[Pseudoxanthoma elasticum. A rare cause of upper digestive hemorrhage]. 1992

G Bonotto, and F Tonetto, and E Baraglia, and M Battistioli, and F Fabi, and M Arrigoni, and R Spolaore
Divisione di Chirurgia Generale, USSL 11-Oderzo, Treviso.

Pseudoxanthoma Elasticum (PXE) is a rare inherited elastic tissue disorder with degeneration of elastic fibres involving mainly skin, eyes and the cardiovascular system. Upper gastro-intestinal hemorrhage occurs in 13% of cases and is often resistant to non-surgical methods of treatment. One case report involving recurrent gastric hemorrhage in a young patient with PXE and treated with total gastrectomy is presented. Diagnosis was made, after surgery, by biopsy of skin lesions of the neck. The characteristics of PXE are reviewed and the case is discussed.

UI MeSH Term Description Entries
D011561 Pseudoxanthoma Elasticum An inherited disorder of connective tissue with extensive degeneration and calcification of ELASTIC TISSUE primarily in the skin, eye, and vasculature. At least two forms exist, autosomal recessive and autosomal dominant. This disorder is caused by mutations of one of the ATP-BINDING CASSETTE TRANSPORTERS. Patients are predisposed to MYOCARDIAL INFARCTION and GASTROINTESTINAL HEMORRHAGE. Gronblad-Strandberg Syndrome,Groenblad-Strandberg Syndrome,Pseudoxanthoma Elasticum, Autosomal Dominant,Pseudoxanthoma Elasticum, Forme Fruste,Pseudoxanthoma Elasticum, Incomplete,Elasticum, Incomplete Pseudoxanthoma,Elasticums, Incomplete Pseudoxanthoma,Gronblad Strandberg Syndrome,Incomplete Pseudoxanthoma Elasticum,Incomplete Pseudoxanthoma Elasticums,Pseudoxanthoma Elasticums, Incomplete,Syndrome, Gronblad-Strandberg
D005260 Female Females
D005743 Gastrectomy Excision of the whole (total gastrectomy) or part (subtotal gastrectomy, partial gastrectomy, gastric resection) of the stomach. (Dorland, 28th ed) Gastrectomies
D006471 Gastrointestinal Hemorrhage Bleeding in any segment of the GASTROINTESTINAL TRACT from ESOPHAGUS to RECTUM. Hematochezia,Hemorrhage, Gastrointestinal,Gastrointestinal Hemorrhages,Hematochezias
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D001706 Biopsy Removal and pathologic examination of specimens from the living body. Biopsies
D012867 Skin The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.
D013270 Stomach An organ of digestion situated in the left upper quadrant of the abdomen between the termination of the ESOPHAGUS and the beginning of the DUODENUM. Stomachs

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