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

Nutritional status in patients with hepatitis C

To assess the nutritional status via the SGA [subjective global assessment] screening tool of patients at all stages of hepatitis C virus [HCV] liver disease. Descriptive study. Out-patient Clinics of the Aga Khan University Hospital, Karachi, conducted from October 2009 to January 2011. Patients with hepatitis C virus infection and their HCV-negative attendants were enrolled from the outpatient clinics, and categorized into 4 groups of 100 patients each: healthy controls [HC], those with chronic hepatitis C infection [CHC], compensated cirrhotics [CC] and decompensated cirrhotics [DC]. The validated subjective global assessment [SGA] tool was used to assess nutritional status. A total of 400 patients were enrolled. Most of the patients in the HC group were class A [best nutritional status]. In contrast, the majority [64%] in the DC group were in the class C [worst status]. The compensated cirrhosis [CC] group showed that 90% of patients were malnourished, while 98% of all patients were malnourished in the DC group, predominantly class C. Most importantly, 14% of patients with chronic hepatitis C [CHC] also scored a B on the SGA; which when compared to HC was statistically significant [p=0.005]. As the groups progressed in their disease from CHC to DC, the transition in nutritional status from A to C between groups was statistically significant. Malnutrition occurs early in the course of HCV, and progresses relentlessly throughout the spectrum of HCV disease

Efficacy of L-ornithine-L-aspartate as an adjuvant therapy in cirrhotic patients with hepatic encephalopathy

To evaluate the efficacy of L-ornithine-L-aspartate [LOLA] as an adjuvant therapy in cirrhotic patients with hepatic encephalopathy [HE]. Randomized placebo controlled study. The Aga Khan University Hospital, Karachi in the year 2003-2004. Patients with HE were randomized to receive LOLA or placebo medicine as an adjuvant to treatment of HE. Number connection test-A [NCT-A], ammonia level, clinical grade of HE and duration of hospitalization were assessed. Out of 120 patients, there were 62 males with mean age of 57 +/- 11 years. Improvement in HE was higher [n=40, 66.7%] in LOLA group as compared to the placebo group [n=28, 46.7%, p=0.027]. In patients with grade I or less encephalopathy, improvement was seen in 6 [35.3%] and 3 [20%] patients in LOLA and placebo groups respectively [p=0.667]. Patients with HE grade II and above showed improvement in 34 [79.1%] and 25 [55.6%] cases in LOLA and placebo group respectively [p=0.019]. On multivariate analysis patients with HE of grade II and above showed prothrombin time, creatinine level and use of LOLA influencing the outcome. Duration of hospitalization was 93.6 +/- 25.7 hours and 135.2 +/- 103.5 hours in LOLA and placebo groups respectively [p=0.025]. No side effects were observed in either groups. In cirrhotic patients with advanced hepatic encephalopathy treatment with LOLA was safe and associated with relatively rapid improvement and shorter hospital stay

Haemobilia: a rare cause of gastrointestinal bleeding

Iatrogenic injury to hepatic duct leading to pseudoaneurysm and haemobilia can occur following laparoscopic cholecystectomy. We report a case of a 60 years old man presenting with haematemesis found to have pseudoaneurysm of accessory hepatic artery 4 months after laparoscopic cholecystectomy. Diagnosis was made by computed tomography [CT] scan followed by celiac and mesenteric artery angiogram. The bleeding was successfully treated with coil embolization

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

Frequency and characteristic features of portal hypertensive gastropathy in patients with viral cirrhosis

To determine the frequency and specific characteristic features of portal hypertensive gastropathy [PHG] in cirrhosis due to viral etiology. Cross-sectional descriptive study. The Aga Khan University Hospital, Karachi, from June 2006 till June 2008. Patients with hepatitis B and C cirrhosis were included who underwent screening esophago-gastro-duodenoscopy [EGD] for varices. Baveno III consensus statement was used for diagnosing PHG on endoscopy and divided them into two subgroups i.e. mild and severe. Data related to platelet/spleen ratio, MELD score and Child Turcotte Pugh [CTP] score indicating severity of cirrhosis were recorded in all patients. Findings were compared by using independent sample t-test. Out of 360 patients who underwent screening EGD, 226 [62.8%] were males. Two hundred and eighty one [78%] had hepatitis C while 79 [22%] suffered from hepatitis B related cirrhosis. Three hundred patients [83.3%] had PHG while 71 [24%] had severe PHG. Higher proportion of esophageal varices [89.7%] was present among those who had PHG [p < 0.001]. On univariate analysis lower platelet counts [117 +/- 55 vs. 167 +/- 90; p < 0.001], increased spleen size [14.1 +/- 2.9 cm vs. 12 +/- 2.4cm; p < 0.001] were found in PHG patients as compared to those without it. Similarly, lower platelet/spleen ratio was noted in patients with severe PHG [916 +/- 400 vs. 1477 +/- 899; p < 0.001]. Furthermore, on multivariate analysis CTP score > 8 MELD score > 12 and platelets/spleen ratio < 900 were significantly associated factors with severe PHG. Frequency of PHG was 83% while severe PHG was seen in 24% cases of viral hepatic cirrhosis. MELD score > 12, CTP score >/= 8 and platelets/spleen ratio