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OBJECTIVE: Patients with inflammatory bowel disease (IBD) frequently suffer from thromboembolic events. A recently identified mechanism for thrombophilia, the poor anticoagulant response to activated protein C, has been suggested as one of the leading risk factors for thrombosis. The aim of this study was to evaluate the frequency of thrombophilic abnormalities, including activated protein C-resistance (APCR), in Greek patients with ulcerative colitis (UC) and Crohn's disease (CD). METHODS: Forty-eight patients with UC, 36 with CD, and 61 matched healthy controls (HC) were studied. Cases with presence of lupus anticoagulant, use of anticoagulants or heparin, and pregnancy were excluded. Disease activity in CD was evaluated by use of the Crohns Disease Activity Index (CDAI) score and in UC by the Truelove-Witts grading system. Plasma levels of protein C, free protein S, antithrombin III (AT-III), activated protein C resistance (APCR), and fibrinogen were determined in IBD patients, as well as in HC. All the cases and controls with abnormal APCR were further studied by genetic testing for the factor V Leiden mutation. RESULTS: Mean fibrinogen levels in UC and CD patients were significantly elevated (p < 0.0001), compared with HC. The mean values of free protein S, as well as mean APCR, were significantly lower in UC and CD patients than in the HC (p < 0.0001). Seven (five UC and two CD) of 84 IBD patients (8.3%) and three of the HC (4.9%) had the factor V Leiden mutation. No significant difference was observed for the other thrombophilic parameters. Fibrinogen levels and profound free protein S deficiency were found related to disease activity. CONCLUSIONS: Thrombophilic defects are common in Greek patients with IBD and they could interfere either in the disease manifestation or in the thrombotic complications. [ABSTRACT FROM AUTHOR]

An ileocaecal tumour in a patient with α-chain disease in remission was studied immunohistochemically by a quantitative immunoperoxidase method. The tumour which was histologically shown to be a plasmacytoma consisted of 68% α cells and 15% λ cells; no κ cells were found. Away from the tumour the pattern of immunoglobulin-producing cells was normal. It is concluded that the abnormal cell causing α-chain disease remains inactive and that the patient developed a plasmacytoma from a different cell clone. [ABSTRACT FROM AUTHOR]

Patients with inflammatory bowel disease (IBD) frequently suffer from thromboembolic events. Anti-cardiolipin (aCL) antibodies have been shown to be associated with thrombosis. Recently, the antibodies against the anti-cardiolipin cofactor beta2-glycoprotein I (a(beta2)GPI) have been found with higher specificity for thrombosis. The presence of these antibodies was assessed in 128 patients with IBD [83 with ulcerative colitis (UC) and 45 with Crohn's disease (CD)] and 100 healthy controls (blood donors). Patients with UC and CD had a significantly higher prevalence of aCL (18.1% and 15.6%, respectively) than healthy controls (HC) (3%). Eleven IBD patients (8.6%) but no HC had a(beta2)GPI. None of the IBD patients with a history of thrombosis had aCL and only one of them (a UC patient with deep vein thrombosis of the right leg) had a high titer of IgG a(beta2)GPI. In conclusion, these data show that both aCL and a(beta2)GPI are significantly associated with IBD but further studies are needed to determine the significance of our findings. [ABSTRACT FROM AUTHOR]

Objective: To study the natural history and outcome of varicella infection developing in steroid treated inflammatory bowel disease. Background: Varicella infection occurring in immunosuppressed or immunocompromised patients is a common problem with a significant mortality. Varicella infection during the course of inflammatory bowel disease has been reported in a small number of patients with at least one fatality. Methods: Four young patients with inflammatory bowel disease who developed varicella infection while on immunosuppressive therapy, steroids, or azathioprine were studied. In each patient the infection was severe, and the three most recently treated patients received acyclovir. Results: All four patients developed severe varicella infection while receiving immunosuppressive therapy for their disease. Three patients were treated with intravenous acyclovir with concomitant reduction of steroid dosage and recovered completely. One patient, treated in 1980 with antibiotics and reduction in steroids, did not receive acyclovir and also survived. Conclusions: Varicella infection is a relatively uncommon occurrence in inflammatory bowel disease. If varicella infection occurs, prompt diagnosis and treatment with acyclovir and concomitant reduction in immunosuppressive therapy (reduction in steroid dosage and discontinuation of azathioprine) should be initiated immediately to limit viremia and avoid fatal complications. [ABSTRACT FROM AUTHOR]

The cellular immune mechanisms of patients with adenocarcinoma of colon or rectum were evaluated by studying the lymphocyte response to PHA stimulation. Our results indicate that the serum of cancer patients causes a decreased blastic transformation and H thymidine uptake of the lymphocytes of patients as well as of normal controls. A defect of the lymphocytes unrelated to serum factors was also present in some cancer patients. However, no relationship was found between the degree of transformation and the presence of metastases in the lymph nodes. [ABSTRACT FROM PUBLISHER]

Objectives: To estimate the frequency of autoimmune hemolytic anemia and Coombs positivity without overt hemolysis in ulcerative colitis, to determine possible subsets of patients with ulcerative colitis susceptible to this complication, and to assess the efficacy of the applied therapeutic modalities. Methods: Three hundred and two patients with ulcerative colitis treated at the University Hospital of Heraklion, Crete, over a 6-yr period were included. Within this group, a subgroup of 152 patients were studied prospectively for the presence of a positive direct Coombs test. Results: Autoimmune hemolytic anemia was diagnosed in five of 302 patients with ulcerative colitis (1.7%). One more patient developed Coombs-positive hemolytic anemia, attributed to sulfasalazine. A positive Coombs test without evidence of hemolysis was found in three of 152 patients (2%). The mean age of all Coombs-positive patients was 50.5 yr, and there was a definitive male preponderance (male: female, 2:1). Autoimmune hemolytic anemia occurred during active colitis in all cases. The mean time between the onset of colitis and the diagnosis of autoimmune hemolytic anemia was 17 months. Three of five patients with autoimmune hemolytic anemia (60%) and seven of nine of all Coombs-positive patients (77.7%) had total colitis. All patients with autoimmune hemolytic anemia were treated initially with large doses of corticosteroids. Three of five (60%) had good hematological responses. One patient responded to the addition of azathioprine, and one underwent splenectomy and proctocolectomy. Conclusions: In this study, the frequency with which autoimmune hemolytic anemia was associated with ulcerative colitis was higher than in previous reports. The complication occurred early in the course of colitis and was related to activity and extent of the disease. In contrast to others studies, we found a preponderance of males. Although corticosteroids and/or immunosuppressive therapy was successful in most. [ABSTRACT FROM AUTHOR]

Plasma carcinoembryonic antigen (CEA) levels were measured by the Z-Gel technique in 138 patients with benign thyroidopathies, 25 patients with thyroid cancer, and 141 normal persons. Levels were raised (above 5 ng/ml) in 2% of the normal subjects, in none of the patients with benign thyroidopathies, and in 20% of the patients with thyroid cancer. They were considerably raised in all cases of medullary carcinoma of the thyroid but they were also high in other histological types of thyroid cancer. Measurement of plasma CEA may be of value in the preoperative diagnosis and follow-up of patients with thyroid cancer. [ABSTRACT FROM PUBLISHER]

Objective: Lipoprotein (a) is recognized as a risk factor for arterial and venous thrombosis, a property that might be related to its structural similarity to plasminogen. Since patients with inflammatory bowel disease frequently suffer from thromboembolic events, we studied the role of lipoprotein (a) in conjunction with lipids and apolipoproteins in Greek patients with ulcerative colitis and Crohn's disease., Methods: Lipoprotein (a), total cholesterol, high-density lipoprotein cholesterol, low-density lipoprotein cholesterol, triglycerides, apolipoprotein A-1 and apolipoprotein B-100 were determined in sera from 129 consecutive fasting Greek patients with inflammatory bowel disease (66 with ulcerative colitis and 63 with Crohn's disease) and from 66 matched healthy controls., Results: In Crohn's disease patients, the mean serum lipoprotein (a) level was significantly higher than in control patients (41.2 mg/dl vs 22.9 mg/dl; P = 0.005). Mean apolipoprotein A-1 and apolipoprotein B-100 levels were significantly lower in Crohn's disease patients than in the controls. In ulcerative colitis patients the mean levels of lipoprotein (a) and apolipoprotein A-1 were not significantly different to the controls, but the levels of apolipoprotein B-100 were significantly lower. Raised levels of lipoprotein (a) of > 30 mg/dl were found in 29 Crohn's disease patients (46%), 15 ulcerative colitis patients (23%) and 11 control patients (17%). Patients with active Crohn's disease had significantly higher mean lipoprotein (a) and lower apolipoprotein A-1 than patients with non-active disease., Conclusions: Our results suggest that Crohn's disease patients have different lipoprotein (a) and apolipoprotein patterns compared to ulcerative colitis patients and healthy controls. These changes in Crohn's disease patients may possibly expose them to a higher risk of thrombosis.

Objectives: Elevated platelet count is a well recognized marker of inflammatory bowel disease (IBD) activity. Thrombopoietin (TPO) is a critical cytokine in the physiological regulation of thrombopoiesis. The aim of this study was to investigate the serum levels of endogenous TPO in patients with IBD, the relationship between platelet counts and TPO levels, and the correlation of TPO with the clinical characteristics of the patients., Methods: TPO levels in 40 patients with Crohn's disease (CD), 63 patients with ulcerative colitis (UC), and in 42 healthy blood donors were assessed by ELISA. Platelet and white blood cell counts as well as C-reactive protein, and erythrocyte sedimentation rate were measured., Results: TPO levels were significantly elevated in patients with CD (mean 124.3 +/- SD 58.0 pg/ml, p < 0.0001) and in patients with UC (mean 152.2 +/- SD 142.3 pg/ml, p < 0.0001), compared to controls (mean 53.4 +/- SD 45.7 pg/ml). TPO levels remained significantly elevated in remission (mean 144.7 +/- SD 131.1 pg/ml, p < 0.0001 compared to controls). Platelets were significantly elevated only in active CD, being normal in inactive disease as well as in all patients with UC. There was no significant correlation between TPO levels and various clinical characteristics of patients with IBD. No significant correlation was found between TPO levels and either platelet counts or white blood cell counts, erythrocyte sedimentation rate, and C-reactive protein., Conclusions: TPO levels are increased in IBD, irrespective of disease activity, platelet counts, and clinical characteristics of the patients. These observations indicate that TPO, apart from being a platelet producer, might have additional functions, probably related to the procoagulant state of IBD.

Background: Carcinoma of the ampulla of Vater is an infrequent tumor that can be diagnosed, early., Patients and Methods: Twenty-four patients with histologically proven carcinoma of the ampulla of Vater were retrospectively studied and their data were analyzed., Results: Most common presenting symptoms were jaundice (67%), weight loss (58%), fever and pain (54%). Endoscopic biopsies which were taken in 19 patients revealed carcinoma or dysplasia in 15 patients and were normal for in the rest. Twelve patients were treated with a Whipple's resection, 5 with local resection, 2 with palliative surgery and 2 received a stent endoscopically. During a mean follow-up period of 25 months, (range 1-82) 12 deaths were noted, and one patient was lost during follow-up., Conclusions: Presenting symptoms, endoscopic and histological findings were similar as in other series. The contribution of duodenoscopy, ERCP and endoscopic biopsy is essential for diagnosis but endoscopic biopsies may be misleading.

Objective: To study the incidence of ulcerative colitis and to analyse the pattern of the disease in the prefecture of Heraklion, Crete., Participants: The population at risk comprised 263,670 inhabitants in the prefecture of Heraklion (2641 km2). The two regional hospitals, five health centres, 109 private family doctors and 145 specialists participated in the study., Methods: A prospective and population-based epidemiological study of ulcerative colitis over five years from 1990 to the end of 1994., Results: Overall, 117 patients with ulcerative colitis (75 males and 42 females) were newly diagnosed during the study period. The mean annual incidence of the disease for the years 1990-1994 was 8.9 per 10(5) inhabitants (95% CI 7.2-10.4). The male to female ratio was 1.8:1. There were no significant difference between the age-specific incidences of the age groups. The majority (51.3%) of the patients were exsmokers and one-third had never smoked. A family history of first-degree relatives positive for inflammatory bowel disease was obtained in 9.6% of our patients., Conclusion: Ulcerative colitis is common in Crete; its incidence is as high as in Northern Europe.

Besides a genetic predisposition, a causal role of various environmental factors have been taken into consideration in the etiology of inflammatory bowel disease (IBD). The most consistent association of environmental factors so far identified is the association between non smoking and ulcerative colitis (UC) as well a between smoking and Crohn's disease (CD). Other factors such as oral contraceptives, refined sugar, perinatal events, childhood infections, microbial agents, and domestic hygiene have been found to be associated with ulcerative colitis and Crohn's disease but further evaluation is required to confirm the consistency and to define the strength of the association. Recent data also suggest that measles virus may persist in intestinal tissue and early exposure to the virus may be a risk factor for development of CD. The further investigation of environmental factors on IBD and the explanation of their role is expected to open new avenues for basic scientific research and may lead to the development of a more rational approach to the prevention and treatment of IBD. The available data suggest that UC and CD are heterogeneous disorders of multifactorial etiology in which hereditary and environmental factors interact to produce the disease.

Two cases of coexisting ulcerative colitis and Sjogren's syndrome are presented. Both patients were women and in both ulcerative colitis preceded the diagnosis of Sjogren's syndrome by several years. The course of ulcerative colitis before the onset of Sjogren's syndrome was quite severe. On the basis of clinical signs and serological and immunogenetic patterns, both patients were classified as suffering from secondary Sjogren's syndrome. Although the combination of the two diseases in the same patient seems to be the result of chance, we think that this combination offers an opportunity for future studies related to the pathogenesis of ulcerative colitis.

The clinical course and prognosis of ulcerative colitis were studied in a group of 205 Greek patients. The study lasted for 6 years and follow-up was achieved in 98% of the patients. In most patients the disease was confined to the left bowel and it was of mild or moderate severity. Mortality was absent during the first attack and low at completion of the study while the cumulative colectomy rate was 14%. Bad prognostic factors leading to severe attacks and colectomy were: extensive disease, young age at onset and continuous symptoms. Compared to highly developed countries, the disease in Greece seems to run a milder course as can be judged by the better outcome of severe attacks and the more favorable short-term prognosis.

Intestinal transit times and daily stool weights were measured in 14 residents of Athens and 22 residents of a small village of Greece. Subjects in rural Greece have significantly shorter transit times (38.4 vs 56.8 hr) and heavier daily stools (172 vs 131 g) than those living in Athens. These findings may be attributable to the more refined diet of the urban Greek population and may explain the different incidence of certain bowel diseases in the urban and rural population of Greece.

A case of alpha-chain disease (alpha-CD) is described presenting as a malabsorption syndrome complicated by severe kalipenic nephropathy. Antibiotics and cyclophosphamide were effective in controlling the malabsorption and thr renal dysfunction but did not affect the basic pathological lesion. Eventually the disease became generalized, affected lymph nodes, liver and bone marrow and caused multiple intestinal perforations. Detailed histological studies failed to demonstrate the development of reticulum-cell sarcoma as a terminal complication of the disease and are suggestive of a single malignant plasmacytic cell process.