Intestinal carcinoid tumor and myotonic dystrophy. A new association?

The occurrence of multiple carcinoid tumors of the small bowel with liver metastases is reported in a patient with a previous myotonic dystrophy. In addition to the association of myotonic dystrophy with multiple endocrine adenomatosis type 2A, hyperparathyroidism, and neurofibromatosis, this case report gives further evidence for the propensity of such patients to develop neuroendocrine tumors.

[Non-surgical treatment of cysts and pseudocysts of the pancreas. A study of a series of 33 cases]. / Traitement non chirurgical des kystes et pseudo-kystes du pancréas. Etude d'une série de 33 cas.

Pancreatic cysts and pseudocysts had to be treated by surgery until 15 years ago. Nowadays they can be aspirated (or drained) either endoscopically or by ultrasonic and computed tomographic guided punctures. The aim of this study was to see if these non-surgical treatments were efficient enough among the actual treatments. From 1984 to 1988, 33 patients were admitted in one single institution with a pancreatic cyst or pseudocyst, 22 of which were a consequence on an acute pancreatitis and 11 complicated a chronic pancreatitis. Ten cysts were connected with the Wirsung channel while 11 others were not, but the ERCP failed to give any accurate information on this point in 12 cases. As a first treatment, we abstained of any invasive procedure in 9 cases, 18 were treated by percutaneous aspiration guided by ultrasound and 6 patients underwent surgery. The mean follow-up was 30 months. All the patients who had no treatment remained symptom free; their cysts improved in 5 cases and disappeared in 4 cases. Among the cysts treated by percutaneous aspiration, 4 had to be operated, 5 disappeared, 3 improved and 6 recurred; the percutaneous aspiration obtained a lasting symptoms' relief for these patients, whatever the connection the cyst had with the Wirsung channel. This study suggests that percutaneous aspiration or drainage guided by ultrasound may be a treatment of the highest quality for acute and chronic pancreatitis cystic formations. However, it seems to act upon the symptoms rather than upon the cystic formations themselves.

[Mononuclear cell activation in Crohn's disease. Evaluation using serum assay of neopterin and interleukin-2 soluble receptors]. / Activation des cellules mononucléées dans la maladie de Crohn. Evaluation par le dosage sérique de la néoptérine et des récepteurs solubles de l'interleukine-2 (RS-IL-2).

To determine the degree of mononuclear blood cell activation in Crohn's disease (CD), 65 patients were prospectively investigated (22 with mild, 26 with moderate and 17 with severe disease). Serum levels of soluble receptors for interleukin-2 (SR-IL-2) were measured by ELISA. In CD patients SR-IL-2 levels were significantly higher (m = 707 +/- 326 U/ml) than in three other groups: 70 controls (m = 258 +/- 87 U/ml, p less than 0.0001); 8 patients with acute infectious colitis (m = 405 +/- 216 U/ml, p less than 0.0001); 101 HIV seropositive subjects (m = 564 +/- 216 U/ml, p less than 0.002). There was a positive correlation between SR-IL-2 level and the Van Hees activity index (r = 0.595, p less than 0.0001). On the other hand, the numbers of activated T cells (CD 3+, HLA DR+), CD 4+, CD 8+ and NK cells did not differ according to the CD activity groups. Furthermore, CD patients treated with steroids (n = 39) did not differ from those without any medication. As a marker of monocyte activation, serum neopterin level was determined by RIA. All CD patients considered as a group, serum neopterin level was 2.89 +/- 1.44 ng/l (n less than 2.5 ng/l). Neopterin level increased with disease activity (1.97 +/- 0.92 vs 3.10 +/- 1.46 vs 3.74 +/- 1.36, p less than 0.01), and was positively correlated with SR-IL-2 (r = 0.609, p less than 0.0001). These results suggest a monocyte-macrophage activation in CD, which parallels disease activity.(ABSTRACT TRUNCATED AT 250 WORDS)

[Arterial thrombotic complications in Crohn's disease]. / Accidents thrombotiques artériels au cours de la maladie de Crohn.

In a series of 230 observations of Crohn's disease, the authors describe 4 cases of arterial thrombosis; two of them involving cerebral arteries. These complications occurred in young women without any notable risk factor for atheroma. All patients had highly active Crohn's disease when arterial thrombosis occurred: two of them had several episodes of thrombosis and three, extraintestinal manifestations. As the arterial thromboses are often severe, rarely foreseeable and the venous thromboses frequent, the point is whether to use anticoagulants. When Crohn's disease is highly active, but only if there are no hemorrhagic lesions, anticoagulants at prophylactic doses may be recommended. How to define more exactly a high risk thromboses population deserves further investigation.

[Socioprofessional consequences of Crohn disease]. / Répercussions socio-professionnelles de la maladie de Crohn.

To evaluate evolutivity and social repercussions of Crohn's disease, the same gastroenterologist interviewed 151 out of 197 patients with Crohn's disease (77.5 percent) who had been hospitalized from 1964 to 1986 in our unit. The other 46 could not be traced or refused to participate. There was no difference regarding age, sex, duration of illness, pattern of lesions and operations between the two groups. At the time of observation, mean follow-up was 7.8 years and two thirds of the patients had inactive disease. During the previous year, the course of the disease was quiescent in 50 percent of all patients; this rate increased with duration of follow-up and time from surgery. A constant rate of stable course was observed (20 percent) irrespective of duration of disease and time of surgery. The interviews were compared with those obtained from an age and sex-matched group of 70 previously healthy subjects admitted to the hospital for less than one month. The education and socioeconomic levels were higher in patients with Crohn's disease than in controls, in spite of the fact that 25 percent of patients whose disease begun during scholarship complained of difficulties at school, and nearly 20 percent of all patients were partially or totally unable to work. Sports and cultural activities were the same in Crohn's disease and in the control group. Tobacco intake was slightly higher at time of diagnosis but it was equal in both groups at the time of the interview. Non allopathic advice or treatment were sought by 48 percent of Crohn's disease patients (7 percent in controls).(ABSTRACT TRUNCATED AT 250 WORDS)

[Monoclonal anti-AFP immunoscintigraphy of human hepatocellular carcinoma. Influence of biological, anatomical and vascular factors]. / Immunoscintigraphie monoclonale anti-AFP du carcinome hépatocellulaire humain. Influence des facteurs biologiques, anatomiques et vasculaires.

The actual interest of immunoscintigraphy for the detection of alphafetoprotein-producing liver tumors was investigated by both visual examination and quantitative analysis in 61 patients with either hepatocellular carcinoma (39 patients, group I), secondary liver cancer (11 patients, group II), or non tumoral liver (9 cirrhosis, 2 healthy liver, group III): All patients received injections of 123I-anti-alphafoetoprotein monoclonal antibodies and planar scans were performed after 28 hours. Only 18 out of 39 hepatocellular carcinoma-bearing patients had a positive scan (46 p. 100). Such a moderate sensibility was due to a striking inhibitory influence of cirrhosis: the positivity rate was 6/24 and 12/15 respectively when hepatocellular carcinoma was and was not associated with cirrhosis (p less than 0.01). Specificity was also moderate (55 p. 100) for detection of hepatocellular carcinoma in tumor-bearing patients (group I and II). In the absence of cirrhosis, the intensity of the tumoral uptake was highly correlated with high serum alphafoetoprotein level (p less than 0.001) and tumor arterial hypervascularization (p less than 0.01). Anti-alphafoetoprotein monoclonal antibody uptake by the extratumoral liver was found to be very specific of hepatocellular carcinoma since it was high in 23 out of 39 patients of group I but in only 1 out of 22 in groups II and III (p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

[Hepatobiliary actinomycosis. Apropos of 3 cases with pseudocancerous aspects]. / Actinomycose hépato-biliaire. A propos de 3 cas d'allure pseudo-cancéreuse.

Three cases of hepatobiliary actinomycosis are reported. In all cases, carcinoma was initially suspected. One patient underwent cytoaspiration under sonographic guidance. Diagnosis was obtained by autopsy in one case, laparotomy and biopsy in the other cases. Confusion with cancer is frequent in actinomycosis, especially in hepatic lesions.

Is there an increased incidence of adenomatous polyps in breast cancer patients?

Using flexible proctosigmoidoscopy, the incidence of adenomatous polyps was studied in 161 patients previously operated on for breast cancer and also in 147 hospitalized controls not presenting with colorectal symptoms. The acceptance and tolerance of the examination were good in both populations. The mean age and length of the colons explored were not statistically different for the two groups. The incidence of adenomas was high in the breast cancer group (14.2%) in comparison to the control group (4.7%) (P less than 0.01). This strong incidence especially concerned very small polyps with a diameter smaller than 3 mm. There were also two polyps with superficial carcinomas in the breast cancer group. Breast cancer does indeed seem to represent a condition with a high risk of colorectal adenomas. Our results prompt us to propose the adoption of systematic screening measures and a surveillance schedule as soon as the breast cancer is found.

[Efficacy of endoscopic pneumatic dilatation in achalasia. Medium-term results]. / Efficacité de la dilatation pneumatique perendoscopique dans l'achalasie. Résultats à moyen terme.

During a seven-year period, thirty patients with achalasia of the esophagus were treated by pneumatic dilatation without complications. Only twenty patients could be followed with a mean follow of 32 +/- 23 months after their initial dilatation. Excellent to good results were obtained in more than 75 p. cent of the group. Most patients have not required a further dilatation. This study suggest to propose a regular supervision of these patients by clinical examination, esophageal manometry and endoscopy.

[Treatment using lymphocytapheresis of severe forms of Crohn's disease. Preliminary results]. / Traitement par lymphocytaphérèses des formes sévères de maladie de Crohn. Résultats préliminaires.

We report our experience in the treatment of severe Crohn's disease by lymphocytapheresis (LCTPH). Twelve patients with severe Crohn's disease unresponsive to prednisone for two years were treated by twelve sessions of LCTPH over a three-week period. Approximately 4.5 x 10(9) lymphocytes were removed at the end of each session, total lymphocyte removal per patient ranging from 1.9 to 10.7 x 10(8)/kg. Sessions were well tolerated. Clinical improvement was observed in all patients at the end of their LCTPH courses. However only fifty per cent of these patients achieved clinical remission of three months and for two of them the remission lasted 8 and 9 months. We examined the immediate effects of cytapheresis on peripheral blood lymphocytes and monocytes. No correlation was noted between the clinical efficacy of LCTPH and the quantity of lymphocytes (T3, T4, T8, NK) and/or monocytes removed. The only significant change was a partial recovery of NK activity in the good responders. The optimal modalities of depletion and the advantages of this treatment in severe Crohn's disease have to be defined in multicentric controlled studies.

[Congenital cystic dilatation of the common bile duct, anomaly of the biliopancreatic junction and cancer of the bile ducts]. / Dilatation kystique congénitale du cholédoque, anomalie de la jonction bilio-pancréatique et cancer des voies biliaires.

The choledochus and pancreatic duct ordinarily enter the duodenum either separately or via a common channel located in the duodenal wall. The usual maximal length of the common channel is approximately 0.5 cm. An anomalous junction of the pancreaticobiliary tract is defined by the presence of an unusually long common channel whose length is 1 cm or longer. During the last 8 years, we studied 9 cases of congenital dilatation of the bile duct. Besides the choledochal cyst, all patients had a common channel which measured 1.5 cm or longer. The mean age of patients (6 females, 3 males) was 36.5 years. Three patients had extrahepatic bile tract carcinoma. One patient, a 51-year old man, had gallbladder carcinoma without stones while the two other patients, a 43-year old woman and a 32-year old man, had carcinoma arising from the wall of the cystic dilatation. These two patients had undergone a simple internal drainage procedure 16 and 8 years before, respectively. Our study suggests that the anomalous junction of the pancreaticobiliary tract, an embryonic anomaly which allows the reflux of pancreatic juice into the bile duct and gallbladder, may be responsible for congenital bile duct dilatation and biliary tract carcinoma. Total excision of the cystic dilatation and the gallbladder with hepaticojejunostomy is the appropriate surgical treatment.

[Specific gastric localization of Wegener's disease]. / Localisation gastrique spécifique de la maladie de Wegener.

We report an unusual gastric ulcer disease in a 52 year old white man which relapsed despite antacids and anti-H2 treatment. During the course of the disease, fever, weight loss, and ocular, oropharyngeal as well as systemic manifestations occurred. Gastric biopsies were normal until macrobiopsy was performed 5 months later demonstrating necrotizing granulomatous vasculitis. Further identical oropharyngeal mucosal lesions and a pulmonary infiltrate occurred supporting the diagnosis of Wegener's disease. The subsequent course was complicated by a massive hemorrhage which required total gastrectomy. Despite specific treatment with cyclophosphamide and steroids, the patient died. Most of the gastrointestinal lesions consistent with Wegener's disease are usually found at necropsy. Clinical gastrointestinal manifestations are scarce and rarely due to the underlying disease. Prognosis depends upon the renal lesions. However, as well, involvement of the digestive tract seems to be a factor of poor evolution of the disease.

[Value of dapsone in the treatment of digestive manifestations of rheumatoid purpura in adults]. / Intérêt de la dapsone dans le traitement des manifestations digestives du purpura rhumatoïde de l'adulte.

The authors report a case of adult rheumatoid purpura with severe gastrointestinal complications which responded extremely well to treatment with Dapsone. Although this drug has been reported to be effective in treating some forms of allergic vasculitis and the cutaneous manifestations of rheumatic purpura, there have not been any previous reports of its beneficial action in the treatment of the gastrointestinal complications of rheumatoid purpura. The mode of action is not fully understood. Dapsone could constitute a valuable therapeutic option together with steroids, parenteral nutrition and plasmapheresis in the treatment of the gastrointestinal complications of rheumatoid purpura.

[Digestive manifestations in a patient with type-IIB multiple endocrine neoplasms]. / Manifestations digestives au cours d'une néoplasie endocrinienne multiple de type IIB.

The IIB or third type of multiple endocrine neoplasia is a very uncommon hereditary disease. It includes a digestive ganglioneuromatosis, a typical dysmorphia, a medullary thyroid cancer (MTC) sometimes a pheochromocytoma and seldom a hyperparathyroidism. We report a Men IIB case in a 19 years old patient who had been suffering from severe constipation and afflicted with a megacolon, known since early infancy. Diagnosis was documented by discovering a colonic ganglioneuromatosis and a MTC. A complete thyroidectomy was performed but cervical node metastasis were already present. The ganglioneuromatosis usually affects the whole alimentary tract, but megacolon although not regularly found occurs most frequently. Awareness of the diagnosis is easier when patients have big lips, eyelids, tongue (due to mucosal neuromas) and marfanoid habitus. The prognosis depends upon the MTC. It occurs early in the life, is often bilateral and has a trend to become uncontrollable in the young adult. The pheochromocytoma is often latent and affects only half of patients. The etiopathogeny of this disease still remains unknown. Family inquiry may help to an earlier diagnosis and therefore to a better prognosis.

[Esophagogastroduodenal localizations of Crohn's disease are frequent]. / Les localisations oesogastroduodénales de la maladie de Crohn sont fréquentes.

The purpose of this study was to determine the endoscopic features, actual frequency and consequences of upper digestive involvement in Crohn's disease (CD). Between April 1973 and April 1985, one or several upper digestive tract endoscopies with biopsies were performed in 129 of 195 patients admitted for ileal and/or colorectal CD. Endoscopic lesions were small and superficial: aphtous and superficial ulcerations, or erythematous areas. The diagnosis of upper digestive involvement was probable in 36 of the 129 patients who underwent upper endoscopies (28 p. 100), certain (microscopic evidence of granuloma) in 21 patients (16 p. 100). Lesions were isolated or multifocal but their preferential sites were the antrum and proximal duodenum. Histologic study of biopsies showed that granuloma were as frequent in upper as in lower intestinal lesions of CD. A frequency of 20 p. 100 seems to us to be more correct than the 5 p. 100 usually admitted. In our experience, upper digestive involvement in CD could not be regarded as a pejorative prognostic factor. It was sometimes possible to identify colitis or a peptic ulcer disease as a lower or upper digestive manifestation of CD, respectively.