The Variable Response to Teduglutide in Pediatric Short Bowel Syndrome: A Single Country Real-Life Experience.

OBJECTIVES: The glucagon-like peptide-2 analog Teduglutide has been shown to enhance intestinal absorption and decrease parenteral nutrition (PN) requirements in short bowel syndrome (SBS). As data in children is limited, we evaluated nationwide real-life experience and treatment outcome in children with SBS. METHODS: Longitudinal data of children treated with Teduglutide for ≥3 months was collected. Data included demographic and medical background, anthropometrics, laboratory assessments and PN requirements. Treatment response was defined as >20% reduction in PN requirement. RESULTS: The study included 13 patients [54% males, median (interquartile range {IQR}) age of 6 (4.7-7) years]. The most common SBS etiology was necrotizing enterocolitis (38%), and median (IQR) small bowel length was 20 (15-40) cm. Teduglutide treatment ranged between 3 and 51 months [median (IQR) of 18 (12-30) months], with 10 patients (77%) treated >1 year. Response to treatment was observed in 8 patients (62%), with a mean [±standard deviation (SD)] treatment duration of 5.9 (±3.2) months. Among responders, 2 patients were weaned off PN and additional 4 decreased PN needs by >40%. There was a median (IQR) reduction in PN volume/kg of 36% (15%-55%) and in PN energy/kg of 27% (6%-58%). Response was not associated with patients' background, and no correlation was found with bowel length or PN dependency at baseline. CONCLUSIONS: Real-life response to Teduglutide is highly variable among children with SBS. While most patients did reach 20% reduction in PN, less achieved further significant reduction or enteral autonomy. No predictive factors of response to treatment were identified, and large multicenter studies are needed to elucidate predictive factors and long-term outcome.

Diagnostic Utility of Increased STIR Signal in the Posterior Atlanto-Occipital and Atlantoaxial Membrane Complex on MRI in Acute C1-C2 Fracture.

BACKGROUND AND PURPOSE: Acute C1-C2 fractures are difficult to detect on MR imaging due to a paucity of associated bone marrow edema. The purpose of this study was to determine the diagnostic utility of increased STIR signal in the posterior atlanto-occipital and atlantoaxial membrane complex (PAOAAM) in the detection of acute C1-C2 fractures on MR imaging. MATERIALS AND METHODS: Eighty-seven patients with C1-C2 fractures, 87 with no fractures, and 87 with other cervical fractures with acute injury who had both CT and MR imaging within 24 hours were included. All MR images were reviewed by 2 neuroradiologists for the presence of increased STIR signal in the PAOAAM and interspinous ligaments at other cervical levels. Sensitivity and specificity of increased signal within the PAOAAM for the presence of a C1-C2 fracture were assessed. RESULTS: Increased PAOAAM STIR signal was seen in 81/87 patients with C1-C2 fractures, 6/87 patients with no fractures, and 51/87 patients with other cervical fractures with 93.1% sensitivity versus those with no fractures, other cervical fractures, and all controls. Specificity was 93.1% versus those with no fractures, 41.4% versus those with other cervical fractures, and 67.2% versus all controls for the detection of acute C1-C2 fractures. Isolated increased PAOAAM STIR signal without increased signal in other cervical interspinous ligaments showed 89.7% sensitivity versus all controls. Specificity was 95.3% versus those with no fractures, 83.7% versus those with other cervical fractures, and 91.4% versus all controls. CONCLUSIONS: Increased PAOAAM signal on STIR is a highly sensitive indicator of an acute C1-C2 fracture on MR imaging. Furthermore, increased PAOAAM STIR signal as an isolated finding is highly specific for the presence of a C1-C2 fracture, making it a useful sign on MR imaging when CT is either unavailable or the findings are equivocal.

A pilot study of the "Continuation of Care" model in "revolving-door" patients.

PURPOSE: The efficiency of continuation of care (COC) treatment by inpatient caregivers as compared to treatment administered by outpatient services for "revolving door" psychiatric patients was tested in this study. Number and days of hospitalization were examined. METHOD: All patients who were hospitalized three times or more during the past 12 months were offered continuing follow-up in the ward, by the same staff, instead of being referred to the outpatient department. Information on number and length of hospitalizations before and after initiation of this care model was retrieved from the hospital computerized database. RESULTS: Of the 36 patients meeting the criteria, 35 patients agreed to participate. The number of hospitalizations in the 18 months following the index hospitalization was 1.79±3.51 as compared to 4.67±1.79 before the index hospitalization (p=0.0002), and the number of days of hospitalization 18 months after was 24±41.65 as compared to 119.71±69.31 before (p<0.0001). CONCLUSION: COC via inpatient follow-up significantly reduces the number and length of hospitalizations in "revolving door" psychiatric patients as compared to the traditional system of follow-up in an outpatient clinic.

n-3 fatty acids ameliorate hepatic steatosis and dysfunction after LXR agonist ingestion in mice.

UNLABELLED: Liver X receptor (LXR) agonists slow atherogenesis, but cause hepatic steatosis and dysfunction in part by increasing expression of sterol regulatory element binding protein 1-c (SREBP1-c), a transcription factor that upregulates fatty acid (FA) synthesis. n-3 FAs decrease hepatic FA synthesis by down-regulating SREBP1-c. To test the hypothesis that n-3 FAs decrease hepatic steatosis in mice given LXR agonist, C57BL/6 mice received daily gavage of an LXR agonist T0901317 (LXR(T)) or vehicle for 4weeks with concomitant intakes chow or high-fat diets enriched in saturated fat (SAT) or n-3 fat (n-3). Mice on LXR(T) and SAT developed hepatomegaly with a large increase in size and number of hepatic lipid droplets; an n-3 diet reduced liver weight/body weight with decreased hepatic steatosis and triglyceride levels. Effects of n-3 diet on hepatic lipogenesis were linked to a blunting of LXR(T) upregulation of hepatic SREBP1-c and FA synthase mRNA. n-3 diets also normalized LXR(T)-mediated increases of plasma ALT and AST levels, whereas SAT diet increased these markers. CONCLUSION: These studies suggest that n-3 FAs when given together with LXR agonists have the potential to improve both hepatic steatosis and hepatotoxicity in humans that might receive LXR agonists to decrease risk of atherosclerosis.

Postrenal transplant non-EBV multiple myeloma of donor origin.

Multiple myeloma occurring after solid organ transplantation is a rare condition, with only a few case reports found in the literature. We report a case of Epstein-Barr virus-negative, posttransplant multiple myeloma in a 67-year-old female, presenting 18 months after renal transplantation. Interestingly, fluorescence in situ hybridization analysis of the tumor revealed a Y chromosome in the majority of the cells, indicating that the neoplasm was derived from the donor kidney. To our knowledge, this represents the first reported case with these features.