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        Outcomes of Portosystemic Shunts in Children with and without Liver Transplantation

        Hamza Hassan Khan,Stuart S. Kaufman,Nada A. Yazigi,Khalid M. Khan 대한소아소화기영양학회 2024 Pediatric gastroenterology, hepatology & nutrition Vol.27 No.1

        Purpose: Limited data exist regarding outcome and morbidity associated with portosystemic shunts in the pediatric transplant population. Our study assesses the outcomes of pediatric patients who underwent a portosystemic shunt procedure, both with and without liver transplantation (LT). Methods: This study retrospectively reviewed the medical records of pediatric patients aged 0–19 years who underwent shunt placement between 2003 and 2017 at a tertiary care center. The analysis included cases of shunt placement with or without LT. Results: A total of 13 pediatric patients were included in the study with median age of 8.8 years. Among the cases, 11 out of 13 (84.6%) underwent splenorenal shunt, 1 (7.7%) underwent a mesocaval shunt, and another 1 (7.7%) underwent a Modified Rex (mesoportal) shunt. Additionally, 5 out of 13 (38.5%) patients had LT, with 4 out of 5 (80.0%) receiving the transplant before shunt placement, and 1 out of 5 (20.0%) receiving it after shunt placement. Gastrointestinal bleeding resulting from portal hypertension was the indication in all cases. A total of 10 complications were reported in 5 patients; the most common complication was anemia in 3 (23.1%) patients. At the most recent follow-up visit, the shunts were functional without encephalopathy, and no deaths were reported. Conclusion: Shunt placement plays a crucial role in the management of patients with portal hypertension. Our study demonstrates favorable long-term outcomes in pediatric patients who underwent shunt placement. Long term shunt outcomes were similar and unremarkable in patients with LT and without LT.

      • The Role of Genetic Variation Near Interferon-Kappa in Systemic Lupus Erythematosus

        Harley, Isaac T. W.,Niewold, Timothy B.,Stormont, Rebecca M.,Kaufman, Kenneth M.,Glenn, Stuart B.,Franek, Beverly S.,Kelly, Jennifer A.,Kilpatrick, Jeffrey R.,Hutchings, David,Divers, Jasmin,Bruner, G Hindawi Publishing Corporation 2010 Journal of biomedicine & biotechnology Vol.2010 No.-

        <P>Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by increased type I interferons (IFNs) and multiorgan inflammation frequently targeting the skin. IFN-kappa is a type I IFN expressed in skin. A pooled genome-wide scan implicated the <I>IFNK</I> locus in SLE susceptibility. We studied <I>IFNK</I> single nucleotide polymorphisms (SNPs) in 3982 SLE cases and 4275 controls, composed of European (EA), African-American (AA), and Asian ancestry. rs12553951C was associated with SLE in EA males (odds ratio = 1.93, <I>P</I> = 2.5 × 10<SUP>−4</SUP>), but not females. Suggestive associations with skin phenotypes in EA and AA females were found, and these were also sex-specific. <I>IFNK</I> SNPs were associated with increased serum type I IFN in EA and AA SLE patients. Our data suggest a sex-dependent association between <I>IFNK</I> SNPs and SLE and skin phenotypes. The serum IFN association suggests that <I>IFNK</I> variants could influence type I IFN producing plasmacytoid dendritic cells in affected skin.</P>

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