Egil Gjone

Primary Sclerosing Cholangitis: A Long-Term Follow-up Study

During the 10-year period from 1 January 1975 to 31 December 1984, primary sclerosing cholangitis (PSC) was diagnosed in 45 patients. Twelve of the patients have died (26.7%), 10 of them of causes related to PSC. Inflammatory bowel disease was found in all patients; ulcerative colitis was found in 37, Crohns disease in 6, and unclassified colitis in 2 patients. Of the patients alive, 27 were submitted to a follow-up study in 1985. At the follow-up examination no general progression of the liver disease, as measured on the basis of clinical examination and levels of transaminases, alkaline phosphatases, and bilirubin, was found. Cholangiographic evaluation in 24 patients showed that the stage of ductal changes progressed from mild to moderate in 3 patients; in the other patients the stage was not altered. Morphologic examination of liver biopsy specimens in patients with a benign clinical course usually showed portal inflammation, fibrosis, and minor signs of piecemeal necrosis, whereas widespread piecemeal necrosis was found in patients who deteriorated and died. The 50% survival since diagnosis of liver disease was calculated to be 17 years in patients with PSC and 50 years in a comparable group among the general population. The estimated survival curve in PSC was displaced to the left, indicating a reduced life expectancy of about 30 years.

Familial Plasma Lecithin: Cholesterol Acyltransferase Deficiency Biochemical Study of a New Inborn Error of Metabolism

Three adult sisters with a new inborn error in lipid metabolism have been studied. They had an almost complete lack of esterified cholesterol in plasma, and a high concentration of plasmafree cholesterol. The plasma concentrations of lecithin were high and of lysolecithin low. These abnormalities were found to be associated with an absence of plasma lecithin: cholesterol acyltransferase. No inhibitors of this enzyme could be demonstrated in the plasma of the patients. The small amounts of cholesterol esters found in the plasma of the patients were found to be formed during intestinal absorption of cholesterol. The two eldest patients had lipemic plasma. The fatty acid composition of the triglycerides were normal. Electrophoresis of the plasma lipoproteins revealed a pathological pattern: no α- and no pre- β-lipoproteins could be demonstrated. Immunoelectrophoresis also revealed an α-lipoprotein deficiency. The data obtained in the present study strongly suggest that the plasma cholesterol esterification r...

Sclerosing Cholangitis in Ulcerative Colitis

In a 5-year period 48 (14%) of 336 patients with ulcerative colitis were found to have hepatobiliary disease. The bile ducts were examined in 35 of these patients, and optimal visualization of both intra- and extra-hepatic bile ducts was obtained in 26. Duct changes compatible with sclerosing cholangitis were found in 14 patients. This finding of sclerosing cholangitis in 4% of all patients admitted with ulcerative colitis by far exceeds previous estimations on the incidence of sclerosing cholangitis in ulcerative colitis. The entire colon was usually affected, and the symptoms of the bowel disease were most often mild or moderate. The age at the onset of the colitis was usually below 20 years in patients with combined ulcerative colitis and hepatobiliary disease. In most patients the hepatobiliary disease gave no symptoms. Biochemical data and the histological findings in the liver biopsies did not distinguish between patients with hepatobiliary disease with and without sclerosing cholangitis. Our follow-up study has so far shown that most patients with sclerosing cholangitis remain asymptomatic for a considerable period of time.

Metronidazole in Crohn's disease. A double blind cross-over clinical trial.

Twenty-two patients with active Crohns disease treated with salazosulfapyridin or prednisone, were in addition given metronidazole 1.000 mg daily or placebo for two months each in a double-blind cross-over study. No statistically significant effect was observed on the overall clinical condition in the 20 patients who completed the trial, although haemoglobin rose and ESR fell significantly in the metronidazole periods. In the 6 patients with colonic involvement only an improvement was registered both in symptoms and laboratory values.

Familial plasma lecithin: cholesterol acyltransferase deficiency.

A woman with familial plasma lecithin: cholesterol acyltransferase (L.C.A.T.) deficiency showed, like the other reported cases, obvious corneal opacity, proteinuria, and moderate anaemia with a slight haemolytic component. In the plasma the concentrations of free cholesterol, triglycerides, and lecithin were high, and those of esterified cholesterol, lysolecithin, and alphalipoprotein were low. L.C.A.T. activity in plasma was 10% of normal. The heparin-induced lipolytic activity in plasma was reduced. The erythrocyte lipid pattern was abnormal and showed the same pattern as earlier described in L.C.A.T. deficiency. The patients brother also probably suffered from the disease and died in uraemia. These are the fourth and fifth known patients with L.C.A.T. deficiency, the first one reported in a male, and the first one with a fatal outcome.

Sclerosing cholangitis in ulcerative colitis. A follow-up study.

In the 5-year period 1974-78, 48 (14%) of 336 patients with ulcerative colitis were found to have hepatobiliary disease. Endoscopic retrograde cholangiography (ERC) was successfully performed in 39 of these 48 patients, and sclerosing cholangitis was demonstrated in 19. One is excluded from this series because Crohns disease was diagnosed at reclassification of the bowel disease. Two of the 18 patients with ulcerative colitis and sclerosing cholangitis have died, one of cholangiocarcinoma and one of an unrelated cause. The remaining 16 patients have been observed for a median period of 6 years (3-13 years) since the diagnosis of hepatobiliary disease. Ten have remained symptom-free, four have had intermittent or non-progressive symptoms, and two have developed symptoms of advanced chronic liver disease. The bilirubin level, which was initially raised in one of the patients, was elevated in four at the follow-up examination. Otherwise the laboratory values have remained stationary. Evidence of a progression of the hepatobiliary disease was found in most patients by repeated liver biopsy and particularly ERC. It is concluded that sclerosing cholangitis may remain asymptomatic for several years. Since progressive cholangiographic changes were often seen without concomitant worsening of symptoms, laboratory data, and liver biopsy findings, it is concluded that these criteria are of limited use in evaluating the progression of this disease.

Epidemiology of Polyps in the Rectum and Sigmoid Colon

Epidemiological studies have suggested an association between diet and colorectal cancer. Case/control studies, however, have been scarce, and studies based on interview with cancer patients who have symptoms from their cancer are inevitably prone to bias. An endoscopic population screening study for detection of colorectal adenomas enabled a double-blind registration of diet during 5 consecutive weekdays. Neither the participant nor the dietitian was informed of the findings at endoscopy. The estimation of 23 nutritional components was based on analysis of local commercial food and on the composition of foods in Norway. Results showed increasing consumption of fat and decreasing consumption of fiber and cruciferous vegetables in the presence of increasing neoplastic changes. The present material will form the basis for dietary-related follow-up studies.

Epidemiology of polyps in the rectum and sigmoid colon: evaluation of nutritional factors

Epidemiological studies have suggested an association between diet and colorectal cancer. Case/control studies, however, have been scarce, and studies based on interview with cancer patients who have symptoms from their cancer are inevitably prone to bias. An endoscopic population screening study for detection of colorectal adenomas enabled a double-blind registration of diet during 5 consecutive weekdays. Neither the participant nor the dietitian was informed of the findings at endoscopy. The estimation of 23 nutritional components was based on analysis of local commercial food and on the composition of foods in Norway. Results showed increasing consumption of fat and decreasing consumption of fiber and cruciferous vegetables in the presence of increasing neoplastic changes. The present material will form the basis for dietary-related follow-up studies.

Epidemiology of polyps in the rectum and sigmoid colon design of a population screening study

The design of a population screening study, including endoscopic screening for polyps in the rectum and sigmoid colon, was tested in a defined population sample of 200 women and 200 men aged 50-59 years. The attendance rate was 81%. A simple bowel cleansing procedure performed immediately before the examination gave satisfactory conditions for endoscopic examination in 94% of attendants. Polyp prevalence was 35%. The present material and results represent the basis for further follow-up studies of risk factors for the development of cancer in the same group of individuals.

Familial Plasma Cholesterol Ester Deficiency a study of the erythrocytes

The erythrocytes of three sisters with plasma cholesterol ester deficiency and lack of plasma lecithin: cholesterol acyltransferase (LCAT) have been studied. The erythrocyte content of cholesterol was markedly increased. Their total lipid phosphorus was normal, lecithin being increased and phosphatidylethanolamine and sphingomyelin decreased. The fatty acid composition of the phospholipids was not normal as an increase in linoleic acid and a decrease in arachidonic and very long chain fatty acids were demonstrated. The absence of LCAT in plasma of the patients may explain the major lipid abnormalities of their red cells.