Jacob Churg

Treatment of Idiopathic Membranous Nephropathy

In a retrospective study of the effect of treatment in biopsy-proved idiopathic membranous nephropathy, 91 adults and 12 children were followed for periods up to 29 years after clinical onset (mean, 6.5 years). Forty-four were treated with corticosteroids, 15 with corticosteroids and immunosuppressants; 44 had no treatment and served as a control group. Clinical cure and improvement were significantly greater in the treated than in the nontreated group (P less than 0.01). The recurrence rate, occurrence of renal failure and probability of death were significantly greater in the nontreated group, although some of these patients eventually showed improvement. Prognosis was better in patients who responded to therapy. These data strongly suggest that steroid therapy is beneficial in patients with membranous nephropathy.

Resolution of renal amyloidosis

A patient with renal amyloidosis and the nephrotic syndrome consequent to extensive infected burns demonstrated both clinical resolution of the nephrotic syndrome and morphologic regression of the renal amyloid deposits over a six year period. The regression of the amyloid deposits was associated with several changes in the glomerular capillary wall resulting in a double capillary wall contour. This case indicates that deposits of amyloid in the kidney may regress and suggests a sequence of events in this resolution.

Ultrastructure of Immune Deposits in Renal Glomeruli

Abstract Immunofluorescence microscopy and electron microscopy are mutually complementary in the characterization of immune deposits in renal glomeruli. Deposits differ in [1] composition: various ...

Intraglomerular fibrin, platelet aggregation, and subendothelial deposits in lipoid nephrosis

We have investigated the formation of fibrin, platelet aggregates, and subendothelial deposits in lipoid nephrosis. Fibrin formation was found in 10 cases of active lipoid nephrosis. Platelet aggregates were found in eight cases and subendothelial deposits in nine. Fibrin and platelets were also found in cases of nephrotic syndrome due to other causes, and in glomerulonephritis. Fibrin was generally absent in lipoid nephrosis in remission and in benign recurrent hematuria. It is suggested that what seems to be a lower incidence in females is more apparent than real and that fibrin or related material may be present in a less easily identifiable form. Steroid therapy apparently had no effect on the presence or absence of fibrin. Most instances were associated with elevated serum cholesterol and alpha(2)-globulin. It is suggested that elevated serum lipids as well as the disease process in the kidney play a role in this phenomenon. It is further suggested that intraglomerular fibrin formation could lead to irreversible renal damage in lipoid nephrosis.

Morphologic and clinical correlates in renal amyloidosis

Morphologic studies and clinical correlations were undertaken in 59 patients with renal amyloidosis. Spicularly arranged amyloid deposits in the glomerular capillary wall were found in all clinical groups but were more frequent and more extensive in primary amyloidosis and multiple myeloma. The severity of proteinuria correlated with the presence of spicules and podocyte destruction rather than with the amount of amyloid in the glomerulus. The spicules were associated with morphologic and clinical evidence of rapid amyloid deposition and a fulminant clinical course. The absence of spicules and the presence of extensive new basement membrane material may produce basement membrane thickening, lamination, and double capillary wall contours, which are associated with mild proteinuria and, rarely, resolution of amyloidosis. Nodular or mixed nodular-diffuse patterns of glomerular amyloid deposits were more frequent in patients with secondary amyloidosis and a longer clinical course. Renal failure generally corresponded to severe glomerular amyloidosis and tubular atrophy. However, a relatively precipitous, usually irreversible decrease in renal function frequently occurred in patients with renal amyloidosis and did not always have a morphologic explanation. The duration of life from the time of biopsy no death in patients with primary amyloidosis (nine months) was markedly shorter than in those with secondary amyloidosis (more than 50 months).

Paraproteinemia and cryoglobulinemia associated with atypical glomerulonephritis and the nephrotic syndrome

Abstract A patient with an atypical from of glomerulonephritis and the nephrotic syndrome, whose serum contained a paraprotein and mixed cryoglobulin and had rheumatoid factor activity, is described. The nephritis was characterized by the presence of numerous intracellular crystals, wire loops and hyaline thrombi in the glomeruli. It appears reasonable to assume that the crystals and other deposits represent precipitated protein, probably related to the paraprotein and cryoglobulin in the serum. It is suggested that the crystals are derived from the paraprotein and that the mixed cryoglobulins (possibly antigen-antibody complexes) give rise to the deposits seen along the basement membrane.

Hereditary nephritis with a characteristic renal lesion.

Abstract Light and electron microscopic studies were performed on renal tissue from four involved members of a family with hereditary nephritis. The two females studied had no apparent deafness or renal insufficiency, but a nonspecific mild increase in mesangial matrix was noted on light microscopic examination of the renal biopsy specimen. The two males had neurosensory deafness. One had advanced renal insufficiency with glomerular sclerosis, whereas the other had no azotemia and less histopathologic change. However, all four had a characteristic ultrastructural lesion consisting of glomerular basement membrane splitting separated by clear spaces with the accumulation of small dense particles between the thin layers. This change may be specific for certain forms of hereditary nephritis.

Renal vein thrombosis and the nephrotic syndrome: Report of two cases with successful treatment of one

Abstract A nephrotic syndrome and bilateral renal vein thrombosis developed in two women. One suffered low back pain, the other oliguric renal failure. Both suffered pulmonary emboli and had exhibited edema, proteinuria and hypertension during pregnancies several years before. Results of thrombectomy and anticoagulant therapy were successful in one patient, although she suffered serious hemorrhage at the biopsy site during anticoagulant therapy. The other patient died of pulmonary embolism. Renal interstitial changes usually associated with renal vein thrombosis were found in both, but neither showed membranous nephropathy (membranous glomerulonephritis). This study suggests that (1) although membranous nephropathy is frequently associated with renal vein thrombosis, it is probably not the result of that condition nor invariably associated with it. (2) Prognosis in renal vein thrombosis with nephrotic syndrome may be partially dependent upon underlying renal disease. (3) Renal biopsy is useful for diagnosis and assaying prognosis. (4) Treatment should consist of thrombectomy and anticoagulants. However, serious hemorrhage may occur in patients recently subjected to biopsy.

Electron microscopy of a feminizing leydig cell tumor of the testis

The ultrastructural characteristics of a feminizing interstitial (Leydig) cell tumor of the testis were compared with those of normal Leydig cells and with the findings described in 10 published cases of Leydig cell tumor. The neoplastic Leydig cells superficially resembled normal Leydig cells. Similarities included abundant smooth endoplasmic reticulum, lipid, and microbodies. Contrastingly, Reinke crystalloids and paracrystalline inclusions were absent and lipochrome pigment and lysosomes very rare. The nuclei were large and contained enlarged, often multiple, nucleoli. The nuclear membranes tended to be irregular and undulating. Cytoplasmic membranous whorls and myelin figures were conspicuous. Fairly homogeneous fibrous septa were evident between single and grouped tumor cells. Despite several individual variations, there is a general resemblance between the neoplastic Leydig cells in this patient and those previously reported. No distinguishing ultrastructural characteristics were discerned between feminizing and virilizing Leydig cell tumors.

Acute Toxicity of Sodium Salts of Sulfapyridine, Sulfathiazole and Sulfamethylthiazole.∗:

Conclusions It has been shown that the acute toxicity of sodium sulfamethylthiazole in rats, as measured by intraperitoneal injection, is almost twice as high as that of sodium sulfathiazole, which in turn is slightly greater than the toxicity of sodium sulfapyridine. The pH and buffer capacities of solutions of the sodium salts, and differences in solubility of the free compounds as factors influencing the toxicity, are discussed.