| Acrodermatitis enteropathica: recent findings concerning clinical features, pathogenesis, diagnosis and therapy. | |
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MedLine Citation:
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PMID: 1278184 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Acrodermatitis enteropathica (AE) was diagnosed in 2 siblings, boy and girl, at the age of 10 and 6 weeks. The family history is unremarkable except for consanguinity 5 generations previously. The clinical symptoms of the 2 patients conformed to the known features of AE, the gastrointestinal involvement loosing its significance with increasing age. In one patient in a stage of exacerbation the serum level of oleic acid (18:1) was lowered and of linoleic (18:2) acid slightly increased while that of arachidonic acid was decreased (Fig. 4). In both patients the serum zinc levels were significantly lowered. Under substitution with ZnSO4 the clinical condition improved and the serum zinc levels returned to normal. Histologically the small bowel mucosa was practically normal. Ultrastructural examination of jejunal biopsies revealed rather unspecific changes in the enterocytes in the form of numerous multivesicular bodies. The Paneth cells sometimes contained irregularly formed inhomogeneous structures within their cytoplasm. In addition the secretory granules varied in size and displayed a granular heteromorphic matrix. Frequently they were confluent and formed giant granules. |
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Authors:
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O H Braun; K Heilmann; W Pauli; J A Rossner; K E Bergmann |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: European journal of pediatrics Volume: 121 ISSN: 0340-6199 ISO Abbreviation: Eur. J. Pediatr. Publication Date: 1976 Mar |
Date Detail:
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Created Date: 1976-09-02 Completed Date: 1976-09-02 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 7603873 Medline TA: Eur J Pediatr Country: GERMANY, WEST |
Other Details:
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Languages: eng Pagination: 247-61 Citation Subset: IM |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Acrodermatitis
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diagnosis*,
drug therapy,
etiology,
genetics,
pathology Age Factors Arachidonic Acids / blood Child, Preschool Female Humans Infant Intestinal Mucosa / pathology Intestine, Small / pathology Linoleic Acids / blood Male Microscopy, Electron Oleic Acids / blood Pedigree Sulfates Zinc / blood, therapeutic use |
| Chemical | |
Reg. No./Substance:
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0/Arachidonic Acids; 0/Linoleic Acids; 0/Oleic Acids; 0/Sulfates; 7440-66-6/Zinc |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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