First registry of adult patients with chronic intestinal failure due to short bowel syndrome in Argentina: The RESTORE project.

BACKGROUND: Short bowel syndrome (SBS) is considered a low prevalence disease. In Argentina, no registries are available on chronic intestinal failure (CIF) and SBS. This project was designed as the first national registry to report adult patients with this disease. METHODS: A prospective multicenter observational registry was created including adult patients with CIF/SBS from approved centers. Demographics, clinical characteristics, nutrition assessment, home parenteral nutrition (HPN) management, surgeries performed, medical treatment, overall survival, and freedom from HPN survival were analyzed. RESULTS: Of the 61 enrolled patients, 56 with available follow-up data were analyzed. At enrollment, the mean intestinal length was 59.5 ± 47.3 cm; the anatomy was type 1 (n = 41), type 2 (n = 10), and type 3 (n = 5). At the end of the interim analysis, anatomy changed to type 1 in 31, type 2 in 17, and type 3 in 8 patients. The overall mean time on HPN before enrollment was 33.5 ± 56.2 months. Autologous gastrointestinal reconstruction surgery was performed before enrollment on 21 patients, and afterward on 11. Nine patients (16.1%) were weaned off HPN with standard medical nutrition treatment; 12 patients received enterohormones, and 2 of them suspended HPN; one patient was considered a transplant candidate. In 23.7 ± 14.5 months, 11 of 56 patients discontinued HPN; Kaplan-Meier freedom from HPN survival was 28.9%. The number of cases collected represented 19.6 new adult CIF/SBS patients per year. CONCLUSION: The RESTORE project allowed us to know the incidence, the current medical and surgical approach for this pathology, as well as its outcome and complications at dedicated centers.

Postsurgical Intestinal Rehabilitation Using Semisynthetic Glucagon-Like Peptide-2 Analogue (sGLP-2) at a Referral Center: Can Patients Achieve Parenteral Nutrition and sGLP-2 Independency?

BACKGROUND: Teduglutide, a semisynthetic analogue of glucagon-like peptide-2 (sGLP-2), increases intestinal absorption of fluids and nutrients, reducing the need for parenteral nutrition (PN). This report aims to describe our experience with sGLP-2 in a cohort of adult patients with short-bowel syndrome. METHODS: This is a prospective observational study on adult patients initially evaluated in our specialized intestinal rehabilitation program that received sGLP-2 from June 2014 to March 2020. RESULTS: Autologous gastrointestinal reconstruction surgery (AGIRS) was performed in 108 patients; 68.5% (74 of 108) achieved intestinal sufficiency with standard medical therapy. Seventeen patients were treated with sGLP-2; 66.5% (8 of 12) received treatment for a mean time of 25.8 weeks (3.4-54.0) and could suspend PN. One patient reinitiated treatment due to renal lithiasis and acute renal failure. Currently, 7 of 12 patients (53.8%) continue without PN for a mean time of 165.6 weeks. Volume, energy, and days of PN were reduced in all patients. No serious adverse events were registered. Four of 7 patients (57.1%) who discontinued PN could also discontinue sGLP-2. Therefore, the use of sGLP-2 increased the overall success rate of PN independency after AGIRS to 76% (82 of 108). CONCLUSION: This study confirmed that sGLP-2 should be considered as part of the standard therapy for postsurgical medical rehabilitation treatment in patients with chronic intestinal failure. We add to the current knowledge that some patients can discontinue both PN and sGLP-2 in the long term, achieving complete recovery of their quality of life.

Telomere length study in celiac disease.

OBJECTIVES: Telomeres are important structures that are critical for maintaining chromosomal integrity and cell surveillance. The aim of this study was to analyze telomere length in patients with celiac disease (CD), a multifactorial disorder with a strong genetic component that exhibits genomic instability and cancer predisposition, particularly T-cell lymphomas. METHODS: Telomere length measured by telomere restriction fragments (TRF) was studied in small intestinal biopsy (SIB) samples and peripheral blood lymphocytes (PBL) from 20 untreated CD patients, distributed according to the clinical form as four asymptomatic, five monosymptomatic, and 11 polysymptomatic individuals. We also analyzed TRF from normal peripheral blood lymphocytes and normal biopsy samples as normal controls. RESULTS: TRF evaluation showed a significant telomere shortening in SIB samples from CD patients (4.21 +/- 0.29 Kb) compared to PBL from the same individuals (9.17 +/- 0.35 Kb) (p < 0.0001), independently of clinical form. Mean TRF peak values from normal biopsy samples were significantly higher (8.33 +/- 0.38 Kb) than those observed in CD biopsy samples (p < 0.001). No differences between TRF values in CD-PBL and normal peripheral blood lymphocytes (8.89 +/- 0.37Kb) were found. CONCLUSIONS: Our findings in patients with CD, a disorder in which the gluten-induced mucosal injury could accelerate telomere shortening, would increase the process of end-to-end fusions resulting in chromosomal changes, supports the hypothesis that genomic instability and telomere reduction may play a role in the cancer predisposition observed in these patients.