Immunoproliferative small intestinal disease [IPSID]

This study describes the frequency, demographics, clinical presentation, endoscopic findings, histopathological features, treatment and outcome of 'Immunoproliferative small intestinal disease' [IPSID]. Archives contained a total of 27 cases of IPSID diagnosed and treated over an 18-year period. A M: F ratio of 2.4:1 was seen with a mean and median ages of 28.7 and 25 years. Most patients [68.8%] presented with abdominal pain and diarrhoea. In the majority [62.5%], duodenum was the primary site of involvement. Endoscopy showed polypoidal, raised or flat lesions. Biopsy findings included blunting or flattening of villi with dense plasma cell infiltrate and lymphoepithelial lesions. Twenty-four cases were categorized as stage A and B [benign and intermediate] and three were categorized as stage C [malignant, diffuse large B-cell lymphoma with plasmacytoid features]. Stage A and B patients responded well to antibiotic treatment [tetracycline] with regression of the lesions while for stage C patients standard CHOP chemotherapy was administered

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

Gastric MALT lymphoma: a rarity

Association of Helicobacter pylori [H.Pylori] infection with gastric 'Mucosa Associated Lymphoid Tissue' [MALT] lymphomas [MALTomas] is well established. In this study the frequency and characteristics of gastric MALToma over a period of 18 years was evaluated. During this period 24 patients with gastric MALToma were diagnosed; out of them, 13 [54.2%] had active H.Pylori infection. The mean and median age was 49.7 and 53.5 years. The male: female ratio was 17:7. The common presenting complaints were epigastric pain [n=10] and dyspepsia [n=9]. Endoscopic findings revealed mild gastric hyperemia [n=16], superficial erosions [n=4] and superficial ulcers [n=4]. It was concluded that the prevalence of gastric MALToma was very low in contrast to a high H. pylori gastritis in the Pakistani population

Size or the number of portal tracts: which matters in a liver biopsy core in chronic hepatitis C?

Liver biopsy has an important role in staging of fibrosis [SoF] and grading of inflammation [GoI] in chronic hepatitis C [CHC] patients. The effect of size and number of portal tracts [NoP] on grading and staging of liver biopsy was evaluated. A total of 150 consecutive liver biopsy core [LBC] of patients with CHC were obtained. There were 98 [65.3%] males. Mean length of LBC was 1.45 +/- 0.48 cm. Mean number of portal tracts [NoP] was 11 +/- 4.6. Mean length of LBC was greater [1.60 +/- 0.45 cm] in stage 4 [n=41; 27.3%] and lesser [1.28 +/- 0.39] in stage 1 [n=23; 15%, p=0.04]. The mean NoP were 8.5, 10.6 and 13.1 in GoI 1, 2 and 3 respectively [p < 0.001]. The mean NoP were 7.6, 11.1, 11.3 and 14.5 in SoF 1, 2, 3 and 4 respectively [p < 0.0001]. There was a good correlation between number of portal tracts and length of LBC [r[2]=0.56]

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

Liver biopsy for histological assessment - the case in favor

Liver biopsy [LB] is the gold standard method for assessment of liver histology. It provides valuable, otherwise unobtainable information, regarding the degree of fibrosis, parenchymal integrity, degree and pattern of inflammation, bile duct status and deposition of materials and minerals in the liver. This information provides immense help in the diagnosis and prognostication of a variety of liver diseases. With careful selection of patients, and performance of the procedure appropriately, the complications become exceptionally rare in current clinical practice. Furthermore, the limitations of sampling error and inter-/intra-observer variability may be avoided by obtaining adequate tissue specimen and having it reviewed by and experienced liver pathologist. Current noninvasive tools are unqualified to replace LB in clinical practice in the face of specific limitations for each tool, compounded by a poorer performance towards the assessment of the degree of liver fibrosis, particularly for intermediate stages

Precipitating factors and the outcome of hepatic encephalopathy in liver cirrhosis

To determine precipitants of hepatic encephalopathy [HE] and their impact on hospital stay and mortality. Cross-sectional, analytical study. The Aga Khan University Hospital, from January 2005 to December 2007. Consecutive patients admitted with different grades of HE were evaluated between January 2005 and December 2007. The precipitants of HE were correlated with the different grades of HE, and length of hospital stay and mortality. Chi-square test was used to compare the proportion of precipitating factors versus hospital stay and grade with significance at p < 0.05. Of the 404 patients 252 [62%] were males. Hepatitis C virus was the cause of cirrhosis in 283 [70%]; Child Turcotte Pugh [CTP] class C was present in 317 [78%] patients. On presentation, 17% patients had grade 1 HE while 44%, 29% and 10% had grades 2, 3 and 4 respectively. The most common precipitant of HE was spontaneous bacterial peritonitis in 83 [20.5%], constipation in 74 [18.3%] and urinary tract infection in 62 [15.3%]. One hundred and forty [35%] patients had >/= 2 precipitating factors while no precipitant was noted in 50 [12%] patients. Mean hospital stay was 4 +/- 3 days. The lesser the number of precipitants, shorter was the length of stay [p < 0.01] and lesser was the grade of HE [p=0.025]. Complete reversal of HE was noted in 366 patients [91%] while the remaining had grade 1 HE on discharge. Nine [2.2%] patients died during the hospital stay. No mortality was noted in patients without precipitants. Patients presenting with >/= 2 precipitating factors and advanced grade of HE had a prolonged hospital stay. Moreover, patients without precipitants had better outcomes

Hepatic angiosarcoma with metastasis to small intestine

Hepatic angiosarcomas are rare tumours with poor prognosis, with patients usually dying within 6 months. Metastases mainly occur in lymph nodes, spleen, lungs, bones and adrenals. Metastasis to small intestine is even rarer. Similarly, primary or metastatic angiosarcomas in small intestine are extremely rare, often present with recurrent gastrointestinal bleeding and anemia, and have an extremely poor prognosis. Both primary or metastatic intestinal angiosarcomas may exhibit epithelioid morphology. It may be very difficult to differentiate between primary and secondary cases in intestine and especially when the tumour exhibits epithelioid morphology

Sclerotherapy plus octreotide versus sclerotherapy alone in the management of gastro - oesophageal variceal hemorrhage

There are different ways for controlling oesophageal variceal bleed which include pharmacological and endoscopic methods. In this study we compare efficacy of octreotide [50 g/hr for 48 hours] combined with sclerotherapy versus sclerotherapy alone in patients with acute bleeding from gastro-oesophageal varices [GOV]. It was a randomized clinical controlled trial conducted at Aga Khan University Hospital, Karachi, from January 1997 to December 1998. We evaluated the role of octreotide [50mcg/hr for 48 hours] combined with sclerotherapy versus sclerotherapy alone in a total of 105 adult cirrhotic patients who had acute bleeding from GOV. Patients were assigned to receive octreotide plus sclerotherapy or sclerotherapy alone. Primary outcome measure was 5-day survival without rebleeding. The hospital stay in days and blood transfusion requirements were also compared in the combined treatment group versus sclerotherapy alone group. Initial control of bleeding was achieved in 46/51 [90.2%] patients who received combined treatment compared to 41/54 [75.9%] patients [p=0.05] in sclerotherapy alone group. Rebleeding after the first 48 hours was less in the octreotide treated patients 2/46 vs. 8/41 patients [p=0.003]. The octreotide treated patients had a better short term [5 days] survival without rebleeding 44/51 vs. 33/54 [p=0.003] and shorter hospital stay, 5.31 +/- 3.87 days vs. 6.63 +/- 3.86 [p=0.008] as compared to sclerotherapy alone group. The blood transfusion requirement was also less in the combined treatment group 3.88 +/- 2.80 vs. 5.37 +/- 3.15 units [p=0.002]. 1] The combination of sclerotherapy, and octreotide infusion over 48 hours is more effective than sclerotherapy alone in the treatment of acute variceal bleeding and prevention of early rebleed in cirrhotic patients. 2] It leads to shorter hospital stay and 3] less blood transfusion requirements. 4] Although early survival without rebleeding is improved, the overall mortality at the end of hospitalization period is similar in the two groups of treated patients

audit of indications and complications of percutaneous endoscopic gastrostomy

To evaluate the indications and complication rate in fifty consecutive patients in whom percutaneous endoscopic gastrostomy [PEG] was attempted. A descriptive study. Fifty consecutive adults referred to our unit for consideration for primary percutaneous endoscopic gastrostomy feeding between November 1999 and October 2001 at The Aga Khan University Hospital, Karachi and Isra University Hospital, Hyderabad were evaluated prospectively. PEG insertions were successful in all fifty patients [29 Males, 21 Females]. The indications for PEG were neurogenic [80%], recurrent aspiration, not the gastric contents [8%], head and neck tumors [6%], esophageal tumor [4%] and dermatomyositis [2%]. Ninety six percent of the patients had the procedure as inpatients while 4% were outpatients when the procedure was performed. Eighty percent of the patients had at least one follow-up after the tube insertion. Major complications were peritonitis [2%] and aspiration of gastric contents [4%]. Minor complications were PEG site infection [16%], accidental tube removal [10%], PEG migration [6%] and PEG blockage [4%]. One patient died due to peritonitis. Oral feed was resumed in 12% of the patients and the tube was removed subsequently. PEG is a feasible and safe procedure in our set up. It provides adequate feeding support and has low mortality rate even in patients, debilitated secondary to their underlying disease

Factors predicting hyperkalemia in patients with cirrhosis receiving spironolactone

To evaluate the factors leading to hyperkalemia in patients with cirrhosis receiving spironolactone. Design: An observational, analytical, case control study. Place and Duration of Study: The Aga Khan University Hospital. Six months. Subjects and One hundred and fifty consecutive patients [100 males, 50 females] with cirrhosis of liver, hospitalized for decompensated disease and receiving spironolactone for at least two weeks before admission, were included in this study. Patients with hyperkalemia [n=67] having potassium level >5 mmol/l were compared with patients who had normal potassium level 5 mmol/l [n=83]. The parameters taken into account were age, gender of the patients, type and dose of diuretics along with concomitant medicines, diastolic blood pressure, edema, ascites, blood urea nitrogen, serum creatinine, electrolytes, bilirubin, albumin, prothrombin time, Child class, and Child Pugh score. Patients with hyperkalemia [K > 5 mmol/l] had higher blood urea nitrogen, serum creatinine and bilirubin levels [p= 0.004, 0.001 and 0.044 respectively]. Their serum sodium and albumin levels were lower [p= 0.000 and 0.017 respectively]. They had advanced cirrhosis with high Pugh score [p= 0.003]. These patients were on higher dose of spironolactone [p =0.001]. Multivariate analysis showed that dose of spironolactone >100 mg /day, serum creatinine >1.3 mg/dl, persistence of ascites and edema, and female gender were important predictors of development of hyperkalemia. Patients with cirrhosis receiving high dose of the diuretic, having edema, ascites and high serum creatinine are at the greater risk of developing hyperkalemia during spironolactone therapy