Calciphylaxis, a poorly understood clinical syndrome: three case reports and a review of the literature.

Systemic calcification syndromes are a recognized complication occurring in some patients with end-stage renal disease (ESRD) and secondary hyperparathyroidism. These patients develop severe livedo reticularis and subcutaneous tissue lesions progressing to frank necrosis and ultimately large areas of eschar. Clinically this syndrome is known as calciphylaxis; these lesions are usually resistant to aggressive debridement, systemic antibiotics, and revascularization procedures. We report three patients with somewhat different clinical presentations but all sharing a common link of exquisitely painful leg ulcers initially being treated as ischemic lesions or venous stasis-type ulcerations. These three patients were diagnosed with calciphylaxis on the basis of clinical, biochemical, and histopathological criteria. Two patients underwent parathyroidectomy late in the progression of their disease, with some resolution of their ulcerative lesions.

Portal hypertension in chronic hepatitis: relationship to morphological changes.

Various anatomical factors were examined which might provide passive resistance to portal venous flow and so cause portal hypertension. Methods included the measurement of portal pressure (WHVPG) in cirrhotic and non-cirrhotic patients, morphological assessment by semiquantitative grading of severity of disease, calculation of hepatocyte size indices, and assessment of volume density of hepatocytes, sinusoids, Disse's space and Disse's space collagen by electron microscopy. The wedged hepatic venous pressure gradient increased with progression of disease and portal hypertension was present before histologically detectable cirrhosis had developed. With increasing progression of disease towards cirrhosis, the relationship between individual and aggregated features and the WHVPG diminished and lost statistical significance. Hepatocyte size increased with progression of histological changes and correlated significantly with increase of WHVPG, both in non-alcoholic and alcoholic patients. Disse's space collagen was increased significantly in non-alcoholic chronic active hepatitis compared with patients with near-normal liver. No significant decrease of sinusoidal space was found. Multiple factors rather than any single feature influence the development of portal hypertension.

Development of an in vitro model for the disruption of extracellular matrix components in the study of congenital renal parenchymal dysmorphism.

We attempted to refine a model system for the study of renal parenchymal malformations and demonstrate its use with a preliminary study of the induction of congenital renal dysmorphism. A technique is described whereby 3-day chick embryos are explanted from their shells and cultured in vitro in specially constructed polyethylene chambers. At 8 days of development, the time of ureteral induction of the metanephric mesenchyme, 6-diazo-5-oxo-norleucine, an inhibitor of proteoglycan biosynthesis, is administered as a single microinjection into the posterolateral body wall. Histological examination of fixed metanephric tissue revealed a gross deficiency in the number of differentiated tubules in norleucine-treated animals. A morphometric analysis was made of the actual area occupied by differentiated tubules in the norleucine-treated and control kidneys between 10 and 15 days of development. This analysis revealed a total tubule surface area of control kidneys of more than 2 times greater than that of embryos injected with norleucine at ureteral induction.

A caval clamp for portacaval shunt in the rat.

An inexpensive caval clamp and stand, designed for use in the performance of portacaval shunt in the rat are described.

Fusion between immunoglobulin-secreting and nonsecreting myeloma cell lines.

The defects in two nonsecreting variant clones of the mouse plasmacytoma MOPC 21 (P3) were studied by tissue culture methods. The variants (NSI and NSIII) do not synthesize detectable heavy chains. NSI synthesizes, but does not secrete, light chains and NSIII does not synthesize light chain. A screening procedure was used allowing the detection of revertant cells secreting immunoglobulin. The method is based on a hemolytic plaque assay using anti-immunoglobulin-coated red cells. No revertants were detected among 2 x 10(7) cells. Both variant lines were fused to another myeloma line (PI) which secretes a complete immunoglobulin and excess light chains. Analysis of the products by isoelectric focusing showed that in the hybrids there was no reactivation of synthesis of the nonexpressed chains. The defects leading to loss of synthesis cannot therefore be complemented in the hybrid lines. The secretion of light chain in NSI, on the other hand, could be complemented in the hybrid but the light chain was only secreted as part of a new immunoglobulin hybrid molecule.