Funding and resource availability for home parenteral nutrition in Australia: A national cross-sectional survey.

BACKGROUND: Home parenteral nutrition (HPN) is a life-saving therapy required for the management of type III intestinal failure, one of the rarest organ failures. It requires a multidisciplinary approach to manage the complexity of the underlying medical, surgical, and nutrition issues, but the current levels of healthcare funding in Australia are unknown. This study aimed to quantify the caseload, staffing, and capacity of existing HPN centers nationally. METHODS: This was a cross-sectional survey inviting centers known to provide HPN care. The survey was designed to capture metrics related to the national framework for the delivery of HPN. These centered on staffing levels, patient load, capacity to audit key outcomes, and service challenges. RESULTS: A total of 24 (89%) of 27 invited centers responded to the survey. There were 17 (71%) adult centers and 7 (29%) pediatric centers. Adult centers managed a median of 12 (interquartile range [IQR]: 6-25) patients vs 16 (IQR: 9-17) in pediatric centers. Several centers did not have dedicated funding for core team members. The total funded clinician time each week per patient was 7 min (IQR: 0-12 min) in adult centers and 14 min (IQR: 10-21 min) in pediatric centers. Fewer than half of centers reported having sufficient resources to regularly audit key metrics. CONCLUSION: The availability of dedicated expertise to manage the highly complex needs of people living with type III intestinal failure is lacking in Australia. Current funding of HPN services falls well short of being sufficient to meet the requirements outlined in the national quality framework.

[The registers of pediatric patients with home artificial nutrition. The Spanish experience]. / Los registros de pacientes pediátricos con nutrición artificial en el domicilio. La experiencia española.

Introduction: Background: the number of infants and children who receive artificial nutrition at home has been steadily increasing over the last decades, as better outcomes for children with chronic conditions have been achieved. In order to evaluate the need of resources to implement the technique it is necessary to know how many patients benefit from home artificial nutrition. This information can be estimated from the register of patients, when available. Methods: in this paper the characteristics of all registers were reviewed, especially those devoted to pediatric patients. Results: only two pediatric registers are active in 2023: the Canadian register and the Spanish one. NADYA register from the Spanish Society for Clinical Nutrition and Metabolism (SENPE) and the recent REPAFI, form the Spanish Society of Pediatric Gastroenterology, Hepatology and Nutrition. The most valuable register from the British Society, BANS, stopped providing information in 2018. Conclusion: despite the fact of acknowledging the importance of having gathered information on the prevalence and incidence of home artificial nutrition, to fit resources to necessities, the number of active registers is quite short.

Introducción: Introducción: el número de pacientes pediátricos que reciben nutrición artificial en el domicilio (NAD) ha crecido en los países occidentales en la medida que ha mejorado el pronóstico de algunas enfermedades graves potencialmente letales. Con el fin de poder estimar la cantidad de recursos necesarios para implementar esta técnica, es necesario conocer el volumen real de pacientes que precisan NAD. Una manera aproximada de conocer estas necesidades puede establecerse a partir de los datos de los registros nacionales de pacientes. Métodos: se revisan las características de los registros de pacientes pediátricos con NAD, tanto los activos como los que no han continuado en el tiempo, con especial atención a los registros españoles. Resultados: solo dos registros de pacientes permanecen activos en 2023, el registro canadiense y los registros españoles de la Sociedad Española de Nutrición Clínica y Metabolismo (SENPE), a través del registro NADYA y el de la Sociedad Española de Gastroenterología Hepatología y Nutrición Pediátrica, registro REPAFI. El registro más completo de la Sociedad Británica, BANS, dejó de publicar resultados en 2018. Conclusiones: a pesar de reconocer la importancia de presentar datos actualizados de prevalencia e incidencia del uso de la NAD, con el fin de adecuar los recursos a las necesidades, el número de registros activos de pacientes pediátricos es excepcional.

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

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 were 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 were analysed in the following categories: epidemiology, demographics, underlying syndrome, 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 REgistr Domaci NUtricni Podpory (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.

Revisión sobre la experiencia en vida real de teduglutida / Review of real-life teduglutide experience

Introducción: la teduglutida es un agonista del péptido relacionado con glucagón (aGLP2) eficaz como tratamiento de pacientes con síndrome de intestino corto (SIC) una entidad que afecta a la calidad de vida, suele precisar de nutrición parenteral domiciliaria (NPD) y genera importantes costes sanitarios. El objetivo de la presente revisión narrativa fue evaluar la experiencia en vida real reportada con teduglutida. Métodos y resultados: en vida real un metaanálisis y estudios publicados con 440 pacientes, indican que teduglutida es efectivo pasado el periodo de adaptación intestinal posterior a la cirugía, reduciendo las necesidades de NPD y en algunos casos permite incluso suspenderla. La respuesta es heterogénea, aumenta progresivamente hasta 2 años después del inicio del tratamiento y alcanza el 82 % en algunas series. La presencia de colon en continuidad es factor predictivo negativo de respuesta precoz, pero un factor predictivo positivo para la retirada de NPD. Los efectos adversos más frecuentes son de origen gastrointestinal en las primeras etapas del tratamiento. Hay complicaciones tardías relacionadas con el estoma o con la aparición de pólipos de colon, aunque la frecuencia de estas últimas es muy baja. En adultos son escasos los datos en mejoría de calidad de vida y en coste eficacia. Conclusiones: teduglutida es efectivo y seguro confirmándose en vida real los datos de los ensayos pivotales para tratamiento de pacientes con SIC y permite reducir o incluso suspender en algunos casos la NPD. Aunque parece coste efectivo son necesarios más estudios para identificar aquellos pacientes con mayor beneficio.(AU)

Background: teduglutide is an agonist of glucagon-related peptide (aGLP2) effective as a treatment for patients with short bowel syndrome (SBS), an entity that affects quality of life, usually requires home parenteral nutrition (HPN) and generates significant health costs. The objective of the present narrative review was to assess the real-life experience reported with teduglutide.Methods and results: in real life, one meta-analysis and studies published with 440 patients indicate that Teduglutide is effective after the period of intestinal adaptation after surgery, reducing the need for HPN and in some cases even allowing it to be suspended. The response is heterogeneous, increasing progressively up to 2 years after the start of treatment and reaching 82 % in some series. The presence of colon in continuity is a negative predictor of early response, but a positive predictive factor for the withdrawal of HPN. The most common side effects are gastrointestinal in the early stages of treatment. There are late complications related to the stoma or the occurrence of colon polyps, although the frequency of the latter is very low. In adults, data on improved quality of life and cost-effectiveness are scarce. Conclusions: teduglutide is effective and safe and data from pivotal trials for the treatment of patients with SBS are confirmed in real life and can reduce or even stop HPN in some cases. Although it seems cost-effective, more studies are needed to identify those patients with the greatest benefit.(AU)

Trend Observations in Home Parenteral Nutrition. Prevalence, Hospitalizations and Costs: Results from a Nationwide Analysis of Health Care Provider Data.

BACKGROUND: The population of patients on home parenteral nutrition (HPN) worldwide is growing. Since only a few counties provide data from national registries long-term observations are valuable to address this specific area of nutrition support. This study is a nationwide analysis determining the trends in the epidemiology of HPN (prevalence, age distribution, death rates), indications for HPN, causes for hospitalizations, and cost analysis of HPN reimbursement in Poland between 2010-2020. METHODS: A retrospective analysis of data obtained from the national health fund (NHF) of Poland on adult patients on HPN. RESULTS: The prevalence of adult patients on HPN in Poland in 2020 was 53.26 per million citizens with a 2.99-fold increase and a growing trend observed from 2010. Significant decrease in the percentage of patients between 18-34, 45-54 and an increase in patients between 65-74 and patients over 75 years old was observed. Trend analysis showed an increase in new patients between 65-74 and a decrease between 35-54. Malnutrition (34.28%), postprocedural disorders of the GI tract (19.61%), intestinal malabsorption/other intestinal diseases (20.41%) and GI obstruction due to cancer (17.36% as primary and 23.16% as secondary diagnosis) were mostly reported as the primary indications for HPN. Cancer patients were mostly gastric, ovarian and colon cancer (34.74%, 17.83% and 12.3%). HPN and total health cost reimbursement increase was 2.6 and 2.57-fold respectively. Costs of HPN and total health care costs in 2020 per patient were € 10,015 and € 16,038, respectively. Overall death risk rate during the first year of nutrition was 0.59 with a significant increase in the observation period p-trend < 0.004. A significant increase in the death rate was observed in patients above 75 years old (estimate 1.629, p-trend < 0.030). Cancer, infection, malnutrition and GI symptoms were the most common indications for hospitalizations of HPN patients. The rate of patients with a maximal length of HPN of 5 months in 2010 was 54.9% and was growing up to 78.1% in 2020. CONCLUSIONS: The prevalence of HPN in Poland is growing. Trends of age distribution show increasing numbers of patients with more advanced age and shorter survival. Costs of HPN are comparable with other European data.

Pediatric Home Parenteral Nutrition in France: A six years national survey.

BACKGROUND AND AIMS: Home Parenteral Nutrition (HPN) is the cornerstone management for children suffering from chronic intestinal failure (CIF). In France, HPN is organized from a network of 7 certified centers located in University Hospitals spread across the national territory. This study aims to review the data involving children on HPN over a 6-years period in France to outline the global and continuous improvement in care. PATIENTS AND METHODS: This cross-sectional study included all children enrolled in any of the 7 French HPN certified centers from January 1st, 2014 to December 31st, 2019. Data was recorded from annual databases provided by each center regarding: age at inclusion, indication and duration of HPN, type of intravenous lipid emulsion (ILE), outcome [PN weaning off, transfer to adult center, death, intestinal transplantation (ITx)], rate of catheter-related bloodstream infections (CRSBIs) for 1000 days of HPN, Taurolidine lock procedure (TLP) use and prevalence of cholestasis defined as conjugated bilirubin ≥20 µmol/l. RESULTS: The number of patients increased by 43.6% from 268 in 2014 to 385 in 2019. According to the year of follow up, the indications for HPN were short bowel syndrome (SBS) (42.3-46.6%), congenital enteropathies (CE) (18.5-22.8%), chronic intestinal pseudo-obstruction syndrome (CIPOS) (13.0-16.3%), long segment Hirschsprung's disease (LSHD) (9.7-13.3%), Crohn's disease (CD) (1.6-2.6%) and other non-primary digestive diseases (NPDD) such as immune deficiency, cancer or metabolic disease (4.0-9.2%). The median age at discharge on HPN decreased from 11.7 months in 2014 to 8.3 months in 2019 (p < .001). By December 31st, 2019, 44.8% of children had left the HPN program after a median duration ranging between 39.9 and 66.4 months. Among these patients, 192 (74.2%) were weaned off PN (94.7% SBS), 41 (15.8%) were transferred to adult centers for CIPOS (42%), SBS (31%) or CE (27%), 21 died (8.1%) - mostly in relation to cancer or immune deficiency - and 5 were transplanted (1.9%): 4 underwent combined liver-intestine transplantation for LSHD (n = 2), SBS, CE and one multivisceral Tx for CIPOS. The use of a composite fish-oil based ILE increased from 67.4% in 2014 to 88.3% in 2019 (p < 0.001). CRBSIs dropped from 1.04 CRSBIs per 1000 days HPN in 2014 to 0.61 in 2019 (p < 0.001) while meantime, the percentage of children receiving TLP increased from 29.4% to 63.0% (p < 0.001). The prevalence of cholestasis (conjugated bilirubin ≥ 20 µmol/l) was low and stable between 4.1 and 5.9% of children during the study period. CONCLUSION: In France, the number of children enrolled in a HPN program continuously increased over a 6 years period. SBS is the leading cause of CIF requiring HPN. The rate of CRBSIs dropped dramatically as the use of TLP increased. Mortality rate was low and mainly in relation to the underlying disease (cancer, immune deficiency). Cholestasis and intestinal Tx remained very rare.

International Latin American Survey on Pediatric Intestinal Failure Team.

There is little data on the experience of managing pediatric Intestinal Failure (IF) in Latin America. This study aimed to identify and describe the current organization and practices of the IF teams in Latin America and the Caribbean. An online survey was sent to inquire about the existence of IF teams that managed children on home parenteral nutrition (HPN). Our questionnaire was based on a previously published European study with a similar goal. Twenty-four centers with pediatric IF teams in eight countries completed the survey, representing a total number of 316 children on HPN. The median number of children on parenteral nutrition (PN) at home per team was 5.5 (range 1-50). Teams consisted of the following members: pediatric gastroenterologist and a pediatric surgeon in all teams, dietician (95.8%), nurse (91.7%), social worker (79.2%), pharmacist (70.8%), oral therapist (62.5%), psychologist (58.3%), and physiotherapist (45.8%). The majority of the centers followed international standards of care on vascular access, parenteral and enteral nutrition, and IF medical and surgical management, but a significant percentage reported inability to monitor micronutrients, like vitamins A (37.5%), E (41.7%), B1 (66.7%), B2 (62.5%), B6 (62.5%), active B12 (58.3%); and trace elements-including zinc (29.2%), aluminum (75%), copper (37.5%), chromium (58.3%), selenium (58.3%), and manganese (58.3%). Conclusion: There is wide variation in how IF teams are structured in Latin America-while many countries have well-established Intestinal rehabilitation programs, a few do not follow international standards. Many countries did not report having an IF team managing pediatric patients on HPN.

Home parenteral nutrition patients on mixed oil lipid emulsion have a higher rate of hospitalizations compare to those on soybean oil- a prospective 2-year cohort study.

BACKGROUND & AIMS: Mixed oil intravenous lipid emulsion (MO ILE) that contains 30% soybean oil (SO), 30% medium chain triglycerides, 25% olive oil and 15% fish oil can benefit hospitalized patients receiving parenteral nutrition (PN) but there are very few studies on its long-term use. Our goal was to evaluate the clinical outcomes of adults receiving home PN (HPN) with MO versus those receiving SO ILE over a 2-year period. METHOD: This is a retrospective analysis of data collected prospectively from a cohort of patients recorded in the Canadian HPN Registry over a 2-year period. HPN patients from academic programs across Canada were entered in the Registry according to a validated protocol. For this study, demographic, nutritional, laboratory and clinical data were extracted from January 1st 2015, when MO lipid emulsion became available in Canada, to July 24th 2019. Clinical data for each patient included: number of hospitalizations, number of hospitalizations related to HPN and number of hospitalization days related to HPN, over a year; incidence of line sepsis per 1000 catheter days and mortality. Data are presented as median (1st, 3rd quartile) for continuous variables and frequency (percentage) for categorical variables. Comparisons between groups were performed using two sample t-test or Wilcoxon Rank Sum tests for continuous variables and Chi-square tests or Fisher's exact tests for categorical variables. Univariate and multiple linear regressions were also carried out. Statistical significance is set at a p-value <0.05. RESULTS: A total of 120 patients were included (MO n = 68, SO n = 52). Significant differences at baseline between the two groups were a higher use of Hickman line (62.12% vs 42%, p = 0.038) and more western Canada based hospital care with MO (75% vs 42.31%, p = 0.0002). The MO group had significantly more hospitalizations (p = 0.001), more hospitalizations related to HPN (p = 0.012) and more hospitalization days related to HPN (p = 0.016) per patient per year compared to SO patients. There was no significant difference between groups for line sepsis per 1000 catheter days (MO: 0.05 (0.0, 1.0) vs SO: 0.0 (0.0, 0.22), p = 0.053) or mortality. All other variables, including biochemical variables, were similar between groups. In a multiple regression analysis, the following factors were significantly associated with a greater number of hospitalizations per patient per year: use of MO, high blood glucose from the last recorded value and having died by the end of the study period. CONCLUSION: This 2-year prospective cohort study suggests an increased risk of hospitalization in HPN patients receiving MO lipid emulsion. The long-term effect of using MO lipid emulsion in HPN patients should be further evaluated using a large randomized controlled trial. THE STUDY WAS REGISTERED IN CLINICALTRIALS.GOV: (NCT02299466).

The prevalence of liver fibrosis according to non-invasive tools in a pediatric home parenteral nutrition cohort.

BACKGROUND & AIMS: Liver biopsy is no viable tool to routinely screen for liver fibrosis in children suffering from chronic intestinal failure (IF). We aim to assess the prevalence of liver fibrosis in a cohort of children with chronic IF by non-invasive tests: transient elastography (TE), aspartate-aminotransferase-to-platelet-ratio-index (APRI) and enhanced liver fibrosis (ELF) score. METHODS: Cross sectional study where patients with chronic IF, receiving parenteral nutrition (PN) for at least 3 months, were enrolled. TE, APRI and ELF score were measured. Using Spearman's rank correlation coefficient and Kruskal-Wallis H test, the correlation between TE, APRI, ELF score and known risk factors for development of intestinal failure-associated liver disease (IFALD) were calculated. RESULTS: 32 patients were included (50% female), median age was 8 years and 4 months, median PN duration was 45 months. Six patients (21%) had TE ≥6.5 kPa, indicating significant fibrosis. Twelve patients (38%) had APRI ≥.5, indicating fibrosis. ELF score indicated moderate fibrosis in 17 patients (63%) and significant fibrosis in 10 patients (37%). TE and APRI correlated significantly with known risk factors for IFALD, but ELF showed poor correlation with known risk factors for IFALD. CONCLUSION: In a cohort of pediatric patients suffering from chronic IF, TE measurement, APRI and ELF test show a varying, but substantial proportion of subjects with fibrosis. The diagnostic value of these tests and their role in the management of pediatric IF must be determined in larger cohorts with liver biopsy as reference standard. TRIAL REGISTRATION: Academic Medical Center medical ethics committee number: METC 2017_185.

Metabolic bone disease in children with intestinal failure is not associated with the level of parenteral nutrition dependency.

BACKGROUND & AIMS: Children on long-term home parenteral nutrition (HPN) are at increased risk of suboptimal growth and metabolic bone disease (MBD) i.e. decreased bone mineral density (BMD). The aims of this cross-sectional study were to assess growth and bone health in children on long term HPN and to identify risk factors for MBD. METHODS: Children above the age of 5 years, stable on HPN for more than 2 years were included. Medical files were reviewed retrospectively and included demographics, gestational age, birth weight and height, indication for PN, age at PN start, duration of PN, number of weekly PN infusions, weight-for-age and height-for-age (SD), body mass index (BMI, kg/m2) as well as blood and urine analyses at the time of Dual X-ray absorptiometry (DXA) measurements. All BMD values were adjusted to statural age which corresponds to the 50th percentile of height. Growth failure (height-for-age ≤ -2SD) and MBD (at least one BMD measurement ≤ -2SD) were analyzed according to the indication of PN, duration of PN and PN dependency index (PNDI) by comparing means and performing logistic regression analysis. PNDI is the ratio of non-protein energy intake in HPN to resting energy expenditure using Schofield equations. RESULTS: Forty children were assessed at 12.4 ± 4.5 years of age. Mean age at PN start was 1.1 ± 3.6 y (median 0.5). The indications for PN were short bowel syndrome (SBS, n = 21), chronic intestinal pseudo-obstruction syndrome (CIPOS, n = 10) and congenital enteropathies (CE, n = 9). The mean number of PN perfusions was 6 ± 1/week. PNDI was 110 ± 30%. The mean serum level of 25-OHD3 was suboptimal at 26.5 ± 9.1 ng/mL (66.2 ± 22.8 nmol/L). The mean concentrations of calcium, phosphorus, and parathyroid hormone (PTH) were in the normal ranges. Eight children (20%) had PTH levels above normal with low 25-OHD3 levels. The mean weight-for-age and height-for-age Z-scores SDS were 0.4 ± 0.9 and -0.5 ± 1.1 respectively. The actual height was lower than genetic target height (p < 0.001). The BMD Z-scores, adjusted to the 50th percentile of height, of the spine, the left femur and the whole body were: -1.1 ± 1.7, -1.2 ± 1.5 and -1.5 ± 1.8 SDS respectively. Children with CE had significantly lower BMD values than those with SBS and CIPOS (p = 0.01). Only two children had bone fractures after a mild trauma (5%). CONCLUSIONS: All children on long-term PN, are at risk of low BMD. High dependency on PN (PNDI>120%) and very long-term PN (>10 years) do not appear to increase the risk of growth failure nor MBD. PN-related bone fractures were rare. Close follow-up remains mandatory.

Results from an Epidemiological Follow-Up Survey on Home Artificial Nutrition in Switzerland from 2010 to 2015.

BACKGROUND: Home artificial nutrition (HAN) is an established treatment for malnourished patients. Since July 2012, the costs for oral nutrition supplements (ONS) are covered by the compulsory health insurance providers in Switzerland if the patient has a medical indication based on the Swiss Society for Clinical Nutrition guidelines. Therefore, the purpose of our study was to analyse the development of HAN, including ONS, before and after July 2012. METHODS: We obtained the retrospective and anonymized data from the Swiss association for joint tasks of health insurers (SVK), who registered patients on HAN. Since not all health insurers are working with SVK, this retrospective study recorded nearly 65% of all new patients on HAN in Switzerland from January 1, 2010, to December 31, 2015. RESULTS: A total of 33,410 patients (49.1% men and 50.9% women) with a mean BMI of 21.3 ± 4.5 kg/m2 and mean age of 68.9 ± 17.8 years were recorded. The number of patient cases on ONS increased from 808 cases in 2010 to 18,538 cases in 2015, while patient cases on home enteral nutrition (HEN) and home parenteral nutrition (HPN) remained approximately the same. The relative distribution of type of HAN changed from 26.2% cases on ONS, 68.7% cases on HEN and 5.1% cases on HPN in 2010 to 86.1% cases on ONS, 12.8% cases on HEN, and 1.1% cases on HPN in 2015. Treatment duration decreased for ONS from 698 ± 637 days to 171 ± 274 days, for HEN from 416 ± 553 days to 262 ± 459 days, and for HPN from 96 ± 206 days to 72 ± 123 days. Mean costs per patient decreased for ONS from 1,330 CHF in 2010 to 606 CHF in 2015. Total costs for HAN increased from 16,895,373 CHF in 2010 to 32,868,361 CHF in 2015. CONCLUSION: Our epidemiological follow-up study showed an immense increase in number of patients on HAN in Switzerland after July 2012. Due to shorter therapy duration and reduced mean costs per patient, total costs were only doubled while the number of patients increased 7-fold.

Evolución de los pacientes con nutrición parenteral domiciliaria: una revisión sistemática de los estudios disponibles / Evolution of patients with home parenteral nutrition: a systematic review of available studies

INTRODUCCIÓN: la nutrición parenteral domiciliaria (NPD) constituye el tratamiento de elección para pacientes con fallo intestinal que no requieren ingreso hospitalario. En esta revisión sistemática, nuestro objetivo fue realizar un análisis epidemiológico y de práctica clínica en NPD. MÉTODOS: la revisión se realizó siguiendo la guía PRISMA. Para ello, se hizo una búsqueda bibliográfica en PubMed® y EMBASE® de los estudios publicados entre 2009 y 2019 en inglés o español que realizaran el seguimiento de pacientes con NPD durante al menos 5 años. Esta búsqueda se completó manualmente. Se excluyeron los artículos centrados únicamente en pacientes oncológicos, una patología específica o embarazadas o bien en complicaciones. RESULTADOS: se identificaron 267 artículos, de los que cumplieron los criterios 9 (3 de población pediátrica y 6 de adultos). En adultos, la principal diferencia entre los estudios fue la patología de base. La indicación mayoritaria fue el síndrome de intestino corto y la causa de exitus, la enfermedad primaria. La mayor parte de la población pediátrica recibió este apoyo en los primeros meses de vida. Además, destaca el mayor porcentaje de conversión a vía oral y el menor número de fallecimientos frente a población adulta. CONCLUSIONES: la utilización de la NPD en patología oncológica está sometida a una amplia variabilidad geográfica. Sería recomendable establecer directrices de uso en enfermos oncológicos y la realización de estudios de calidad que aporten información rigurosa y homogénea

INTRODUCTION: home parenteral nutrition (HPN) is a fundamental treatment for patients with intestinal failure who do not require hospitalization. We aimed to conduct an epidemiological and clinical practice analysis of HPN through a systematic review. METHODS: the systematic review was conducted according to the PRISMA guidelines. A search was performed using the Healthcare Databases Advanced Search of PubMed® and EMBASE®, to identify articles which followed patients treated with HPN for at least 5 years, published between 2009 and 2019 in English or Spanish language. In addition, we manually retrieved other publications of interest. We excluded articles about subgroups of patients with a specific pathology, cancer or pregnant patients. We excluded studies collecting exclusively HPN complications. RESULTS: a total of 267 references were identified, of which 9 met criteria (3 of pediatric population and 6 of adults). In adults, the main difference found between publications was the underlying pathologies. The most common indication was short bowel syndrome and the main cause of exitus was the underlying pathology. Most of the pediatric patients received this support in the first months of life. In addition, children showed a higher conversion rate to oral intake and a lower number of deaths when compared to adults. CONCLUSIONS: the use of HPN in cancer pathology is subject to wide geographic variability. It would be advisable to establish indication guidelines in patients with cancer and conduct quality studies, which provide rigorous and homogeneous information

Clinical characteristics and predictive factors of survival of 761 cancer patients on home parenteral nutrition: A prospective, cohort study.

BACKGROUND: Robust data reporting the survival of cancer patients on home parenteral nutrition (HPN) are lacking. The aim of this prospective, cohort study was to investigate clinical characteristics, predictive factors, and overall survival (OS) of adult-malnourished cancer patients eligible for HPN according to the European guideline recommendations. METHODS: During the study period, 1658 cancer patients were consecutively evaluated in a tertiary university hospital. Of these, 761 who received HPN were grouped into four cohorts according to the provision of supplemental PN (SPN) or total (TPN) and whether they received chemotherapy (CT+ or CT- ): SPN/CT+ (n = 376), TPN/CT+ (n = 99), SPN/CT- (n = 191), and TPN/CT- (n = 95). Patient demographics, nutritional status, cancer-related characteristics, and prognostic scores assessed at HPN start. The primary outcome was OS. RESULTS: Median OS was 8.9, 4.3, 5.7, and 2.2 months for the SPN/CT+ , TPN/CT+ , SPN/CT- , and TPN/CT- cohorts, respectively. In multivariable analysis, predictors showing significant association with decreased survival were patient cohorts, modified Glasgow Prognostic Score (1 and 2 scores), weight loss (>15%) in the 3 months before HPN start, and TNM IV stage while protective factors of survival were Karnofsky Performance Status (>50), albumin level (>3.5 g/dL), oral protein intake, BMI (>20.5), and weight at HPN start. CONCLUSION: For the first time, in four different cohorts of cancer patients on HPN, clinical characteristics and survival were compared. This large study showed that survival is significantly correlated with patient characteristics at HPN start and that the presence of favorable factors may determine even a fourfold increase in survival. These data are expected to assist physicians in the appropriate prescription of HPN.

[Evolution of patients with home parenteral nutrition: a systematic review of available studies]. / Evolución de los pacientes con nutrición parenteral domiciliaria: una revisión sistemática de los estudios disponibles.

INTRODUCTION: Introduction: home parenteral nutrition (HPN) is a fundamental treatment for patients with intestinal failure who do not require hospitalization. We aimed to conduct an epidemiological and clinical practice analysis of HPN through a systematic review. Methods: the systematic review was conducted according to the PRISMA guidelines. A search was performed using the Healthcare Databases Advanced Search of PubMed® and EMBASE®, to identify articles which followed patients treated with HPN for at least 5 years, published between 2009 and 2019 in English or Spanish language. In addition, we manually retrieved other publications of interest. We excluded articles about subgroups of patients with a specific pathology, cancer or pregnant patients. We excluded studies collecting exclusively HPN complications. Results: a total of 267 references were identified, of which 9 met criteria (3 of pediatric population and 6 of adults). In adults, the main difference found between publications was the underlying pathologies. The most common indication was short bowel syndrome and the main cause of exitus was the underlying pathology. Most of the pediatric patients received this support in the first months of life. In addition, children showed a higher conversion rate to oral intake and a lower number of deaths when compared to adults. Conclusions: the use of HPN in cancer pathology is subject to wide geographic variability. It would be advisable to establish indication guidelines in patients with cancer and conduct quality studies, which provide rigorous and homogeneous information.

INTRODUCCIÓN: Introducción: la nutrición parenteral domiciliaria (NPD) constituye el tratamiento de elección para pacientes con fallo intestinal que no requieren ingreso hospitalario. En esta revisión sistemática, nuestro objetivo fue realizar un análisis epidemiológico y de práctica clínica en NPD. Métodos: la revisión se realizó siguiendo la guía PRISMA. Para ello, se hizo una búsqueda bibliográfica en PubMed® y EMBASE® de los estudios publicados entre 2009 y 2019 en inglés o español que realizaran el seguimiento de pacientes con NPD durante al menos 5 años. Esta búsqueda se completó manualmente. Se excluyeron los artículos centrados únicamente en pacientes oncológicos, una patología específica o embarazadas o bien en complicaciones. Resultados: se identificaron 267 artículos, de los que cumplieron los criterios 9 (3 de población pediátrica y 6 de adultos). En adultos, la principal diferencia entre los estudios fue la patología de base. La indicación mayoritaria fue el síndrome de intestino corto y la causa de exitus, la enfermedad primaria. La mayor parte de la población pediátrica recibió este apoyo en los primeros meses de vida. Además, destaca el mayor porcentaje de conversión a vía oral y el menor número de fallecimientos frente a población adulta. Conclusiones: la utilización de la NPD en patología oncológica está sometida a una amplia variabilidad geográfica. Sería recomendable establecer directrices de uso en enfermos oncológicos y la realización de estudios de calidad que aporten información rigurosa y homogénea.

[Home and Ambulatory Artificial Nutrition (NADYA) Group Report. Home parenteral nutrition in Spain, 2018]. / Nutrición parenteral domiciliaria en España 2018. Informe del Grupo de Nutrición Artificial Domiciliaria y Ambulatoria NADYA.

INTRODUCTION: Aim: to communicate home parenteral nutrition (HPN) data obtained from the HPN registry of the NADYA-SENPE group (www.nadya-senpe.com) for the year 2018 Material and methods: descriptive analysis of the data collected from adult and pediatric patients with HPN in the NADYA-SENPE group registry from January 1st, 2018 to December 31st, 2018. Results: there were 278 patients from 45 Spanish hospitals (54.7% women), 23 children and 255 adults, which represent a prevalence rate of 5.95 patients/million inhabitants/year 2018. The most frequent diagnosis in adults was "palliative cancer" (22.0%), followed by "others". In children it was Hirschsprung's disease together with necrotizing enterocolitis, with four cases (17.4%). The first indication was short bowel syndrome in both children (60.9%) and adults (35.7%). The most frequently used type of catheter was tunneled in both children (81.0%) and adults (41.1%). Ending 75 episodes, the most frequent cause was death (52.0%) and change to oral feeding (33.3%). Conclusions: the number of centers and collaborating professionals in the registry of patients receiving HPN remains stable, as well as the main indications and reasons for termination of HPN.

INTRODUCCIÓN: Objetivo: comunicar los datos de nutrición parenteral domiciliaria (NPD) obtenidos del registro del grupo NADYA-SENPE (www.nadya-senpe.com) del año 2018. Material y métodos: análisis descriptivo de los datos recogidos de pacientes adultos y pediátricos con NPD en el registro NADYA-SENPE del 1 de enero al 31 de diciembre de 2018. Resultados: se registraron 278 pacientes (54,7% mujeres), 23 niños y 255 adultos, procedentes de 45 hospitales españoles, lo que representa una tasa de prevalencia de 5,95 pacientes/millón de habitantes/año 2018. El diagnóstico más frecuente en adultos fue "oncológico paliativo" (22,0%), seguido de "otros". En niños fue la enfermedad de Hirschsprung junto con la enterocolitis necrotizante, con cuatro casos (17,4%). El primer motivo de indicación fue síndrome de intestino corto tanto en niños (60,9%) como en adultos (35,7%). El tipo de catéter más utilizado fue el tunelizado tanto en niños (81,0%) como en adultos (41,1%). Finalizaron 75 episodios, la causa más frecuente fue el fallecimiento (52,0%) y el paso a vía oral (33,3%). Conclusiones: el número de centros y profesionales colaboradores en el registro de pacientes que reciben NPD se mantiene estable, así como las principales indicaciones y los motivos de finalización de la NPD.

Home parenteral nutrition provision modalities for chronic intestinal failure in adult patients: An international survey.

BACKGROUND & AIMS: The safety and effectiveness of a home parenteral nutrition (HPN) program depends both on the expertise and the management approach of the HPN center. We aimed to evaluate both the approaches of different international HPN-centers in their provision of HPN and the types of intravenous supplementation (IVS)-admixtures prescribed to patients with chronic intestinal failure (CIF). METHODS: In March 2015, 65 centers from 22 countries enrolled 3239 patients (benign disease 90.1%, malignant disease 9.9%), recording the patient, CIF and HPN characteristics in a structured database. The HPN-provider was categorized as health care system local pharmacy (LP) or independent home care company (HCC). The IVS-admixture was categorized as fluids and electrolytes alone (FE) or parenteral nutrition, either commercially premixed (PA) or customized to the individual patient (CA), alone or plus extra FE (PAFE or CAFE). Doctors of HPN centers were responsible for the IVS prescriptions. RESULTS: HCC (66%) was the most common HPN provider, with no difference noted between benign-CIF and malignant-CIF. LP was the main modality in 11 countries; HCC prevailed in 4 European countries: Israel, USA, South America and Oceania (p < 0.001). IVS-admixture comprised: FE 10%, PA 17%, PAFE 17%, CA 38%, CAFE 18%. PA and PAFE prevailed in malignant-CIF while CA and CAFE use was greater in benign-CIF (p < 0.001). PA + PAFE prevailed in those countries where LP was the main HPN-provider and CA + CAFE prevailed where the main HPN-provider was HCC (p < 0.001). CONCLUSIONS: This is the first study to demonstrate that HPN provision and the IVS-admixture differ greatly among countries, among HPN centers and between benign-CIF and cancer-CIF. As both HPN provider and IVS-admixture types may play a role in the safety and effectiveness of HPN therapy, criteria to homogenize HPN programs are needed so that patients can have equal access to optimal CIF care.

Incisional hernia and enterocutaneous fistula in patients with chronic intestinal failure: prevalence and risk factors in a cohort of patients referred to a tertiary centre.

AIM: This study aims to determine the prevalence of incisional hernia (IH) and enterocutaneous fistula (ECF) in patients with intestinal failure (IF) referred to a tertiary centre and to identify factors associated with their development. METHOD: A retrospective case note review was undertaken of a prospectively maintained database of all patients on home parenteral nutrition between 2011 and 2016 at a UK tertiary referral centre for IF. Risk factors were identified using binary logistic regression. RESULTS: The database search identified 447 patients, of whom 349 (78.1%) had surgery prior to developing IF. Eighty-one (23.2%) patients had an IH and 123 (35.2%) had an ECF at the time of referral. Of these, 51 (14.6%) had both IH and ECF. IH was associated with a high body mass index (P = 0.05), a history of a major surgical complication resulting in IF (P = 0.01), previous emergency surgery (P = 0.04), increasing number of operations (P = 0.02) and surgical site infection (SSI; P = 0.01). ECF was associated with complications relating to earlier surgery. (P ≤ .001), previous treatment with an open abdomen (P = 0.03), SSI (P = 0.001), intra-abdominal collection (P ≤ 0.001) and anastomotic leak (P = 0.02). CONCLUSION: In this series, patients with IF had a prevalence of IH which was more than double that expected following elective laparotomy (about 10%) and one in three had an ECF. Risk factors for IH and ECF are discussed.

Needs-based quality of life in adults dependent on home parenteral nutrition.

BACKGROUND & AIMS: Home parenteral nutrition (HPN) provides life sustaining treatment for people with chronic intestinal failure. Individuals may require HPN for months or years and are dependent on regular intravenous infusions, usually 12-14 h overnight between 1 and 7 days each week. This regime can have adverse impact on the life of people dependent on the treatment. The aim of this study was to establish mean values for the Parenteral Nutrition Impact Questionnaire (PNIQ) and to determine the effect of disease, frequency of infusions per week and patient characteristics on quality of life of patients fed HPN. METHOD: The PNIQ was distributed to patients across nine UK HPN clinics. Data were analysed using linear regression, with PNIQ score as the dependent variable and potential confounders as independent variables. Unadjusted and adjusted models are presented. Higher PNIQ scores reflect poorer quality of life. RESULTS: Completed questionnaires were received from 466 people dependent on HPN. Mean PNIQ score was 11.04 (SD 5.79). A higher PNIQ score (effect size 0.52, CI 0.184 to 0.853) was recorded in those dependent on a higher frequency of HPN infusions per week. Respondents with cancer had a similar mean PNIQ score to those with inflammatory bowel disease (mean 10.82, SD 6.00 versus 11.04, SD 5.91). Those with surgical complications reported a poorer QoL (effect size 3.03, CI 0.642 to 5.418) and those with severe gastro-intestinal dysmotility reported a better QoL (effect size -3.03, CI -5.593 to -0.468), compared to other disease states. CONCLUSIONS: This large cohort study of quality of life in chronic intestinal failure demonstrates that HPN impacts individuals differently depending on their underlying disease. Furthermore, since the number of HPN infusions required per week is inversely related to an individual's needs-based quality of life, therapies that reduce PN burden should lead to an improvement in QoL.

[Spanish home enteral nutrition registry of the year 2016 and 2017 from the NADYA-SENPE Group]. / Registro del grupo nadya-senpe de nutrición enteral domiciliaria en españa; años 2016 y 2017.

INTRODUCTION: Objective: to present the results of the Spanish home enteral nutrition (HEN) registry of the NADYA-SENPE group for the year 2016 and 2017. Material and methods: from January 1st 2016 to December 31st 2017, the HEN registry was recorded and afterwards a further descriptive and analytical analysis was done. Results: in 2016, 4,578 active patients were recorded and prevalence was 98.33 patients per one million inhabitants; in 2017, 4,777 patients were recorded, with a prevalence of 102.57 per one million inhabitants; 50.8% were males in 2016 and 50.5% in 2017. During the period 2016-17, median age was 71.5 years (IIQ 57-83), 1,558 HEN episodes were finished and the main cause was death (793 patients, 50.89%). Adult males were younger than females (65.3 vs. 73.3 years, p-value < 0.001). The most frequent diagnosis was the neurological disorder that presents with aphagia or severe dysphagia (59%). Nasogastric tube was the most frequent administration route (48.3%) and it is the most widely used in elderly patients (p < 0.001). One hundred and twenty-six pediatric patients were registered (57.1% females). Median age at the beginning of HEN in children was four months. "Other disorders" was the most recorded diagnostic group (41.3%), followed by the group of neurological disorder that presents with aphagia or severe dysphagia. Regarding children, 57.6% were fed through gastrostomy and the younger ones were fed through nasogastric tube (p-value 0.001). Conclusions: the number of patients in the registry, as well as the number of participating centers, is progressively increasing. The main characteristics of the patients have not changed. Despite the increase in diagnostic possibilities in the pediatric population, the classification within the group of "Other pathologies" is quite significant.

INTRODUCCIÓN: Objetivo: exponer los resultados del registro de nutrición enteral domiciliaria (NED) de los años 2016 y 2017 del Grupo NADYA-SENPE. Material y métodos: se recopilaron los pacientes introducidos en el registro del 1 de enero al 31 de diciembre de 2016 y la mismas fechas de 2017 para proceder al análisis descriptivo y analítico de los datos. Resultados: en el año 2016 se obtuvieron 4.578 pacientes activos (prevalencia = 98,33 pacientes/millón de habitantes) y en 2017 fueron 4.777 (prevalencia = 102,57). Por sexos, hubo un 50,8% de varones en 2016 y un 50,5% en 2017. En el periodo 2016-17, la edad mediana fue de 71,5 años (IIQ 57-83); asimismo, finalizaron 1.558 episodios de NED y la causa principal fue el fallecimiento (793 pacientes, 50,89%). Los varones adultos fueron más jóvenes que las mujeres (65,3 vs. 73,3 años, p-valor < 0,001) y el diagnóstico más frecuente fue la enfermedad neurológica que cursa con afagia o disfagia severa (59%). La sonda nasogástrica (SNG) fue la vía de acceso más utilizada (48,3%) y se observa, además, que esta es la vía que se utiliza en los pacientes más ancianos (p < 0,001). Se registraron 126 pacientes pediátricos (57,1% niñas). La edad mediana de inicio de la NED fue de cuatro meses. Otras patologías fue el grupo diagnóstico más registrado (41,3%), seguido por la enfermedad neurológica que cursa con afagia o disfagia severa. Se alimentaban a través de gastrostomía en el 57,6% de los casos. Se observó que los niños más pequeños eran los que se alimentaban preferentemente por SNG (p-valor 0,001). Conclusiones: el número de pacientes del registro, así como el número de centros participantes, se va incrementando progresivamente. Las principales características de los pacientes no han variado. A pesar del aumento de posibilidades diagnósticas en la población pediátrica, llama la atención la clasificación dentro del grupo de Otras patologías.

Registro del Grupo NADYA-SENPE de Nutrición Enteral Domiciliaria en España; años 2016 y 2017 / Spanish home enteral nutrition registry of the year 2016 and 2017 from the NADYA-SENPE Group

Objetivo: exponer los resultados del registro de nutrición enteral domiciliaria (NED) de los años 2016 y 2017 del Grupo NADYA-SENPE. Material y métodos: se recopilaron los pacientes introducidos en el registro del 1 de enero al 31 de diciembre de 2016 y la mismas fechas de 2017 para proceder al análisis descriptivo y analítico de los datos. Resultados: en el año 2016 se obtuvieron 4.578 pacientes activos (prevalencia = 98,33 pacientes/millón de habitantes) y en 2017 fueron 4.777 (prevalencia = 102,57). Por sexos, hubo un 50,8% de varones en 2016 y un 50,5% en 2017. En el periodo 2016-17, la edad mediana fue de 71,5 años (IIQ 57-83); asimismo, finalizaron 1.558 episodios de NED y la causa principal fue el fallecimiento (793 pacientes, 50,89%). Los varones adultos fueron más jóvenes que las mujeres (65,3 vs. 73,3 años, p-valor < 0,001) y el diagnóstico más frecuente fue la enfermedad neurológica que cursa con afagia o disfagia severa (59%). La sonda nasogástrica (SNG) fue la vía de acceso más utilizada (48,3%) y se observa, además, que esta es la vía que se utiliza en los pacientes más ancianos (p < 0,001). Se registraron 126 pacientes pediátricos (57,1% niñas). La edad mediana de inicio de la NED fue de cuatro meses. Otras patologías fue el grupo diagnóstico más registrado (41,3%), seguido por la enfermedad neurológica que cursa con afagia o disfagia severa. Se alimentaban a través de gastrostomía en el 57,6% de los casos. Se observó que los niños más pequeños eran los que se alimentaban preferentemente por SNG (p-valor 0,001). Conclusiones: el número de pacientes del registro, así como el número de centros participantes, se va incrementando progresivamente. Las principales características de los pacientes no han variado. A pesar del aumento de posibilidades diagnósticas en la población pediátrica, llama la atención la clasificación dentro del grupo de Otras patologías

Objective: to present the results of the Spanish home enteral nutrition (HEN) registry of the NADYA-SENPE group for the year 2016 and 2017. Material and methods: from January 1st 2016 to December 31st 2017, the HEN registry was recorded and afterwards a further descriptive and analytical analysis was done. Results: in 2016, 4,578 active patients were recorded and prevalence was 98.33 patients per one million inhabitants; in 2017, 4,777 patients were recorded, with a prevalence of 102.57 per one million inhabitants; 50.8% were males in 2016 and 50.5% in 2017. During the period 2016-17, median age was 71.5 years (IIQ 57-83), 1,558 HEN episodes were finished and the main cause was death (793 patients, 50.89%). Adult males were younger than females (65.3 vs. 73.3 years, p-value < 0.001). The most frequent diagnosis was the neurological disorder that presents with aphagia or severe dysphagia (59%). Nasogastric tube was the most frequent administration route (48.3%) and it is the most widely used in elderly patients (p < 0.001). One hundred and twenty-six pediatric patients were registered (57.1% females). Median age at the beginning of HEN in children was four months. "Other disorders" was the most recorded diagnostic group (41.3%), followed by the group of neurological disorder that presents with aphagia or severe dysphagia. Regarding children, 57.6% were fed through gastrostomy and the younger ones were fed through nasogastric tube (p-value 0.001). Conclusions: the number of patients in the registry, as well as the number of participating centers, is progressively increasing. The main characteristics of the patients have not changed. Despite the increase in diagnostic possibilities in the pediatric population, the classification within the group of "Other pathologies" is quite significant