Renal disease in nail-patella syndrome: Clinical and morphologic studies

Renal disease in nail-patella syndrome: Clinical and morphological studies. Clinical and morphological features of seven patients with the nail-patella syndrome are described. Progression to renal failure after a prolonged period of asymptomatic proteinuria is reported. Kidney tissue from these seven patients studied by light, immunofluorescent and electron microscopy demonstrated abnormalities characteristic of this disease. Focal glomerular basement membrane thickening was observed by light microscopy. Immunofluorescent microscopy showed focal glomerular basement membrane and arteriolar staining with serum proteins, predominantly IgM and β1C. Electron microscopy revealed markedly abnormal glomerular basement membranes containing bundles of cross-striated fibrils. These fibrils were more readily demonstrated in phosphotungstic acid-stained sections. The data presented suggest that the inborn error of connective tissue metabolism of the nail-patella syndrome is associated with renal disease as the result of deposition of collagen moieties in glomerular basement membranes with subsequent alterations of glomerular structure and function

Immunologic aspects of the nephrotic syndrome

The nephrotic syndrome is a clinical entity characterized by proteinuria, hypoalbuminemia, edema and hyperlipidemia. All the features of this syndrome are ultimately related to increased permeability of the glomerular capillary to protein. A specific disease entity in its mildest form may result in mild proteinuria insufficient to cause hypoalbuminemia and the other physiological manifestations of the nephrotic syndrome; the same disease in another patient or at another time in the same patient may cause marked proteinuria and the nephrotic state. The principal difference between proteinuria alone and that associated with the nephrotic syndrome in any specific disease would therefore appear to be quantitative, although it is likely that other factors play a role

A review of varietal change in roots, tubers and bananas: consumer preferences and other drivers of adoption and implications for breeding

This review of the literature on varietal change in sub-Saharan Africa looks in detail at adoption of new varieties of bananas in Uganda, cassava in Nigeria, potato in Kenya, sweetpotato in Uganda and yams in C^ote d'Ivoire. The review explored three hypotheses about drivers of varietal change. There was a strong confirmation for the hypothesis that insufficient priority given to consumer-preferred traits by breeding programmes contributes to the limited uptake of modern varieties (MVs) and low varietal turnover. Lack of evidence meant the second hypothesis of insufficient attention to understanding and responding to gender differences in consumer preferences for quality and post-harvest traits was unresolved. The evidence on the third hypothesis about the informal seed system contributing to slow uptake of MVs was mixed. In some cases, the informal system has contributed to rapid uptake of MVs, but often it appears to be a barrier with inconsistent varietal naming a major challenge

Empirical Legal Studies Before 1940: A Bibliographic Essay

The modern empirical legal studies movement has well-known antecedents in the law and society and law and economics traditions of the latter half of the 20th century. Less well known is the body of empirical research on legal phenomena from the period prior to World War II. This paper is an extensive bibliographic essay that surveys the English language empirical legal research from approximately 1940 and earlier. The essay is arranged around the themes in the research: criminal justice, civil justice (general studies of civil litigation, auto accident litigation and compensation, divorce, small claims, jurisdiction and procedure, civil juries), debt and bankruptcy, banking, appellate courts, legal needs, legal profession (including legal education), and judicial staffing and selection. Accompanying the essay is an extensive bibliography of research articles, books, and reports

The glomerular mesangium

The one million or so glomeruli present in each of our kidneys are remarkably constructed. It is here that the ultrafiltrate of plasma is formed to be economically reabsorbed by tubules, leaving behind substances of no further use to the body. It is also here that the movement of macromolecules across the peripheral glomerular capillary is impeded by the basement membrane and by fixed polyanions in the capillary wall. Also within the glomerulus is an anatomically distinct region—the mesangium. The traffic of macromolecules through this zone appears to be significantly influenced by changes in the peripheral glomerular capillary as well as by other experimental manipulations. In human glomerulonephritis, immune deposits are found here, often exclusively. Morphologic abnormalities have ranged from the mesangial matrix expansion and the characteristic intercapillary nodule formation of diabetic nephropathy to the prominent proliferation of membranoproliferative glomerulonephritis and the segmental changes seen in a host of different diseases