• Journal of Chest Surgery
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• 제43권3호
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• pp.336-339
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• 2010

제 4형 Ehlers-Danlos 증후군 혈관 침범형 환자에서 발생한 자발적 혈흉을 개흉술 후 거즈를 충전 압박하여 성공적으로 지혈하였다. 지혈이 어려울 것으로 예상되는 환자에서 지혈 목적의 개흉 수술은 최후의 방법으로 신중히 고려되어야 할 것으로 보인다.

• The Journal of Advanced Prosthodontics
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• 제7권2호
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• pp.178-182
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• 2015

Ehlers-Danlos syndrome is an autosomal dominant hereditary disorder of connective tissue, while familial gigantiform cementoma is a condition that usually manifests as multiple radiopaque cementum-like masses throughout the jaws. This case report discusses the oral management and prosthetic rehabilitation of two patients presenting familial gigantiform cementoma with Ehlers-Danlos Syndrome.

• Journal of Chest Surgery
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• 제33권11호
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• pp.910-914
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• 2000

Ehlers-Danlos 증후군 중 제4형은 다른 형들과 마찬가지로 피부 손상이 쉽게 오고, 쉽게 멍이 들고, 관절이 잘 휘어지는 등의 신체적인 특징을 가기지만 대혈관을 침범하여 때로는 치명적인 출혈을 일으키기도 한다. 아주 작은 충격이나 힘에도 혈관들이 쉽게 찢어지거나 때로는 심각한 손상을 임기 때문에 수술 적인 치료를 하는데 많은 위험이 있을 수 있고 심지어는 이득을 얻지 못하는 경우도 있다. 저자들은 Ehlers-Danlos 증후군을 동반한 급성 대동맥박리의 성공적인 수술적 치료를 보고하는 바이다.

• 대한수의학회지
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• 제47권1호
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• pp.91-94
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• 2007

tisue disease of dog and cat, resembling Ehlers-Danlos syndrome (EDS) in man. EDS is characterizedby loose, hyperextensible and, fragile skin, it is attributed to mutations in connective tisue gene. Thesemutations cause defects in type I or II collagen synthesis and as a result lack of strength or elasticityto skin, joint, ligament and vessels. EDS-affected animals often experience subcutaneous hematomas thathave long bleeding times. The 4-years old male Yorkshire terrier was evaluated because of subcutaneoushematoma after stifle surgery. Clinical examination revealed a thin and hyperextensible skin and jointlaxity. The degree of skin extensibility index was 23.4%, marked skin stretchy. Clinical diagnosis wasconfirmed by histophathological examination of a skin biopsy revealing reduced packing density ofcollagen fiber of skin.

• 대한수의학회지
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• 제56권1호
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• pp.53-55
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• 2016

A 1-year-old Korean domestic short-haired cat presented with skin hyperextensibility and a severely macerated wound on the skin of the dorsal part of the neck. Diagnostic studies including histopathology and skin extensibility index revealed congenital cutaneous asthenia (Ehlers-Danlos syndrome). In this cat, the skin wounds and defects were successfully managed with standard wound management and cosmetic surgery. Although skin hyperextensibility is persistent, the cat has lived well without other complications to date. To the best of our knowledge, this is the first report of cutaneous asthenia in a cat in Korea.

• Journal of Yeungnam Medical Science
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• 제33권1호
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• pp.44-47
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• 2016

Primary dissection of the renal artery is rare. Spontaneous renal artery dissection can be associated with diseases such as medial degeneration, neurofibromatosis, syphilitic arteritis, tuberculosis, polyarteritis nodosa, Marfan syndrome, fibromuscular dysplasia, or Ehlers-Danlos syndrome (EDS). Among these causes, EDS related renal artery dissection is very rare worldwide and has not been previously reported in Korea. EDS are a group of heritable connective tissue disorders characterized by fragility of the skin and hypermobility of the joints. We describe the case history of a young man who presented with left side flank pain, hypermobility of the hand joints and showed left renal artery dissection on computed tomography and angiography that turned out to be the first complication of vascular type EDS.

• 대한두개안면성형외과학회지
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• 제20권2호
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• pp.130-133
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• 2019

Ehlers-Danlos syndrome (EDS) is an inherited disorder of collagen biosynthesis and structure, characterized by skin hyperextensibility, joint hypermobility, aberrant scars, and tissue friability. Besides the skin, skeleton (joint) and vessels, other organs such as the eyes and the intestine can be affected in this syndrome. Accordingly, interdisciplinary cooperation is necessary for a successful treatment. Three basic surgical problems are arising due to an EDS: decreased the strength of the tissue causes making the wound dehiscence, increased bleeding tendency due to the blood vessel fragility, and delayed wound healing period. Surgery patients with an EDS require an experienced surgeon in treating EDS patients; the treatment process requires careful tissue handling and a long postoperative care. A surgeon should also recognize whether the patient shows a resistance to local anesthetics and a high risk of hematoma formation. This report describes a patient with a wide open wound on the foot dorsum and delayed wound healing after the primary approximation of the wound margins.

• Journal of Korean Neurosurgical Society
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• 제55권2호
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• pp.92-95
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• 2014

Ehlers-Danlos syndrome (EDS) is a rare inherited connective disease. Among several subgroups, type IV EDS is frequently associated with spontaneous catastrophic bleeding from a vascular fragility. We report on a case of carotid-cavernous fistula (CCF) in a patient with type IV EDS. A 46-year-old female presented with an ophthalmoplegia and chemosis in the right eye. Subsequently, seizure and cerebral infarction with micro-bleeds occurred. CCF was completely occluded with transvenous coil embolization without complications. Thereafter, the patient was completely recovered. Transvenous coil embolization can be a good treatment of choice for spontaneous CCF with type IV EDS. However, every caution should be kept during invasive procedure.

• Journal of Korean Neurosurgical Society
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• 제48권6호
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• pp.528-531
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• 2010

Ehlers-Danlos syndrome (EDS) type IV is characterized by its clinical manifestations, which are easy bruising, thin skin with visible veins, and rupture of arteries, uterus, or intestines. Arterial complications are the leading cause of death in vascular EDS because they are unpredictable and surgical repair is difficult due to tissue fragility. The authors report a case presented with cervical radiculopathy due to a segmental fusiform aneurysm of the cervical vertebral artery. Transfemoral cerebral angiography (TFCA) was done to verify the aneurysmal dilatation. However, during TFCA, bleeding at the puncture site was not controlled, skin and underlying muscle was disrupted and profound bleeding occurred during manual compression after femoral catheter removal. Accordingly, surgical repair of the injured femoral artery was performed. At this time it was possible to diagnose it as an EDS with fusiform aneurysm on cervical vertebral artery. Particularly, cervical fusiform aneurysm is rare condition, and therefore, connective tissue disorder must be considered in such cases. If connective tissue disorder is suspected, the authors suggest that a noninvasive imaging modality, such as, high quality computed tomography angiography, be used to evaluate the vascular lesion to avoid potential arterial complications.

• Journal of Genetic Medicine
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• 제16권1호
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• pp.43-47
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• 2019

Ehlers-Danlos syndrome (EDS) VIII is an autosomal dominant inherited connective tissue disorder characterized by intractable periodontal inflammation, absence of gingiva, pretibial plaques, skin hyperextensibility, joint hypermobility, and tissue fragility with onset in the childhood or adolescence. In a recent report, heterozygous variants of the C1R or C1S related to the classical complement pathway were identified in families with history of EDS VIII. The current report describes a Korean 34-year-old female carrying a novel missense variant of C1R c.925T>G (p.Cys309Gly) and exhibiting early severe periodontitis, skin fragility, and joint hypermobility. The patient also had frontal, parietal, and temporal white matter brain lesions without definite vascular abnormalities on brain magnetic resonance imaging, which have not been surveyed meticulously in EDS VIII. Considering the genetic alteration of classic complement pathways in this condition, it is necessary to carefully observe multisystemic inflammation processes such as changes in brain white matter.