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Text
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Citation List Month
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URL Address
<a href="http://doi.org/10.1097/00005176-200209000-00029" target="_blank" rel="noreferrer">http://doi.org/10.1097/00005176-200209000-00029</a>
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Diagnosis and management of MNGIE syndrome in children: case report and review of the literature
Publisher
An entity responsible for making the resource available
Journal Of Pediatric Gastroenterology And Nutrition
Date
A point or period of time associated with an event in the lifecycle of the resource
2002
Subject
The topic of the resource
Child; Female; Humans; Parenteral Nutrition; adolescent; Tomography; X-Ray Computed; Duodenum/radiography; Gastrointestinal Diseases/physiopathology/radiography/therapy; Mitochondrial Encephalomyopathies/physiopathology/radiography/therapy; Stomach/radiography; Total; Ubiquinone/therapeutic use; Vitamins/therapeutic use
Creator
An entity primarily responsible for making the resource
Teitelbaum JE; Berde CB; Nurko S; Buonomo C; Perez-Atayde AR; Fox VL
Description
An account of the resource
Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) syndrome is a rare disorder that presents in childhood; however, marked delay in diagnosis is common. We report a case and review the literature describing the typical features that should alert pediatricians to the diagnosis. We also describe a novel management strategy for providing symptomatic relief.
2002
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1097/00005176-200209000-00029" target="_blank" rel="noreferrer">10.1097/00005176-200209000-00029</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Type
The nature or genre of the resource
Journal Article
2002
Adolescent
Backlog
Berde CB
Buonomo C
Child
Duodenum/radiography
Female
Fox VL
Gastrointestinal Diseases/physiopathology/radiography/therapy
Humans
Journal Article
Journal Of Pediatric Gastroenterology And Nutrition
Mitochondrial Encephalomyopathies/physiopathology/radiography/therapy
Nurko S
Parenteral Nutrition
Perez-Atayde AR
Stomach/radiography
Teitelbaum JE
Tomography
Total
Ubiquinone/therapeutic use
Vitamins/therapeutic use
X-Ray Computed
-
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
Citation List Month
Backlog
URL Address
<a href="http://doi.org/10.1097/00000478-199809000-00014" target="_blank" rel="noreferrer">http://doi.org/10.1097/00000478-199809000-00014</a>
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
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Mitochondrial neurogastrointestinal encephalomyopathy: diagnosis by rectal biopsy
Publisher
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The American Journal Of Surgical Pathology
Date
A point or period of time associated with an event in the lifecycle of the resource
1998
Subject
The topic of the resource
Female; Humans; adolescent; Q3 Literature Search; Electron; Microscopy; Muscles/pathology; DNA; Biopsy/methods; Intestinal Pseudo-Obstruction/pathology; Mitochondrial Myopathies/pathology; Mitochondrial/analysis; Rectum/innervation/pathology
Creator
An entity primarily responsible for making the resource
Perez-Atayde AR; Fox V; Teitelbaum JE; Anthony DA; Fadic R; Kalsner L; Rivkin M; Johns DR; Cox GF
Description
An account of the resource
A 14-year-old girl with the mitochondrial neurogastrointestinal encephalopathy syndrome had an 8-year history of intestinal pseudoobstruction with abdominal pain, persistent vomiting, gastric and duodenal dilatation, and duodenal diverticulosis. The child appeared chronically malnourished and had severe growth failure. Multisystem involvement was evident with the presence of ptosis, external ophthalmoplegia, muscle wasting, peripheral neuropathy, and diffuse white matter disease seen on magnetic resonance imaging. Lactic acidosis and increased cerebrospinal fluid protein were observed. Mitochondrial enzyme analysis of fresh-frozen skeletal muscle revealed a respiratory chain defect. Molecular genetic studies showed multiple mitochondrial DNA deletions. Pathologic findings in the intestine included atrophy of the external layer of the muscularis propria and an increased number of abnormal-appearing mitochondria in ganglion and smooth-muscle cells. Microvesicular steatosis was observed in liver, skeletal, and gastrointestinal smooth muscle, and Schwann cells of peripheral nerve. Brightly eosinophilic inclusions in the cytoplasm of gastrointestinal ganglion cells were visible by light microscopy, which were confirmed to be megamitochondria by ultrastructural studies. This is the first report of abnormal mitochondria observed in intestinal ganglion and smooth-muscle cells in this syndrome.
1998
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1097/00000478-199809000-00014" target="_blank" rel="noreferrer">10.1097/00000478-199809000-00014</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Type
The nature or genre of the resource
Journal Article
1998
Adolescent
Anthony DA
Backlog
Biopsy/methods
Cox GF
DNA
Electron
Fadic R
Female
Fox V
Humans
Intestinal Pseudo-Obstruction/pathology
Johns DR
Journal Article
Kalsner L
Microscopy
Mitochondrial Myopathies/pathology
Mitochondrial/analysis
Muscles/pathology
Perez-Atayde AR
Q3 Scoping Review Results
Rectum/innervation/pathology
Rivkin M
Teitelbaum JE
The American Journal Of Surgical Pathology