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Biomedical subjects

R Jaffe

Publications and source records attributed to R Jaffe.

At least 217 records · Page 12Linked to original sources
PubMed1982

Ductuloinsular tumors of the pancreas: a light, electron microscopic and immunohistochemical study.

Most pancreatic tumors are of a single cell type and are identified delta as duct, acinar, or islet cell neoplasms. The authors report on three examples with both duct and endocrine characteristics as seen by light microscopy; two with further confirmation of endocrine differentiation by electron microscopy; and one by immunocytochemistry. Mixed differentiation of this sort can be understood by reference to the embryonic pancreas, which develops from the small intestine and forms ducts, intercalated ducts, acini, and islets, with their different cell types. The merging and intermingling of different cell prevents the identification of one specific cell of origin. It also suggests that the neoplastic process here may not be a clonal proliferation from a single cell mutation as this is generally understood. Alternative explanations are briefly mentioned.

Adenoma, Islet Cell↗
PubMed1982

Familial occurrence of renal and intestinal disease associated with tissue autoantibodies.

Chronic tubulointerstitial renal disease and villous atrophy of the small intestine occurred in two first cousins. Both had protracted diarrhea with malabsorption and died despite intensive parenteral alimentation. In one patient signs of generalized proximal tubular dysfunction developed, followed by nephrotic syndrome and progressive renal insufficiency. A renal biopsy specimen disclosed severe tubulointerstitial disease and membranous glomerulopathy. In this patient, circulating immune complexes were detected and granular deposits of IgG and C3 were seen in the intestinal epithelial cells by direct immunofluorescence. Antiintestinal antibodies (IgG class) were demonstrated by indirect immunofluorescence. The other patient had interstitial nephritis but no glomerular abnormality. On direct immunofluorescence, both patients had confluent granular staining of the renal tubular basement membranes. These immunopathologic studies suggest a common immunologic mechanism in the pathogenesis of the renal and gastrointestinal disorders in these infants.

Autoantibodies↗
PubMed1982

Myocardial aneurysm in association with disseminated cytomegalovirus infection.

An infant with disseminated cytomegalovirus infection and apical aneurysm of the left ventricle died. At autopsy the coronary arteries were anatomically normal, but there was occlusion of the left anterior descending artery with an inflammatory lesion and corresponding organized thrombus. It seemed likely that cytomegalovirus infection acquired in utero may have induced an endothelial lesion, leading to thrombosis, occlusion, apical myocardial infarction, and eventual aneurysm formation.

Cytomegalovirus Infections↗
PubMed1982

Metanephric development in serum-free organ culture.

A new mouse metanephric organ culture system has been developed to study mammalian renal development. The system permits in vitro organotypic differentiation in a serum-free, hormone supplemented medium consisting of Dulbecco's minimal essential medium (MEM) and Ham's F12 medium supplemented with insulin, 5 microgram/ml; PGE1, 25 ng/ml; T3, 3.2 pg/ml; hydrocortisone, 5 microgram/ml; and transferrin, 5 microgram/ml. In this system, metanephric development continues morphologically beyond the S-shaped tubule stage. A well differentiated proximal tubule forms with a well defined brush border, specialized intercellular connections, and an apical endocytic network. In addition, a unique devascularized glomerulus, with highly differentiated podocytes surrounding areas of basement membrane, forms entirely from epithelial elements. The present organ culture model goes beyond the limitations of previously described systems in that it does not require separation of nephrogenic blastema from ureteric bud, nor require animal serum or nonspecific tissue extracts for metanephric development. The model is thus suited for morphological, biochemical, and endocrinological study of normal and abnormal renal organogenesis.

Animals↗
PubMed1982

Neonatal adrenoleukodystrophy: clinical, pathologic, and biochemical delineation of a syndrome affecting both males and females.

We describe the detailed clinical, pathologic, and biochemical features of brother and sister with the neonatal onset form of adrenoleukodystrophy, together with evidence of the biochemical defect. When compared with reports of previous cases, it becomes clear that this is a newly described clinical entity with remarkable uniformity of signs and very different from the usual childhood form. Some pathologic features are shared, including the morphologic abnormality of the adrenal in both neonatal and childhood forms, but deposition of abnormally metabolized lipids is more systemic and widespread in the neonatal form. The biochemistry of the disease is presented in both children and parents. Plasma values of long-chain fatty acid C26:0 are 0.328 +/- 0.18 micrograms/ml in a control population and 0.381 +/- 0.312 micrograms/ml in the father and mother. Values for C26:0 in the plasma of childhood adrenoleukodystrophy are 1.62 +/- 0.87 micrograms/ml and in our two cases, 2.79 micrograms/ml in the male, 1.83 micrograms/ml in the female. The basic biochemical defect appears to be a diminished capacity to oxidize these fatty acids leading to accumulation in cholesterol esters. Fatty acid oxidation to CO2 by cultured skin fibroblasts was 51% of control value for stearic acid, 5% for lignoceric acid in the male, and 39% of control value for stearic acid, 5% for lignoceric acid in the female. The genetics of this disease is different; whereas childhood adrenoleukodystrophy is X-linked, the neonatal onset form affects males and females equally and is most probably autosomally recessive in inheritance.

Adrenal Glands↗
PubMed1982

Flexible fiberoptic sigmoidoscopy.

The 35-cm flexible fiberoptic proctosigmoidoscope is a cost-effective instrument for the family physician. Nonendoscopists have mastered its use with no reported complications. Patient tolerance is high compared to tolerance for the rigid scope. The pathology yield per procedure is two to four times greater than that reported with the rigid sigmoidoscope. Yields with the 35-cm instrument have matched those documented with the 65-cm fiberoptic instrument.

Aged↗
PubMed1981

Isolation, characterization and localization of a 45 000 molecular weight, soluble glycoprotein from the lung in pulmonary alveolar proteinosis.

A carbohydrate-rich, water-soluble glycoprotein has been isolated in pure form from delipidated lung lavage fluid from a patient with pulmonary alveolar proteinosis, in a three-step procedure involving ion-exchange and gel filtration chromatography. The molecular weight of the glycoprotein was determined to be 45 900 by sedimentation equilibrium analysis in the analytical ultracentrifuge and 45 000 by polyacrylamide gel electrophoresis in the presence of sodium dodecyl sulfate, indicating a single polypeptide chain. Nearly half of the mass of the glycoprotein is comprised of carbohydrate that is contributed by 24 residues sialic acid, 23 residues N-acetylglucosamine, 6 residues N-acetylgalactosamine, 19 residues galactose, 4 residues mannose, 1 residue fucose and 1 residue glucose per mol. Unlike a number of collagen-related glycoproteins that have been isolated by others from insoluble lung contents in pulmonary proteinosis, the water-soluble glycoprotein described in the present report does not contain hydroxyproline or hydroxylysine and contains less than 10% of its amino acid residues as glycine. Using rabbit antibodies directed against our purest preparation of material and an immunoperoxidase staining procedure, the 45 000 molecular weight glycoprotein was localized to the thin film of fluid lining the surfaces of alveoli in normal human lungs.

Amino Acids↗
PubMed1981

Entactin, a novel basal lamina-associated sulfated glycoprotein.

A sulfated glycoprotein, entactin, of apparent molecular weight 158,000 has been isolated from an extracellular basement membrane-like matrix. This matrix is elaborated in cell culture by a mouse endodermal cell line. Antibodies prepared in rabbits against this sulfated glycoprotein react with mouse and rat basement membranes from a variety of tissues. These antibodies also react in a specific manner with a discrete component of mouse and rat kidney glomeruli. The electrophoretic mobility of this component is identical to that of entactin. The mouse kidney antigen, as shown by immunoelectron microscopic studies, is predominantly localized at the surface of epithelial cells of tubules and glomeruli adjacent to the basement membrane. Some antigen is also present in the basal lamina adjacent to the epithelial cells. Entactin is distinct from the basement membrane-associated protein GP-2, a protein similar to laminin. Entactin differs from GP-2 in electrophoretic mobility, cyanogen bromide peptide fragmentation pattern, immunological cross-reactivity, and incorporation of H235SO4. Entactin is insensitive to treatment with chrondroitinase ABC. It is suggested that this molecule plays a role in the interaction of the extracellular matrix and the cell surface.

Amino Acids↗
PubMed1981

Tamm-Horsfall protein in lymph nodes.

We found masses of amorphous waxy pale staining material in the peripheral sinuses of lymph nodes removed with renal tumors. The material was histochemically and immunohistochemically identical to Tamm-Horsfall protein. It was found also in tubular casts, renal tubules, and intrarenal lymphatics in the resected kidneys. Tamm-Horsfall protein was restricted to the compressed residual kidney and was not seen in the tumors. Under certain circumstances Tamm-Horsfall protein escapes from the tubules into the renal interstitium and may produce a local inflammatory reaction. It also gains access to intrarenal lymphatics and thence to regional lymph nodes. This may be relevant to the evolution of an antibody response.

Autoantibodies↗
PubMed1981

Neuroma in the region of the atrioventricular node.

A three month old infant died of apparent sudden infant death syndrome. Autopsy showed a neuroma in the region of the atrioventricular node, unassociated with neurofibromatosis. The atrioventricular node and bundle of His were normal. It was not possible to ascribe the sudden death to the neuroma.

Atrioventricular Node↗
PubMed1981

Immunolocalization of entactin, a sulfated basement membrane component, in rodent tissues, and comparison with GP-2 (laminin).

Entactin is a sulfated glycoprotein in the extracellular basement membrane like matrix produced by M1536-B3 cells, a mouse endodermal line derived from an embryonal carcinoma. It has a molecular weight of 158,000 and is chemically and immunologically distinguishable from GP-2 (laminin) and fibronectin. Antibodies produced against entactin and GP-2 react with subepithelial and vascular basement membranes in rat lung, liver, spleen, and kidney and mouse placenta and kidney when examined by light microscopy. Both antibodies yield staining around the marginal sinus of the white pulp of the spleen. Antientactin reacts with basement membrane and mesangium in rat glomeruli, and anti-GP2 does not. Ultrastructurally, staining in kidneys is strongest at epithelial or endothelial cell membranes bordering basement membranes, with only moderate staining of the basement membrane proper. Intracellular staining is not present. The location of entactin suggests that it has a role in the interaction of cells with extracellular matrix, possibly in adhesion. Lack of intracellular staining suggests that the tissues studied are not actively producing entactin or GP-2 and that these substances may be fairly stable in adult organisms.

Basement Membrane↗
PubMed1980

Immunoglobulin production in lymphomatoid granulomatosis and relation to other "benign" lymphoproliferative disorders.

Immunoperoxidase technics were used to examine the immunoglobulin content of sections of pulmonary tissue from two typical cases of lymphomatoid granulomatosis and two cases of pneumonic processes initially diagnosed as lymphomatoid granulomatosis but representing different processes on review. Both "typical" cases and one of the others had a predominantly mixed pattern of all immunoglobulins. One "typical" case showed a focus of exclusively IgG/K staining, which corresponded to histologic malignancy. Less than 1% of cells stained in the fourth case. These results demonstrate that several different processes may fit the morphologic criteria of lymphomatoid granulomatosis; that there is a group of cases that typify lymphomatoid granulomatosis clinically and histologically, and that these cases represent a B-cell proliferation that is initially polyclonal but may evolve into immunoblastic sarcoma. "Typical" cases are similar to other lymphoreticular proliferations with malignant potential, such as angioimmunoblastic lymphadenopathy and Sjögren's syndrome.

B-Lymphocytes↗
PubMed1980

Pancreatic islet cell damage. Its occurrence in neonatal coxsackievirus encephalomyocarditis.

Pancreata from five infants with culture-proven coxsackievirus encephalomyocarditis were studied for evidence of islet cell damage. Four of the five showed islet cell change, varying from clusters of cells with pyknotic nuclei to total islet necrosis. The lesion appeared to be characteristic of coxsackievirus and was not seen in the pancreata of neonates with other neonatal systemic viral infections. This confirms that coxsackievirus shows tropism for insular tissue and may play a role in the genesis of some cases of juvenile diabetes mellitus. Immunostaining was used to ascertain the specificity of the lesions. Damage to cells other than beta cells could be clearly demonstrated. The finding that all islet cell types may be involved lends support to the theory that juvenile diabetes mellitus may be a genetically determined failure to reconstitute the beta cells after viral injury.

Coxsackievirus Infections↗
PubMed1980

Pancreatic pathology in hyperinsulinemic hypoglycemia of infancy.

Pancreas from 10 children with idiopathic hyperinsulinemic hypoglycemia was examined using histochemical and immunostaining techniques. The children ranged from newborn to 9 months in age. Sections were studied with particular reference to islet cell distribution in patients and controls, and quantitative assessments were made of islet size, relative cell-type distribution, and total area of pancreas occupied by endocrine tissue. Four had islet cell adenomatosis, three of these focal and one generalized. The others had a subtle morphologic abnormality seen best on immunostained sections and characterized by loss of the usual centrilobular congregation, irregular islet contours, a generalized of small packets of endocrine cells throughout the acinar tissue, and islet cell hypertrophy. We have termed this constellation "endocrine cell dysplasia." The range of islet cell area found in the controls using immunostaining was substantially higher than previously reported. In addition, we found no increase in mean total endocrine area in the cases with endocrine dysplasia when compared to age-matched controls. Both classic and beta-cell nesidioblastosis were common to patients and controls alike, appeared to decrease with age, and thus could not be considered as the morphologic substrate of hyperinsulinism in this age group.

Female↗