CEROID STORAGE DISEASE IN CHILDHOOD

PEDIATRICS ◽  
1959 ◽  
Vol 23 (6) ◽  
pp. 1091-1102
Author(s):  
Ella H. Oppenheimer ◽  
Edward C. Andrews
Keyword(s):  
Smooth Muscle ◽  
Vitamin E ◽  
Intestinal Mucosa ◽  
Storage Disease ◽  
Cirrhosis Of The Liver ◽  
The Other ◽  
Vitamin E Deficiency ◽  
Nutritional Disturbance ◽  
History Of ◽  
Parenchymal Cells

Two cases of ceroid storage in childhood are reported. In both cases there was a long history of nutritional disturbance and cirrhosis of the liver was found at necropsy. In one case the storage of ceroid followed the pattern of so-called "lipidoses," with massive numbers of ceroid-filled cells in the spleen, liver and intestinal mucosa. In the other case the deposition of ceroid occurred mostly in macrophages in the liver. No ceroid was found in smooth muscle or other parenchymal cells as in vitamin E deficiency.

scholarly journals Brown Bowel Syndrome Is a Rare and Commonly Missed Disease: A Case Report and Literature Review

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Joaquin Ponce-Zepeda ◽  
Wenchang Guo ◽  
Giorgioni Carmen ◽  
Daniel Moon Kim ◽  
Gregory C. Albers ◽  
...  
Keyword(s):  
Vitamin E ◽  
Small Bowel ◽  
Chronic Diarrhea ◽  
Muscularis Propria ◽  
Mitochondrial Damage ◽  
Vitamin E Deficiency ◽  
Case Presentation ◽  
History Of ◽  
Resected Small Bowel

Background. Brown bowel syndrome (BBS) is a rare gastrointestinal condition, and vitamin E deficiency has been considered to be a main contributor. However, vitamin E deficiency has been found in only a few patients throughout the published literature studies and its cutoff lab value for diagnosis is not entirely clarified. Case Presentation. A 56-year-old female patient with a history of congenital bowel obstruction (repaired at birth) presented with bloating, abdominal pain, and chronic diarrhea. Endoscopy identified unremarkable gastrointestinal mucosa except a few small polyps in the colon. A partial obstruction was detected by a small bowel follow-through series and then confirmed by CT scan. The resected small bowel was significantly dilated with a thickened brown wall and extensive serosal adhesion. Microscopic examination revealed unremarkable mucosa, but dense granular brown pigments were identified in the cytoplasm of the smooth muscle cells in the muscularis propria. These deposits resulted to be lipofuscin, and BBS was diagnosed. The patient was asymptomatic at 9-month follow-up after surgery without vitamin E supplement. Conclusion. Mitochondrial damage with lipofuscin deposition is at the root of BBS pathogenesis. Any etiology associated with mitochondrial damage can cause this disease, and vitamin E deficiency is just one of them. Dysmotility from extensive serosal adhesion could be a possible etiology for this patient. Due to overlapping symptoms, lipofuscin deposition primarily in the muscularis propria, and unclear serum value of vitamin E, this syndrome is often missed in routine clinical practice from the superficial biopsy. A transmural biopsy is necessary for a definite diagnosis.

AVITAMINOSIS E IN CYSTIC FIBROSIS OF THE PANCREAS

PEDIATRICS ◽  
1958 ◽  
Vol 22 (3) ◽  
pp. 494-506
Author(s):  
William A. Blanc ◽  
Joseph D. Reid ◽  
Dorothy H. Andersen
Keyword(s):  
Cystic Fibrosis ◽  
Smooth Muscle ◽  
Vitamin E ◽  
Gastrointestinal Tract ◽  
Striated Muscle ◽  
Vitamin E Deficiency ◽  
Severe Vitamin ◽  
Second Year ◽  
Avitaminosis E ◽  
Ceroid Pigment

Ceroid-pigment is present in the smooth muscle fibers of the gastrointestinal tract of patients with cystic fibrosis of the pancreas. It is first seen during the second year of life. It is found in all patients of 5 years of age and older, and the amount increases with age. It has not been found in other pediatric conditions with the exception of biliary atresia and cirrhosis of the liver. Extensive ceroid deposit probably represents an expression of prolonged and severe vitamin-E deficiency, related to protracted malabsorption of fat-soluble tocopherols. Alterations of striated muscle appear to be rare and minimal in cystic fibrosis of the pancreas.

The Cellular Origin in Atheromatous Lesion

1970 ◽  
Vol 28 ◽  
pp. 202-203
Author(s):  
T. M. Murad ◽  
H. A. I. Newman ◽  
K. F. Kern
Keyword(s):  
Smooth Muscle ◽  
Smooth Muscle Cells ◽  
Rabbit Aorta ◽  
Mixed Population ◽  
The Other ◽  
Cellular Origin ◽  
Ultrastructural Studies ◽  
Atherosclerotic Lesions ◽  
Show Evidence ◽  
Early Lesion

The origin of lipid containing cells in atheromatous lesion has been disputed. Geer in his study on atheromatous lesions of rabbit aorta, suggested that the early lesion is composed mainly of lipid-laden macrophages and the later lesion has a mixed population of macrophages and smooth muscle cells. Parker on the other hand, was able to show evidence that the rabbit lesion is primarily composed of lipid-laden cells of smooth muscle origin. The above studies and many others were done on an intact lesion without any attempt of cellular isolation previous to their ultrastructural studies. Cell isolation procedures have been established for atherosclerotic lesions through collagenase and elastase digestion Therefore this procedure can be utilized to identify the cells involved in rabbit atheroma.

Theodor W. Adorno

2018 ◽  
Author(s):  
Colby Dickinson
Keyword(s):  
The Other ◽  
History Of Thought ◽  
History Of ◽  
The One

In his somewhat controversial book Remnants of Auschwitz, Agamben makes brief reference to Theodor Adorno’s apparently contradictory remarks on perceptions of death post-Auschwitz, positions that Adorno had taken concerning Nazi genocidal actions that had seemed also to reflect something horribly errant in the history of thought itself. There was within such murderous acts, he had claimed, a particular degradation of death itself, a perpetration of our humanity bound in some way to affect our perception of reason itself. The contradictions regarding Auschwitz that Agamben senses to be latent within Adorno’s remarks involve the intuition ‘on the one hand, of having realized the unconditional triumph of death against life; on the other, of having degraded and debased death. Neither of these charges – perhaps like every charge, which is always a genuinely legal gesture – succeed in exhausting Auschwitz’s offense, in defining its case in point’ (RA 81). And this is the stance that Agamben wishes to hammer home quite emphatically vis-à-vis Adorno’s limitations, ones that, I would only add, seem to linger within Agamben’s own formulations in ways that he has still not come to reckon with entirely: ‘This oscillation’, he affirms, ‘betrays reason’s incapacity to identify the specific crime of Auschwitz with certainty’ (RA 81).

The World after the End of the World

2017 ◽  
Vol 39 (2) ◽  
pp. 265-276 ◽  
Author(s):  
Kas Saghafi
Keyword(s):  
The Other ◽  
The World ◽  
History Of ◽  
The Absolute ◽  
End Of The World

In several late texts, Derrida meditated on Paul Celan's poem ‘Grosse, Glühende Wölbung’, in which the departure of the world is announced. Delving into the ‘origin’ and ‘history’ of the ‘conception’ of the world, this paper suggests that, for Derrida, the end of the world is determined by and from death—the death of the other. The death of the other marks, each and every time, the absolute end of the world.

Med. Dr. Adalbert Wraný (1836–1902) – doctor of medicine, mineral collector and donor to the National Museum in Prague

2019 ◽  
Vol 188 (1) ◽  
pp. 95-146
Author(s):  
Martin Bohatý ◽  
Dalibor Velebil
Keyword(s):  
Natural Science ◽  
Natural Sciences ◽  
The Other ◽  
National Museum ◽  
Industrial Chemistry ◽  
Pediatric Pathology ◽  
Geological Institute ◽  
History Of ◽  
Precious Stones

Adalbert Wraný (*1836, †1902) was a doctor of medicine, with his primary specialization in pediatric pathology, and was also one of the founders of microscopic and chemical diagnostics. He was interested in natural sciences, chemistry, botany, paleontology and above all mineralogy. He wrote two books, one on the development of mineralogical research in Bohemia (1896), and the other on the history of industrial chemistry in Bohemia (1902). Wraný also assembled several natural science collections. During his lifetime, he gave to the National Museum large collections of rocks, a collection of cut precious stones and his library. He donated a collection of fossils to the Geological Institute of the Czech University (now Charles University). He was an inspector of the mineralogical collection of the National Museum. After his death, he bequeathed to the National Museum his collection of minerals and the rest of the gemstone collection. He donated paintings to the Prague City Museum, and other property to the Klar Institute of the Blind in Prague. The National Museum’s collection currently contains 4 325 samples of minerals, as well as 21 meteorites and several hundred cut precious stones from Wraný’s collection.

La ética del Otro: una introducción al pensamiento de Emmanuel Lévinas

2019 ◽  
Vol 1 (1) ◽  
pp. 5-14
Author(s):  
Carlos Alvaréz Teijeiro
Keyword(s):  
Interpersonal Relationships ◽  
Emmanuel Levinas ◽  
Radical Change ◽  
The Other ◽  
Western Philosophy ◽  
Ethical Implications ◽  
Essential Question ◽  
History Of ◽  
The Relationship ◽  
First Philosophy

Emmanuel Lévinas, the philosopher of ethics par excellence in the twentieth century, and by own merit one of the most important ethical philosophers in the history of western philosophy, is also the philosopher of the Other. Thereby, it can be said that no thought has deepened like his in the ups and downs of the ethical relationship between subject and otherness. The general objective of this work is to expose in a simple and understandable way some ideas that tend to be quite dark in the philosophical work of the author, since his profuse religious production will not be analyzed here. It is expected to show that his ideas about the being and the Other are relevant to better understand interpersonal relationships in times of 4.0 (re)evolution. As specific objectives, this work aims to expose in chronological order the main works of the thinker, with special emphasis on his ethical implications: Of the evasion (1935), The time and the Other (1947), From the existence to the existent (1947), Totality and infinity: An essay on exteriority (1961) and, last, Otherwise than being, or beyond essence (1974). In the judgment of Lévinas, history of western philosophy starting with Greece, has shown an unusual concern for the Being, this is, it has basically been an ontology and, accordingly, it has relegated ethics to a second or third plane. On the other hand and in a clear going against the tide movement, our author supports that ethics should be considered the first philosophy and more, even previous to the proper philosophize. This novel approach implies, as it is supposed, that the essential question of the philosophy slows down its origin around the Being in order to inquire about the Other: it is a philosophy in first person. Such a radical change of perspective generates an underlying change in how we conceive interpersonal relationships, the complex framework of meanings around the relationship Me and You, which also philosopher Martin Buber had already spoken of. As Lévinas postulates that ethics is the first philosophy, this involves that the Other claims all our attention, intellectual and emotional, to the point of considering that the relationship with the Other is one of the measures of our identity. Thus, “natural” attitude –husserlian word not used by Lévinas- would be to be in permanent disposition regarding to the meeting with the Other, to be in permanent opening state to let ourselves be questioned by him. Ontology, as the author says, being worried about the Being, has been likewise concerned about the Existence, when the matter is to concern about the particular Existent that every otherness supposes for us. In conclusion it can be affirmed that levinasian ethics of the meeting with the Other, particular Face, irreducible to the assumption, can contribute with an innovative looking to (re)evolving the interpersonal relationships in a 4.0 context.

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