Metabolic profile of children with extrahepatic portal vein obstruction undergoing meso-Rex bypass

Background: Extrahepatic portal vein obstruction (EHPVO) in children is often associated with growth restriction, which improves after the restoration of portal venous flow with a meso-Rex bypass, but the physiologic mechanism is unknown. The purpose of this study was to investigate the mechanism of growth delay in children with EHPVO by detailing the metabolic and nutritional profile before and after meso-Rex bypass. / Methods: Twenty consecutive children with EHPVO were prospectively studied before and 1 year after meso-Rex bypass. Caloric balance was determined by investigating caloric intake via a calorie count, total energy expenditure via a doubly labeled water isotope assay and stool caloric loss by bomb calorimetry. Laboratory markers of nutrition and growth hormone resistance were tested. / Results: Fifteen of the 20 children underwent successful meso-Rex bypass at a median age of 4.3 years. Prealbumin level was abnormally low (14.6 ± 3.0 mg/dL) at surgery but improved (17.0 ± 4.3 mg/dL) 1 year later (P = 0.026). Mean insulin-like growth factor 1 (IGF-1) level at baseline was 1.57 standard deviations below normal. IGF-1 levels increased from 88.3 ± 38.9 to 117.3 ± 54.5 ng/mL in the year after surgery (P = 0.047). Caloric intake divided by basal metabolic rate (1.90 ± 0.61), total energy expenditure (97.2 ± 15.0% of expected), and stool caloric losses (3.7 ± 1.8% of caloric intake) were all normal at baseline. / Conclusions: Children with EHPVO suffer from malnutrition and growth hormone resistance, which may explain their well-established finding of growth restriction. Prealbumin and IGF-1 levels improve after a successful meso-Rex bypass

Unexpected outcome from Trousseau syndrome

<p>Abstract</p> <p>Background</p> <p>Unprovoked superficial thrombophlebitis and subsequent venous thromboembolism are well-described albeit rare presenting features of advanced visceral malignancy that often manifest too late for curative intervention to be beneficial.</p> <p>Case Presentation</p> <p>We present the first reported case of early gastric carcinoma presenting with these paraneoplastic phenomena in an otherwise healthy farmer. The early presentation allowed for a curative partial gastrectomy, which itself was complicated by the presence of a deep vein thrombosis extending into the inferior vena cava. Fortunately, stabilization of the clot allowed for surgery to proceed without the need for a caval filter. The patient was referred for adjuvant chemotherapy and has since made an excellent recovery.</p> <p>Conclusions</p> <p>This case provides new evidence for the presentation of superficial thrombophlebitis in early gastric carcinoma and the potential for curative surgery in such instances. A high index of suspicion and a prompt diagnostic workup are essential for timely planning and execution of surgery in these early albeit rare presentations.</p

Inherited liver shunts in dogs elucidate pathways regulating embryonic development and clinical disorders of the portal vein

Congenital disorders of the hepatic portal vasculature are rare in man but occur frequently in certain dog breeds. In dogs, there are two main subtypes: intrahepatic portosystemic shunts, which are considered to stem from defective closure of the embryonic ductus venosus, and extrahepatic shunts, which connect the splanchnic vascular system with the vena cava or vena azygos. Both subtypes result in nearly complete bypass of the liver by the portal blood flow. In both subtypes the development of the smaller branches of the portal vein tree in the liver is impaired and terminal branches delivering portal blood to the liver lobules are often lacking. The clinical signs are due to poor liver growth, development, and function. Patency of the ductus venosus seems to be a digenic trait in Irish wolfhounds, whereas Cairn terriers with extrahepatic portosystemic shunts display a more complex inheritance. The genes involved in these disorders cannot be identified with the sporadic human cases, but in dogs, the genome-wide study of the extrahepatic form is at an advanced stage. The canine disease may lead to the identification of novel genes and pathways cooperating in growth and development of the hepatic portal vein tree. The same pathways likely regulate the development of the vascular system of regenerating livers during liver diseases such as hepatitis and cirrhosis. Therefore, the identification of these molecular pathways may provide a basis for future proregenerative intervention

The significance of margins in pediatric Non-Rhabdomyosarcoma soft tissue sarcomas: Consensus on surgical margin definition harmonization from the INternational Soft Tissue SaRcoma ConsorTium (INSTRuCT)

Background: Margin status following surgery in children, adolescents, and young adults with soft tissue sarcomas is controversial and has been defined differently by various specialties, with definitions changing over time and by cooperative group. The International Soft Tissue Sarcoma Consortium (INSTRuCT) is a collaboration of the Children's Oncology Group (COG) Soft Tissue Sarcoma Committee, European pediatric Soft Tissue sarcoma Study Group (EpSSG), and the European Cooperative Weichteilsarkom Studiengruppe (CWS) devoted to improving patient outcomes by pooling and mining cooperative group clinical trial data. Methods: The INSTRuCT non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) working group aimed to develop international harmonized recommendations regarding surgical margin assessment and definitions in children and adolescents with soft tissue tumors. Results and Conclusion: This review addresses accepted principles and areas of controversy, including the perspectives of surgeons, pathologists, radiation oncologists, and pediatric oncologists, to develop a framework for building common guidelines for future research