Hypocomplementemic autosomal recessive hemolytic uremic syndrome with decreased factor H

We describe the clinical course, complement components, and pathological findings of 10 infants with autosomal recessive hemolytic uremic syndrome (HUS). All patients were members of one extended highly inbred Bedouin kindred. The median age of presentation was 2 weeks (range 1-20 weeks). Eight patients died, 2 patients are alive, on dialysis. Renal biopsies revealed thrombotic microangiopathy with a predominant early arteriolar involvement and subsequent development of ischemic glomerular changes. Immunofluorescence was positive for C3 in glomeruli. All patients had low complement components levels during and between relapses, and in some this was evident soon after birth and prior to the onset of symptoms. This deficiency could not be normalized by repeated plasma transfusions. Biosynthetic labelling of patients' fibroblasts demonstrated normal rates of C3 protein synthesis. Serum factor H levels were greatly decreased or absent in 4 patients tested and moderately decreased in 15 of 23 healthy unaffected siblings and patients. This defect may cause complement activation and consumption, possibly at the endothelial cell level.     

Target oriented network intelligence collection: effective exploration of social networks

World Wide Web - Target Oriented Network Intelligence Collection (TONIC) is a crawling process whose goal is to find social network profiles that contain information about a given target. Such...     

A comparison of distinct modes of tumor cell death in Hodgkin's disease using morphology and in situ DNA fragmentation

The study examined the morphology and frequency of cell death occurring spontaneously in lymph nodes from patients with Hodgkin's disease. In addition to necrosis, which was infrequent and usually in patches, we document two cell types showing features of individual cell death: mummy cells end apoptotic cells. Mummy cells present no evidence of DNA fragmentation, but show electron microscopic features of "dark cells." Apoptotic Hodgkin-Reed-Sternberg cells are found frequently and are easier to demonstrate by in situ and labeling of fragmented DNA than by light microscopy only. In many cases phagocytosis of apoptotic cells is also documented. The significance of these findings to the limited number of Hodgkin-Reed-Sternberg cells in most cases of Hodgkin's disease is discussed.     

Crystallization of hydrogenated amorphous silicon films by exposure to femtosecond pulsed laser radiation

To crystallize hydrogenated amorphous silicon films on glass substrates, pulsed Ti-sapphire laser radiation is used, with a pulse duration less than 30 fs. The initial films are grown by plasma-enhanced chemical-vapor deposition at the temperatures 200 and 250°C. The structural properties of the initial films and films treated with laser radiation pulses are studied by Raman spectroscopy. The conditions for complete crystallization of the films grown on glass substrates to thicknesses of up to 100 nm and hydrogen content of up to 20 at % are established. The conditions provide the fabrication of highly homogeneous films by scanning laser treatments. It is found that, if the hydrogen content in the film is 30–40 at %, the crystallization is an inhomogeneous process and laser ablation is observed in some areas of the films.