Norsa L.: Teduglutide in short bowel syndrome
Bergamascheria ancora! Yes, the Bergamo team have made available for a podcast yet another member, Dr Lorenzo Norsa, a professor of paediatrics there. Dr Norsa has been a fellow in paediatric gastroenterology in Israel and in France. In Paris he became well-versed in treatment of short bowel syndrome, working with the prominent Necker team . . . perhaps their publications form a suite Bergamasque. (Apologies, M Debussy.) He speaks today on the use of teduglutide, an agent that mimicks the effects of the small-bowel enterocyte mitotic stimulant and anti-apoptotic life-prolonger glucagon-like peptide 2. Teduglutide increases the number of functional enterocytes, lengthening villi and deepening crypts, but does not go deeper, so to speak ; hyperplasia of the muscular coats and their elements is not induced, and the effects of teduglutide recede when the drug is withdrawn. Perhaps this will not matter after puberty and its growth spurts are over, with less energy demand, but that remains to be seen. A registry of patients is being compiled to permit better prognostication and more efficient dosage with what is a very expensive drug : That early deployment of teduglutide improves overall results is known, but when to withdraw or how to modulate teduglutide administration are still open questions.
Literature :
Lambe C et al. Long-term treatment with teduglutide: A 48-week open-label single-center clinical trial in children with short bowel syndrome. Am J Clin Nutr 2023 Jun 117(6):1152-1163. doi: 10.1016/j.ajcnut.2023.02.019. Epub 2023 May 3. PMID: 37270289.
Norsa L et al. Nutrition and intestinal rehabilitation of children with short bowel syndrome: A position paper of the ESPGHAN committee on nutrition. Part 1: From intestinal resection to home discharge. J Pediatr Gastroenterol Nutr 2023 Aug 177(2):281-297. doi: 10.1097/MPG.0000000000003849. Epub 2023 May 31. PMID: 37256827.
Norsa L et al. Nutrition and intestinal rehabilitation of children with short bowel syndrome: A position paper of the ESPGHAN committee on nutrition. Part 2: Long-term follow-up on home parenteral nutrition. J Pediatr Gastroenterol Nutr 2023 Aug 177(2):298-314. doi: 10.1097/MPG.0000000000003850. Epub 2023 May 31. PMID: 37256821.
Germán-Díaz M et al. Early use of teduglutide in paediatric patients with intestinal failure is associated with a greater response rate: A multicenter study. Eur J Pediatr 2024 Apr 26. doi: 10.1007/s00431-024-05577-5. Online ahead of print. PMID: 38664251.
Dr. Norsa´s favourite song: Piccolo Stella senza cielo - Ligabue https://open.spotify.com/track/0HpKuz1HfXxRzNnUqfwzgF?si=5de78bc63c994e5c
ESPGHAN favourite Songs can be found on Spotify https://open.spotify.com/playlist/0YIHKjxITLEm9XNyHyypTo