Sabrina E. Sanchez

The effect of lipid restriction on the prevention of parenteral nutrition-associated cholestasis in surgical infants

PURPOSE Surgical infants requiring long-term parenteral nutrition (PN) are at risk for parenteral nutrition-associated liver disease (PNALD). The purpose of this study was to determine the effect of a lipid restricted PN regimen in preventing the development of PNALD in surgical infants. METHODS In 2009, we implemented a lipid restricted strategy in surgical infants expected to be on long-term PN using a soy-based lipid emulsion at a goal provision of 1g/kg/day throughout a patients entire PN course. An experimental cohort of surgical infants treated with lipid restriction from 2009 to 2011 (n=82) was retrospectively compared to a control cohort of infants from 2005 to 2008 receiving standard intravenous lipid dosing (n=132). A multivariable relative risk regression model was constructed analyzing the association between lipid restriction and PNALD. RESULTS Patients admitted during the lipid restriction era had reduced daily lipid provisions compared to the control group (p<0.001). There were no significant differences in demographic or measured clinical characteristics between the two groups. A significant reduction in the incidence of PNALD was demonstrated in the lipid restricted group compared to the control group (22% vs. 43%, p=0.002). On multivariable relative risk regression, patients treated with standard lipid provisions were 1.77 times more likely to develop PNALD than patients who were lipid restricted (95% CI: 1.2-2.7; p=0.007). CONCLUSION Restriction of intravenous soy-based lipid in PN-fed surgical infants is associated with a reduction in the incidence of liver disease. Early lipid restriction should be considered in all surgical infants who require PN as a preventative measure against PNALD.

Ultrashort bowel syndrome in children.

Objective: Recent data have demonstrated improved survival in children with intestinal failure. We hypothesized that this trend would also be observed in children with ultrashort bowel syndrome. Methods: A prospective database from Seattle Childrens Intestinal Failure Program was used to evaluate outcomes and morbidities of consecutive patients with 10 cm or less of small bowel enrolled in the program since 2005. Data are listed as median (range). Results: Five patients were identified with a bowel length of 6 (1–10) cm and follow-up of 54 (43–61) months. All children have survived and are currently between 3.5 and 5.5 years of age. One patient underwent isolated intestinal transplantation and one patient is currently listed for intestinal transplantation. The transplanted child is fully enterally fed. The other patients remain at least partially dependent on parenteral nutrition. None of these patients have current evidence of parenteral nutrition-associated liver disease. Patients have required extensive care after referral to our program, including 18 (15–32) visits to the emergency room, 152 (114–273) days of inpatient care, and 6 (5–9) central line–associated blood stream infections. Conclusions: Long-term survival in children with ultrashort bowel length is possible after referral to an intestinal failure program, although extensive medical management is required. These children may be reasonable candidates for long-standing intestinal rehabilitation as a bridge to intestinal transplantation.

Hepatic artery thrombosis and liver malignancy in pediatric liver transplantation

BACKGROUND Hepatic artery thrombosis (HAT) remains a significant cause of graft failure and mortality after pediatric liver transplantation. Conditions not associated with hepatic failure, such as liver tumors, may be more prone to thrombotic problems after transplant. We hypothesized that liver transplant for hepatic malignancies may be associated with increased rates of HAT in the posttransplant period. METHODS We conducted a retrospective review of pediatric patients (age, 0-21 years) who underwent primary liver transplantation at a free-standing childrens hospital from 1990 to 2009. We reviewed cause of underlying liver disease, age, sex, weight, occurrence of HAT, use of antiplatelets and anticoagulants perioperatively, as well as reintervention, retransplant, and death. RESULTS A total of 129 children underwent 146 liver transplants, and 15 (12%) patients developed HAT. Nine liver transplants were performed for hepatic malignancy, and 4 (44%) of these patients developed HAT (relative risk, 4.85; 95% confidence interval, 1.9-12.2; P = .0015). All 4 children with hepatic malignancy and HAT required reintervention, including 3 retransplants (75%). One of these patients died. CONCLUSIONS Hepatic artery thrombosis occurs approximately 5 times more often and appears to be more morbid in children with hepatic malignancy after transplantation. Prospective evaluation of prophylactic anticoagulation regimens in the setting of hepatic malignancy requiring transplantation is warranted.

Health-related quality of life in children with intestinal failure.

Objectives: Survival of infants with intestinal failure (IF) has increased in the past decade; however, data on their health-related quality of life (HRQOL) are lacking. We hypothesized that HRQOL would be lower among children with IF compared with that of healthy children. Methods: We performed a cross-sectional study of the HRQOL of children enrolled in the outpatient intestinal rehabilitation program at Seattle Childrens Hospital using the PedsQL 4.0 Generic Core Scales parent proxy-report and the Family Impact Module questionnaires. Parents were asked 2 open-ended questions pertaining to the suitability and completeness of the PedsQL to assess their and their childs HRQOL. Results: Parents of 23 children with IF completed the questionnaires. Compared with norms for healthy children, parents reported significantly lower total PedsQL scores for children ages 1 to 2 years (mean difference −13.16, 95% confidence interval [CI] −21.86 to −4.46; P = 0.003) and 2 to 6 years (mean difference −15.57, 95% CI −22.66 to −8.48; P < 0.001). Scores were also lower for children younger than 1 year (mean difference −6.43, 95% CI −13.93 to 1.07), although this test was not statistically significant. No measured demographic or clinical characteristics were associated with HRQOL. The majority of parents (65%) said the PedsQL failed to address important effects of IF on children and their families. Conclusions: Children with IF and their parents have a decreased HRQOL compared with healthy children as measured by the PedsQL survey. A disease-specific module or separate HRQOL questionnaire is needed for a more comprehensive assessment of HRQOL in children with IF.

Neurodevelopmental and Cognitive Outcomes in Children With Intestinal Failure.

Objectives: Recent advances in medical and surgical management have led to improved long-term survival in children with intestinal failure. Yet, limited data exist on their neurodevelopmental and cognitive outcomes. The aim of the present study was to measure neurodevelopmental outcomes in children with intestinal failure. Methods: Children enrolled in a regional intestinal failure program underwent prospective neurodevelopmental and psychometric evaluation using a validated scoring tool. Cognitive impairment was defined as a mental developmental index <70. Neurodevelopmental impairment was defined as cerebral palsy, visual or hearing impairment, or cognitive impairment. Univariate analyses were performed using the Wilcoxon rank-sum test. Data are presented as median (range). Results: Fifteen children with a remnant bowel length of 18 (5–85) cm were studied at age 17 (12–67) months. Thirteen patients remained dependent on parenteral nutrition. Twelve (80%) subjects scored within the normal range on cognitive testing. Each child with cognitive impairment was noted to have additional risk factors independent of intestinal failure including cardiac arrest and extreme prematurity. On univariate analysis, cognitive impairment was associated with longer inpatient hospital stays, increased number of surgical procedures, and prematurity (P < 0.02). In total, 4 (27%) children demonstrated findings consistent with neurodevelopmental impairment. Conclusions: A majority of children with intestinal failure demonstrated normal neurodevelopmental and cognitive outcomes on psychometric testing. These data suggest that children with intestinal failure without significant comorbidity may be at low risk for long-term neurodevelopmental impairment.

The Safety and Immunogenicity of Rotavirus Vaccination in Infants With Intestinal Failure

BACKGROUND Young children with intestinal failure are at risk for complications from rotavirus gastroenteritis. To date, the safety and immunogenicity of rotavirus vaccines in these children are not known. We hypothesized that rotavirus vaccination would be safe and confer immunity to infants with intestinal failure and a history of abdominal surgery. METHODS The study population consisted of infants with early intestinal failure who required abdominal surgery and parenteral nutrition for necrotizing enterocolitis, gastroschisis, jejunoileal atresia, or meconium peritonitis. Subjects received a rotavirus vaccine series at the appropriate age. Safety assessments were performed for the first 5 days after each vaccine dose. Viral stool shedding and serum rotavirus antigen were measured at multiple time points after each dose of the vaccine. A 3-fold increase in rotavirus immunoglobulin A titer at study completion defined seroconversion. RESULTS Fifteen infants were enrolled and 14 infants completed the study protocol. Mild diarrhea, emesis, and fever were noted after vaccination in 33%, 40%, and 7% of subjects, respectively. No subject experienced postvaccine intussusception, viremia, dehydration, or required alterations in nutritional care. Viral stool shedding was noted in 47%, but only 1 child shed virus beyond 2 weeks postvaccination. All subjects who were not considered immune at baseline developed seroconversion to rotavirus after vaccination. CONCLUSIONS In infants with intestinal failure, rotavirus vaccination appears to be safe and immunogenic. We found no evidence for altered viral shedding in this population. Given the profound consequences associated with rotavirus infection and potential benefits of rotavirus vaccine in this cohort, multicenter studies focusing on vaccine efficacy are warranted.

Vesicoureteral reflux and febrile urinary tract infections in anorectal malformations: A retrospective review

BACKGROUND Multiple studies document a correlation between anorectal malformations (ARMs) and vesicoureteral reflux (VUR), development of urinary tract infections (UTIs), and subsequent renal disease. We aimed to determine which patient characteristics are associated with VUR and UTI in this population. METHODS A retrospective review of ARM patients at a free-standing childrens hospital from January 1996 to December 2011 was performed. Logistic regression was used to investigate the associations between VUR and UTI and ARM classification and co-morbid diagnoses. RESULTS Of 190 patients, 41 (31%) received a diagnosis of VUR. Thirty-one of the 190 patients had at least one febrile UTI (16%). Of these, only 16 (51%) had a diagnosis of VUR. On multivariable logistic regression, the only patient variable associated with VUR was having an ectopic kidney (p=0.026). Similarly, the presence of GU malformations was the closest variable associated with developing a UTI (p=0.073). CONCLUSIONS In ARM patients, VUR as well as UTIs are associated with the presence of GU malformations. Thus, voiding cystourethrogram (VCUG) testing should be pursued when there are other caudal and GU abnormalities, regardless of fistula location. Antibiotic prophylaxis for UTI should be considered in children with ARM and any GU malformation, not only VUR.

Isolated appendiceal typhlitis masquerading as perforated appendicitis in the setting of acute lymphoblastic leukemia

Abdominal pain is common during chemotherapy for childhood leukemia. Clinically differentiating typhlitis from appendicitis can be difficult. We present an 8-year-old boy with abdominal pain in the setting of acute lymphoblastic leukemia and neutropenia. Following appendectomy for presumed appendicitis, pathology revealed appendiceal typhlitis. Diagnostic and treatment considerations are discussed.

Lipid management in pediatric intestinal failure.

Purpose of reviewLipids are a crucial component in parenteral nutrition but their prolonged use can be associated with parenteral nutrition-associated liver disease (PNALD), which may lead to significant morbidity and mortality. The purpose of this review is to discuss the current state of lipid management in pediatric intestinal failure, especially as it relates to the prevention of PNALD. Recent findingsLipid restriction has emerged as a successful strategy in both the treatment and prevention of PNALD in parenteral nutrition-dependent children. There is concern, however, that lipid restriction can lead to essential fatty acid deficiency. This, in turn, may affect neurodevelopmental outcomes. A wide variety of lipid emulsion formulations is available throughout the world although only one, a soybean-based product, is Food and Drug Administration-approved for use in the pediatric population. To date, there are no definitive data favoring one lipid emulsion composition over another. Summary: Pediatric intestinal failure patients are at high risk for the development of PNALD. Although this may be managed, and even prevented, with lipid restrictive strategies, the development of essential fatty acid deficiency in this setting remains a concern. Further studies are needed to determine the ideal lipid emulsion and dose to be used in parenteral nutrition-dependent children.

Colocolonic intussusception in a four-yr-old with a heart transplant: A case report and review of the literature

Sanchez S, Javid P, Ricca R, Avansino J. Colocolonic intussusception in a four‐yr‐old with a heart transplant: A case report and review of the literature. Pediatr Transplantation 2011.