Document Detail


Ehlers-Danlos syndrome type IV with gastric adenocarcinoma.
MedLine Citation:
PMID:  18672610     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Ehlers-Danlos syndrome has many subtypes. The vascular type (type IV) is characterized by thin, translucent skin, easy bruising, characteristic facial appearance, and arterial, intestinal, and/or uterine fragility. OBJECTIVES: To encourage a better understanding of vascular EDS as a basis for early diagnosis, prevention, and management of complications. A first case of EDS type IV with adeno-carcinoma of the stomach in Thailand was reported and literature was reviewed. RESULT: A 62-year-old Thai priest was admitted in Priest Hospital because of progressive muscle weakness of both legs with neurogenic claudication from compression fracture of L1-2. Abdominal aortic aneurysm were detected with upper gastrointestinal hemorrhage, esophagogastroduodenoscopy showed diffuse gastric body swelling and erythema resulting in chronic gastritis. Gastric biopsy was indicative of adenocarcinoma of the stomach and gastrectomy was done. Dermatologists were consulted due to generalized cutaneous pain, easy bruising following venepuncture, and EKG padding. A vascular EDS type IV was diagnosed. CONCLUSION: After gastrectomy, the patient became drowsy and unconscious from profuse recurrent cerebral hemorrhage and expired.
Authors:
Rajyani Kanechorn Na Ayuthaya; Nonthaya Patthamapasphong; Thanyachai Sura; Nucha Niumpradit; Objoon Trachoo
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of the Medical Association of Thailand = Chotmaihet thangphaet     Volume:  91 Suppl 1     ISSN:  0125-2208     ISO Abbreviation:  J Med Assoc Thai     Publication Date:  2008  
Date Detail:
Created Date:  2008-08-04     Completed Date:  2008-09-10     Revised Date:  2008-09-18    
Medline Journal Info:
Nlm Unique ID:  7507216     Medline TA:  J Med Assoc Thai     Country:  Thailand    
Other Details:
Languages:  eng     Pagination:  S166-71     Citation Subset:  IM    
Affiliation:
Department ofDermatology, Phramongkutklao Hospital, Thailand.
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MeSH Terms
Descriptor/Qualifier:
Adenocarcinoma / complications*,  physiopathology,  surgery
Collagen Type III / genetics
Ehlers-Danlos Syndrome / etiology*,  genetics
Fatal Outcome
Gastrectomy
Humans
Male
Middle Aged
Stomach Neoplasms / complications*,  physiopathology,  surgery
Chemical
Reg. No./Substance:
0/COL3A1 protein, human; 0/Collagen Type III

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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