Recurrent BRAF Gene Fusions in a Subset of Pediatric Spindle Cell Sarcomas: Expanding the Genetic Spectrum of Tumors With Overlapping Features With Infantile Fibrosarcoma.

Infantile fibrosarcomas (IFS) represent a distinct group of soft tissue tumors occurring in patients under 2 years of age and most commonly involving the extremities. Most IFS show recurrent ETV6-NTRK3 gene fusions, sensitivity to chemotherapy, and an overall favorable clinical outcome. However, outside these well-defined pathologic features, no studies have investigated IFS lacking ETV6-NTRK3 fusions, or tumors with the morphology resembling IFS in older children.

Failure to thrive, interstitial lung disease, and progressive digital necrosis with onset in infancy.

Key teaching points • SAVI is a recently described interferonopathy resulting from constitutive action of STING and up-regulation of IFN-β signaling. • SAVI is characterized by facial erythema with telangiectasia, acral/cold-sensitive tissue ulceration and amputations, and interstitial lung disease. It has overlapping features with Aicardi-Goutières syndrome and familial chilblain lupus.

Clinicopathological and immunohistological features in childhood IgA nephropathy: a single-centre experience.

Background: IgA nephropathy is a glomerular disease diagnosed by renal biopsy and is characterized by a highly variable course ranging from a completely benign condition to rapidly progressive renal failure. We aimed to evaluate the clinical, histopathological and inflammatory characteristics of children with IgA nephropathy.

Methods: Data of 37 patients with IgA nephropathy diagnosed between the years 1980 and 2008 were retrospectively reviewed.

Effects of pentoxifylline in adriamycin-induced renal disease in rats.

Tumor necrosis factor-alpha (TNF-alpha) is a mediator of inflammation in human and animal renal disease. Pentoxiphylline (PTX) is an inhibitor of TNF-alpha. In this study we examined the effects of PTX on TNF-alpha, proteinuria, nitrite production, and apoptosis in an experimental model of Adriamycin (ADR) nephropathy in rats.