CRP and gangrene: CRP: 45 mg/L  ESR: 40 mm/h  DSA: multiple microaneurysms involving bilateral interlobar and segmental branches of renal artery, branches of gastroduodenal artery, distal branches of SMA and IMA  GI Biopsy:  Ulcer, ischemic, gangrene, perforation in ileum. Chronic inflammation in recto-sigmoid junction  Plasma ADA2 activity: 1.1 mU/g protein mL  Plasma ADA2 activity (Father): 42.5 mU/g protein mL  Plasma ADA2 activity (Mother): 69.5 mU/g protein mL