Ultrasound Elastography as a Non-Invasive Method to Monitor Liver Disease in Children with Short Bowel Syndrome: Updated Results.

PURPOSE: The purpose of the study was to determine the accuracy of acoustic radiation force impulse (ARFI) ultrasound elastography in assessing the degree of liver disease in children with short bowel syndrome (SBS). METHODS: A prospective observational cohort study of patients with SBS who underwent a liver biopsy and ARFI elastography was performed. Mean shear wave speed (SWS) and stage of fibrosis was evaluated using t-tests. Receiver operating characteristic curves (ROC) were generated and the area under the curves (AUC) estimated in order to assess the accuracy of SWS measurements to discriminate between stages of fibrosis. RESULTS: Thirty-seven paired elastography and biopsy samples from 31 patients were included. The median age was 0.6 years, and 61% were male. There was a significant positive correlation between stage of fibrosis and mean SWS (ß=0.16 m/s increase per stage, p=<0.001). ROC analysis revealed that mean SWS had good accuracy for discriminating between mild liver fibrosis (F0-F1) and moderate to severe fibrosis (F2-F4) (AUC=0.80, 95% CI 0.65-0.95). In addition, ROC analysis demonstrated that mean SWS can also accurately discriminate between mild to moderate fibrosis (F0-F2) and more severe fibrosis (F3-F4) (AUC=0.84, 95% CI 0.71-0.96). CONCLUSION: ARFI elastography is an accurate, non-invasive method to monitor liver disease in children with SBS. TYPE OF STUDY: Retrospective Cohort Study LEVEL OF EVIDENCE: II.

Resources for the Provision of Nutrition Support to Children in Educational Environments.

The use of nutrition support outside of institutional settings has contributed to maintaining the health, well-being, and nutrition status of many medically complex children. As these children grow and enter educational settings, there is a need for awareness of the care that these children require for nutrition support therapy. This document is designed to raise awareness to these needs, provide best practice educational resources for those involved in the supervision or provision of nutrition support to children in an educational environment, and promote safe and effective care. Care of children requiring nutrition support is an ongoing and shared partnership among the educational team, medical team, homecare team, and parents/caregivers. Care is individualized to the specific child and may include provision of nutrition support therapy while in the school setting, maintenance of a nutrition access device, and monitoring to safely prevent or act on signs of potential complications. Suggested roles and responsibilities of those involved with nutrition support care are discussed; however, all interventions and routine care must be in accordance with physician's orders, school nurse privileges and competencies, and state and local regulations.

A pilot study of ultrasound elastography as a non-invasive method to monitor liver disease in children with short bowel syndrome.

BACKGROUND: Our objective was to evaluate the accuracy of acoustic radiation force impulse (ARFI) elastography in determining extent of liver fibrosis in pediatric patients with short bowel syndrome (SBS). METHODS: This prospective cohort study included pediatric patients with SBS who underwent ultrasound with ARFI measurements of shear wave speed (SWS) and liver biopsy within 30days of each other between 12/2014-9/2015. The mean and median SWS were compared to the stage of fibrosis. Receiver operating characteristic curves (ROC) were also generated to assess the classification accuracy of SWS measurements for mild (F0-F1) versus moderate/severe fibrosis (F2-F4). RESULTS: Twelve patients were included with median age 1.4years and median weight 11.3kg. The median direct bilirubin level was 0.3mg/dL at the time of biopsy. On liver biopsy, 6 patients had mild fibrosis (F1), 3 had septal fibrosis (F3), and 3 had cirrhosis (F4). The area under the ROC curve for elastography to differentiate moderate/severe liver fibrosis from mild disease was 0.83 (95CI 0.58-1.00) and 0.86 (95CI 0.63-1.00) for the median SWS and mean SWS, respectively. CONCLUSIONS: These early results suggest that ARFI ultrasound elastography may be a reliable non-invasive method to monitor liver fibrosis in pediatric patients with SBS. TYPE OF STUDY: Prospective observational cohort. LEVEL OF EVIDENCE: 3.

Accuracy of radiographic estimation of small bowel dimensions in pediatric patients with short bowel syndrome.

BACKGROUND: The decision to perform autologous intestinal lengthening in patients with short bowel syndrome (SBS) depends on total bowel length and the diameter and length of dilated segments. This study evaluated the accuracy of radiologic measurements of intestinal length and diameter. METHODS: Patients who underwent an intestinal lengthening procedure with preoperative upper gastrointestinal study (UGI) were identified from 10/2012 through 1/2015. Measurements of total length and diameters and lengths of dilated segments on UGI were compared to intraoperative measurements using Spearman's rank correlation coefficients and Bland-Altman plots. RESULTS: Fourteen patients underwent 15 lengthening procedures. Median age was 3.6years. Most common causes of SBS were complicated gastroschisis (43%) and small bowel atresia (36%). Intra-operative bowel lengths prior to performing lengthening procedures ranged from 21 to 170cm. The median measurements of radiographic and operative measurements (respectively) were total bowel lengths 77cm and 69cm (r=0.93, p<0.0001), maximum diameters 7.7cm and 7cm (r=0.86, p=0.001), and lengths of dilated segments 13cm and 14cm (r=0.41, p=0.36). CONCLUSIONS: The correlation between UGI and operative measurements suggests that prediction of total bowel length and maximum diameter of dilation is accurate and can assist with operative planning.

Incidence, pattern, and etiology of elevated liver enzymes in pediatric inflammatory bowel disease.

OBJECTIVES: Patients with inflammatory bowel disease (IBD) often develop elevated liver enzymes (ELE), which are frequently a benign, transient finding, but may be related to treatment or IBD-associated liver diseases. Distinguishing benign from pathologic ELE is crucial for focused diagnostic and therapeutic interventions. We sought to characterize the incidence, character, chronicity, degree, and etiology of ELE in children with IBD. METHODS: Institutional review board-approved retrospective review of all of the patients with IBD (2-21 years) seen between October 2009 and October 2012 with >9 months of follow-up were included in the study. We examined body mass index, disease activity, extent, phenotype, concurrent medications, and character, chronicity, degree of enzyme elevation, and final diagnosis. RESULTS: A total of 219 of 514 patients with IBD had ≥1 episode of ELE. Five patients were excluded for preexisting liver disease, leaving 214 patients (Crohn disease [CD]: 14.8 ±â€Š3.5 years, 46% girls; ulcerative colitis [UC]: 14.4 ±â€Š4.2 years, 37% girls). One hundred forty-eight patients (69%) had a hepatic, 17 (8%) cholestatic, and 49 (23%) mixed character of ELE. There were no significant differences in character, chronicity, or degree of ELE between CD and UC (P = 0.71, P = 0.58, P > 0.33). Of the 128 patients with sufficient data to determine chronicity, 98 (77%) had transient elevations, (CD: n = 66, 75% and UC: n = 32, 80%). Episodes of ELE were idiopathic in 87% of patients with IBD. A final diagnosis of idiopathic ELE was associated with a lower degree of ELE elevation (P < 0.0001). CONCLUSIONS: Pediatric patients with IBD commonly experience transient, idiopathic ELE. Our findings suggest that higher degrees of ELE, specifically alanine aminotransferase, are associated with an etiology that requires more extensive evaluation.

Safety of pediatric percutaneous liver biopsy performed by interventional radiologists.

OBJECTIVE: National data suggest that pediatric percutaneous liver biopsy is increasingly being performed by interventional radiologists rather than pediatric gastroenterologists. The objective of the present report is to describe the safety and effectiveness of percutaneous liver biopsy performed by interventional radiologists in a large cohort of children and to compare the results with the existing literature on biopsies performed by pediatric gastroenterologists. PATIENTS AND METHODS: The medical records of 249 children undergoing ultrasound-guided percutaneous liver biopsy by interventional radiologists were reviewed for adverse events and success of obtaining tissue. Two hundred ninety-four biopsies were reviewed. RESULTS: There were no deaths. There were 2 instances of a 2-g or greater drop in hemoglobin following biopsy, neither of which was associated with clinical signs of hemorrhage. A small, asymptomatic pneumothorax quickly resolved without treatment. One patient developed Klebsiella sepsis 48  hours after biopsy. In all but 1 case, an adequate sample size was obtained. This low incidence of adverse events compares favorably with existing published reports of morbidity and mortality following percutaneous liver biopsy performed by pediatric gastroenterologists. CONCLUSIONS: Ultrasound-guided percutaneous liver biopsy performed by experienced pediatric interventional radiologists in a children's hospital setting is as safe and effective as biopsy performed by pediatric gastroenterologists.

Presentation and outcome of histoplasmosis in pediatric inflammatory bowel disease patients treated with antitumor necrosis factor alpha therapy: a case series.

BACKGROUND: Antitumor necrosis factor alpha (aTNF) therapies are commonly used in the treatment of pediatric inflammatory bowel disease (IBD). However, inhibition of the TNF-alpha pathway predisposes to serious infections, including histoplasmosis, which is the most common invasive fungal infection in individuals on aTNF therapy and carries a high mortality rate when associated with delayed diagnosis. Few data exist on the frequency, presentation, and appropriate treatment of pediatric patients with histoplasmosis on aTNF therapy. METHODS: Following Institutional Review Board approval, cases were identified then reviewed with their primary gastroenterologist and infectious disease specialists. RESULTS: Herein we describe histoplasmosis in five pediatric patients receiving aTNF therapy for IBD in an endemic area. CONCLUSIONS: Histoplasmosis is an important complication of treatment with TNF-alpha neutralizing agents. Children with IBD treated with aTNF therapy who develop the infection may present with minimal pulmonary symptoms. While discontinuation of aTNF therapy is important initially, few data exist to determine when and how aTNF therapy can be reinstituted. Recognition of Histoplasma capsulatum is often delayed due to the overlap of symptoms with some of the extraintestinal manifestations of IBD and other more prevalent infectious complications.

Myectomy-myotomy for long segment Hirschsprung's disease in a patient with Haddad syndrome.

Hirschsprung's disease is a rare entity with an incidence of 1 in 5000 live born infants. Long segment Hirschsprung's disease occurs in approximately 5% to 10% of this patient population and is defined as a transition zone proximal to the sigmoid colon (Bodian M, Carter CO, Ward BC. Hirschsprung's disease. Lancet. 1951;1:302-309). The association of congenital central hypoventilation syndrome (also known as Ondine's curse) and Hirschsprung's disease is termed Haddad syndrome.