcal/kg and high volume of iv fluids (2300 ml; 85 ml/kg) administered for 17 hours/day. She had 7-8 semiliquid bowel movements/day (output 2000 ml/day) and frequent hospital re-admissions owing to catheter-related infections. She only had a single central venous access for PN administration. The written informed consent of the parents was obtained for the administration of teduglutide and the hospital approved its subcutaneous administration in a dose of 0.05 mg/kg/day.

Results: The evolution of PN volume, calorie requirements, days of PN administration and plasma citrulline, before and during teduglutide treatment are shown in Table 1.

Prior to treatment, weight, height and body mass index (BMI) were 27.2 kg (+0.86 SD), 135 cm (+2.04 SD) and 14.9 (-0.39 SD), respectively. After 4 weeks of treatment, less frequent bowel movements, change in stool form (more consistency) and increased urine output were noted, weaning off PN support was initiated. At 22 weeks, the patient only required PN 2 days/week which was stopped at week 30 of treatment. In parallel, a rise in serum citrulline was observed.

After 18 months of treatment we decreased dose to 0.025 mg/Kg/day and after follow-up of 24 months, the patient remained free of PN at this daily dose. Nowadays, bowel movement frequency is 2-3 per day (type 5 on the Bristol scale). Weight (31.6; -0.24 SD) and BMI remain stable (14.8; -0.85 SD), while her height has increased to 146 cm (+1.5 SD).

Conclusions: Teduglutide has allowed this child, a candidate for intestinal transplantation, to achieve complete PN-free status with a great improvement in her quality of life.