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Biomedical subjects

E Grishman

Publications and source records attributed to E Grishman.

At least 19 recordsLinked to original sources

Eosinophilic temporal and systemic arteritis.

We describe a 39-year-old patient with an unusual type of bilateral temporal arteritis characterized histologically by inflammation, diffuse eosinophilic infiltration, destruction of elastic tissue, and fibrosis. In addition, the patient had a history of systemic vasculitis, peripheral eosinophilia, eosinophilic lymphadenitis, and membranous glomerulonephritis. The patient has been followed up for 14 years and is well controlled on moderate doses of steroids. We propose that this patient suffers from an immune reaction to an unknown, possibly infectious, antigen.

Adult↗

Disseminated giant cell arteritis.

A 76-yr-old woman with widespread giant cell arteritis and polymyalgia rheumatica is described. The patient had an unusual simultaneous involvement of the cranial (temporal) arteries demonstrated by biopsy, and of large elastic arteries (aorta and its major branches), medium-sized and small muscular arteries, arterioles and vasa vasorum found at autopsy. Coronary arteritis was responsible for the fatal myocardial infarction.

Aged↗

Renal failure due to tubular obstruction by large protein casts in patients with massive proteinuria.

We describe two patients with nephrotic syndrome whose histologic findings on renal biopsy and at autopsy are characterized by numerous large protein casts in dilated cortical tubules, as well as podocyte swelling with effacement of foot processes in the glomeruli. Both patients progressed rapidly to renal failure. The casts and the protein precipitate in the Bowman's capsule were found to be composed of varying proportions of albumin and globulin but contained no Tamm-Horsfall protein. This suggests a glomerular rather than tubular origin of proteins. Light and electron microscopic examination of tubules revealed changes mainly due to compression by the casts. We propose that tubular obstruction due to the large casts was the main cause of renal failure.

Acute Kidney Injury↗

Intravenous pulse cyclophosphamide in the treatment of type IV lupus nephritis.

A retrospective study compared two groups with type IV lupus nephritis with very similar activity and chronicity indices on renal biopsy. One group was treated with intermittent pulse cyclophosphamide (IPC), and the other was not. The IPC group demonstrated a greater decrease in serum creatinine at 6 months, 12 months and most recent follow-up intervals (p < 0.01, p < 0.05, p < 0.001). Reduction in proteinuria was similar in the two groups. Two of eight in the no-IPC group progressed to ESRD while only one of twelve in the IPC group developed ESRD. The findings suggest that IPC preserves renal function in Type IV nephritis. Furthermore it is suggested that a lower cumulative dosage than previously reported may reduce toxicity without a significant loss of efficacy.

Creatinine↗

Vascular lesions in lupus nephritis.

Three groups of kidney specimens from patients with systemic lupus erythematosus (SLE) were examined for histologic evidence of vascular lesions in small arteries and arterioles. Group 1 consisted of 24 autopsy kidneys from patients who died before the advent of steroid therapy, and Group 2, of 26 more recent autopsy specimens from patients treated with steroids and/or immunosuppressive drugs. Group 3 comprised 276 renal biopsies. Group 1 showed characteristic subendothelial eosinophilic deposits in small arteries and arterioles of 8 cases; Group 2 showed similar lesions in 5 specimens, while 3 others revealed evidence of resorption of deposits. Deposits were characterized by clumping and were delimited toward the media by a thick basement membrane. Only one case showed necrotizing arteritis resembling polyarteritis nodosa. Group 3 presented vascular deposits in 19 cases and thrombotic microangiopathy in 2. Electron microscopic appearance of some of the deposits is described. Immunofluorescence microscopy showed a mixture of IgG, IgA, and IgM in 7 cases, a finding that was not seen in a group of non-lupus patients with various vascular lesions. Vascular deposits are generally rare in systemic lupus erythematosus, although in autopsies widely scattered involvement of arteries and arterioles was seen in nearly 1/3 of the cases. The deposits were more common in male patients. The evolution of the lesions could be followed through various stages to eventual sclerosis, particularly in patients treated with steroids or immunosuppressants. Some deposits appeared to resolve after treatment. Patients with vascular deposits had more severe glomerular disease and a more serious clinical course. Thrombotic microangiopathy appears to be a secondary phenomenon whose pathogenesis is unknown.

Adolescent↗

Colonic obstruction in a child with von Recklinghausen's neurofibromatosis.

A child is reported who developed partial colonic obstruction from an intussuscepting polypoid mass in the transverse colon. A 5 cm plexiform ganglioneurofibroma was found at laparotomy. Examination of the entire gastrointestinal tract revealed no other neurogenic tumors. Gastrointestinal involvement by neurofibromatosis in children is rare; however, children with von Recklinghausen's disease who complain of vague abdominal symptoms should be evaluated for gastrointestinal tumors.

Child↗

Progressive systemic sclerosis with the nephrotic syndrome and acquired factor X deficiency.

A patient with progressive systemic sclerosis (PSS) involving skin, digit, esophagus, and lung developed the nephrotic syndrome and rapidly progressive renal insufficiency. Renal pathology revealed capillary collapse, cellular crescents, arteriolar hyaline deposition, and mesangial proliferation. On immunofluorescence IgM, C3, and fibrinogen were present in mesangium and capillary walls. Prebiopsy coagulation screening revealed a factor X deficiency which caused substantial prolongation of the partial thromboplastin time without an overt bleeding diathesis. The acquired factor X deficiency resolved after fresh frozen plasma and vitamin K administration, although some spontaneous improvement was noted. Nephrotic syndrome may occasionally be seen in the acute fulminant form of PSS and should not deter diagnosis of PSS.

Capillaries↗

Blind pouch syndrome: a case report.

The blind pouch syndrome is associated with a spectrum of diseases affecting the gastrointestinal tract that includes: malabsorption, ulceration, bleeding, and perforation. The clinical signs and symptoms of anemia, weight loss, abdominal pain, vomiting, and intermittent intestinal obstruction can be found. Occasionally, constipation or more often diarrhea is an important manifestation. A case report of this entity with related radiological and pathological findings secondary to a side-to-side anastomosis is presented and discussed.

Adolescent↗

Hodgkin's lymphoma in an adolescent with systemic lupus erythematosus.

An adolescent female with systemic lupus erythematosus (SLE) with diffuse proliferative lupus nephritis subsequently contracted Hodgkin's lymphoma, mixed cellularity type. Prior to the diagnosis of Hodgkin's lymphoma, hypogammaglobulinemia developed with IgG deficiency. The nephritis, which improved on steroid therapy, remitted entirely after nitrogen mustard, vincristine, procarbazine, and prednisone (MOPP) therapy. The hypogammaglobulinemia also remitted after chemotherapy for Hodgkin's lymphoma. The literature concerning the association of SLE and Hodgkin's lymphoma is reviewed. The significance of this concurrence is discussed in regard to the possible pathophysiology and the clinical management.

Adolescent↗

Nephronophthisis with massive proteinuria.

A 23-year-old male whose uncle died of nephronophthisis, and whose pathology is also discussed, presented with 5 g of protein in a 24-hour urine collection. Nephrogenic diabetes insipidus and salt wasting were present in addition to azotemia. Characterization of the proteinuria, including elevated alpha globulins by electrophoresis and markedly elevated urinary beta-microglobulins by radioimmunoassay (49.55 mg/L) indicated predominantly tubular proteinuria. A percutaneous renal biopsy showed normal glomeruli, interstitial inflammation and fibrosis, and tubular atrophy. Electron microscopy revealed notable alterations of the tubular basement membrane.

Adult↗

Cell coat of podocytes in patients with nephrotic syndrome.

Renal biopsies from 23 patients with the nephrotic syndrome and five patients with slight or no proteinuria were examined for the presence of cell coat of podocytes by light and electron microscopy. Of those with nephrotic syndrome, five had minimal change disease, nine focal glomerular sclerosis, six membraneous nephropathy and three amyloidosis. Colloidal iron and phosphotungstic acid stains were used for the demonstration of anionic and neutral polysaccharide components of the cell coat. On light microscopy, the colloidal iron reaction showed a reduction in intensity of the stain in glomeruli of patients with massive proteinuria, as compared to those with slight or no proteinuria. On electron microscopy, only the cell coat lining the surface of the foot processes disappeared parallel to the loss of these structures, while the coat covering the surface facing the urinary space remained unchanged with both stains.

Cell Membrane↗

Patterns of renal injury in systemic lupus erythematosus: light and immunofluorescence microscopic observations.

Light and immunofluorescent microscopic patterns of lupus nephritis in 203 biopsies, 1 nephrectomy, and 20 autopsies from 179 patients were analyzed. The latest World Health Organization (WHO) classification was used. Seventy patients had diffuse lupus nephritis, 43 mesangial, 19 membranous, 19 focal, and 16 minimal change; 2 patients had advanced sclerosing nephritis. Nine patients were difficult to classify by light microscopy, but 3 of these could be classified with the help of immunofluorescence microscopy. Strict definition, especially of category III (focal and segmental lupus nephritis) is important, since this lesion has a tendency to heal, and patients with few immune deposits outside the segmental lesions have a rather good prognosis. Category V (membranous lupus nephritis) should probably be limited to membranous lesions with pure subepithelial deposits or with subepithelial and mesangial deposits, while membranous changes associated with diffuse or focal proliferative lesions are better classified as Category IV (diffuse lupus nephritis). It was observed that steroid treatment reduces the amount of deposits, especially those in the subendothelial and mesangial locations. The amount of proliferation is also reduced, but in a considerable proportion of cases, it is replaced by sclerosis. Therefore, interpretation of biopsy patterns must take prior therapy into consideration. Immunofluorescence findings in the glomeruli correlated quite well with light microscopic patterns. Active interstitial inflammation, which is most common in diffuse lupus nephritis, was only observed in the presence of tubulointerstitial immune deposits. Acute arteritis was much more common in autopsy than in biopsy specimens pointing to its ominous nature. It was concluded that combined examination of biopsies by light and immunofluorescence microscopy as well as electron microscopy, and strict categorization of lesions are valuable diagnostic and prognostic aids. Their usefulness is considerably enhanced if certain clinical data, such as prior therapy are taken into consideration.

Antigen-Antibody Complex↗

Focal segmental lupus nephritis.

Nineteen renal biopsies and one autopsy from fifteen patients with focal segmental lupus nephritis were examined by light and electron microscopy, and seven biopsies also by immunofluorescence microscopy. Histopathologic data were correlated with clinical course. Patients were selected on the basis of strict histologic criteria, and had to have a minimum of 2 years clinical follow-up after biopsy. All patients were treated with steroids. It could be demonstrated by light and electron microscopy that segmental lesions go through several stages of evolution, starting with mesangial proliferation, followed by necrosis and terminating as a nodule composed mainly of collagen. The necrotic lobule sometimes contains electron dense deposits, but no wire loop lesions. Prognosis was generally good with an average of 6.3 year survival of 86%. Factors contributing to outcome include number of involved glomeruli, amount of mesangial deposits outside the segmental lesions, and the presence of vasculitis. The pathogenesis of segmental lesions probably differs from that of diffuse lupus nephritis, constituting a localized reaction, independent of immune deposits in the remainder of the glomerulus, and with a considerable tendency to heal, particularly when treated with steroids.

Adolescent↗

Light- and electron-microscopic histochemistry of Fabry's disease.

A histochemical study was performed on light- and electron-microscopic level in a case of Fabry's disease. The patient underwent kidney transplantation for renal failure and died of heart failure 6 months later. Patient's tissues were studied at the light- and electron-microscopic levels with various embedding and staining techniques for lipids and carbohydrates. Two peroxidase-labeled lectins (from Ricinus communis and from Bandeiraea simplicifolia) known to have affinity for alpha- and beta-D-galactose, were strongly reactive with the storage material on frozen sections. The ultrahistochemical and extraction tests showed that the typical granules had a variable reactivity and morphologic characteristics in different cells, probably reflecting different composition. A small number of typical deposits were also observed in the transplanted kidney. This is the first reported case of recurrence of the storage disease in the allograft. Of interest was also the fact that the patient's blood inhibited normal alpha-galactosidase activity, suggesting a possible inhibitor-related mechanism in the pathogenesis of the recurrence.

Adult↗