incidentally discovered asymptomatic para-aortic paraganglioma with peutz-jeghers syndrome

Peutz-Jeghers syndrome [PJS] is an autosomal dominant inherited disorder characterized by mucocutaneous melanin pigmentation and gastrointestinal [GI] tract hamartomatous polyps and an increased risk of malignancy. In addition to polyposis, previous studies have reported increased risk of GI and extra GI malignancies in PJS patients, compared with that of the general population. The most common extraintestinal malignancies reported in previous studies are pancreatic, breast, ovarian and testicular cancers. We report the case of a 17-year-old boy who presented with generalized weakness, recurrent sharp abdominal pain and melena, had exploratory laparotomy and ileal resection for ileo-ileal intussusception. Pigmentation of the buccal mucosa was noted. An abdominal computed tomography scan [CT] revealed multiple polyps in small bowel loops. Gastroscopy revealed multiple dimunitive polyps in stomach and pedunculated polyp in duodenum. Colonoscopy revealed multiple colonic polyps. Pathological examination of the polyps confirmed hamartomas with smooth muscle arborization, compatible with Peutz-Jeghers polyps. CT scan guided left para-aortic lymph node biopsy revealed the characteristic features of extra-adrenal para-aortic paraganglioma. Although cases of various GI and extra GI malignancies in PJS patients has been reported, the present case appears to be the first in literature in which the PJS syndrome was associated with asymptomatic extraadrenal para-aortic paraganglioma. Patients with PJS should be treated by endoscopic or surgical resection and need whole-body screening

Factors predicting the recurrence of spontaneous bacterial peritonitis in patients with cirrhosis

To evaluate the frequency of recurrence of spontaneous bacterial peritonitis [SBP] in patients with end stage liver disease and the factors responsible for it. Descriptive study. The Aga Khan University Hospital, Karachi, from November 2008 till November 2009. Patients with cirrhosis who were admitted at AKUH with diagnosis of SBP during the study period were included. Any episode of SBP after resolution of the first index case of SBP within one year was considered as recurrence. Out of 238 cirrhotic patients, 157 [66%] had single, while 81 [34%] had recurrent episodes of SBP. History of using proton pump inhibitors [PPI] and diuretics was found in 113 [47.5%] and 139 [58.4%] patients respectively. Only 58 [24.4%] patients were on prophylactic antibiotic therapy. Univariate analysis revealed that the female gender [52%], and presence of porto-systemic encephalopathy [PSE, 31%] were statistically significant [p=0.03] among those who had recurrent SBP. On multivariate analysis bilirubin level of > 1.0 mg [OR=7.03; 95%CI=1.55-32], protective factor of hepatitis B [OR 0.31; 95%CI=0.13-0.70] and presence of urinary tract infection [UTl] [OR=2.24; 95%CI=0.99-5.09] were significant in patients with recurrent SBP. Recurrent SBP was noticed in 34% patients. Serum bilirubin level of > 1.0 mg, protective factor of HBV and presence of UTl were significant factors present in patients with recurrent SBP