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Objectives: Few data are available to describe the changes in incidence of pediatric-onset inflammatory bowel disease (IBD). The aim of this study was to describe changes in incidence and phenotypic presentation of pediatric-onset IBD in northern France during a 24-year period., Methods: Pediatric-onset IBD (<17 years) was issued from a population-based IBD study in France between 1988 and 2011. Age groups and digestive location were defined according to the Paris classification., Results: 1,350 incident cases were recorded (8.3% of all IBD) including 990 Crohn's disease (CD), 326 ulcerative colitis (UC) and 34 IBD unclassified (IBDU). Median age at diagnosis was similar in CD (14.4 years (Q1=11.8-Q3=16.0)) and UC (14.0 years (11.0-16.0)) and did not change over time. There were significantly more males with CD (females/males=0.82) than UC (females/males=1.25) (P=0.0042). Median time between onset of symptoms and IBD diagnosis was consistently 3 months (1-6). Mean incidence was 4.4/10 5 for IBD overall (3.2 for CD, 1.1 for UC and 0.1 for IBDU). From 1988-1990 to 2009-2011, a dramatic increase in incidences of both CD and UC were observed in adolescents (10-16 years): for CD from 4.2 to 9.5/10 5 (+126%; P<0.001) and for UC, from 1.6 to 4.1/10 5 (+156%; P<0.001). No modification in age or location at diagnosis was observed in either CD or UC., Conclusions: In this population-based study, CD and UC incidences increased dramatically in adolescents across a 24-year span, suggesting that one or more strong environmental factors may predispose this population to IBD.

Objective: We report the cases of two patients with the complete CREST variant (calcinosis, Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly, telangiectasia) of systemic sclerosis (SSc) who developed autoimmune hepatitis., Results: Our findings suggest that autoimmune hepatitis can be considered to be one of the liver manifestations associated with SSc. Our data also indicate that, because liver involvement may precede skin manifestations, evaluation for SSc is appropriate when autoimmune hepatitis is noted, and that the evaluation should include clinical examination, testing for antinuclear antibodies (especially for anticentromere antibodies) and nailfold capillaroscopy., Conclusions: From a practical point of view, our two cases emphasize that suspicion of autoimmune hepatitis in SSc patients presenting with cytolytic hepatitis will help to achieve both accurate diagnosis and optimal management.

Objective: To identify risk factors for gastrointestinal bleeding (GIB) among users of non-aspirin, non-steroidal anti-inflammatory drugs (NANSAIDs)., Design: Case-control study., Participants: A total of 120 patients aged over 60 years and using NANSAIDs were hospitalized between January 1988 and September 1992 for GIB related to erosions or ulceration of the gastroduodenal mucosa. A group of 100 general practitioners selected two controls matched for age and sex, receiving NANSAIDs and without GIB, for each patient., Methods: The same questionnaire was used to interview patients and controls about their medical history, use of NANSAIDs and other drugs, alcohol and tobacco use, recent stress and nutritional status., Results: The adjusted odds ratios (OR) for the risk factors related to the pattern of NANSAID use were 3.39 [95% confidence interval (CI) 1.77-6.47] when the intake of NANSAIDs was followed by decubitus, 3.00 (95% CI 1.54-5.85) when NANSAIDs were taken before a meal, 6.05 (95% CI 2.10 17.43) with a high dose of NANSAIDs, 5.87 (95% CI 2.00-17.25) with recent NANSAID use, 3.35 (95% CI 1.47-7.64) with NANSAIDs associated with aspirin use, 3.46 (95% CI 1.15-10.36) with more than one NANSAID, and 10.70 (95% CI 1.06-108.07) when NANSAIDs were associated with corticosteroids. The patient-related risk factors and their OR were 9.94 (95% CI 3.29-24.28) for irregular food intake, 3.94 (95% CI 1.45-10.69) for previous peptic ulcer, 3.71 (95% CI 1.26-10.89) for recent weight loss, 4.44 (95% CI 1.48-13.30) for heavy alcohol abuse, 2.92 (95% CI 1.36-6.26) for recent stress and 5.26 (95% CI 1.19-23.33) for a past history of GIB., Conclusion: This study identified a group at 'high risk' for GIB which would benefit from the development of a prophylactic therapy.

Gaucher's disease is characterized by accumulation of glucocerebroside (caused by an autosomally inherited deficiency of glucocerebrosidase) in the cells of the reticuloendothelial system. We report the clinical, laboratory, radiologic features of 17 patients with type 1 Gaucher's disease.

The pharmacokinetics of pyrazinamide (Pirilène) and its metabolites are evaluated in ten subjects with hepatic insufficiency, after an oral dose of 19.3 +/- 0.6 mg.kg-1 and the results are compared to those of a group of nine healthy subjects (control group). The results exhibit a marked reduction of the pyrazinamide total clearance (0.48 vs 0.84 ml.min-1.kg-1) and an increase in half-life from 9.19 h to 15.07 h in the patients group. The area under the curve of pyrazinoic acid (the main metabolite) is increased from 97 to 280 mg.h.l-1 with a half-life twice as much as that of the control group. The hepatic insufficiency entails a marked reduction of the common posology as well as a closer survey of the biologic hepatic parameters and of uric acid the renal elimination of which is inhibited by pyrazinoic acid.

The authors describe a recent case of agenesis of the right lobe of the liver. Such cases are rare and can be diagnosed with state of the art imaging modalities, ultrasound and computed tomography, which permit early diagnosis and help avoid liver complications of cirrhosis, cholangiocarcinoma and hepatoma. This type of congenital anomaly can be associated with biliary tract disease, portal hypertension or other congenital anomalies.

The aim of this work was to assess the usefulness of somatostatin in acute peptic ulcer bleeding. Sixty-four patients with endoscopic evidence of duodenal ulcer bleeding completed a double-blind trial to compare the effectiveness of somatostain (6 mg i.v. per day) with that of placebo; incidence of treatment failure (i.e., rebleeding or persistent haemorrhage) was not different in the somatostatin and in the placebo groups (respectively 24% and 23%). Because the efficacy of cimetidine had previously been demonstrated in gastric ulcer bleeding, somatostatin was tested against cimetidine (1.6 g per day) in 50 patients with gastric ulcer haemorrhage. The treatment failed to control bleeding in 15% of somatostatin and in 17% of cimetidine treated subjects (n.s.). Both treatments were well tolerated and blood requirements were not different in the trial groups. These results show that the efficacy of somatostain in peptic ulcer bleeding is not different from placebo in duodenal ulcers and not different from cimetidine in gastric ulcers.

A 17 year-old girl with chronic idiopathic intestinal pseudo-obstruction is reported. Abnormalities of smooth intestinal muscle were shown on light and electron microscopic studies of the excised small intestine and led to the diagnosis of visceral myopathy based on the following features: vacuolar degeneration of intestinal smooth muscle cells with replacement by fibrous preferential involvement of the external longitudinal muscle layer normal myenteric plexus. For the first time similar ultrastructural changes were found on histological study of the colon. Manometric studies revealed a diffuse disease involving the esophagus, small bowel, and bladder. Anorectal abnormalities, never described before, were reported. Family involvement was shown by abnormal esophageal and anorectal manometries in the patient's brother and by paternal history of fatal small intestine occlusion without mechanical obstruction. The prognosis of severe forms of visceral myopathy is generally poor because of the inefficiency of drugs. In this case, after a long period of parenteral nutrition with maintenance of a good nutritional status, a terminal ileostomy (with a special procedure to avoid evagination) associated with a second stage total colectomy allowed to stop parenteral nutrition.

The present study of hepatic mixed function oxidase activity was carried out by determining antipyrine clearance in 49 patients presenting with energy malnutrition (group E, n = 26) or global protein-calorie malnutrition (group P, n = 23). A control group (group C, n = 25) was composed of subjects with good nutritional status. The metabolic clearance rate and weight-corrected clearance in group P were significantly lower than those in groups E and C. The weight clearances in the latter two groups were not significantly different, suggesting that mixed function oxidase activity decreases only in protein-calorie malnutrition. Antipyrine clearance was studied again in 27 patients after nutritional rehabilitation with artificial nutrition for 31 +/- 4 days (means +/- SEM). Concomitant with an improvement in nutritional state, clearances tended towards normal values in group P (n = 17) and were not significantly modified in group E (n = 10). It is thus important to take the type of malnutrition into account in pharmacological studies of malnourished humans in order to correctly adapt therapeutic doses.

The aim of this study was to assess, by a pragmatic controlled trial, the value of propranolol in the prevention of recurrent bleeding in patients with cirrhosis. From January 1982 to December 1984, 368 cirrhotic patients were admitted for bleeding episode due to portal hypertension. Ninety-nine patients were included in the trial 24 h after cessation of bleeding (5 days on the average after hospital admission) and were randomly assigned to 2 groups: 51 patients receiving propranolol at doses that reduced the resting heart rate by 25 p. 100 and 48 receiving neither treatment nor placebo. Ninety-one per cent presented a rupture of varices, and 9 p. 100 acute gastric erosions: 94 p. 100 had an alcoholic cirrhosis. The distribution according to Child Turcotte's classification was grade A: 45.5 p. 100, B: 45.5 p. 100, C: 100 p. 100. The two groups were not significantly different excepted for previous bleeding episodes. The cumulative percentages of recurrent bleeding were not significantly different: between the treated and untreated groups (60 p. 100 vs. 68 p. 100 at 18 months); between the compliant patients (n = 39) and untreated and not compliant patients altogether; between the treated and untreated patients of Child's grade A on the one hand, and patients grades B and C on the other hand. The curves of cumulative survival were not significantly different between treated and untreated patients (66 p. 100 vs. 78 p. 100 at 18 months).(ABSTRACT TRUNCATED AT 250 WORDS)

The present work was conducted in patients with severe cimetidine-resistant duodenal ulcer, in order to correlate the response (i. e. healing or no healing of the ulcer) to ranitidine therapy and the results of 24 h gastric pH monitoring performed under therapeutic conditions. Twenty patients who fulfilled the criteria of cimetidine resistance (i. e. with an ulcer not healed after a treatment with cimetidine given at a dose of at least 1 g/d for at least 6 weeks) received 150 mg ranitidine twice daily for 6 weeks and were then controlled endoscopically; ulcer was healed in 8 cases, not healed in 12 cases. Nocturnal gastric acid inhibition was significantly greater in patients who responded to ranitidine than in those whose ulcer did not heal. During daytime the gastric pH profiles were not different between these two groups. Nocturnal pH score was also calculated in each patient by reference to the mean inhibitory effect observed in 8 normal subjects in whom gastric pH was monitored in the same therapeutic conditions. In 7 patients the nocturnal pH score was greater than 10; none of these patients was healed after 6 weeks ranitidine treatment. Among the 13 patients with a pH score lower than 10, 8 had an ulcer healed at the end of the ranitidine course. In 5 cases gastric pH monitoring was repeated after continuation of the ranitidine treatment; healing of ulcer was observed in 4 cases, always associated with a nocturnal pH score lower than 10 (in 2 cases after increasing the dosage of ranitidine).(ABSTRACT TRUNCATED AT 250 WORDS)