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 i...
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| Format: | Book |
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MDPI AG,
2021-08-01T00:00:00Z.
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| LEADER | 00000 am a22000003u 4500 | ||
|---|---|---|---|
| 001 | doaj_ece0d85e9d7f4dd1bdbc64cbf12490d8 | ||
| 042 | |a dc | ||
| 100 | 1 | 0 | |a Nicole Zeky |e author |
| 700 | 1 | 0 | |a Celia Short |e author |
| 700 | 1 | 0 | |a Brent Keith |e author |
| 700 | 1 | 0 | |a Randall D. Craver |e author |
| 700 | 1 | 0 | |a Jessica A. Zagory |e author |
| 245 | 0 | 0 | |a Segmental Absence of Intestinal Musculature in a Child with Type IV Ehlers-Danlos Syndrome |
| 260 | |b MDPI AG, |c 2021-08-01T00:00:00Z. | ||
| 500 | |a 10.3390/children8080680 | ||
| 500 | |a 2227-9067 | ||
| 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. | ||
| 546 | |a EN | ||
| 690 | |a Ehlers-Danlos | ||
| 690 | |a vascular Ehlers-Danlos | ||
| 690 | |a intestinal perforation | ||
| 690 | |a segmental absence of intestinal musculature | ||
| 690 | |a Pediatrics | ||
| 690 | |a RJ1-570 | ||
| 655 | 7 | |a article |2 local | |
| 786 | 0 | |n Children, Vol 8, Iss 8, p 680 (2021) | |
| 787 | 0 | |n https://www.mdpi.com/2227-9067/8/8/680 | |
| 787 | 0 | |n https://doaj.org/toc/2227-9067 | |
| 856 | 4 | 1 | |u https://doaj.org/article/ece0d85e9d7f4dd1bdbc64cbf12490d8 |z Connect to this object online. |