Outcome of adult patients receiving parenteral support at home: 36 years' experience at a tertiary referral centre.

BACKGROUND AND AIMS: Patients with intestinal failure often need long-term home parenteral support (PS). We aimed to determine how the underlying diagnosis, complications and survival had changed over the last 36 years in the UK's largest IF centre. METHODS: 978 adult home PS patient records were analysed from January 1979 until October 2016. The age, sex, underlying aetiology, complications and survival was compared over 5-year periods. RESULTS: Pre-1990 to 2011-2016, numbers increased from 29 to 451, the mean age of patients increased from 31 ± 16.5 to 52 ± 17.6 years. The percentage of patients with IF due to surgical complications increased (3.4%-28.8%, p < 0.001)), while those with inflammatory bowel disease decreased (37.9%-22.6%, p < 0.001). Complication of home PS reduced: catheter related blood stream infections (CRBSI) 71.4% to 42,2%, CVC thrombosis 34.5%-5.3%. Intestinal failure associated liver disease (IFLAD) 10.3%-1.8%. Patients with dysmotility, scleroderma and a congenital aetiology had the highest incidence of CRBSI and CVC Thrombosis. Overall survival was greater pre-1995 [HR 0.2-0.4 (p = 0.02)] most likely associated with an increase in mean age. Survival for patients without malignancy was 90%, 66%, 55%, 45%, 33% and 25% at 1,5, 10, 15, 20 and 30 years respectively. Multivariate analysis demonstrated a relationship between survival and age of starting home PS; type of home PS; presence or absence of the colon in continuity; and underlying aetiology. CONCLUSION: Demand for home PS is increasing in particular for advanced malignancy, post-surgical complications and older more co-morbid patients. Complications of home PS are reducing over the last 30 years and 10-year survival for non-malignant aetiologies improving. Survival and changes in aetiology in intestinal failure.

Assessment and management of patients with intestinal failure: a multidisciplinary approach.

Intestinal failure (IF) is a condition characterized by the inability to maintain a state of adequate nutrition, or fluid and electrolyte balance due to an anatomical or a physiological disorder of the gastrointestinal system. IF can be an extremely debilitating condition, significantly affecting the quality of life of those affected. The surgical management of patients with acute and chronic IF requires a specialist team who has the expertise in terms of technical challenges and decision-making. A dedicated IF unit will have the expertise in patient selection for surgery, investigative workup and planning, operative risk assessment with relevant anesthetic expertise, and a multidisciplinary team with support such as nutritional expertise and interventional radiology. This article covers the details of IF management, including the classification of IF, etiology, prevention of IF, and initial management of IF, focusing on sepsis treatment and nutritional support. It also covers the surgical aspects of IF such as intestinal reconstruction, abdominal wall reconstruction, and intestinal transplantation.

Identification of coeliac disease triggering glutenin peptides in adults.

OBJECTIVE: Coeliac disease affects approximately 1% of Northern American and European populations. It is caused by an inappropriate immune response to dietary gluten. Gluten comprises of two major protein fractions: gliadins and glutenins. Glutenins have recently been found to be toxic to coeliac individuals. Proliferation assays suggest in some but not all paediatric coeliac individuals there may be immunological stimulation with high molecular weight (HMW) glutenins. Less evidence pertains to low molecular weight (LMW) glutenins. The aim is to assess adaptive, T-cell driven, and innate immune response in adult coeliac individuals towards HMW glutenin peptide, glut04, and LMW glutenin peptide, glt156. MATERIALS AND METHODS: Coeliac patients were recruited attending endoscopy for routine monitoring. Adaptive immune response towards glut04 and glt156 was measured by proliferation assays and measurement of interferon-γ secretion in 28 T-cell lines. The innate immune response was assessed by measurement of enterocyte cell height (ECH) in coeliac small intestinal biopsies following overnight incubation in organ culture chambers in a further nine individuals. RESULTS: There were 3/28 and 2/28 positive proliferation results using gluten-sensitive T-cells with glut04 and glt156, respectively. All coeliac biopsies tested in organ culture chambers demonstrated clear reduction in ECH with peptic-tryptic digest of whole industrial gluten, glut04 and glt156 when compared to negative control ovalbumin (p < 0.005). Three individuals had both T-cell and organ culture study data. Their proliferation assays showed no stimulation of the T-cells. CONCLUSIONS: This study demonstrates glutenin epitopes glut04 and glt156, while minor T-cell epitopes, are important in their ability to trigger the innate immune response.

Celiac disease: management of persistent symptoms in patients on a gluten-free diet.

AIM: To investigate all patients referred to our center with non-responsive celiac disease (NRCD), to establish a cause for their continued symptoms. METHODS: We assessed all patients referred to our center with non-responsive celiac disease over an 18-mo period. These individuals were investigated to establish the eitiology of their continued symptoms. The patients were first seen in clinic where a thorough history and examination were performed with routine blood work including tissue transglutaminase antibody measurement. They were also referred to a specialist gastroenterology dietician to try to identift any lapses in the diet and sources of hidden gluten ingestion. A repeat small intestinal biopsy was also performed and compared to biopsies from the referring hospital where possible. Colonoscopy, lactulose hydrogen breath testing, pancreolauryl testing and computed tomography scan of the abdomen were undertaken if the symptoms persisted. Their clinical progress was followed over a minimum of 2 years. RESULTS: One hundred and twelve consecutive patients were referred with NRCD. Twelve were found not to have celiac disease (CD). Of the remaining 100 patients, 45% were not adequately adhering to a strict gluten-free diet, with 24 (53%) found to be inadvertently ingesting gluten, and 21 (47%) admitting non-compliance. Microscopic colitis was diagnosed in 12% and small bowel bacterial overgrowth in 9%. Refractory CD was diagnosed in 9%. Three of these were diagnosed with intestinal lymphoma. After 2 years, 78 patients remained well, eight had continuing symptoms, and four had died. CONCLUSION: In individuals with NRCD, a remediable cause can be found in 90%: with continued gluten ingestion as the leading cause. We propose an algorithm for investigation.

Pharmacotherapy and management strategies for coeliac disease.

INTRODUCTION: Coeliac disease is a common disease that affects approximately 1% of Northern European and American populations. Evidence suggests it is caused by an inappropriate immune response in genetically susceptible patients to dietary gluten found in wheat, rye, barley and, in a small minority of patients, oats. Treatment involves a lifelong gluten-free diet. This diet limits nutritional variety and is costly and difficult to maintain. AREAS COVERED: This review covers the current treatment options available and discusses novel emerging therapies for coeliac disease. EXPERT OPINION: Novel therapies are still in early stages of development and therefore, at present, a gluten-free diet remains the treatment of choice in coeliac disease due to its low side-effect profile. A replacement for a gluten-free diet would be superior to an adjunct; in this case dietary modification of gluten may well have the least side effects, be tolerated by a wider group of coeliac patients and therefore be accepted. Search terms used: Pubmed, Medline and clinicaltrials.gov were searched with 'celiac disease' and 'therapy' as MESH terms. Patent database was searched using the term 'celiac disease'. Conference attendance at DDW Chicago 2011 and Columbia 2010 was also used to gain further information from conference abstracts.