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BackgroundMalnutrition is a common management problem in patients with cystic fibrosis (CF). Various approaches to supplemental nutrition by both parenteral and enteral routes have been used.AimTo analyse the efficacy and acceptability of supplemental overnight feeding using a percutaneous endoscopic gastrostomy (PEG) in patients with CF.Patients53 patients with CF (43 adults; age >17 years) with severe pulmonary disease.MethodsThe technical success and complications of PEG insertion were documented together with changes in nutritional and pulmonary status of the cohort.ResultsPEG tubes were successfully inserted in all patients, with immediate complications (respiratory depression) in two (4%) and late complications in 13 (25%). Feeding was well tolerated by 50/51 (98%) of the cohort during a mean (SEM) follow up of 14.5 (2.1) months. The adult cohort had a significant increase in weight and body mass index at six months which was maintained at 12 months. Serum albumin concentration remained stable at six months but had fallen by 12 months, although the differences were not statistically significant. These results were reflected in the paediatric cohort. Pulmonary function in those followed up for one year had apparently stabilised, but the number of admissions to hospital over the year before and the year after PEG did not change. Half of the cohort were accepted for heart-lung/lung transplantation, the improvement in nutritional status being a prerequisite for this.ConclusionSupplemental PEG tube feeding is well tolerated and results in a significant improvement in nutritional status and an apparent stabilisation of pulmonary function in severely malnourished CF patients with advanced pulmonary disease.

Background and Study Aims: Treatment options for un-resectable hepatic hilar strictures include percutaneous or endoscopic stent insertion, using either standard Teflon or self-expanding metal endoprostheses. The use of Teflon stents is complicated by stent migration and high occlusion rates. Published series of endoscopically placed metal stents have not concentrated on their use in the treatment of high-grade hilar strictures (Bismuth grades II and III). We therefore undertook a prospective and open pilot study to evaluate the efficacy of endoscopically placed metal endoprostheses in the palliation of jaundice due to high-grade hilar strictures. Patients and Methods: Self-expanding metal stents were placed endoscopically in 17 patients (nine women, eight men; median age 64 years, range 33-77). Of the 17 malignancies 11 patients (65%) had a diagnosis of cholan-giocarcinoma, and 12 (71%) had Bismuth grade III strictures. Results: The stents were inserted successfully in all 17 patients. Adequate drainage, as demonstrated by a significant reduction in bilirubin, was achieved in 15 (88 %); the two patients in whom drainage failed had extensive intrahepatic disease. Early complications (cholangitis) developed in one patient (6%), and there were seven late complications (41 %) in five patients (four occluded stents and three cases of duodenal compression). The stent patency appeared to be prolonged (median stent patency 12 months, range 1.5-24 months, in patients surviving five months or more). The median survival was ten months (range 1-48 months) after stent insertion. All of the patients died during follow-up. Conclusions: These results suggest that endoscopically placed metal stents offer effective palliation for high-grade hilar malignancies, although controlled trials comparing the use of metal and Teflon endoprostheses in this patient group are required.

Variceal haemorrhage complicates the clinical course of chronic liver disease in about 30% of patients.1Mortality for the index bleed is as high as 50%,2with a 30% mortality for subsequent recurrent bleeds. The rate of recurrent haemorrhage in those who survive the initial bleeding episode is as high as 100% over two years. The initial resuscitation of the patient is of paramount importance, with protection of the airway, particularly in patients with encephalopathy, and restoration of the circulating volume (box 1). #### Box 1 - Initial resuscitation Establishing adequate venous access, in some cases through a central venous catheter, is essential to ensure ease of fluid replacement and adequate monitoring of the patient. In patients with ascites, the elderly patients, and those with associated medical conditions such as ischaemic heart disease, the right atrial pressure may not accurately reflect left sided heart pressures so fluid replacement should be monitored with a Swan- Ganz catheter. Detailed attention to fluid replacement is important for any gastrointestinal bleed and even more so in patients with established chronic liver disease. These patients may have reduced vascular tone and fail to mobilise pooled venous blood from the splanchnic circulation.3Failure to achieve prompt volume replacement may jeopardise renal and hepatic function, a major factor in the morbidity and mortality associated with a variceal bleed.3It is equally important to avoid overfilling as this may precipitate rebleeding, so the right atrial pressure should be maintained between 4 and 8 mm Hg. Immediate transfusion of colloid (crystalloid in the form of saline should be avoided in patients with chronic liver disease because of impaired renal sodium excretion and the development …

Summary Background ‘Re-infection’ with Helicobacter pylori after eradication has been estimated to occur in 0–14% of patients, although most so-called ‘re-infections’ occur within the first year following ‘eradication’ and many may actually be due to recrudescence of a temporarily suppressed infection. Aim To study the true re-infection rate, we have studied re-infection rates after eradication therapy by excluding the first year's data, minimizing the possible confounding effect of recrudescence. Methods All patients tested for H. pylori infection following eradication therapy between 1987 and 2004 were evaluated. Testing was carried out by urea breath test and gastric biopsy. Patients were included if they were found to be negative for H. pylori infection by testing at least 1 year following eradication and underwent at least one further test for H. pylori. Results 1162 patients met the inclusion criteria with median post-eradication follow-up of 3 years (1.5–14) including 4668 tests; 3319 years of follow-up were analysed. Thirteen cases of re-infection occurred (re-infection rate 0.4% per year). Conclusions This large study of H. pylori re-infection avoided cases of recrudescence by excluding the first post-eradication year. True re-infection is probably less common than previously thought.

BACKGROUND: The treatment for endstage cystic fibrosis is, where appropriate, double-lung, heart-lung or, occasionally, heart-lung-liver transplantation. Optimising the timing of transplantation depends upon an accurate prediction of survival, but while current criteria give some guidance to this, they are not based upon statistically derived prognostic models. METHODS: Data collected prospectively on 403 patients with cystic fibrosis, recruited between 1969 and 1987 (cohort A), were analysed by log rank and univariate Cox regression analysis to determine variables that accurately predict survival. The significant variables were then subject to time dependent multivariate Cox regression analysis to generate a prognostic model. The model was validated, within the study population, using split sample testing, and was subsequently validated in a further cohort of patients recruited since October 1988 (cohort B). RESULTS: One hundred and eighty eight (50.4%) of the study cohort died within the study period. Percentage predicted forced expiratory volume in one second (FEV1), percentage predicted forced vital capacity (FVC), short stature, high white cell count (WBC), and chronic liver disease (as evidenced by the presence of hepatomegaly) were negatively correlated with survival. These variables, when combined into a prognostic index, accurately predicted one year survival in the study population and in the cohort recruited since 1988. CONCLUSION: This prognostic index may prove valuable in predicting prognosis in other cohorts with cystic fibrosis and thereby improve the timing of transplantation.

Introduction Coeliac disease is a common condition, affecting up to 1 in 100 individuals in the UK. The average age of diagnosis is now over the age of 40 with 20% of newly diagnosed cases being over 60. In those over 60, the delay in diagnosis may be particularly long. The purpose of this audit was to evaluate the appropriate use of tissue transglutaminase (tTG) and duodenal biopsy in the diagnosis of celiac disease in a cohort of DGH patients analysed retrospectively. Methods 114 consecutive patients with a reactive anti tTG > 3 were identified from the Ipswich Hospital Pathology laboratory database between January and June 2011. Those with an anti tTG titre > 7 have positive results in our laboratory. The cohort was divided into positive and negative groups. The duodenal biopsy (DDB) results of the positive group were documented. If the patient had not had a DDB the notes were reviewed or relevant clinician contacted. The negative group’s IgA level was recorded to determine the likelihood of a false negative result. Results Of 114 patients identified from the database with an anti tTG ≥ 3, 69 were female and 45 male. There were 63 positives and 51 negatives. In the positive serology group, 48 patients were already known to have coeliac disease, 4 had a negative biopsy and 11 have not had a DDB. Of these 11 patients only 2 had been referred to gastroenterology, 3 had been given a diagnosis of coeliac disease without a DDB and 6 had not been informed of their results. In the negative serology group 19 (16 adult and 3 children) were known to have coeliac disease and were on a gluten free diet. Of the remaining 27 adult patients, only 7 (26%) had had their IgA checked, while only 3 of 5 children had had their IgA tested. 2 of these 3 were IgA deficient. The other 2 had not been tested. None of the IgA deficient cohort had undergone a duodenal biopsy to exclude a false negative result. Conclusion The results of this audit demonstrate inconsistent application of the national guidelines(1) for the diagnosis of celiac disease. Of greatest concern was the cohort of adult patients who were labelled as having coeliac disease without a confirmatory biopsy. There was also a significant cohort of IgA deficient patients in the negative group who may have had celiac disease. There is clearly a need for further on-going education of all healthcare professionals regarding appropriate testing to diagnose coeliac disease to ensure appropriate treatment and prior to labelling an individual with a lifelong diagnosis. Disclosure of Interest None Declared. Reference Ciclitira P J, Dewar D H, McLaughlin S D, Sanders DS (2010) The Management of Adults with Coeliac Disease, British Society of Gastroenterology

Background/Aims: There are well-documented systemic haemodynamic changes associated with chronic liver disease. Patients with cystic fibrosis may develop chronic liver disease, but it is not known whether these systemic haemodynamic changes develop and, if they do, whether they are influenced by the associated chronic lung disease. We therefore undertook a study to document the circulatory status of cystic fibrosis patients with and without chronic liver disease. Methods: Fifty-six subjects with cystic fibrosis were studied. Systemic haemodynamic and pulmonary parameters, in sub-groups both with (LD) and without (NLD) liver disease, were measured at rest and during measured exertion. Cystic fibrosis-related chronic liver disease was diagnosed using previously validated ultrasound criteria. Patients underwent assessment at rest and in the fourth minute of seated bicycle exercise at 25W. Heart rate (ECG), blood pressure (semiautomated sphygmomanometer), aortic blood velocity (pulsed Doppler suprasternal probe), arterial oxygen saturation (pulse oximeter) and respiratory variables (pneumotachometer with expired gas analysis by an automatic system) were measured. Results: A complete data set was available for 45 patients (22 LD) at rest and 40 patients (19 LD) on exercise. The patients were well matched for age, sex, height, weight, and pulmonary function. Patients with chronic liver disease had a hyperkinetic circulation while ventilatory variables before and during exercise were similar for the two groups. There was evidence that the circulatory changes were exacerbated by both deteriorating hepatic and pulmonary function. Conclusions: Cystic fibrosis patients with chronic liver disease have a hyperdynamic circulation similar to that documented in other forms of chronic liver disease. These circulatory changes are exacerbated by deteriorating hepatic and pulmonary function.

The optimum treatment of gastric varices has still to be defined. Lesser curve gastric varices may be treated by injection sclerotherapy, but this has a limited role in the treatment of fundal gastric varices. Surgical intervention is commonly needed but carries a high mortality in patients with advanced liver disease. This study evaluated the use of thrombin for the treatment of gastric varices in 11 consecutive patients (nine with fundal, two with high lesser curve varices), identified as having bled from this site. Bovine thrombin (1000 U/ml) was injected intravariceally (mean volume 5.5 ml, range 2-10 ml) producing initial haemostasis in all 11 cases. Varices were considered thrombosed or obliterated in all patients after a median of two injection episodes (range 1-3). After a median follow up of nine months only one patient had rebled from a gastric varix. Thrombin may represent a valuable alternative injectate for the treatment of gastric varices.

There have been striking advances in the management of complications of cirrhosis in the 50 years since biliary cirrhosis was recognised as a feature occurring in a minority of patients with cystic fibrosis. Many questions remain unanswered with regard to its pathogenesis, its effect on other systems, morbidity and mortality and to its optimum management. Factors which may initiate liver disease and/or cause progressive biliary fibrosis leading to biliary cirrhosis in some cystic fibrosis patients are being defined but much is still poorly understood and further research is required. Clinical and pathological studies confirm that cirrhosis is frequently asymptomatic and only slowly progressive, with the prevalence rising with age. In a recent large epidemiological study, however, it was found that the age related prevalence fell in patients over the age of 20 years although deaths from liver disease were rare. Does the presence of liver disease cause premature respiratory death in teenagers? Current treatments control variceal bleeding, the important sequel for most patients with biliary cirrhosis, with less morbidity and mortality than in the past but require reappraisal as newer techniques become available. Endoscopic radiologically controlled methods are emerging as an important adjunct to the surgical control of biliary complications which cause symptoms in up to 4%. Ursodeoxycholic acid improves liver function tests but its effect on hepatic fibrosis and portal hypertension will only be demonstrated by large scale prospective controlled trials. Should liver transplantation have a larger role in management? In this chapter we have attempted to summarise the current state of knowledge and to assess the efficacy of present management.

For the first time the endoscopist has more than one option for the management of gastro-oesophageal varices. It is now feasible to select the appropriate therapy on the basis of the clinical setting. Acute injection sclerotherapy remains a quick and simple technique for the control of active bleeding from oesophageal varices, and could be followed two or three days later by banding ligation. Earlier obliteration of varices with this technique may offer the prospect of only two or three sessions of therapy. The availability of the tissue adhesives and thrombin as injectates for fundal gastric varices provide the option of an initial attempt at endoscopic therapy in this high risk group.