Segmental Absence of Intestinal Musculature in a Child with Type IV Ehlers-Danlos Syndrome
Patients with vascular Ehlers-Danlos syndrome (vEDS) have a defect in the formation of type III collagen. This defect puts patients at risk of vascular rupture, uterine rupture, and bowel perforations. The segmental absence of intestinal musculature is a rare histopathologic finding, wherein there is a lack of a muscularis propria layer in the intestinal wall. Although typically documented in the literature in neonates or adults, it can be seen in children of other ages. This is a case report of a patient who exhibits both rare entities, which has not been described in the literature to date.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2021 |
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| Erschienen: |
2021 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:8 |
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| Enthalten in: |
Children (Basel, Switzerland) - 8(2021), 8 vom: 05. Aug. |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Zeky, Nicole [VerfasserIn] |
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| Links: |
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| Themen: |
Case Reports |
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| Anmerkungen: |
Date Revised 30.08.2021 published: Electronic Citation Status PubMed-not-MEDLINE |
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| doi: |
10.3390/children8080680 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM329814796 |
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| 520 | |a Patients with vascular Ehlers-Danlos syndrome (vEDS) have a defect in the formation of type III collagen. This defect puts patients at risk of vascular rupture, uterine rupture, and bowel perforations. The segmental absence of intestinal musculature is a rare histopathologic finding, wherein there is a lack of a muscularis propria layer in the intestinal wall. Although typically documented in the literature in neonates or adults, it can be seen in children of other ages. This is a case report of a patient who exhibits both rare entities, which has not been described in the literature to date | ||
| 650 | 4 | |a Case Reports | |
| 650 | 4 | |a Ehlers–Danlos | |
| 650 | 4 | |a intestinal perforation | |
| 650 | 4 | |a segmental absence of intestinal musculature | |
| 650 | 4 | |a vascular Ehlers–Danlos | |
| 700 | 1 | |a Short, Celia |e verfasserin |4 aut | |
| 700 | 1 | |a Keith, Brent |e verfasserin |4 aut | |
| 700 | 1 | |a Craver, Randall D |e verfasserin |4 aut | |
| 700 | 1 | |a Zagory, Jessica A |e verfasserin |4 aut | |
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