Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0740577 (acute abdominal pain)
 1,982 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Marfan syndrome is multisystem connective tissue disorder that primarily involves the skeletal, cardiovascular, and ocular systems. The gastrointestinal complications in Marfan syndrome are rare, with only a few case reports described in the literature. We present a 25-year-old woman who presented with acute abdominal pain for 1 day. The imaging features revealed complex diaphragmatic hiatus hernia with organoaxial gastric volvulus. This is a unique case report about an adult patient with Marfan syndrome who presented with symptomatic paraesophageal hernia and organoaxial gastric volvulus.
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PMID:Unusual presentation of adult Marfan syndrome as a complex diaphragmatic hiatus hernia. 2494 65

Paraesophageal hernias are considered to be benign entities which are usually managed conservatively. We present a case of a middle-aged male with no previous history of esophageal hernia who presented with acute chest and abdominal pain. The patient was diagnosed to have a type 2 paraesophageal hernia with gastro-thorax. Laparotomy was performed during which it was found that herniated segment of the stomach had strangulated and gangrenous. Thoracotomy was performed and gangrenous stomach segment resected. A roux-en-Y esophago-jejunostomy was performed. Diaphragmatic defect was plicated. Patient recovered with adequate post operative support. A review of the literature revealed that paraesophageal hernias presenting as acute abdominal pain is a rare clinical entity and those with gastric gangrene is even rarer, with high mortality rates. We suggest that paraesophageal hernias require to be managed actively considering the seriousness of potential complications and the relative safety of newer elective surgical modalities. A high index of suspicion is needed in order to avoid missing this diagnosis in patients presenting with chest pain.
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PMID:Gastric Gangrene Due to a Strangulated Paraesophageal Hernia-a Case report. 2597 48