X-linked agammaglobulinemia - first case with Bruton tyrosine kinase mutation from Pakistan.

Non-occlusive mesenteric ischemia (NOMI) is a severe pathological condition characterized by signs and symptoms of bowel obstruction, intestinal necrosis resulting from acute and/or chronic inadequate blood perfusion, in the absence of an organic vascular obstruction detectable by imaging techniques. A 64 years old man case with a history of Parkinson's disease in high-functioning levodopa treatment is presented. Clinical and radiological signs of intestinal obstruction were observed. He underwent surgical operation with total colectomy and terminal ileostomy for generalized secondary peritonitis due to perforation of sigmoid colon. Ischemic pancolitis was first suspected. In third post-operative day a contrastenhanced CT scan was performed in the evidence of fever and sub-occlusive symptoms. It was found absence of reliable evidence of vascular changes; superior mesenteric artery and vein patency is maintained A NOMI was then diagnosed. NOMI represents about 0.04% of mesenteric artery diseases. It is correlated with a poor prognosis with a mortality estimated of 70-90%. Parkinson's disease, considering neurodegenerative alterations that characterize it, can be considered as a predisposing factor. The combined treatment with high doses of levodopa and vasodilators, such as PGE (Prostaglandin E), can contribute to an improvement in prognosis.