| Primary lymphatic dysplasia in children: chylothorax, chylous ascites, and generalized lymphatic dysplasia. | |
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MedLine Citation:
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PMID: 3769996 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Primary lymphatic "dysplasia", a congenital maldevelopment, interferes with function of the lymphatic system and causes effusion of chyle or lymph into the limbs and pleural or peritoneal cavity. Between 1955 and 1982, 38 Mayo Clinic patients were found to have a chylous effusion or dysplasia of the lymphatic system. In 22, the condition was secondary to surgery or other medical problems and in 16 it was primary. These cases were separated into three categories: chylothorax, chylous ascites, and generalized lymphatic dysplasia. Conservative therapy, such as a restricted fat diet or total parenteral nutrition with repeated thoracentesis or paracentesis, was effective in the children with isolated abnormalities of the lymphatic system (75% resolution rate, no deaths). All five children with documented generalized dysplasia reported in the literature had died; of the three reported here, one has died and two have become progressively worse. |
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Authors:
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D M Smeltzer; G B Stickler; R E Fleming |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: European journal of pediatrics Volume: 145 ISSN: 0340-6199 ISO Abbreviation: Eur. J. Pediatr. Publication Date: 1986 Sep |
Date Detail:
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Created Date: 1986-12-11 Completed Date: 1986-12-11 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: 286-92 Citation Subset: IM |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Child Child, Preschool Chylothorax / congenital, diagnosis*, pathology Chylous Ascites / congenital, diagnosis*, pathology Female Humans Infant Infant, Newborn Lymphatic Diseases / congenital, diagnosis*, pathology Male |
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