Clinical characteristics of Crohn's disease in 72 families.

BACKGROUND & AIMS: Familial aggregation argues for genetic susceptibility to Crohn's disease. The aim of this study was to compare the age of onset and the clinical features of Crohn's disease between patients with familial disease and those with sporadic disease and investigate the concordance for disease location and type among relatives with Crohn's disease. METHODS: Seventy-two families with 2 (n = 55), 3 (n = 8), 4 (n = 6), and 5 or more (n = 3) affected first-degree relatives were selected for the study. A population of 1377 patients with sporadic nonfamilial Crohn's disease was used for comparison. RESULTS: Clinical data were obtained from 176 patients with familial Crohn's disease (79 men and 97 women). Median age at onset was younger in familial Crohn's disease than in sporadic cases: 22 vs. 26.5 years (P < 0.01). In familial cases, fewer patients had exclusively colonic involvement and more patients had both small bowel and colonic involvement. Among relatives of families with 2 affected members, 56% were concordant for disease location and 49% for disease type. These percentages reached 83% and 76%, respectively, within families with more than 2 affected members. CONCLUSIONS: Patients with familial Crohn's disease are characterized by an early age at onset with more extensive disease and may represent a homogeneous clinical subgroup with a particularly strong genetic influence.

[Severe and recurrent digestive hemorrhage: an uncommon form of colonic schistosomiasis]. / Hémorragie digestive grave et récidivante: une forme exceptionnelle de bilharziose colique.

Schistosoma mansoni infection is frequent in certain areas of the world in which it is endemic. It is characterised by colonic and hepatic lesions. Gastrointestinal hemorrhages due to rupture of esophageal varices in case of hepatic involvement or moderate rectal bleeding due to colonic involvement may also be observed. We report a case of colonic Schistosoma mansoni infection presenting exclusively with recurrent episodes of serious gastrointestinal hemorrhage without hepatic lesions. All diagnostic investigations were negative. The diagnosis was only established on histological examination of the operative left hemicolectomy carried out with the utmost emergency for the serious recurrent hemorrhage. Histological examination revealed the presence of mucosal micro-ulcers, capillary neovascularization in the submucosa and serosa, and the presence of fibrous nodules and giant cell granulomas surrounding the eggs of Schistosoma mansoni in the serosa. This case is original by its clinical presentation and the difficulty to diagnose the Schistosomiasis.

Brain noradrenergic systems modulate the ceco-colonic myoelectric activity in rats.

The role of the brain noradrenergic systems in the control of the ceco-colonic myoelectric activity was investigated in rats following lesions with intracerebroventricular (icv) or intracisternal (ic) injection of 6-hydroxydopamine (6-OHDA). Controls received the vehicle alone. The ceco-colonic myoelectric activity was recorded 3 weeks later in conscious rats chronically fitted with electrodes. After icv injection of 6-OHDA, lesions of rostral and caudal (spinal) noradrenergic systems were observed whereas only spinal noradrenergic systems were lesioned after ic injection. This differential pattern of lesions was followed by a differential pattern of ceco-colonic myoelectric activity. In fasted animals, a significant increase of the long spike burst (LSB) frequency (nb min-1) was observed after icv injection of 6-OHDA whereas no modification was observed after ic injection of the neurotoxic. After a 6-g pelleted rat diet, a significant increase of the LSB frequency was also observed in the icv lesioned group when compared to controls. No modification of the ceco-colonic noradrenergic innervation was observed, thus confirming the central selectivity of these lesions. Lesions of central noradrenergic systems modify the LSB frequency in rats; the rostral noradrenergic systems seem to play the major role.

[Is the intestinal lavage with a solution of mannitol effective in the prevention of post-hemorrhagic hepatic encephalopathy in patients with liver cirrhosis? Results of a randomized prospective study]. / Le lavage intestinal par une solution au mannitol est-il efficace dans la prévention de l'encéphalopathie hépatique post-hémorragique chez le malade atteint de cirrhose?

OBJECTIVES: The aim of this study was to evaluate the usefulness of whole gut irrigation with a mannitol solution in the prevention of hepatic encephalopathy in patients with cirrhosis and bleeding oesophageal or gastric varices. METHODS: The randomized prospective study included 40 patients with a mean age of 59.6 +/- 9.6 years. Bleeding was controlled by a Blakemore or Linton haemostatic tube, in all cases. The patients were divided into a "mannitol" group (n = 20) treated by whole gut irrigation with a 5 L solution containing 40 g/L of mannitol, and a "control" group (n = 20). The two groups did not differ for age, sex, aetiology of cirrhosis or gastrointestinal bleeding, Child-Pugh classification, delay of minimum fare or the number of transfused blood units. The onset of hepatic encephalopathy was investigated within the first 48 hours after the beginning of gastrointestinal bleeding. The grade of hepatic encephalopathy was established using an index with 4 criteria (mental status, asterixis, number connection test, arterial ammonia concentrations), each with 5 grades of severity. RESULTS: There were no adverse side-effects in the mannitol group. The incidence of hepatic encephalopathy (5 vs 30%) and the length of hospitalization (8.2 +/- 2.3 vs 13.6 +/- 3.1 days) were significantly decreased in the mannitol group (P < 0.05). Mortality was not different between the 2 groups (P = 0.4). CONCLUSION: Whole gut mannitol irrigation appears to be an effective, simple, inexpensive, well tolerated technique in the prevention of hepatic encephalopathy after variceal bleeding in patients with cirrhosis.

[Can early recurrent hemorrhage in gastroduodenal ulcer be predicted?]. / Peut-on prédire la récidive hémorragique précoce des ulcères gastroduodénaux?

OBJECTIVE: To evaluate clinical, biological and endoscopic predictive factors of early recurrent bleeding from gastric or duodenal ulcers. PATIENTS AND METHODS: Seventy six patients (26 females and 50 males) with a mean age of 65.9 years (SD = 17.2) were included in a prospective study. Among the 76 patients, there were 39 NSAIDs users (51.3%). An endoscopy was performed systematically until 12 hours after admission. Treatment modalities were identical for all patients. The predictive value of 11 factors was evaluated through an univariate and multivariate analysis. RESULTS: Three factors had independent significant predictive value: a) the number of blood units used to treat a shock and to increase the haemoglobin level up to 100 g/L (P < 0.05); b) "high endoscopic risk" of recurrent bleeding including Forrest Ia, IIa and IIb ulcers (P < 0.05); c) a non steroidal anti-inflammatory treatment was associated with no recurrent bleeding contrary to the other factors (P < 0.05). Based on a second multivariate analysis including clinical factors only, a predictive score was calculated: 5 + number of blood units -5 x (NSAID = 0/1). The cut off point with maximum discrimination was 6 (specificity = 79.6%; sensitivity = 77.2%). CONCLUSION: A combination of clinical and endoscopic factors is useful to predict ulcer recurrent bleeding. Our clinical predictive score is interesting because of its simplicity. Its predictive value is of interest but have to be evaluated in another sample of patients.

Role of upper esophageal reflex and belch reflex dysfunctions in noncardiac chest pain.

Fifty-four patients examined for noncardiac chest pain (NCCP), showing no esophageal motor disorder or gastroesophageal reflux disease compatible with NCCP, were subjected to an intraesophageal balloon distension test and a study of the belching reflex provoked by intraesophageal air injection. Thirty-three control subjects were also studied, allowing us to define high-threshold belchers (group I) as those who belched during two of three 40-ml distensions and low-threshold belchers (group II) as those who did not. The balloon distension test induced NCCP in 64% of the patients in group I, and in 14% of the patients in group II (P < 0.01). High-threshold belching was a factor favoring the positivity of the balloon distension test. This result supports the hypothesis that esophageal distension by air due to a belching disorder may be the mechanism responsible for NCCP in some patients with an abnormal sensitivity to balloon distension.

Chronic gastritis: prevalence in the French population. CIRIG.

Chronic gastritis (CG) which can be associated with severe complications, is a frequent phenomenon in gastroenterological practice. No data concerning the prevalence of GC are available in France. The aim of this study was to evaluate the prevalence of CG on biopsy specimens in the French population. All outpatients coming from different towns of seven French areas and undergoing an upper endoscopy on one or two randomly selected consecutive days were included in the study. A case report form was filled out and 5 biopsy specimens were taken from the fundus and antrum. Pathologists graded the specimens on the basis of Whitehead's classification. Seven hundred and forty-two patients were enrolled by 102 private gastroenterologists. Mean age was 53 years; 52% were males. Endoscopically, the gastric mucosa was abnormal in 53%. Superficial and atrophic CG was diagnosed in 53% of cases. Superficial gastritis was observed in 101 patients (14%), antral chronic atrophic gastritis (CAG) in 189 (26%) and fundic CAG in 17 (2%). Patients with CAG were significantly older than the other patients. There were more smokers and heavy alcohol drinkers in the group of CAG patients. Endoscopy and histology were in accordance in only 55.2% of the patients: most cases of CAG were not detected by endoscopy. These results are similar to other studies in European populations. The principal pattern among the various types of gastritis was CAG. The other forms were rare. Autoimmune gastritis was uncommon in this French population (4% of the CAG patients).

[Churg-Strauss syndrome with rectal localization and exudative enteropathy]. / Syndrome de Churg et Strauss avec localisation rectale et entéropathie exsudative.

The authors report the case of a 61-year old patient with a Churg-Strauss syndrome revealed by abdominal pain. Investigations showed a diffuse inflammatory digestive tract involvement, documented by endoscopy associated with protein-losing enteropathy. Deep rectal biopsy revealed vasculitis in an otherwise macroscopically normal rectum. Faced with acute and life-threatening course of disease, emergency medical treatment with steroids and cyclophosphamide led to rapid regression of clinical, biological and radiological abnormalities.

Involvement of hypothalamic noradrenergic systems in the modulation of intestinal motility in rats.

Selective lesions of the noradrenergic systems of the paraventricular nucleus (PVN) of the hypothalamus with 6-hydroxydopamine (6-OHDA) lengthen the periodicity of the migrating myoelectric complex (MMC), an index of intestinal motility, in rats. These lengthening effects resemble those obtained after lesions of the locus coeruleus (LC), thus suggesting that noradrenergic terminals from LC to the PVN are involved in this modulation.

Locus ceruleus modulates migrating myoelectric complex in rats.

The role of the locus ceruleus (LC) in the control of migrating myoelectric complex (MMC) was investigated in rats with lesions induced by injections of 6-hydroxydopamine (6-OHDA). Control animals received the vehicle alone. MMC was recorded in conscious rats chronically fitted with electrodes. After 6-OHDA was injected into the LC, lesions of the LC were total, partial (mostly rostral), or ineffective. The MMC period was significantly longer in animals with a total or partial lesion but was unchanged in animals with an ineffective lesion. No lesion of other brain noradrenergic nuclei was observed. The longer MMC period is comparable to that obtained after intracerebroventricular injection of 6-OHDA, which is responsible for a more diffuse destruction of brain noradrenergic systems, including LC itself. Bilateral lesions of the central tegmental tract, which carries ascending noradrenergic axons from the medullary and pontine cell groups outside the LC, do not alter the MMC cycle. Consequently, the LC is most likely the major brain noradrenergic candidate for modulating the MMC pattern in rats.

Chronic intestinal pseudoobstruction with myopathy and ophthalmoplegia. A muscular biochemical study of a mitochondrial disorder.

The association of chronic intestinal pseudoobstruction with ophthalmoplegia has been reported previously in visceral myopathies. We report a case of this association in which muscle mitochondria had a crystalline appearance, a dense core, and decreased cytochrome c oxidase and succinate cytochrome c reductase activities. The absence of evident mitochondrial DNA deletion in the skeletal muscle of this patient does not exclude the possibility of localized deletion or mutation of mitochondrial DNA in digestive muscle.

[Barrett's esophagus, gastroesophageal acid reflux and duodenogastric reflux during the digestive and postprandial period]. / Endobrachyoesophage, reflux gastro-oesophagien acide et reflux duodénogastrique en période inter-digestive et post-prandiale.

Pathologic gastroesophageal acid reflux appears to be involved in the pathogenicity of Barrett's esophagus. The possible pathogenic role of duodenogastric reflux, however, has been suggested by several studies. The aim of this prospective study was to assess the prevalence of acid or duodenogastric reflux in patients with Barrett's esophagus. Nine patients with histologically proven Barrett's esophagus (mean length: 7.7cm; range: 2-13 cm) were studied by esophageal manometry and 24 hour pHmetry. Duodenogastric reflux was measured in the interdigestive period by aspiration and during the postprandial period using an isotopic method. The results of these different investigations were compared with healthy volunteers (n = 20 to 27). Three patients had complicated Barrett's esophagus (Barrett's ulcer: n = 2, high-grade dysplasia: n = 1). The results of the different investigations showed that a) all patients had abnormal acid exposure and an esophageal motor dysfunction (decrease in lower esophageal sphincter pressure, amplitude and duration of contractions and increase in percentage of peristaltic dysfunction); b) none of the patients had any pathologic duodenogastric reflux neither in the interdigestive nor in the postprandial period. These results a) confirm the high prevalence of acid reflux in patients with Barrett's esophagus, b) show that bile or pancreatic secretions are not involved in the pathogenicity of Barrett's esophagus.