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Biomedical subjects

M Elleder

Publications and source records attributed to M Elleder.

At least 163 records · Page 9Linked to original sources
PubMed1977

alpha-D-mannosidase activity in histiocytosis X.

A histochemical study of enzymatic activities was undertaken in five cases of histiocytosis X (two localized bone forms, two generalized forms, and one involving mainly the skin), each of which revealed characteristic structural features at the optical and ultrastructural levels. A confirmation was made of the original assumption of high acid alpha-D-mannosidase activity, i.e. activity described in human Langerhans intraepidermal cells (Elleder, 1975). In the control group of tumors, with the exception of urticaria pigmentosa, enzyme activity was either at trace level or altogether absent. Acid alpha-D-mannosidase activity therefore appears to be the first biochemical feature common to both histiocytosis X and the Langerhans cells. The significance of the finding for the present theory of the histogenesis of the above tumors is discussed.

Biopsy↗
PubMed1977

Peripheral nervous system affection in experimental lipidosis induced by 4,4'-diethylaminoethoxyhexesterol.

A picture of generalized phosphoglyceride and cholesterol storage was induced, in keeping with literary data, by the experimental administration of 4,4'-diethylaminoethoxyhexesterol to rats. An asset of this model lies in the discovery that considerable storage occurs in the peripheral nervous system in contrast to the CNS, whose resistance to hexesterol is generally known. The significance of this finding is briefly discussed.

Animals↗
PubMed1977

So-called neuronal ceroid-lipofuscinosis. Histochemical study with evidence of extractibility of the stored material.

Histochemical study of so-called neuronal ceroid-lipofuscinoses (NCL) showed that the stored material is extractable in the unfixed state especially with alkalized or acidified chloroform-methanol mixtures when compared with other solvents. The extractability was strongly reduced or almost abolished by fixation with formaldehyde. Identical results were obtained with the type one storage material (see Elleder, 1977) in all late infantile cases and in a juvenile case studied, in which, contrary to the infantile form, the stored material displayed a significantly higher degree of extractability. As far as the extractability of the type two storage material is concerned insufficient data have been accumulated, but it seems that it does not differ significantly from the first one. In the control group of lipopigments ceroid was found to be much more extractable under identical conditions than matured lipofuscin which was almost entirely resistant to all extraction procedures. The significance of the results is discussed.

Adolescent↗
PubMed1977

Histochemical diagnosis of lipidoses.

Contemporary possibilities for the histochemical diagnosis of lipidoses are demonstrated in examples of phospholipidoses, Gaucher's disease, Fabry's disease, sulphatidosis, gangliosidosis and neuronal ceroid-lipofuscinoses.

Biopsy↗
PubMed1977

Possibilities for the cytochemical diagnosis of enzymopathies.

The authors review the contemporary uses of histochemistry for the diagnosis of enzymopathies. Enterokinase, lactase, sucrase and trehalase deficiency can be diagnosed by histochemical methods. In glycogenoses, glycogen storage and glucose-6-phosphatase, acid alpha-glucosidase and phosphorylase deficiencies can be demonstrated. In mucopolysaccharidoses, the accumulation of acid muco-substances and changes in lysosomal enzyme activities can be demonstrated.

Biopsy↗
PubMed1976

Prolonged methanol fixation of soluble mucosubstances in mucopolysaccharidoses.

A simple and efficient method for the demonstration of highly water soluble acid mucosubstances in cold microtone sections is described. It consists of prolonged treatment of cold microtome sections with methanol (for at least 1 h) and subsequent staining with 0.1% azure A in distilled water or in 30% methanol. The procedure is recommended particularly for the bioptical examination of mucopolysaccharidoses.

Azure Stains↗
PubMed1975

Niemann-Pick disease (Crocker's type C): A histological study of the distribution and qualitative differences fo the storage process.

A histochemical study is reported of regional differences of the lipid storage in a case of Niemann-Pick disease (NPD) type C. Besides tissues known to be affected (reticulo-endothelium, hepatocytes, nervous system), storage was demonstrated in adrenal cortical spongiocytes, sweat glands, renal glomerular and tubular cells, smooth muslce, excretory tubules of some salivary glands, ependyma and in choroid plexus. In most tissues were stored sphingomyelin, cholesterol and a small amount of a glycosphingolipid. In the endothelium of cerebral and spinal vessels the main stored lipid was a glycosphingolipid. The significance of these regional differences are discussed and their study is recommended as a useful counterpart to the biochemical investigation.

Adrenal Cortex↗
PubMed1975

Studies in lipid histochemistry. XIII. The OPA (osmiumtetroxide-periodic acid-alpha-naphthylamine) method for the detection of apolar lipids.

A new procedure for the detection of apolar lipids is described. It is a modification of the OTAN method (Adams, 1959) using periodic acid which oxidatively removes lower osmium derivatives from polar sites only, leaving those in apolar lipids intact and demonstrable with alpha-naphthylamine. Control steps for the exclusion of the possible interference of some less polar complex lipids and of lipopigments are described. The described technic is superior to the conventionally used sudan dyes due partly to the fact that only aqueous solutions are employed thus excluding any extraction of lipids, partly to the more distinct coloration.

1-Naphthylamine↗
PubMed1975

Activity of alpha-d-mannosidase in human Langerhans epidermal cells.

The histochemical examination of bioptic samples of the human skin revealed the strikingly high activity of an acid-alpha-D-mannosidase in Langerhans cells. This enzyme is highly soluble and its activity "in situ" can be demonstrated practically only with the semipermable membrane technic. The significance of this finding is briefly discussed.

Biopsy↗
PubMed1975

Lipidosis with a predominant storage of phosphoglycerides (phospholipidosis type II--Baar, Wiedemann).

A case of a 27 month old girl suffering from a rare form of lipidosis is described. Clinical symtoms consisted of a moderate hepatosplenomegaly and a progressive psychomotor retardation. Bioptical examination of the liver, appendix and skin revealed a pronounced lipid storage in histiocytes, hepatocytes, vascular endothelium and in peripheral nervous system. Histochemically, a generalized storage of phosphoglycerides and cholesterol was found. It was accompanied with a moderate amount of sphingomyelin and a variable amount of glycolipids (predominantly glycosphingolipids), the latter being stored mainly in the peripheral nervous system and in the vascular endothelium. Chromatographically, an increased concentration of lysobisphosphatidic acid and cholesterol could be detected. The ultrastructure of storage cytosomes was rather pleomorphic often with concentrically lamellar appearance. Further details of the investigation are described and the relation of this case to those described by Baar and Hickmans (1956) and Wiedemann et al. (1972) is stressed. Due to a strong evidence that this group of diseases represents a new type of phospholipid storage disease the name "Phospholipidosis Type II" (Baar-Wiedemann) or "Phosphoglyceridosis" is proposed, whereas "Phospholipidosis Type I" or "Sphingomyelinosis" should be reserved for the classical Niemann-Pick complex.

Appendix↗
PubMed1975

[Phosphoglyceridosis].

On the basis of a bioptic examination of the appendix, skin and a liver specimen, the diagnosis of phospholipidosis was made in a girl aged 27 months. In contrast to the Niemann-Pick's complex of sphingomyelinoses, phosphoglycerides were stored in larger amounts than spingomyelin. The disease should be undoubtedly included under one heading with the so-called "kephalinosis" [11] and with the cases described by Wiedemann et al. [10]. The terms "Type II phospholipidosis [Baar-Wiedemann's disease]" or, briefly, phosphoglyceridosis, appear to be most adequeate for designating the diseases in question. The disorder can be diagnosed on the basis of iron hematoxylin staining, visualizing all phospholipids. In Niemann-Pick's sfingomyelinosis, alkaline hydrolysis does not alter the colour, whereas in the phosphoglyceridosis under discussion the colour is substantially reduced or even desappears after alkaline hydrolysis.

Child, Preschool↗
PubMed1975

[Lipids in the renal veins and disseminated intravascular coagulation].

Marked steatosis of glomeruli and of cortical arterioles is a peculiar and hitherto neglected change occuring in generalized Shwartzman's reaction as well as in the haemolytic-uremic syndrome. The fatty deposits originate during the transient hyperlipemia occurring in both the above entities. No comparable steatosis takes place in disseminated intravascular coagulation. In disseminated intravascular coagulation occurring in hyperlipemic rats, however, steatosis of glomeruli as well as of cortical arterioles is present so that the picture resembles generalized Shwartzman's reaction. Such a comparison poses a lot of further pathogenetic problems.

Animals↗
PubMed1975

[Problem in the determination of calcinosis by means of hematoxylin].

The histochemical analysis of a number of dystrophic and metastatic calcifications revealed that the haematoxylinophilia of calcified tissues is not based on the inorganic component, but on their organic component. In most of the cases the material examined contained protein on the basis of its staining properties, as well as neutral and acid mucosubstances and, most probably, also covalently bound phosphate. Exceptionally the organic component appeared to be unequivocally acidic phospholipid, tigtly bound to an inorganic component. The significance of the findings has been discussed.

Benzopyrans↗