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Biomedical subjects

J D Elema

Publications and source records attributed to J D Elema.

At least 73 records · Page 4Linked to original sources

Simultaneous occurrence of pulmonary interstitial fibrosis and alveolar cell carcinoma in one family.

The coexistence of interstitial pulmonary fibrosis and alveolar cell carcinoma is well known. The familial occurrence of a combination of these two entities, however, is very rare. We present a family of which five members had diffuse interstitial pulmonary fibrosis. Three of them had in addition alveolar cell carcinoma. In a sixth family member, evidence of alveolar cell carcinoma was present without proven interstitial fibrosis. An autosomal dominant trait is suggested as the mode of inheritance of both interstitial fibrosis and alveolar cell carcinoma in this family.

Adenocarcinoma, Bronchiolo-Alveolar↗

Glomerular mesangium. Analysis of the increased activity observed in experimental acute aminonucleoside nephrosis in the rat.

Kinetic studies have revealed increased mesangial macromolecular uptake in acute aminonucleoside of puromycin (PAN) nephrosis in the rat. The mechanisms leading to this increased activity, however, are poorly understood. Therefore, we studied mesangial function and ultrastructure in rats 8 days after intravenous injection of 6 mg. of PAN per 100 gm. of body weight. One hour after intravenous injection of 20 mg. of colloidal carbon per 100 gm., no differences in mesangial carbon could be detected between PAN rats and controls. After 24 hours, the amount of mesangial carbon was significantly higher in PAN rats; rates of disappearance did not differ. Ultrastructural examination revealed no differences in localization of mesangial carbon at the 1-hour interval. After 24 hours, mesangial cells of PAN rats showed an increased number of lysosomes filled with carbon; the number of carbon particles in the mesangial matrix was not increased. Using an ultrastructural immunoperoxidase technique, we found increased amounts of endogenous IgG in the extracellular space of segmental mesangial lobules of PAN rats. IgG was mainly present in matrix substance of electron-lucent aspect, and quantitative morphometric analysis revealed an increase in volume of this loose permeable matrix component (mesangial channels). No increased staining of IgG was found in the lacis area indicating that there was no increased mesangial egress of macromolecules in PAN nephrosis. The increased volume of the permeable mesangial channels in segmental mesangial lobules of PAN rats may lead to a pooling of tracer material with consequent increased phagocytosis by mesangial cells.

Acute Disease↗

The localization of Hodgkin's disease in lymph nodes. A study with immunohistological, enzyme histochemical and rosetting techniques on frozen sections.

Lymphoid tissue of 51 patients with Hodgkin's disease was studied with immunohistological, enzyme histochemical and rosetting techniques for the detection of B and T cells in frozen sections. In lymph nodes of patients with lymphocyte predominance type of Hodgkin's disease, the majority of the lymphocytes in the involved areas were normal B lymphocytes of polyclonal origin. This was also true for nodular sclerosis cases with a predominance of lymphocytes. Surrounding Sternberg-Reed cells small clusters of T lymphocytes could be demonstrated. In mixed cellularity and also in nodular sclerosis with a mixed cellular pattern only small residual areas of B lymphocytes were present, whereas relatively large numbers of T lymphocytes were found in the involved areas. In lymphocyte depletion B lymphocytes were scarce and T lymphocytes were present in small number. It is concluded that different patterns of lymphocyte population can be discerned in the subtypes of Hodgkin's disease. A predominance of B lymphocytes is found in cases with lymphocyte predominance and thus is a prognostic favourable sign. A predominance of T lymphocytes as found in cases with a mixed cellular pattern with or without nodular sclerosis is therefore not a favourable sign in general but may indicate progressive disease. The possible reasons for the presence of large numbers of B or T lymphocytes in lymphoid tissue affected by Hodgkin's disease are discussed.

B-Lymphocytes↗

Intracranial malignant lymphomas. A morphologic and immunocytologic study of twenty cases.

Fifteen primary intracranial reticulum cell sarcomas and five cases with an additional solitary extracranial tumor mass have been studied. For comparison, seven extracranial malignant non-Hodgkin lymphomas and normal lymphoid tissue were included. The methods used on formalin-fixed paraffin-embedded tissue sections were an immunoperoxidase technique for the demonstration of intracellular immunoglobulins, microglial staining, Gomori's reticulin, methylgreen-pyronin, Giemsa, diastase resistant PAS, Mallory's PTAH and H&E. Electron microscopy was performed in one primary brain tumor. According to histopathologic criteria all tumors could be classified as malignant non-Hodgkin lymphomas, predominantly of the pleomorphic immunocytic or of the immunoblastic type; follicular lymphomas were notably absent. In all cases intracellular immunoglobulins were demonstrable in tumor cells and in a majority of the tumors these were monoclonal. Thus, all malignant lymphomas proved to be of B cell origin with demonstrable cytoplasmic immunoglobulin production. Based on the microglial staining more than half of the malignant lymphomas could also be classified as microgliomas. As a comparable staining was present in non-Hodgkin lymphomas outside the CNS, microglioma characteristics are not associated with intracranial growth.

Adult↗

Infantile histiocytosis X.

Skin biopsies and a lymphnode of three children with infantile Histiocytosis-X (Letter-Siwe Disease) were studied with enzymehistochemical and sheep-erythrocyte rosetting techniques. The majority of cells making up the infiltrates of skin and lymphnode showed rather weak acid phosphatase and nonspecific esterase activity but considerable leucyl-beta-naphtylamidase activity. Sheep-erythrocyte rosetting techniques performed on frozen sections indicated the presence of receptors for the Fc fragment of IgG, but no receptors for C3 could be demonstrated. Cells with the same enzymehistochemical characteristics could be found in thymus-dependent areas of normal spleen, of normal and reactive lymphnodes and in thymic medulla but not in B-cell areas or thymic cortex. It is suggested that Histiocytosis-X cells belong to the Mononuclear Phagocyte System and that they are related to or identical with cells normally present in the thymus dependent areas of the lymphoid tissue involved with the functioning of cell-mediated immunity.

Acid Phosphatase↗

The significance of intracytoplasmic proteins in Reed-Sternberg cells.

Lymphoid tissue of 42 patients with Hodgkin's disease was studied with immunohistological techniques on the light microscopic and ultrastructural level. The presence of IgG in some Reed-Sternberg (R-S) cells was confirmed, and in addition serial sections and a double staining technique revealed that these cells also contained both kappa and lambda light chains. Furthermore two serum proteins, human serum albumin and alpha-1-antitrypsin, were demonstrated in the same positive R-S cells. The ultrastructural localization of the immunoglobulin and of human serum albumin was not related to any protein synthesizing organelle or to structures related to endocytosis. It is suggested that the presence of immunoglobulin in R-S cells is the result of a disturbance of the cell wall integrity with subsequent nonspecific diffusion of immunoglobulin and other serum proteins into the cell. The presence of IgG therefore can not be taken as an argument for a B-cell origin of R-S cells. Possible mechanisms for the cell wall damage are discussed.

Adolescent↗

Familial pulmonary veno-occlusive disease: a case report.

A case of pulmonary veno-occlusive disease in a male infant who died at the age of 3 months is presented. Both intra- and extrapulmonary veins were involved. Two years earlier a brother had died of the same disease at the age of 8 weeks, but, in that case, the disease was restricted to the intrapulmonary veins. It is suggested that the disease may have been caused by a viral infection, the mother acting as a carrier. The simultaneous occurrence of intra- and extraparenchymal pulmonary vein occlusion indicates that some instances of isolated extraparenchymal pulmonary vein atresia or obstruction may also have been examples of pulmonary veno-occlusive disease.

Humans↗

The glomerular mesangium: uptake and transport of intravenously injected colloidal carbon in rats.

Collodial carbon, 70 mg/100 g, was injected into rats which were sacificed for histologic study of the kidneys at intervals of five minutes to seven weeks. Transient thrombocytopenia and albuminuria were observed. Uptake of carbon by the mesangium of glomeruli was maximal at 32 hr and gradually decreased thereafter. Semiquantitative analysis of the distribution of carbon particles within glomeruli revealed a predominately peripheral localization during early time periods, and increased relative concentration of particles within more central zones and in the lacis area at the vascular pole of the glomerulus at two to seven weeks, indicating that one of the mechanisms for clearance of materials from the mesangium was movement of particles in the direction of the lacis area. Sequential electron microscopic studies showed that carbon particles moved through fenestrae in the endothelium covering the mesangium into channels between mesangial cells. Observations at later intervals suggested that carbon eventually reached the vascular pole by being passed on from one mesangial cell to the next. Vasoactive amines or other substances derived from platelets may play an important role in initiating the process of mesangial uptake.

Albuminuria↗

Two cases of phosphoenolpyruvate carboxykinase deficiency.

Two children are described who suffered from hypoglycemia and liver impairment. Assays of gluconeogenic enzymes in liver samples taken immediately after death demonstrated a deficiency of phosphoenolpyruvate carboxykinase, a key enzyme of gluconeogenesis. Post mortem examination demonstrated massive fat deposition in liver and kidney and to a lesser extent in other tissues. The fatty changes in liver and kidney could be explained by the absence of phosphoenolpyruvate carboxykinase, which would cause an alteration in the mitochondrial-cytosolic processes related to gluconeogenesis.

Fatty Acids↗

Unilateral renal disease in the rat. II. Glomerular mesangial uptake of colloidal carbon in unilateral aminonucleoside nephrosis and nephrotoxic serum nephritis.

Unilateral renal disease was produced in rats by left renal perfusion in situ with the aminonucleoside of puromycin or with nephrotoxic serum. The uptake of colloidal carbon by the glomerular mesangium was increased in kidneys perfused with aminonucleoside or nephrotoxic serum compared to contralateral kidneys. The quantities of carbon within the glomerular mesangium remained increased in aminonucleoside perfused kidneys 14 days later. These studies demonstrate that alterations in glomerular mesangial function in aminonucleoside nephrosis and nephrotoxic serum nephritis are related to renal factors rather than differences in host milieu and may be involved in the pathogenesis of the morphologic lesions seen in chronic proteinuria.

Animals↗

Changes in the renin-angiotensin-aldosterone system and in sodium and potassium balance during development of renal hypertension in rats.

1. The relationships between the renin-angiotensin-aldosterone system, sodium and potassium balance and systolic blood pressure were studied during development of moderate (160-180 mmHg; clip i.d. 0.25 mm) and severe (200-230 mmHg; clip i.d. 0.20 mm) renal hypertension in rats with an undisturbed contralateral kidney. 2. In severely hypertensive rats renin activity in the peripheral plasma increased from day 9, by which time the systolic blood pressure was elevated to 160-180 mmHg. The rate of total corticosteroid and aldosterone production in vitro increased from day 14 and plasma renin substrate concentration increased from day 24. In moderately hypertensive rats, none of these changes occurred. 3. During the first 10 days after the application of 0.25 and 0.20 mm clips, sodium and potassium retention/g gain in body weight were higher than in sham-operated controls. During the next 10 days, the positive balance stabilized in animals with a 0.25 mm clip whereas, in animals with a 0.20 mm clip, sodium and potassium balance returned to the level of the sham-operated controls through increased renal losses. Despite these changes the systolic pressure rose further in animals with a 0.20 mm clip. 4. The initial sodium retention could be a factor in the early rise of blood pressure and could account for the delay in the rise of peripheral plasma renin activity. The subsequent loss of the retained sodium and potassium during the development of severe hypertension could have facilitated the rise in peripheral plasma renin activity, but did not initiate this rise.

Adrenal Cortex Hormones↗

Focal and segmental glomerular hyalinosis and sclerosis in the rat.

A glomerular disease spontaneously developing in Wistar rats was studied by light and electron microscopy and by immunofluorescence techniques. The disease is characterized by the local subendothelial deposition of hyaline material leading to increase of mesangial matrix and the development of adhesions. Immunofluorescence shows deposition of complement and IgM and to a lesser degree also of IgG in these lesions. There is a constant relationship of these early changes with the vascular pole of the glomerulus. It is confirmed that female rats are resistent to the disease as are male rats fed a sodium-deficient diet. A higher protein excretion was found in normally fed male rats as compared to female rats and to rats on a sodium-deficient diet. These differences already existed before the normally fed male rats developed glomerular disease. From these studies it is suggested that an appropriate name for this disease would be focal and segmental glomerular hyalinosis and sclerosis and that hemodynamic factors could be an important etiologic mechanism. The histopathology of the disease bears a striking resemblance to focal sclerosing glomerulopathy with segmental hyalinosis sometimes found in kidneys of patients with an idiopathic nephrotic syndrome.

Animals↗