98. Gynecologic Complaints in Pediatric and Adolescent Females with Ehlers-Danlos Syndrome

Ehlers-Danlos Syndrome (EDS) is a connective tissue disorder resulting in abnormal collagen synthesis leading to skin, joint, ligament, blood vessel and organ abnormalities. Studies in adults show an association with heavy menstrual bleeding, dysmenorrhea, and other gynecologic concerns, but there are no studies looking at gynecologic problems an adolescent population with EDS. We aimed to survey gynecologic complaints and management in a pediatric and adolescent population with EDS.
Source: Journal of Pediatric and Adolescent Gynecology - Category: OBGYN Authors: Source Type: research

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Ehlers-Danlos Syndrome (EDS) is a connective tissue disorder resulting in abnormal collagen synthesis leading to skin, joint, ligament, blood vessel and organ abnormalities. Studies in adult women show an association with heavy menstrual bleeding, dysmenorrhea, and pelvic organ prolapse. We aimed to evaluate gynecologic complaints in pediatric and adolescent patients with EDS and their management by Pediatric and Adolescent Gynecology (PAG) Providers.
Source: Journal of Pediatric and Adolescent Gynecology - Category: OBGYN Authors: Source Type: research
Conclusions There are only four reports of V-EDS causing splenic rupture in the literature to date. These patients were all adults and only one had not previously been diagnosed with V-EDS. All underwent splenectomy. While V-EDS presenting with abdominal visceral rupture in children has been reported, this is the first report of a child with V-EDS presenting with splenic rupture. It is the only case of splenic rupture secondary to V-EDS that has been managed minimally invasively by embolization. [...] Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals: Table of contents  | &nb...
Source: European Journal of Pediatric Surgery Reports - Category: Surgery Authors: Tags: Review Article Source Type: research
Authors: Sakai K, Toda M, Kyoyama H, Nishimura H, Kojima A, Kuwabara Y, Kobayashi Y, Kikuchi S, Hirata Y, Moriyama G, Watanabe W, Akutsu K, Nakai M, Yamada T, Gemma A, Uematsu K Abstract Type III collagen is the major protein in the walls of blood vessels and hollow organs; it is decreased in patients with vascular Ehlers-Danlos syndrome (EDS). A 52-year-old man was admitted for severe back pain, and right hemothorax was suspected by chest computed tomography. Immediately after embolization for bleeding bronchial artery, aortic dissection occurred and was treated conservatively in the intensive-care unit. Vascular ...
Source: Internal Medicine - Category: Internal Medicine Tags: Intern Med Source Type: research
Ehlers –Danlos syndrome (EDS) type IV (EDS-IV) is an inherited connective-tissue disorder of malformation of collagen resulting in poor blood-vessel integrity and is associated with spontaneous catastrophic bleeding from major arteries, making endovascular therapies challenging. Recent publications (1,2) advocate the safety of performing endovascular procedures in patients with vascular manifestations associated with EDS-IV. However, the present report describes a power-injection arterial injury in a patient with EDS-IV.
Source: Journal of Vascular and Interventional Radiology : JVIR - Category: Radiology Authors: Tags: Letter to the Editor Source Type: research
Authors: Qureshi AA, Friedman AJ Abstract Since the first reported cases in 2007, idiopathic mast cell activation syndrome has been increasingly recognized. Understanding of the cutaneous manifestations of this condition is imperative for dermatologists given the substantial clinical heterogeneity in its presentation and high estimated prevalence. A review of PubMed® and SCOPUS® databases was performed in order to investigate the most common dermatologic manifestations of idiopathic mast cell activation syndrome. Evidence to date suggests that flushing, pruritus, and clotting dysfunction or bleeding disorde...
Source: Journal of Drugs in Dermatology - Category: Dermatology Tags: J Drugs Dermatol Source Type: research
ConclusionsOur results show that nearly half of patients with EDS have an abnormal BSS, which, in 90% of cases, appear, at least in part, to be attributable to platelet function abnormalities. Abnormalities of primary hemostasis may contribute to the risk of bleeding in patients with EDS.
Source: Journal of Thrombosis and Haemostasis - Category: Hematology Authors: Tags: Original Article Source Type: research
CONCLUSIONS: Our results show that nearly half of patients with EDS have abnormal BSS, that in 90% of cases appears at least in part justified by platelet function abnormalities. Abnormalities of primary hemostasis may contribute to the risk of bleeding in patients with EDS. This article is protected by copyright. All rights reserved. PMID: 30312027 [PubMed - as supplied by publisher]
Source: Thrombosis and Haemostasis - Category: Hematology Authors: Tags: J Thromb Haemost Source Type: research
Ehlers-Danlos syndrome is a hereditary disorder characterized by defective collagen synthesis. Ehlers-Danlos type VIII is autosomal dominant and is distinguished from other subtypes by severe periodontitis leading to premature loss of teeth. A 4-member family diagnosed with Ehlers-Danlos syndrome type VIII was referred from the genetic department for oral evaluation. A 30-year-old woman reported early tooth loss. Her 26- and 27-year-old brothers and her 5-year-old daughter reported tooth loss, gingival bleeding, and dental mobility.
Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics - Category: ENT & OMF Authors: Tags: Oral and Maxillofacial Pathology Source Type: research
CONCLUSION: We reported an extremely rare case of pEDS with missense mutation in C1R in a Chinese family, with coexistence C1R and COL3A1 mutations in the proband who was suspected to suffer from vascular EDS as well. To our knowledge, this is the first case of coexistence of two forms of EDS in a single individual. This article is protected by copyright. All rights reserved. PMID: 30025171 [PubMed - as supplied by publisher]
Source: Journal of Clinical Periodontology - Category: Dentistry Authors: Tags: J Clin Periodontol Source Type: research
Ehlers-Danlos Syndrome (EDS) is a connective tissue disorder resulting in abnormal collagen synthesis, leading to skin, joint, ligament, blood vessel and other organ abnormalities. Studies in adult women with EDS show that 33-76% report heavy menstrual bleeding (HMB) and 72-93% report dysmenorrhea. The purpose of this study was to evaluate gynecologic complaints in pediatric and adolescent patients with EDS and their management by Pediatric and Adolescent Gynecology (PAG) providers.
Source: Journal of Pediatric and Adolescent Gynecology - Category: OBGYN Authors: Source Type: research
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