Ehlers-Danlos Syndrome Presenting with Infarction of Stomach | oneedsvoice

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Ehlers-Danlos Syndrome Presenting with Infarction of Stomach

key information

source: Journal of the Royal Society of Medicine

year: 1988

authors: Shaikh NA, Turner DT

summary/abstract:

Ehlers-Danlos syndrome was first described by Ehler (1901) and well documented by Danlos (1908). It is an inherited disorder of connective tissue with many complications involving the gastrointestinal system, but infarction of the stomach has not previously been reported.

Case report
The patient, a 17-year-old girl, a known case of Ehler-Danlos Syndrome(EDS), presented with a 14 h history of generalized abdominal pain, worse in the upper abdomen. The pain radiated to the back. She had been vomiting, starting 2 h after onset of pain and continued with approximately 30-60 min intervals. She had one loose motion 8 h prior to admission; there was no mucus nor blood in the stools. Her previous malformations which had required surgical correction had been scoliosis, and dislocated hip and knee joints. On examination, she was toxic, febrile with a pulse of 130/min and a blood pressure of 130/95 mmHg. Abdominal examination revealed a cyanotic tinge to the skin from nipple line down. It was distended with generalized tenderness and guarding. The bowel sounds were present.

organisation: Pilgrim Hospital Lincolnshire

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