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J K Ramage

Publications and source records attributed to J K Ramage.

At least 19 recordsLinked to original sources
PubMed2006

Development of a disease-specific Quality of Life questionnaire module for patients with gastrointestinal neuroendocrine tumours.

Quality of life (QoL) measurements are increasingly being used as an end point in cancer clinical trials. Standard generic QoL questionnaires may not assess symptoms produced by neuroendocrine tumours. Here we report the development of a disease-specific quality of life score questionnaire for patients with neuroendocrine tumours of the gut to supplement the EORTC core cancer questionnaire, the QLQ-C30. Phases 1-3 of the EORTC quality of life group guidelines for module development were used to design the new questionnaire. Forty-one relevant issues (questions) were generated after an extensive literature search. Following interviews of 15 health care workers and 35 patients, a 35 question provisional questionnaire was constructed. This was translated into seven European languages and pre-tested in 180 patients resulting in a 21-item module that will be validated in an international clinical trial. The EORTC QLQ-NET21 provides a site-specific module to supplement the QLQ-C30 for patients with neuroendocrine tumours.

Adolescent↗
PubMed2004

Are renal microgranulomas related to inflammatory tubular destruction?

Two cases of renal microgranuloma formation are reported, one in a patient with known Crohn's disease and another in a case of acute renal allograft rejection. In both cases, the microgranulomas arose as a result of inflammatory tubular destruction, in a manner analogous to that seen in patients with ulcerative colitis arising adjacent to ruptured epithelial crypts in the large intestine. Microgranulomas may occur at multiple anatomical sites in Crohn's disease, although renal microgranulomas are very rare. Non-specific inflammatory tubular destruction should be considered as a cause of renal microgranuloma formation, in addition to systemic granulomatous diseases, such as tuberculosis, sarcoid, or Crohn's disease, when granulomas are seen in the presence of inflammatory tubular destruction in renal biopsies.

Adult↗
PubMed2003

Measurement of quality of life in carcinoid/neuroendocrine tumours.

Quality of life is multi-dimensional, including issues relating to symptoms from the disease but also social, emotional, functional and financial domains. Debate remains on the true definition of quality of life and its measurement. Quality of life measurements are best done by patients themselves, although, in some situations a proxy such as carer or relative can be substituted. Healthcare workers can over- or underestimate overall quality of life. Currently used devices for measuring quality of life in cancer include the European Organization for Research and Treatment of Cancer (EORTC) QLQ-C30, which is a generic tool for all cancers and which requires the use of add-on modules for specific cancers. We are developing a separate module for carcinoid/neuroendocrine tumours, in accordance with the EORTC guidelines on module development, which will be translated into five languages and will be available for use throughout Europe.

Carcinoid Tumor↗
PubMed2000

Clinical features, diagnosis and outcome of acute portal vein thrombosis.

The clinical features of acute portal vein thrombosis (APVT) are poorly defined in the literature. The proportion that progress to chronic PVT and the influences of various treatments are unknown. Between 1996 and 1998, nine patients presented to our hospital with varying upper gastrointestinal symptoms. They were found to have APVT by colour flow Doppler ultrasound, which was confirmed by CT scanning. All were tested for procoagulant tendencies and then treated with intravenous heparin for 7 days and warfarin for 3 months. Colour flow Doppler ultrasound or CT was done regularly to assess response to treatment. There was complete resolution of thrombus in five patients. Four patients had procoagulant tendencies identified; of these the thrombus resolved in two cases and in two cavernous transformation occurred. In most cases, the thrombus disperses on heparin and warfarin, although the effect of this therapy is unknown. A randomized trial of thrombolytic therapy may be appropriate, in an attempt to reduce the rate of progression to chronic PVT.

Acute Disease↗
PubMed2000

Churg-Strauss syndrome and granulomatous cholangiopathy.

Churg-Strauss syndrome is a vasculitis that is known to have gastroenterological manifestations. There have been no reports associating it with liver disease. We present a case of this syndrome in a man who was found to develop a co-existent cholangiopathy. It is likely that this represents an associated granulomatous reaction consequent to a hepatic manifestation of the vasculitis.

Adult↗
PubMed1996

Imaging secondary neuroendocrine tumours of the liver: comparison of I123 metaiodobenzylguanidine (MIBG) and In111-labelled octreotide (Octreoscan).

Functional imaging of neuroendocrine tumours with Octreoscan and I123-metaiodobenzylguanidine (MIBG) is important for assessment prior to various therapies and assessing response. The two imaging methods have not been directly compared in hepatic neuroendocrine tumours. Patients (n = 18) were studied with both imaging techniques. The sensitivity of Octreoscan was 94%, and that of MIBG 39%. No previously occult primary sites were detected. Concurrent octreotide therapy did not reduce the sensitivity of Octreoscan. Widespread bone metastases were seen in two post-liver-transplant patients using Octreoscan. Octreoscan is a sensitive means of detecting hepatic neuroendocrine tumours, and the more specific technique. MIBG has poor sensitivity, reducing its clinical utility. Therapy with I131-MIBG is likely to be applicable to relatively few patients.

3-Iodobenzylguanidine↗
PubMed1996

p53 Protein overexpression in cholangiocarcinoma arising in primary sclerosing cholangitis.

The protein encoded by the p53 tumour suppressor gene plays an important part in the regulation of cell growth. Abnormalities of this gene represent one of the most common genetic changes in the development of human cancers. This study investigated the expression of p53 protein in cholangiocarcinoma arising in association with primary sclerosing cholangitis (PSC). Of the 14 patients with cholangiocarcinoma studied, 13 had underlying PSC. The expression of p53 protein was detected immunohistochemically in paraffin wax embedded liver specimens, after microwave pretreatment. The expression of p53 protein was shown in the cholangiocarcinoma tissue of 11 of 14 (78.5%) patients. In five of 10 patients, the accumulation of p53 protein highlighted the presence of neoplastic cells in biliary tissue separate from the main tumour. These cells were mainly located in the septal bile ducts or in the accessory glands, or both, but occasionally also in large portal areas at the periphery of nerves and lymphatics, and in one case in the mucosa of an extrahepatic bile duct. No p53 protein was detectable in liver tissue obtained at the time of transplantation in 15 patients with PSC but not cholangiocarcinoma. These results show that cholangiocarcinoma development in PSC is commonly associated with abnormalities of p53 and that these occur at a late stage in the development of the malignant process. Staining for p53 protein could represent an additional criterion for the diagnosis of cholangiocarcinoma development in patients with PSC.

Adult↗
PubMed1995

Overview: the management of metastatic carcinoid tumors.

Chemotherapy, IFN, octreotide, and hepatic artery embolization can be useful for palliation, both of local symptoms and of the carcinoid syndrome, but they have little effect on tumor progression, MIBG therapy is still experimental but may have a future role. OLT should be considered as a palliative procedure in those patients with severe symptoms and no evidence of extrahepatic disease.

Antineoplastic Agents↗
PubMed1995

Orthotopic liver transplantation in the treatment of metastatic neuroendocrine tumors of the liver.

The place of orthotopic liver transplantation (OLT) in the management of metastatic hepatic neuroendocrine tumors has not been adequately defined. The present report is concerned with patient survival, disease recurrence, and symptom relief in 11 such patients in a single center who, at the time of transplantation, had no extrahepatic tumor. All patients obtained complete symptom relief initially but tumor recurrence was observed in 6 of the 11 cases (5 carcinoid and 1 apudoma) at a median of 11 months (range 3.5-26). Five patients have died, 4 in the carcinoid group with recurrence and one from chronic rejection in the other apudoma group. Of the 6 patients currently alive one of 2 carcinoids and one of 4 other apudomas have tumor recurrence. Recurrent deposits were found predominantly in bone and in the transplanted liver. Actuarial survival post transplant was 82% and 57% at 1 and 5 years respectively. It is concluded that OLT is effective at controlling symptoms from secreting carcinoid deposits in the liver. Although the tumor will recur in most cases, this is not necessarily associated with early return of symptoms. Prolonged disease free survival is more likely in the non carcinoid apudoma group.

Adult↗
PubMed1995

Ribavirin therapy for hepatitis C infection following liver transplantation.

Hepatitis C infection following orthotopic liver transplantation may lead to progressive chronic graft dysfunction. In this study, seven liver transplant recipients with chronic allograft dysfunction due to hepatitis C infection (one acquired and six recurrent infections) were treated with oral ribavirin for 6 months. Symptoms of lethargy, nausea and anorexia improved in all patients within 2 weeks of starting the drug, with a fall in serum AST of at least 40% by this time. Ribavirin-induced haemolysis was clinically significant in three patients, necessitating a reduction in the daily dose of ribavirin from 1.2 g to 0.2 g. Comparison of the pre- and post-treatment biopsy specimens in the four patients who tolerated the full dose of ribavirin and who had normal AST levels at the end of 6 months of treatment showed significant histological improvement with reduction in either lobular or periportal inflammation in all of the patients and a reduction in periportal fibrosis in one patient. HCV RNA remained detectable in serum in all of the patients at the end of the study.

Female↗
PubMed1995

Serum tumor markers for the diagnosis of cholangiocarcinoma in primary sclerosing cholangitis.

BACKGROUND/AIMS: The diagnosis of cholangiocarcinoma in primary sclerosing cholangitis (PSC), even with the use of current imaging techniques and brush cytology, is difficult and particularly important in patients being assessed for liver transplantation. This study investigated the accuracy of serum levels of a combination of the tumor markers carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9 (CA19-9) in the diagnosis of cholangiocarcinoma in patients with PSC. METHODS: Seventy-four patients with PSC were studied. Fifteen patients had tumors (11 occult on imaging), 22 had severe PSC that necessitated transplantation (with explanted liver known to be free of tumor), and 37 patients had stable PSC. RESULTS: An index of the two serum tumor markers [using the formula CA19-9 + (CEA x 40)] gave an accuracy of 86% in diagnosis of cholangiocarcinoma, with 10 of the 15 cases of cholangiocarcinoma having an increased value compared with none in a group of 22 comparable cases with no tumor. In addition, 6 of the 11 patients with occult tumors had abnormal values. Ultrasonography, computerized tomographic scanning, and endoscopic retrograde cholangiopancreatography were poor predictors of the presence of tumor. CONCLUSIONS: A combination of serum tumor markers will identify most occult tumors and will improve selection of appropriate cases for orthotopic liver transplantation.

Adult↗
PubMed1995

Hepatic venous outflow obstruction in patients with polycystic liver disease: pathogenesis and treatment.

Polycystic liver disease is commonly asymptomatic but may present with hepatomegaly, abdominal distension, and dull abdominal pain. Transudative ascites is a rare manifestation in these patients but may occur when portal hypertension is present resulting from associated hepatic fibrosis or after deroofing procedure of a cyst. Exudative ascites might suggest hepatic venous outflow obstruction. Four cases are described where hepatic venous outflow obstruction occurred in patients with polycystic liver disease. Three patients had orthotopic liver transplantation and one had a mesocaval shunt. Of the two patients that survived orthotopic liver transplantation both have shown considerable improvement in their symptoms. None of the patients had any confirmed procoagulant disorder. The mechanism of hepatic venous outflow obstruction in these patients seems to be mechanical compression of hepatic veins by the cysts and associated formation of thrombi in small hepatic vein tributaries. Patients with severe polycystic kidney/liver disease are at risk of hepatic venous outflow obstruction and the onset of this complication is heralded by tender hepatomegaly and presence of exudative ascites.

Adult↗
PubMed1994

Accuracy of radiology in detection of hepatocellular carcinoma before liver transplantation.

BACKGROUND/AIMS: Recurrence of hepatocellular carcinoma (HCC) after orthotopic liver transplantation (OLT) depends on tumor size and number; the accuracy of radiology in detecting HCC is uncertain. This study compared tumor size at radiological and pathological examination in 30 patients with HCC who underwent OLT. METHODS: Pre-OLT radiology included ultrasonography (US), computerized tomography (CT), and hepatic angiography (HA). Sensitivity of radiology was calculated by comparison with pathology. RESULTS: Radiology missed HCC in two patients but showed five with small (< 4 cm) and eight with large (> 4 cm) lesions. Multicentricity was shown in 15 cases at radiological examination and 24 at pathological examination, including one incidental and 4 of 5 radiologically small HCC. Mean tumor bulk was 3.4, 74, 338, and 1375 cm3, respectively, in patients with incidental, small, large, and multicentric HCC at radiology. Sensitivities of US, CT, and HA in detection of HCC (and multicentricity) were 80% (16.6%), 86.6% (58.3%), and 90% (58.3%). No recurrence was found in patients with incidental or small HCC; even when multicentric. CONCLUSIONS: US, CT, and HA identified patients with small or large tumor bulk, but sensitivity in detecting satellites was poor; their finding in patients with radiologically small or incidental tumors does not affect outcome. The failure of US, CT, and HA to detect satellites must be considered in patients undergoing partial hepatectomy for HCC.

Carcinoma, Hepatocellular↗