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

Gondolesi, Gabriel; Ortega, Mariana; Doeyo, Mariana; Buncuga, Martín; Pérez, Carolina Alejandra; Mauriño, Eduardo; Costa, F.; Barrio, Silvia de; Manzur, Alejandra; Donnadio, Luciana; Matoso, Dolores; Clariá, Rodrigo Sanchez; Crivelli, Adriana; Solar, Héctor

doi:10.1002/jpen.2387

Cited by 8 publications

(1 citation statement)

References 42 publications

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“…This information is invaluable for healthcare professionals and policy-makers as well as researchers. Although many other countries have already established their registries [4][5][6][7][8], there is still a noticeable lack of published data.…”

Section: Introductionmentioning

confidence: 99%

The Czech Home Parenteral Nutrition Registry REDNUP: Comprehensive Analysis of Adult Patients’ Data

Koudelková,

Waldauf,

Wohl

et al. 2024

Ann Nutr Metab

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Introduction: Home parenteral nutrition (HPN) is the primary treatment modality for patients with chronic intestinal failure, one of the least common organ failures. This article provides a retrospective analysis of the data collected on HPN patients in the Czech Republic over the past 30 years. Methods: National registry data was collected using a standardised online form based on the OASIS registry (Oley–A.S.P.E.N. Information System) across all centres providing HPN in the Czech Republic. Data collected prospectively from adult patients in the HPN program was analysed in the following categories: epidemiology, demographics, underlying syndrome, and diagnosis, complications, and teduglutide therapy prevalence. Results: The registry identified a total of 1,838 adult patient records, reflecting almost 1.5 million individual catheter days. The prevalence of HPN has risen considerably over the last few decades, currently reaching 5.5 per 100,000 population. The majority of patients have short bowel syndrome and GI obstruction, with cancer being the most prevalent underlying disease. Catheter-related bloodstream infections have been the most prevalent acute complication. However, the incidence in 2022 was only 0.15 per 1,000 catheter days. The study also observed an increase in the prevalence of patients on palliative HPN over the last decade. Conclusion: This study presents a thorough analysis of data from the Czech REDNUP registry. It shows an increasing prevalence of HPN, namely in the palliative patient group. The sharing of national data can improve understanding of this rare condition and facilitate the development of international guidelines.

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Section: Introductionmentioning

confidence: 99%

The Czech Home Parenteral Nutrition Registry REDNUP: Comprehensive Analysis of Adult Patients’ Data

Koudelková,

Waldauf,

Wohl

et al. 2024

Ann Nutr Metab

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Short bowel syndrome related intestinal failure outcomes in Latin America: Insights from the RESTORE Registry

Rumbo,

Solar,

Ortega

et al. 2024

J Parenter Enteral Nutr

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BackgroundShort bowel syndrome is considered a low prevalence disease. The scant information available about intestinal failure in Latin America was the driving force to expand this registry.MethodsA prospective, multicenter observational registry was created for patients with chronic intestinal failure short bowel at specialized centers in Latin America. Demographics, clinical characteristics, nutrition assessment, parenteral nutrition management, intestinal rehabilitation, related complications, clinical outcome, and survival were analyzed.ResultsFrom May 2020 to July 2023, 167 patients (115 adults, 52 children) from 20 centers were enrolled. For the adults, the mean age was 37.2 ± 18 years, 48% were female, and the mean follow‐up was 22.6 ± 18.3 months. The main etiology was surgical resections (postsurgical complications: 37%; ischemia: 25%); the mean intestinal length was 73 ± 55 cm. The complications were as follows: infections: 0.4/1000 catheter‐days; thrombosis: 0.24/1000 catheter‐days; liver disease: 2.6%. The outcomes were as follows: 28% were rehabilitated, 15% died, 9.6% were lost to follow‐up, 0.9% underwent transplant, and 45.6% continued follow‐up. For the children, the mean age 48 ± 52 months, 48% were female, 52% were premature. The mean follow‐up was 17.2 ± 5.6 months; the mean remaining intestinal length was 38 ± 45 cm. The leading etiologies were atresia (25%), NEC (23%), and gastroschisis (21%). The complication were as follows: infections: 2/1000 catheter‐days; thrombosis: 2.22/1000 catheter‐day; 25% developed liver disease. The outcomes were as follows: 7.7% died, 3.8% were rehabilitated, and 88.5% continued follow‐up.ConclusionThe RESTORE amendment served as a registry and educational tool for the participating teams. The aspiration is to objectively show current aspects of intestinal failure in the region and carry them to international standards. Including all Latin American countries and etiologies of chronic intestinal failure besides short gut would serve to complete this registry.

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Epidemiology, survival, costs, and quality of life in adults with short bowel syndrome

Winkler

Tappenden

2023

Nut in Clin Prac

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Short bowel syndrome (SBS) is a rare disorder with known physical, psychosocial, and economic burdens and significant morbidity and mortality. Many individuals with SBS require long-term home parenteral nutrition (HPN). The incidence and prevalence of SBS is difficult to determine because it is often based on HPN usage and may not account for those who receive intravenous fluids or achieve enteral autonomy. The most common etiologies associated with SBS are Crohn's disease and mesenteric ischemia. Intestinal anatomy and remnant bowel length are prognostic for HPN dependency, and enteral autonomy confers a survival advantage. Health economic data confirm that PN-related costs are higher for hospitalizations than at home; yet significant healthcare resource utilization is necessary for successful HPN, and patients and families report substantial financial distress that impacts quality of life (QOL). An important advancement in QOL measurement is the validation of HPN-and SBS-specific QOL questionnaires. In addition to the known factors negatively impacting QOL, such as diarrhea, pain, nocturia, fatigue, depression, and narcotic dependency, research has shown that the volume and number of PN infusions per week is associated with QOL.Although traditional QOL measurements describe how underlying disease and therapy influence life, they do not assess how symptoms and functional limitations affect the QOL of patients and caregivers. Patient-centered measures and conversation focused on psychosocial issues helps patients with SBS and HPN dependency better cope with their disease and treatment. This article presents a brief overview of SBS, including epidemiology, survival, costs, and QOL.

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

Cited by 8 publications

References 42 publications

The Czech Home Parenteral Nutrition Registry REDNUP: Comprehensive Analysis of Adult Patients’ Data

The Czech Home Parenteral Nutrition Registry REDNUP: Comprehensive Analysis of Adult Patients’ Data

Short bowel syndrome related intestinal failure outcomes in Latin America: Insights from the RESTORE Registry

Epidemiology, survival, costs, and quality of life in adults with short bowel syndrome

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