Expenditure and survival of adult patients with intestinal failure due to short bowel syndrome: real-world evidence from Southern Finland.

OBJECTIVES: Comprehensive follow-up data from the largest hospital district in Finland was used to assess hospital-based healthcare resource utilization (HCRU) and expenses, incidence and prevalence, survival, and effect of comorbidities/complications on survival of adult patients with intestinal failure due to short bowel syndrome (SBS-IF). METHODS: This study utilized electronic healthcare data covering all ≥18-year-old patients with SBS-IF at the Hospital District of Helsinki and Uusimaa in Finland between 2010 and 2019. Patients were followed from SBS-IF onset until the end of 2020 or death and compared to birth year and sex-matched control patients without SBS-IF. RESULTS: The study included 77 patients with SBS-IF (cases) and 363 controls. Cases had high HCRU; the cumulative expenses were about tenfold compared to the controls, at the end of the study (€123,000 vs. €14,000 per patient). The expenses were highest during the first year after SBS-IF onset (€53,000 per patient). Of the cases with a median age 62.5 years, 51.9% died during study time. The median survival was 4.4 years from SBS-IF onset and cases died 13.5 times more likely during the follow-up compared to controls. Mortality risk was lower in female cases (hazard ratio (HR) 0.46; 95% confidence intervals (CI) 0.24, 0.9) and higher with presence of comorbidities (Charlson comorbidity index HR 1.55; 95% CI 1.2, 2.0) and mesenteric infarction (HR 4.5; 95% CI 1.95, 10.36). The incidence of adult SBS-IF was 0.6 per 100,000 adults. CONCLUSION: Our study demonstrates a high demand for healthcare support and elevated mortality in adult SBS-IF-patients. Our results suggest that the presence of comorbidities is a key driver for mortality.

Neighborhood Income Is Associated with Health Care Use in Pediatric Short Bowel Syndrome.

OBJECTIVE: To evaluate associations between neighborhood income and burden of hospitalizations for children with short bowel syndrome (SBS). STUDY DESIGN: We used the Pediatric Health Information System (PHIS) database to evaluate associations between neighborhood income and hospital readmissions, readmissions for central line-associated bloodstream infections (CLABSI), and hospital length of stay (LOS) for patients <18 years with SBS hospitalized between January 1, 2006, and October 1, 2015. We analyzed readmissions with recurrent event analysis and analyzed LOS with linear mixed effects modeling. We used a conceptual model to guide our multivariable analyses, adjusting for race, ethnicity, and insurance status. RESULTS: We included 4289 children with 16 347 hospitalizations from 43 institutions. Fifty-seven percent of the children were male, 21% were Black, 19% were Hispanic, and 67% had public insurance. In univariable analysis, children from low-income neighborhoods had a 38% increased risk for all-cause hospitalizations (rate ratio [RR] 1.38, 95% CI 1.10-1.72, P = .01), an 83% increased risk for CLABSI hospitalizations (RR 1.83, 95% CI 1.37-2.44, P < .001), and increased hospital LOS (ß 0.15, 95% CI 0.01-0.29, P = .04). In multivariable analysis, the association between low-income neighborhoods and elevated risk for CLABSI hospitalizations persisted (RR 1.70, 95% CI 1.23-2.35, P < .01, respectively). CONCLUSIONS: Children with SBS from low-income neighborhoods are at increased risk for hospitalizations due to CLABSI. Examination of specific household- and neighborhood-level factors contributing to this disparity may inform equity-based interventions.

Pediatric Short Bowel Syndrome: Real-World Evidence on Incidence and Hospital Resource Use From a Finnish Data Lake.

OBJECTIVES: Little is known about the epidemiology and healthcare burden of pediatric intestinal failure (IF). We aimed to assess the incidence, prevalence, healthcare resource utilization (HCRU), and related costs of pediatric short bowel syndrome (SBS) using follow-up data from the largest hospital district in Finland. METHODS: This retrospective registry study utilized electronic healthcare data covering all pediatric patients with SBS-IF born between 2010 and 2019 at the Hospital District of Helsinki and Uusimaa in Finland. Patients were followed from birth until the end of 2020 and compared to control patients, all from the same hospital system. RESULTS: In total, 38 patients with SBS-IF and 1:5 matched controls were included, with median follow-up time of almost 6 years from birth. Over half of the patients were born early preterm (gestational age ≤30 weeks). The incidence of pediatric SBS-IF was 24 per 100,000 live births. The HCRU was higher compared to controls and most of the inpatient days incurred during the first year of the SBS-IF patients' life. The average hospital-based HCRU costs were €221,000 for the first year and €57,000 for whole follow-up annually. The costs were higher for the early preterm patients and accumulated mainly from inpatient days. CONCLUSIONS: SBS-IF is a rare disease with a relatively low number of patients treated at each hospital district. The burden on the hospital system, as well as the patient's family, is especially high at the onset as the newborns with SBS-IF spend a significant part of their first year of life in the hospital.

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

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.

Real-world etiologies and treatments of pediatric short bowel syndrome in Japan.

BACKGROUND: Short bowel syndrome (SBS) is a rare disease that can result in intestinal failure (IF). Short bowel syndrome intestinal failure leads to stunted growth and development and high mortality rates. The primary goal of treatment is to enhance intestinal adaptation and nutrient absorption. Parenteral nutrition (PN) is used to support this process until enteral autonomy can be restored. Some patients experience prolonged partial or complete dependency on PN and face an increased risk of life-threatening catheter-related bloodstream infections and intestinal failure-associated liver disease. This study aimed to provide real-world insights into the patient characteristics and treatment dynamics of PN-dependent children with SBS-IF in Japan. METHODS: This retrospective observational study used anonymized information from a large hospital-based medical insurance database to identify pediatric patients who received PN for ≥6 months between April 2008 and January 2020. The primary endpoint was weaning from PN. Secondary endpoints included duration and complications of PN. RESULTS: Forty-eight children (mean age, 2.9 years) were eligible for inclusion. The most common causes of SBS-IF were mechanical bowel obstruction, functional bowel disorders, and Hirschsprung's disease. Twenty-two patients (45.8%) were weaned from PN during the study. The mean time to first weaning was 464.2 days and five patients (22.7%) restarted PN. The mean total duration of PN was 692.6 days in weaned patients and 1,170.9 days in unweaned patients. The most frequent complications were sepsis, catheter infections (both 79.2%), and liver dysfunction (64.6%). CONCLUSIONS: Pediatric patients with SBS-IF faced difficulties when weaning off PN and rates of life-threatening complications were high.

Severe acute respiratory syndrome coronavirus 2 antibody prevalence in adult patients with short bowel syndrome-A German multicenter cross-sectional study.

BACKGROUND: Not all patients suffer from a severe course of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, demanding a definition of groups at risk. Short bowel syndrome (SBS) has been assumed to be a risk factor, because of the complexity of disease, the need for interdisciplinary care, and frequent contact with caretakers. We aimed to establish data on the course of infection and prevalence of SARS-CoV-2 seropositivity in SBS patients in Germany. METHODS: From January 2021 until January 2022 a total of 119 patients from three different tertiary care centers with SBS were included. All patients received an antibody test against the nucleocapsid (N) antigen and were asked to fill out a questionnaire, which included frequency of contact with medical personnel, risk behavior and worries. RESULTS: Sixty-seven percent of SBS patients received parenteral nutrition with a median of 6 days per week. The seroprevalence of SARS-CoV-2 antibodies was 7.6% (n = 9). Seven patients with positive antibodies had coronavirus disease 2019 (COVID-19) with a mild course. None of the patients were hospitalized or needed further treatment. There was no difference in willingness to take risks in SARS-CoV-2 antibody-positive and -negative patients (P = 0.61). Patients were predominantly worried about the economy (61%) and transmitting COVID-19 (52%), less frequent (26%) about receiving insufficient medical treatment. CONCLUSION: These are the first clinical results concerning SARS-CoV-2 seropositivity and COVID-19 disease in patients with SBS. The seropositivity is comparable to national data, which we attribute to increased risk awareness and avoidance. Further studies are warranted to investigate effects of COVID-19 infection in SBS patients.

Etiologies and treatments of chronic intestinal failure-short bowel syndrome (SBS) in Japanese adults: a real-world observational study.

PURPOSE: Short bowel syndrome (SBS) with intestinal failure (SBS-IF) requires long-term parenteral nutrition (PN). This study investigated the real-world etiologies of SBS, treatment patterns, and PN-related outcomes among adult patients with SBS-IF in Japan. METHODS: This retrospective, observational cohort study was based on data from April, 2008 to January, 2020 from one of the largest hospital-based claim databases in Japan. Analyzed patients were aged ≥ 16 years, had received continuous PN for ≥ 6 months, and had SBS or undergone SBS-related surgery with a diagnosis of a causative disease. The primary endpoint was PN weaning. RESULTS: We analyzed data for 393 patients. The most frequent causes of SBS-IF were ileus (31.8%), Crohn's disease (20.1%), and mesenteric ischemia (16.0%). Of 144/393 (36.6%) patients who were weaned off their PN, 48 (33.3%) were subsequently restarted on PN. Of 276/393 (70.2%) patients whose PN was initiated in hospital, 156 (56.5%) transitioned to home management. The mean duration of initial PN was 450.4 and 675.5 days for patients who were able or unable to be weaned off PN, respectively. Sepsis (67.4%), catheter-related bloodstream infections (49.1%), and liver disorders (45.0%) were the most reported PN-related complications. CONCLUSIONS: Most patients with SBS-IF in Japan could not be weaned off PN and suffered life-threatening complications.

An overview of the current management of short-bowel syndrome in pediatric patients.

Short-bowel syndrome (SBS) is defined as a state of malabsorption after resection or loss of a major portion of the bowel due to congenital or acquired factors. This article presents an overview on the recent management of pediatric SBS. The pediatric SBS population is very heterogeneous. The incidence of SBS is estimated to be 24.5 per 100,000 live births. The nutritional, medical, and surgical therapies available require a comprehensive evaluation. Thus, multidisciplinary intestinal rehabilitation programs (IRPs) are necessary for the management of these complex patients. The key points of focus in IRP management are hepato-protective strategies to minimize intestinal failure-associated liver disease; the aggressive prevention of catheter-related bloodstream infections; strategic nutritional supply to optimize the absorption of enteral calories; and the management and prevention of small bowel bacterial overgrowth, nephrocalcinosis, and metabolic bone disease. As the survival rate of children with SBS currently exceeds 90%, the application of small bowel transplantation has been evolving. The introduction of innovative treatments, such as combined therapy of intestinotrophic hormones, including glucagon-like peptide-2, may lead to further improvements in patients' quality of life.

Changes in adult home parenteral nutrition practice over 25 years.

BACKGROUND AND AIMS: Home Parenteral Nutrition (HPN) is the established treatment of intestinal failure. This study considers the changes in practice in a single UK centre over the past twenty-five years. METHODS: Data was culled from a database used for clinical care and maintained prospectively. RESULTS: Two hundred and five patients were included from 1993 to 2018. Patient numbers increased from 22 during 1999-2003 to 158 during 2014-2018. The median age at discharge increased from 52 years during 1999-2003 to 59 years during 2014-2018. Thirty percent of patients discharged during 1999-2003 had Crohn's disease, reducing to 14% during 2014-2018. Fifteen percent of patients discharged during 1999-2003 had small bowel fistula or obstruction in comparison to 44% during 2014-2018. Only 18 patients were treated with palliative intent, the majority in recent years. An increasing number of patients required help with HPN care over the years. Survival in non-palliative patients was 85% at 1 year, 67% at 3 years, 53% at 5 years and 42% at 10 years. The majority of deaths were due to underlying disease and only 5 of 55 deaths were attributed to HPN alone. HPN dependence in non-palliative patients was 73% at 1 year, 59% at 3 years, 56% at 5 years and 43% at 10 years. Fifty eight patients stopped HPN after reconstructive surgery. Patients experienced 5.1 admissions/1000 HPN days (64.7 admission days/1000 HPN days). Admission rate did not change over the years though the percentage due to catheter problems fell from 52% to 40% while the percentage due to underlying disease or unrelated cause rose. CONCLUSIONS: The increase in numbers, age and dependency of HPN patients requires increasing resource and consideration of new models of service. Many patients with short bowel syndrome now survive to old age and the care needs of the HPN patient who has become elderly can be complex. A significant proportion of patients are being referred for HPN as a bridge to reconstructive surgery after surgical complication and this requires close involvement of gastrointestinal surgeons in HPN teams. The need for hospital admissions remains a burden for HPN patients and there is scope for changes in service provision to try to reduce hospital days.

Characteristics of adult patients with chronic intestinal failure due to short bowel syndrome: An international multicenter survey.

BACKGROUND AND AIMS: The case-mix of patients with intestinal failure due to short bowel syndrome (SBS-IF) can differ among centres and may also be affected by the timeframe of data collection. Therefore, the ESPEN international multicenter cross-sectional survey was analyzed to compare the characteristics of SBS-IF cohorts collected within the same timeframe in different countries. METHODS: The study included 1880 adult SBS-IF patients collected in 2015 by 65 centres from 22 countries. The demographic, nutritional, SBS type (end jejunostomy, SBS-J; jejuno-colic anastomosis, SBS-JC; jejunoileal anastomosis with an intact colon and ileocecal valve, SBS-JIC), underlying disease and intravenous supplementation (IVS) characteristics were analyzed. IVS was classified as fluid and electrolyte alone (FE) or parenteral nutrition admixture (PN). The mean daily IVS volume, calculated on a weekly basis, was categorized as <1, 1-2, 2-3 and >3 L/day. RESULTS: In the entire group: 60.7% were females and SBS-J comprised 60% of cases, while mesenteric ischaemia (MI) and Crohn' disease (CD) were the main underlying diseases. IVS dependency was longer than 3 years in around 50% of cases; IVS was infused ≥5 days/week in 75% and FE in 10% of cases. Within the SBS-IF cohort: CD was twice and thrice more frequent in SBS-J than SBS-JC and SBS-JIC, respectively, while MI was more frequent in SBS-JC and SBS-JIC. Within countries: SBS-J represented 75% or more of patients in UK and Denmark and 50-60% in the other countries, except Poland where SBS-JC prevailed. CD was the main underlying disease in UK, USA, Denmark and The Netherlands, while MI prevailed in France, Italy and Poland. CONCLUSIONS: SBS-IF type is primarily determined by the underlying disease, with significant variation between countries. These novel data will be useful for planning and managing both clinical activity and research studies on SBS.

Vitamin D status in children with intestinal failure who have achieved enteral autonomy.

BACKGROUND: Vitamin D deficiency is highly prevalent in children with intestinal failure (IF) who receive parenteral nutrition (PN), but data on vitamin D status after achieving enteral autonomy (EA) are limited. We aimed to evaluate the prevalence of vitamin D deficiency in this population while exploring clinical variables that may be associated with its development. METHODS: A retrospective review was performed on 29 children with IF who had achieved EA. Deficiency was defined as a mean serum 25-hydroxyvitamin D <30 ng/ml. DATA RESULTS: Sixty-six percent of children had at least one deficient level during the study period, with 38% being deficient based on the mean vitamin D levels. Eighty-four percent had radiologic evidence of osteopenia. Compared with the sufficient group (n=18), the deficient group (n=11) received higher daily mean vitamin D doses (2246 vs 920 IU; P=.02), had shorter remnant small-bowel length (53.8 vs 82.1 cm; P=.03), and were PN dependent for a longer duration (1.3 vs 0.58 years; P=.01). Univariate analyses revealed longer remnant gut length (odds ratio [OR] = 1.03; P=.04) and shorter duration of PN (OR = 0.26; P=.04) to be significantly associated with sufficient vitamin D status. CONCLUSION: Vitamin D deficiency and osteopenia are highly prevalent in pediatric patients with a history of IF who have achieved EA, despite enteral supplementation with higher than standard doses. Shorter remnant small-bowel length and longer duration of PN were associated with vitamin D deficiency. These findings emphasize the importance of prolonged surveillance and highlight the need for alternate dosing regimens.

Prevalence of home parenteral nutrition in children.

BACKGROUND: Use of HPN in paediatrics in the UK has increased rapidly over the last 20 years but the prevalence of HPN has been challenging to define. Clinicians in the UK have noted an evolving complexity of cases and perceive improved outcomes and increased acceptability of long-term PN. These factors combined have the potential to increase the burden on existing paediatric gastroenterology services in the UK. METHODS: A national database was interrogated to define the prevalence of HPN in children in the UK and to explore outcomes for patients receiving HPN. RESULTS: Since 2015, 525 children were notified to the database; of these patients, mortality was <5% and intestinal transplant occurred in 1%. In 2019, 389 children received HPN in the UK; this is nearly double the number last reported in 2012 and is a prevalence of 30 per million children. Short bowel syndrome is the largest category of these patients. However, a poorly defined group including those with multisystem disease has increased 10 fold since 2012 and is now the second largest category. CONCLUSIONS: Long term HPN in childhood is safe and associated with good survival and low risk of the need for intestinal transplantation.

Pediatric Feeding Disorder in Children With Short Bowel Syndrome.

OBJECTIVE: We aimed to characterize the prevalence of pediatric feeding disorder (PFD) in short bowel syndrome (SBS) and study factors contributing to the persistence of PFD. METHODS: Single-center retrospective study of patients diagnosed with SBS at age 6 months or younger. Data were collected in 3-month intervals through age 2 years, and every 6 months through age 4 years. Demographic information, anthropometric data, and details regarding nutrition support were recorded and analyzed. RESULTS: We reviewed 28 patients. Of the 21 patients who were weaned off parenteral nutrition, 57.1%, 81.0%, 90.5%, and 100.0% achieved this by 12, 24, 36, and 48 months of age, respectively. Of the 13 patients who were weaned off enteral nutrition, 30.8%, 69.2%, 76.9%, and 100.0% achieved this by 12, 24, 36, and 48 months, respectively. DISCUSSION: The prevalence of PFD was 100.0%, 76.5%, 68.8%, and 70.0% at 1, 2, 3, and 4 years of age, respectively. All patients who exhibited resolution of PFD had an underlying etiology of necrotizing enterocolitis. Median small bowel percentage remaining was greater in patients who exhibited resolution of PFD compared to those who did not. Except for the group of patients seen at 4 years of age, a larger percentage of patients with vomiting/history of requirement of postpyloric feeds were seen among patients with PFD compared to those without PFD. CONCLUSION: PFD is prevalent in children with SBS. Although prevalence decreases over time, children with PFD will continue to require more medical attention than children that do not.

Neurodevelopmental and Growth Outcomes of Extremely Preterm Infants with Short Bowel Syndrome.

OBJECTIVE: To determine if preterm infants with surgical necrotizing enterocolitis (sNEC) or spontaneous intestinal perforation (SIP) with short bowel syndrome (SBS) have worse neurodevelopmental and growth outcomes than those with sNEC/SIP without SBS, and those with no necrotizing enterocolitis, SIP, or SBS. STUDY DESIGN: We undertook a retrospective analysis of prospectively collected data from infants born between 22 and 26 weeks of gestation in the National Institute of Child Health and Human Development Neonatal Research Network centers from January 1, 2008, to December 31, 2016. Survivors were assessed at 18-26 months corrected age by standardized neurologic examination and Bayley Scales of Infant and Toddler Development, Third Edition. The primary outcome was moderate-severe neurodevelopmental impairment. Growth was assessed using World Health Organization z-score standards. Adjusted relative risks were estimated using modified Poisson regression models. RESULTS: Mortality was 32%, 45%, and 21% in the 3 groups, respectively. Eighty-nine percent of survivors were seen at 18-26 months corrected age. Moderate-severe neurodevelopmental impairment was present in 77% of children with SBS compared with 62% with sNEC/SIP without SBS (adjusted relative risk, 1.22; 95% CI, 1.02-1.45; P = .03) and 44% with no necrotizing enterocolitis, SIP, or SBS (adjusted relative risk, 1.60; 95% CI, 1.37-1.88; P < .001). Children with SBS had lowcognitive, language, and motor scores than children with sNEC/SIP without SBS. At follow-up, length and head circumference z-scores remained more than 1 SD below the mean for children with SBS. CONCLUSIONS: Preterm infants with sNEC/SIP and SBS had increased risk of adverse neurodevelopmental outcomes at 18-26 months corrected age and impaired growth compared with peers with sNEC/SIP without SBS or without any of these conditions.

Micronutrient deficiencies in pediatric short bowel syndrome: a 10-year review from an intestinal rehabilitation center in China.

PURPOSE: Vitamins and trace elements are essential nutrients for growth and intestinal adaptation in children with short bowel syndrome (SBS). This study aimed to assess micronutrients' status during and after weaning off PN in pediatric SBS. METHODS: This retrospective study evaluated the follow-up of 31 children with SBS between Jan 2010 and Sep 2019. Clinical data were reviewed from the patients' electric medical record. Serum electrolytes, trace elements, vitamin B12, vitamin D, and folate concentrations were collected before and after enteral autonomy. RESULTS: Thirty-one SBS cases were reviewed (median onset age 11 days after birth, 51.6% boys, mean PN duration 4 months, and mean residual small intestine length 58.2 cm). Median duration of follow-up was 10 months (interquartile range [IQR]: 4, 19). The common micronutrient deficiencies were zinc (51.6%), copper (38.7%), vitamin D (32.3%), and phosphorus (25.8%) after the transition to EN. The proportion of patients deficient in vitamin D decreased dramatically from 93.5% to 32.3% (P < 0.001), and serum concentrations of vitamin D increased significantly (27.4 ± 12.3 vs. 60.3 ± 32.9 nmol/l, P = 0.03) after achieving full enteral feeding more than 1 month. Additionally, serum magnesium levels significantly increased (0.76 ± 0.17 vs. 0.88 ± 0.14 mmol/l, P = 0.03). Hemoglobin levels elevated significantly after weaning off PN (104.3 ± 10.7 vs. 117.8 ± 13.7 g/l, P = 0.03). CONCLUSIONS: Micronutrient deficiencies remain a common problem in pediatric SBS through intestinal rehabilitation. Therefore, we strongly recommend supplementation of more vitamin D and trace elements (zinc, copper, and phosphorus) under regular monitoring during long-term intestinal rehabilitation.

Predictors for short bowel syndrome in Crohn's disease.

BACKGROUND AND AIM: Patients with Crohn's disease (CD) are at risk for short bowel syndrome (SBS). We investigated independent predictors for SBS in these patients to allow the development of preventive strategies. METHODS: All adult patients seen at the Nancy University hospital for CD or SBS between 2012 and 2019 were eligible for inclusion in this case-control study. Each CD patient with SBS was matched to 9 controls. RESULTS: 410 CD patients were included (369 without SBS and 41 with SBS). Subjects with SBS underwent significantly more bowel resections (median value of 3 vs 1, p<0.0001) and median time before the first surgery was not different than controls (6 vs 4 years, p=0.59). A higher need for parenteral support was found in end-jejunostomy SBS than in jejunocolic and jejunoileal SBS (70.6% vs 25% and 0%, p=0.0031). Montreal B1 behavior (OR 0.02, CI 95% 0-0.08) and budesonide treated-patients (OR=0.03, CI 95% 0.003-0.2) were at lower risk of SBS, while IV steroid treated-patients were at higher risk (OR=8.5, CI 95% 3.0-24.9). CONCLUSION: Montreal B1 behavior, IV steroids and budesonide use are influencing predictors for this complication. These predictors should be assessed in daily clinical practice to prevent SBS occurrence.

Etiology of and predictive factors for chronic intestinal failure requiring long term parenteral support in the last two decades: A retrospective study.

BACKGROUND & AIMS: Chronic intestinal failure (CIF) has been long-recognized, however the underlying etiology and risk factors have not been historically well-studied. We aim to study the underlying etiologies of CIF and predictive factors for long-term parenteral support (PS). METHODS: We retrospectively identified patients with newly diagnosed CIF who received PS to maintain nutrition at the Cleveland Clinic between 2000 and 2017. Long-term PS was defined as a duration of more than 3 months. Univariable and multivariable logistic regression analyses were performed to identify the predictors of the need for long-term PS. RESULTS: We identified 350 patients with CIF, 150 (43%) and 200 (57%) were diagnosed before and after 2010, respectively. The most common etiology was Crohn's disease (CD) in both cohorts (34.7% versus 30.5%, p = 0.41). Graft-versus-host-disease (GVHD) was a less frequent cause of CIF after 2010 (12.7% versus 2.5%, p = 0.0002). The type of PS was mostly total parenteral nutrition before and after 2010, 95% and 96%, respectively (p = 0.55). On univariable analysis, absence of ileocecal valve (p < 0.0001), ischemic bowel disease (p = 0.009), and whole colon resection (p = 0.033) were associated with the need for long-term PS. On multivariable analysis, absence of ileocecal valve (OR 2.19, p = 0.011) and ischemic bowel disease (OR 3.04, p = 0.003) remained statistically significant predictors of long-term PS. CONCLUSION: In our cohort of patients with CIF, CD remains the leading etiology over the last 20 years, whereas GVHD is less common after 2010. The absence of ileocecal valve and ischemic bowel disease were reliable predictive factors for requiring long-term PS.

Hospital Charges Associated with Central Venous Stenosis in Pediatric Patients Requiring Long-Term Central Venous Access.

OBJECTIVE: To evaluate the hospital charges associated with central venous stenosis in pediatric patients requiring long-term central venous catheters, via associated charges and hospital length of stay (LOS). STUDY DESIGN: This institutional review board-approved retrospective review identified pediatric patients with central venous catheters and either short bowel syndrome (SBS) or end-stage renal disease (ESRD) diagnosed between 2008 and 2015 using the Pediatric Health Information System. These 2 cohorts were selected because long-term central venous access is commonly required for survival. Prevalence of central venous stenosis, total number of admissions, procedures, LOS, and associated charges were recorded. Statistical analysis performed with Wilcoxon nonparametric and 2-sample t test with a significance of P < .05. RESULTS: Of 4952 patients with SBS and 4665 patients with ESRD, 169 (3.4%) patients with SBS and 191 (4.1%) patients with ESRD were diagnosed with central venous stenosis (360 patients total [3.7%]). The cumulative median admissions and LOS was higher in patients with SBS with central venous stenosis (15 admissions and 156 days) vs those without central venous stenosis (5 admissions and 110 days) (P < .001). The cumulative median number of admissions and LOS was higher in patients with ESRD with central venous stenosis (13 admissions and 72 days) vs those without central venous stenosis (7 admissions and 42 days) (P < .001). The mean cumulative charges for patients with SBS with central venous stenosis were higher than for those without central venous stenosis ($1.89 million vs $1.11 million, respectively) (P < .001). Similarly, the mean cumulative charges for patients with ESRD with central venous stenosis were higher than for those without central venous stenosis ($1.17 millions vs $702 000, respectively) (P < .001). CONCLUSIONS: Pediatric patients with central venous stenosis have significantly higher total charges, imaging charges, number of admissions, and longer LOS. Attention to mitigate the incidence of central venous stenosis in pediatric patients requiring long-term central venous access is warranted.

Understanding short bowel syndrome: Current status and future perspectives.

Short bowel syndrome (SBS) is a rare malabsorptive disorder as a result of the loss of bowel mass mostly secondary to surgical resection of the small intestine. Other causes are vascular diseases, neoplasms or inflammatory bowel disease. The spectrum of the disease is widely variable from single micronutrient malabsorption to complete intestinal failure, depending on the remaining length of the small intestine, the anatomical portion of intestine and the function of the remnant bowel. Over the last years, the management of affected patients has remarkably improved with the increase in patients' quality of life and survival, mainly thanks to advances in home-based parenteral nutrition (PN). In the last ten years new treatment strategies have become available together with increasing experience and the encouraging results with new drugs, such as teduglutide, have added a new dimension to the management of SBS. This review aims to summarize the knowledge available in the current literature on SBS epidemiology, pathophysiology, and its surgical (including intestinal lengthening procedures and intestinal transplantation) and medical management with emphasis on the recent advances. Moreover, this review attempts to provide the new understanding and recent approaches to SBS complications such as sepsis, catheter thrombosis, and intestinal failure-associated liver disease.

Colon polyps in patients with short bowel syndrome before and after teduglutide: Post hoc analysis of the STEPS study series.

BACKGROUND & AIMS: Teduglutide promotes intestinal growth and is approved for the treatment of short bowel syndrome and intestinal failure (SBS-IF). Based on the pharmacologic activity and preclinical findings, teduglutide can potentially induce proliferative colonic mucosal changes. The aim of this study is to report the occurrence of colorectal polyps in adult patients with SBS-IF who received teduglutide in clinical studies conducted to date. METHODS: A post hoc analysis of the completed Study of Teduglutide Effectiveness in Parenteral Nutrition-Dependent Short Bowel Syndrome Subjects (STEPS) clinical study series (NCT00798967, EudraCT 2008-006193-15; NCT00930644, EudraCT 2009-011679-65; NCT01560403) evaluated electronic case report form data for baseline colonoscopies (performed before treatment) and for surveillance or end-of-study (performed after treatment with teduglutide 0.05 mg/kg/day for 24 and 36 months) post-exposure procedures. RESULTS: In the STEPS studies, 73 patients treated with teduglutide had a baseline colonoscopy. No post-exposure colonoscopy was scheduled in STEPS. In STEPS-2/3, 50 of 65 patients with remnant colon (77%) underwent a protocol-mandated post-exposure colonoscopy. Colon polyps were reported at baseline in 12% (9/73) of patients and post-exposure in 18% (9/50) of patients. Two had polyps both at baseline and post-exposure. On histology, available for 7 patients, 5 had adenomas (1 serrated, 4 tubular) and none had malignancy or high-grade dysplasia. CONCLUSION: These data support recommendations for colonoscopic screening before teduglutide therapy and subsequent on-therapy colonoscopic surveillance for patients with SBS-IF. Further studies are required to assess the risk of polyp formation in patients with SBS-IF and the most appropriate colon polyp surveillance strategies.