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.

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.

Prevalence of eosinophilic gastrointestinal diseases in children with short bowel syndrome: A single center study.

Patients with short bowel syndrome (SBS) have multiple risk factors for eosinophilic gastrointestinal diseases (EGIDs) including increased risk for intestinal dysbiosis and food allergy compared to their counterparts with normal anatomy. However, there is limited data on the prevalence of EGIDs in children with SBS. We aimed to define the prevalence of EGIDs in an SBS cohort and its association with different risk factors via a retrospective chart review of patients with SBS at Children's National Hospital. The prevalence of eosinophilic esophagitis in our SBS cohort was 10%, eosinophilic gastritis was 4.9%, and eosinophilic enteritis was 4.9%. SBS patients with history of allergy or atopy were more likely to have esophageal and intestinal eosinophilia on biopsy than patients without allergy. The prevalence of EGIDs in our SBS cohort is significantly higher than in the general population and may be associated with allergic polarization.

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.

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.

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.

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.

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.

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.

Predicting Morbidity and Mortality in Neonates Born With Gastroschisis.

BACKGROUND: Gastroschisis is an increasingly common congenital abdominal wall defect. Due to advances in neonatal critical care and early surgical management, mortality from gastroschisis and associated complications has decreased to less than 10% in most series. However, it has been recognized that the outcome of gastroschisis has a spectrum and that the disorder affects a heterogeneous cohort of neonates. The goal of this study is to predict morbidity and mortality in neonates with gastroschisis using clinically relevant variables. METHODS: A multicenter, retrospective observational study of neonates born with gastroschisis was conducted. Neonatal characteristics and outcomes were collected and compared. Prediction of morbidity and mortality was performed using multivariate clinical models. RESULTS: Five hundred and sixty-six neonates with gastroschisis were identified. Overall survival was 95%. Median hospital length of stay was 37 d. Sepsis was diagnosed in 107 neonates. Days on parenteral nutrition and mechanical ventilation were considerable with a median of 27 and 5 d, respectively. Complex gastroschisis (atresia, perforation, volvulus), preterm delivery (<37 wk), and very low birth weight (<1500 g) were associated with worse clinical outcomes including increased sepsis, short bowel syndrome, parenteral nutrition days, and length of stay. The composite metric of birth weight, Apgar score at 5 min, and complex gastroschisis was able to successfully predict mortality (area under the curve, 0.81). CONCLUSIONS: Clinical variables can be used in gastroschisis to distinguish those who will survive from nonsurvivors. Although these findings need to be validated in other large multicenter data sets, this prognostic score may aid practitioners in the identification and management of at-risk neonates.

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.

Clinical Features and Outcomes of Patients With Acute Mesenteric Ischemia and Concomitant Colon Ischemia: A Retrospective Cohort Study.

BACKGROUND: Early identification of patients with acute mesenteric ischemia (AMI) involving the large bowel may play a decisive role in improving the prognosis of AMI. This study aims to compare the outcomes between patients with isolated AMI and AMI patients with colon involvement (CI) and to identify the predictors of worse outcomes. The different surgical modalities for AMI patients with CI were also evaluated. METHODS: This retrospective cohort study included 199 AMI patients admitted from January 2005 to January 2014. Based on colonoscopy and pathology reports, 39 patients were diagnosed as AMI with CI, and 160 were AMI patients without CI. The clinical outcomes and different surgical modalities were compared. Risk factors of 30-d mortality and short bowel syndrome (SBS) were identified. RESULTS: The 30-d mortality (10% versus 49%, P < 0.01) and SBS incidence (19% versus 49%, P < 0.01) were higher in AMI patients with CI than AMI patients without CI. AMI patients with CI have higher rate of bowel resection (68% versus 95%, P < 0.001) and second-look laparotomy (25% versus 54%, P < 0.001) than patients with AMI alone. For AMI patients with CI, emergent laparotomy was associated with shorter hospital stay (P = 0.04) and less incidence of SBS (74% versus 25%, P < 0.001) than initial endovascular therapy. Patients with ostomy had less repeated bowel resection (11% versus 63%, P = 0.001) and rate of SBS (21% versus 79%, P < 0.001) than patients with primary bowel anastomosis. Serum procalcitonin level and colon ischemia were risk factors of 30-d mortality and SBS for AMI. CONCLUSIONS: AMI patients with CI represent a special cohort of AMI patients with higher risk of poor outcome. Compared to initial endovascular therapy, emergent laparotomy was associated with shorter length of hospital stay and reduced incidence of SBS.

Treatment With Biologic Agents Has Not Reduced Surgeries Among Patients With Crohn's Disease With Short Bowel Syndrome.

BACKGROUND & AIMS: Little is known about the effects of biologic agents used to treat Crohn's disease (CD) on its long-term complications, such as short bowel syndrome and intestinal failure (SBS-IF). We evaluated trends in small bowel resections and health care utilization among patients with CD with and without SBS-IF. METHODS: We collected data on the National Inpatient Sample on 2,989,185 patients hospitalized with CD in the United States before the time period in which CD was treated with biologic agents (1993-1997) and after biologic therapy became widespread (1998-2014). We used Poisson and linear regression analyses to evaluate trends for small bowel resections and health care utilization among patients with CD with and without SBS-IF. Multivariable models were adjusted for age, sex, Charlson-Deyo comorbidity index, payer source, hospital size, region, and teaching status. RESULTS: The proportions of patients who underwent resection did not significantly change during the period before biologic therapy (121.8 per 1000 hospitalizations in 1993 to 110.1 per 1000 hospitalizations in 1997; P trend =.14) but decreased significantly during the period after biologic therapy began (99.0 per 1000 hospitalizations in 1998 to 64.6 per 1000 hospitalizations in 2014; P trend < .01). However, among patients with SBS-IF, similar proportions of patients underwent resection during the period before biologic therapy (0.7 per 1000 hospitalizations in 1993 to 0.7 per 1000 hospitalizations in 1997; P trend = .92) and during the period after biologic therapy (0.6 per 1000 hospitalizations in 1998 to 0.7 per 1000 hospitalizations in 2014; P trend = .06). Rates of hospitalization for patients with SBS-IF increased from 16.5 per 1000 hospitalizations in 1998 to 19.5 per 1000 hospitalizations in 2014 (P trend < .01). SBS-IF hospitalizations were associated with longer lengths of stay (P < .01) and greater total charges (P < .01). CONCLUSIONS: In a study of the United States population, we found that the use of biologic agents to treat CD reduced the proportion of patients undergoing resection, but not among patients with SBS-IF. These findings indicate that biologic agents reduce some but not all features of CD. Studies are needed to identify patients at risk for SBS-IF, prevent and treat this complication, and identify new treatments.

[Mesenteric edema as a prenatal ultrasound sign of poor prognosis in gastroschisis]. / El edema mesentérico como signo ecográfico prenatal de mal pronóstico en gastrosquisis.

INTRODUCTION/AIM OF THE STUDY: Gastroschisis is a congenital malformation with an easy and early prenatal diagnosis, however, it has a variable post-natal outcome. Our aim was to determine if certain ultrasound markers or early delivery were related with a worse postnatal outcome. PATIENTS AND METHODS: Retrospective study of a cohort of patients with gastroschisis diagnosed between 2005-2014, with emphasis on prenatal ultrasounds, gestational age at delivery and post-natal outcome. Oligohydramnios, peel, mesenteric edema, fixed and dilated bowel with loss of peristalsis and small wall defect were considered ultrasonographic markers associated with poor prognosis. Outcome variables included: length-of-stay, complications, nutritional and respiratory factors. Non-parametric statistical analysis were used with p < 0,05 regarded as significant. RESULTS: Clinical charts of 30 patients with gastroschisis were reviewed (17M/13F). Gestational age at diagnosis was 20 (12-31) and at delivery 36 (31-39) weeks (33% of the patients over 36+3 weeks). A 73% of the patients presented at least one ultrasonographic marker factor during follow-up. Univariate analysis showed that mesenteric edema was associated with poor outcome variables: short-bowel syndrome (p= 0,000), PN-dependence (p= 0,007) and intestinal atresia (p= 0,02). The remaining risk factors analysed, including late delivery (> 36+3 weeks) were not associated with length-of-stay, ventilatory support, digestive autonomy, complications or mortality. CONCLUSIONS: Neither the presence of ultrasonographic markers classically associated with unfavorable outcomes, nor early delivery (< 36 weeks) resulted in worse postnatal outcome. Mesenteric edema was the only alarming ultrasound marker and that may suggest the need of closer follow-up.

INTRODUCCION: La gastrosquisis es una anomalía congénita de fácil diagnóstico prenatal y pronóstico postnatal variable. Nuestro objetivo es determinar si los signos ecográficos prenatales o el momento del parto se relacionan con peor pronóstico postnatal. PACIENTES Y METODOS: Se realiza un estudio retrospectivo de la cohorte de pacientes con gastrosquisis diagnosticados entre 2005-2014, registrando las ecografías prenatales, edad gestacional al parto y evolución postnatal. Se valoraron los hallazgos ecográficos prenatales: oligohidramnios, peel, edema mesentérico, asas fijas, aperistálticas y/o dilatadas y defecto pequeño de pared. Se consideraron variables resultado: la estancia hospitalaria, complicaciones, mortalidad y factores nutricionales y respiratorios. Se utilizaron pruebas no paramétricas, considerándose significativo un valor p < 0,05. RESULTADOS: Se analizaron 30 pacientes con gastrosquisis (17V/13M). La edad gestacional al diagnóstico fue de 20 (12-31) y al parto de 36 (31-39) semanas (33% mayores de 36+3 semanas). El 73% de los pacientes presentaron al menos un signo ecográfico de mal pronóstico. El análisis univariante asoció el edema mesentérico al síndrome de intestino corto (p= 0,000), falta de autonomía digestiva (p= 0,007) y mayor incidencia de atresia (p= 0,02). El resto de los factores, incluyendo la edad gestacional > 36+3 semanas, no tuvieron repercusión negativa en términos de estancia, asistencia respiratoria, autonomía digestiva, complicaciones o mortalidad. CONCLUSIONES: Ni la presencia de signos ecográficos considerados generalmente como desfavorables ni la tendencia a acercar el parto a la semana 36ª tienen repercusiones significativas en el curso postnatal. Únicamente el edema mesentérico parece un signo alarmante que sugiere la necesidad de aumentar la frecuencia de intervenciones (ecografías, pruebas de bienestar fetal).

Management and Complications of Short Bowel Syndrome: an Updated Review.

Short bowel syndrome (SBS) is defined as loss of bowel mass from surgical resection, congenital defects, or disease. Intestinal failure (IF) includes the subset of SBS unable to meet nutrition needs with enteral supplements and requires parenteral nutrition (PN). The parenteral treatment of SBS is now a half-century old. Recent pharmacologic treatment (GLP-2 analogues) has begun to make a significant impact in the care and ultimate management of these patients such that the possibility of reducing PN requirements in formerly PN-dependent patients is a now a real possibility. Finally, newer understanding and possible treatment for some of the complications related to IF have more recently evolved and will be an emphasis of this report. This review will focus on developments over the last 10 years with the goal of updating the reader to new advances in our understanding of the care and feeding of the SBS patient.

Peripartum and neonatal outcomes of small-for-gestational-age infants with gastroschisis.

OBJECTIVES: Neonates with gastroschisis are often small for gestational age (SGA) based on population nomograms. Our objective was to evaluate the effect of SGA on perinatal and neonatal outcomes in cases of gastroschisis. METHODS: This is a retrospective study of neonates with prenatally diagnosed gastroschisis from two academic centers between 2008 and 13. Perinatal and neonatal outcomes of neonates with SGA at birth were compared with appropriate-for-gestational-age (AGA) neonates. The primary composite outcome was defined as any of the following: neonatal sepsis, short bowel syndrome at discharge, prolonged mechanical ventilation (upper quartile for the cohort), bowel atresia or death. RESULTS: We identified 112 cases of gastroschisis, 25 of whom (22%) were SGA at birth. There were no differences in adverse peripartum outcomes between SGA and AGA infants. No difference was found in the primary composite neonatal outcome (52% vs 36%, p = 0.21), but SGA infants were more likely to have prolonged mechanical ventilation (44% vs 22%, p = 0.04) and prolonged length of stay (LOS) (52% vs 22%, p = 0.007). After adjusting for GA at delivery, SGA remained associated with prolonged LOS (OR = 4.3, CI: 1.6-11.8). CONCLUSION: Among infants with gastroschisis, SGA at birth is associated with a fourfold increase in odds for prolonged LOS, independent of GA.

The pharmacologic treatment of short bowel syndrome: new tricks and novel agents.

Short bowel syndrome (SBS) is a manifestation of massive resection of the intestines resulting in severe fluid, electrolyte, and vitamin/mineral deficiencies. Diet and parenteral nutrition play a large role in the management of SBS; however, pharmacologic options are becoming more readily available. These pharmacologic agents focus on reducing secretions and stimulating intestinal adaptation. The choice of medication is highly dependent on the patient's symptoms, remaining anatomy, and risk versus benefit profile for each agent. This article focuses on common and novel pharmacologic medications used in SBS, including expert advice on their indications and use.

Economic evaluation in short bowel syndrome (SBS): an algorithm to estimate utility scores for a patient-reported SBS-specific quality of life scale (SBS-QoL™).

PURPOSE: Condition-specific preference-based measures can offer utility data where they would not otherwise be available or where generic measures may lack sensitivity, although they lack comparability across conditions. This study aimed to develop an algorithm for estimating utilities from the short bowel syndrome health-related quality of life scale (SBS-QoL™). METHODS: SBS-QoL™ items were selected based on factor and item performance analysis of a European SBS-QoL™ dataset and consultation with 3 SBS clinical experts. Six-dimension health states were developed using 8 SBS-QoL™ items (2 dimensions combined 2 SBS-QoL™ items). SBS health states were valued by a UK general population sample (N = 250) using the lead-time time trade-off method. Preference weights or 'utility decrements' for each severity level of each dimension were estimated by regression models and used to develop the scoring algorithm. RESULTS: Mean utilities for the SBS health states ranged from -0.46 (worst health state, very much affected on all dimensions) to 0.92 (best health state, not at all affected on all dimensions). The random effects model with maximum likelihood estimation regression had the best predictive ability and lowest root mean squared error and mean absolute error, and was used to develop the scoring algorithm. CONCLUSIONS: The preference-weighted scoring algorithm for the SBS-QoL™ developed is able to estimate a wide range of utility values from patient-level SBS-QoL™ data. This allows estimation of SBS HRQL impact for the purpose of economic evaluation of SBS treatment benefits.

Bowel-associated dermatosis-arthritis syndrome in an adolescent with short bowel syndrome.

Bowel-associated dermatosis-arthritis syndrome (BADAS) is a neutrophilic dermatosis, characterized by the occurrence of arthritis and skin lesions related to bowel disease with or without bowel bypass. We report an unusual case of BADAS in a 15-year-old white male with congenital aganglionosis of the colon and hypoganglionosis of the small intestine and multiple bowel surgeries in childhood complicated by short bowel syndrome. He presented with recurrent peripheral polyarthritis, tenosynovitis, and painful erythematous subcutaneous nodules located on the dorsolateral regions of the legs and on the dorsa of the feet. Histological examination disclosed a neutrophilic dermatosis confirming the diagnosis of BADAS.Although an uncommon disease, especially at pediatric age, it is important to evoke the diagnosis of BADAS in children and adolescents with bowel disease, because treatment options and prognosis are distinct from other rheumatologic conditions.