Systemic vasculitis presenting with massive bowel infarction.

Gastrointestinal involvement in systemic vasculitis occurs in up to 30% of patients. Fortunately intestinal infarction is a rare complication, but if present carries a high mortality, and swift management of the underlying vasculitis is crucial. Two such cases are described.     

Hemorrhagic fever with renal syndrome presenting with hemophagocytic lymphohistiocytosis

Hemophagocytic lymphohistiocytosis--which is associated with a variety of infections, malignant neoplasms, autoimmune diseases, and immunodeficiencies--is an uncommon syndrome with a rapidly fatal outcome. We describe the first case of hemorrhagic fever with renal syndrome due to Hantaan virus presenting with reactive hemophagocytosis.     

Ehlers-Danlos syndrome: case and pedigree report and review

OBJECTIVE: To report a case and a firstly described pedigree with Ehlers-Danlos syndrome in China. METHODS: Clinical materials of a case of Ehlers-Danlos syndrome type IV and a pedigree of 6 members with 4 generations were analyzed. Dilated internal jugular vein in the proband was removed operatively. The diagnosis, surgical treatment, and postoperative complications were retrospectively summarized. Etiology, clinical characteristics and classification of Ehlers-Danlos syndrome were also analysed. RESULTS: Vessels of the proband in the pedigree were crisp and easily lacinated during the precedure for removal of his internal jugular vein. Repeat postoperative hemotomas were found though complete stanching was made during operation. The patient successfully recovered after prompt debridement in operative theatre and needle sucking. The other 5 members of the pedigrees all had the triads of Ehlers-Danlos syndrome, but no combined vascular diseases were found in them. CONCLUSIONS: Though the morbidity rate was extremely low, green attention should be paid to the high mortality rate and complication of Ehlers-Danlos syndrome during surgical management of in patients with Ehlers-Danlos syndrome type IV. Surgeons should be aware of the ponderance of its complications and combined diseases to avoid fatal intraoprative vascular lacination and incontrollable hamorrhage.