Neonatal congenital microvillus atrophy
Identifieur interne : 001820 ( Main/Exploration ); précédent : 001819; suivant : 001821Neonatal congenital microvillus atrophy
Auteurs : N. Pecache [Australie] ; S. Patole [Australie] ; R. Hagan [Australie] ; D. Hill [Australie] ; A. Charles [Australie] ; J M Papadimitriou [Australie]Source :
- Postgraduate Medical Journal [ 0032-5473 ] ; 2004-02.
English descriptors
- KwdEn :
- Teeft :
- Absent corpus callosum, Atrophy, Brief review, Brush border, Characteristic inclusions, Cmva, Congenital, Congenital microvillous atrophy, Congenital microvillus atrophy, Diarrhoea, Early diagnosis, Early onset, Gastroenterol, Hydrolysed formula, Ileocaecal valve, Inclusion, Intestinal, Intestinal transplantation, Intractable diarrhoea, Late onset, Literature review, Microvillous, Microvillous inclusion disease, Microvillus, Microvillus inclusion disease, Milk formula, Native colon, Neonatal, Neonatal period, Nutr, Parenteral nutrition, Pathol, Pediatr, Pediatr gastroenterol nutr, Pediatr pathol, Positive material, Protracted diarrhea, Secretory diarrhoea, Severe dehydration, Small bowel transplantation, Syndrome, Transplantation, Ultrastruct pathol, Western australia.
Abstract
Congenital microvillous atrophy (CMVA) is the leading cause of neonatal secretory diarrhoea with onset either in the first 72 hours of life (early onset) or at 6–8 weeks after birth (late onset). To date over 30 cases have been reported worldwide. The prognosis for this life threatening condition continues to be poor. Therapeutic agents like somatostatin and epidermal growth factor are either ineffective or of marginal benefit. Overall five year survival after small bowel transplantation is currently ∼50%. The following brief review is aimed towards helping neonatologists/perinatologists in the early diagnosis, and management of CMVA and in counselling the parents appropriately.
Url:
- https://api.istex.fr/ark:/67375/NVC-N233KVMW-3/fulltext.pdf
- http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1742937
DOI: 10.1136/pmj.2003.007930
Affiliations:
Links toward previous steps (curation, corpus...)
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<term>Atrophy</term>
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<term>Characteristic inclusions</term>
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<term>Congenital microvillous atrophy</term>
<term>Congenital microvillus atrophy</term>
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<term>Protracted diarrhea</term>
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<front><div type="abstract" xml:lang="en">Congenital microvillous atrophy (CMVA) is the leading cause of neonatal secretory diarrhoea with onset either in the first 72 hours of life (early onset) or at 6–8 weeks after birth (late onset). To date over 30 cases have been reported worldwide. The prognosis for this life threatening condition continues to be poor. Therapeutic agents like somatostatin and epidermal growth factor are either ineffective or of marginal benefit. Overall five year survival after small bowel transplantation is currently ∼50%. The following brief review is aimed towards helping neonatologists/perinatologists in the early diagnosis, and management of CMVA and in counselling the parents appropriately.</div>
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