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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>