[Recklinghausen's disease with digestive localizations associated with gastric acid hypersecretion suggesting Zollinger-Ellison syndrome]. / Maladie de Recklinghausen à localisations digestives associée à une hypersécrétion gastrique acide evoquant un syndrome de Zollinger-Ellison.

We report the case of a 49 year old male patient who had Recklinghausen's disease associated with hyperchlorhydria. The principal features of Recklinghausen's disease were cutaneous localizations and countless digestive tumors, found mainly on the small bowel. The ultrastructural aspect of these tumors was neurinoma or schwannoma. Basal acid gastric hypersecretion and a positive secretin test were highly suggestive of a Zollinger-Ellison syndrome. Gastrinoma was not found at laparotomy even though a gastrin gradient had been demonstrated by pancreatic venous sampling. In a patient with Recklinghausen's disease, neuroendocrine tumors (APUDomas) should be looked for systematically.

[Tropical sprue. Apropos of a case observed in the Paris region]. / La sprue tropicale. A propos d'un cas observé en région parisienne.

A case of tropical sprue in a 73-year old parisian male returning from endemic areas is reported. Clinical manifestations were megaloblastic anemia, malabsorption and protein-losing enteropathy. Biopsy of the jejunal mucosa showed partial villous atrophy with thickening of the basement membrane region; fat droplets were observed inside and close to the epithelial cells on electron microscopy study. Fat intestinal absorption after a test meal was delayed. Clinical, biological and histological improvement was obtained with tetracycline and folic acid therapy. The particular clinical and anatomical features of this case, as well as the difficulties for establishing the diagnosis of tropical sprue in non-endemic areas are analyzed.

[Multifocal cryptogenetic stenosing enteritis: an autonomous entity?]. / L'entérite sténosante multifocale cryptogénétique: une entité autonome?

Multifocal idiopathic stenosing enteritis has been described as a relapsing disease affecting the small bowel. The main anatomical characteristics are multiple non-specific inflammatory strictures. Six cases (4 men, 2 women) presenting this disorder were followed up for 3 to 22 years. Five patients were young. Iterative obstruction or chronic diarrhea and loss of weight were the outstanding clinical features. Hyposideremic anemia and protein-losing enteropathy were common. The anatomical substrate was numerous (2-19) short annular strictures situated in the ileum (75 p. 100). These strictures presented shallow ulcerations and underlying fibrosis which never extended beyond the submucosa. The small bowel wall was otherwise normal and did not show mucosal or vascular abnormalities at microscopic examination. In case of relapse, strictures developed distal to or in close contact with previous suture lines. Cultures for pathogenic bacteria were constantly negative. The natural history of these 6 cases and of 7 other previously published cases suggests that this disease is an autonomous disorder which must be differentiated from other inflammatory bowel disease entities such as Crohn's disease or tuberculosis.

Clonidine (Dixarit) for menopausal flushing.

Clonidine, 0.05 mg twice daily, was evaluated in a multicentre, randomized, placebo-controlled, double-blind crossover study in 66 patients who had had menopausal flushing for less than 1 year. Although the placebo effect was substantial, clonidine reduced the frequency of attacks significantly more than did placebo. In three of the four trials the patients' comparisons of symptoms before and after crossover indicated significantly greater improvement when the crossover was from placebo to clonidine rather than the reverse. The frequency, severity and duration of attacks were reduced by clonidine in 78%, 89% and 88% of the patients respectively, and by placebo in 50%, 53% and 50%. Side effects were minimal and their pattern was the same for clonidine as for placebo. Clonidine's action as a peripheral vascular stabilizer makes it potentially useful for the treatment of menopausal flushing. It would be prudent to include clonidine at the beginning of treatment so that its efficacy could be assessed in each individual. Its use would enhance the effects of the usual management of the menopausal syndrome, which consists of explanation, reassurance and, sometimes, the use of tranquillizers. Clonidine is a symptomatic medication that makes flushing more tolerable and should reduce the number of patients who would otherwise be exposed to the risks of estrogens.

[Caecal perforation in a patient with Sezary syndrome treated by corticosteroids (author's transl)]. / Perforation caecale au cours d'un syndrome de Sezary traité par corticoïdes.

The authors report of a case of caecal perforation in a patient with Sezary syndrome who was submitted to systemic corticosteroids a few days before that complication. Caecal perforations during corticosteroid treatment appear to be rare, the known causes being essentially carcinoma, ischaemia and colectasia in inflammatory colitis. In the case reported, there was no associated intestinal lesion. The prognosis is usually severe, mainly on account of the lack of explicit symptoms.

Is steroid dependent Crohn's disease a separate entity?

Crohn's disease has been sometimes considered as a syndrome including different entities. In this prospect we tried to assess whether steroid dependent Crohn's disease could be a separate sub-group. Eighty five patients (mean follow-up: 6 years) with documented Crohn's disease were classified into 3 groups: 1--patients never treated with steroids (NS) (N = 37); 2--patients in whom steroids had been given but had been withdrawn (NSD) (N = 37); 3--patients dependent on continuous steroid therapy (SD) (N = 11). Ten variables were considered: age at onset, sex, CDAI, cumulative topography of lesions, extra-intestinal symptoms, albuminemia, ESR, surgical operations, annual frequency of relapses. Monofactorial analysis (analysis of variance and CHI2 test) showed group SD to be significantly different from group NS and in term of age at onset, CDAI, ESR, annual frequency of relapses, extra-intestinal symptoms, surgical operations. In contrast, a multivariate analysis of correspondences applied to the 3 groups and to 9 dichotomous variables showed that group SD is not a separate entity, but the limit of a continuum extending from group NS to group NSD. This was ascertained by a CHI2 test applied to the dichotomous variables. Thus, within the limits of this study Crohn's disease appears to be a homogeneous entity rather than a heterogeneous syndrome.